Craniofacial Anomalies, Part 2 - Plastic Surgery Internal

CRANIOFACIAL EMBRYOLOGY 

To understand craniofacial anomalies and the context 

in which the anomalies occur, a complete 

understanding of embryogenesis of this region is 

imperative. Sperber1 presents a complete description 

of the processes of embryogenesis of the craniofacial 

skeleton, excerpted here. 

The primary germ layers of the embryo, the ectoderm 

and endoderm, form in the inner cell mass of 

the blastocyst. The ectoderm differentiates into 

cutaneous and neural portions by about day 20. 

Neural crest cells make up the most important structures 

in craniofacial biology. Neural crest ectomesenchyme 

has great migratory propensity and is the major 

source of connective tissue throughout the body. 

Neural crest cells differentiate into cartilage, bone, 

ligaments, muscles, and arteries. Any disruption in 

the orderly migration and differentiation of these 

cells can have severe consequences, manifested as 

congenital defects. 2 

The crucial period of organogenesis takes place in 

the first 12 weeks of gestation, and it is during this 

time that the majority of congenital craniofacial 

anomalies occur. 1,2 The earliest signs of the future 

face make their appearance at approximately day 

23–24 of embryonic life as paired mandibular processes 

of the first branchial arch. Neural crest cells 

make up the most important structures in the head. 

Next, the medial nasal processes combine with the 

intervening forebrain to form the frontonasal pro- 

CRANIOFACIAL ANOMALIES II: 

SYNDROMES AND SURGERY 

Delora L Mount MD 

cess, which is destined to become the future forehead 

and the dorsum of the nose. The lateral nasal 

folds separate the olfactory pits from the gradually 

developing eye region. By the end of embryonic 

week 5, the maxillary and mandibular processes have 

begun to increase in size but have not yet fused. It is 

not until week 6 that definitive jaws are formed. 

By the end of week 8, the face assumes most of 

the characteristics that make it recognizable as 

human. The face derives from five prominences 

that surround the future mouth, the single frontonasal 

and the paired maxillary and mandibular processes 

(Fig 1). 

The grooves between these facial prominences 

usually disappear by day 46–47 of gestation. A persisting 

groove will result in a congenital facial cleft. 

ETIOLOGY OF CRANIOFACIAL ANOMALIES 

Table 1 represents suspected contributions to 

the development of congenital craniofacial abnormalities. 

3,4 

The possibility of identifying distinct genetic aberrations 

in craniofacial dysmorphology has improved 

significantly in recent times. Specific genetic abnormalities 

resulting in syndromic craniosynostoses and 

facial dysostoses will be presented later in this issue. 

In addition to the genetic component, distinct 

environmental causes have been identified, includ- 

Fig 1. Embryonic development of the human face at (a) 5, (b) 6, and (c) 7 weeks. BG, first branchial groove; FNP, frontonasal prominence; 

LNP, lateral nasal prominence; MDP, mandibular prominence; MNP, medial nasal prominence; MXP, maxillary prominence. (Reprinted 

with permission from Sperber GH: Craniofacial Embryology, 4th ed. London, Wright, 1989.)

SRPS Volume 10, Number 17, Part 2 

ing radiation, infection, maternal factors, and chemical 

exposures. 

Radiation. Large doses of radiation have been 

associated with microcephaly. 

Infection. The children of mothers affected with 

toxoplasmosis, rubella, or cytomegalovirus show 

increased frequency of facial clefts as well as concomitant 

hand and ocular abnormalities. 

Maternal idiosyncrasies. Mothers of cleft lip and 

palate children have been noted to have a higherthan-normal 

incidence of the phenylketonuria disorder. 

The oculoauriculovertebral (OAV) spectrum has 

been seen with unusual frequency in infants of diabetic 

mothers. 5 Many studies associate maternal 

factors such as age, weight, and general health as 

potential causes of malformation. 

Chemicals. Vitamin deficiency states are associated 

with an increased incidence of cleft lip and 

2 

TABLE 1 

Causes of Congenital Deformities in Man 

(Reprinted with permission from Slavkin HC: Developmental 

Craniofacial Biology. Philadelphia, Lea & Febiger, 1979.) 

palate; which may be reduced with vitaminsupplementation 

diets for the mothers. Numerous 

studies have demonstrated a reduced incidence of 

facial clefts after maternal supplementation with folic 

acid. 6 

Vitamin A, its derivatives, and related compounds 

such as isotretinoin (Accutane) have been implicated 

in the development of facial clefting and hemifacial 

microsomia. Maternal smoking is associated with 

craniosynostosis 7 and facial clefts. 8 

Additional substances are implicated in increased 

risk of craniofacial anomalies, such as chlorpheniramine, 

chlordiazepoxide, and nitrofurantoin exposure 

and craniosynostosis. 9 

CLASSIFICATION 

To date no single classification system has been 

devised that accurately describes all congenital craniofacial 

anomalies. In 1981 the Committee on 

Nomenclature and Classification of Craniofacial 

Anomalies of the American Cleft Palate Association10 grouped craniofacial disorders according to their 

diverse etiology, anatomy, and treatment. They propose 

a practical and simple classification system in 

which five categories of deformity are identified, as 

follows: 

I Clefts (Tessier classification) 

II Synostoses 

III Atrophy/hypoplasia 

IV Neoplasia/hyperplasia 

V Unclassified 

I — CLEFTS, DYSPLASIAS, AND DYSOSTOSES 

Anatomic Classification 

In 1976 Tessier11 described an anatomic classification 

system whereby a number is assigned to each 

of the malformations according to its position relative 

to the sagittal midline (Fig 2). 

For orientation, the orbit is divided into two hemispheres: 

The lower lid with cheek and lip demonstrate 

facial clefts, the upper lid demonstrates cranial 

clefts. Two Tessier classification schemes exist, one 

for the skeleton and one for soft tissue. Numerous 

instances may occur where there is discrepancy 

between the two classification systems—for example, 

a subject with a bony cleft but no soft-tissue cleft, or

CENTRIC 

Corresponding Cranial 

Facial Clefts Extension of Facial Clefts 

No. 0 No. 14 

No. 1 No. 13 

No. 2 No. 12 

No. 3 No. 11 

ACENTRIC 

Corresponding Cranial 

Facial Clefts Extension of Facial Clefts 

No. 4 No. 10 

No. 5 No. 9 

No. 6 

No. 7 

No. 8 

Fig 2. Tessier’s classification of craniofacial clefts. Localization on 

the soft tissues (above) and skeleton (below). (Reprinted with 

permission from Tessier P: Anatomical classification of facial, 

cranio-facial, and latero-facial clefts. J Maxillofac Surg 4:69, 

1976; list from Whitaker LA, Pashayan H, and Reichman J: A 

proposed new classification of craniofacial anomalies. Cleft 

Palate J 18:161, 1981.) 

where skeletal cleft and soft-tissue cleft are not in the 

same position. Nevertheless, Tessier’s scheme remains 

in wide use today because it is relatively easy to learn 

for communicating with other clinicians. David et 

al 12 illustrate a complete series of these craniofacial 

clefts in 3D CT scans. 


The tissue-deficiency disorders—arhinencephaly 

and holoprosencephaly—are secondary to failure of 

cleavage of the embryonic holoprosencephalon and 

of the normal longitudinal split into cerebral hemispheres. 

The tissue-excess deformities range from a 

slight midline notch of the upper lip to severe orbital 

hypertelorism. 

The holoprosencephaly malformation represents 

a hypoplastic No. 14 cleft in association with a tissue 

deficiency or a tissue excess. 13,14 Cohen and Sulik 15 

present a modern analytic review of the holoprosencephalic 

disorders. Central nervous system 

findings and craniofacial anatomy are discussed, syndromes 

and associated anomalies are updated, and 

the differential diagnosis is reviewed. 

Elias, Kawamoto, and Wilson 16 reviewed holoprosencephaly 

and midline facial anomalies in an 

attempt to redefine their classification and management. 

They note that true holoprosencephaly 

encompasses a series of midline defects of the brain 

and face, and in most cases is associated with severe 

malformations of the brain which are incompatible 

with life. At the other end of the spectrum are 

patients with midline facial defects and normal or 

near-normal brain development. 

Embryologic Classification 

Van der Meulen and coworkers13 tried to correlate 

clinical features of the disorders with embryologic 

events. 

Site of dysplasia/dysostosis and associated cleft: 

Frontosphenoidal = Tessier 9 

Frontal dysplasia = Tessier 10 and 11 

Interfrontal dysplasia = Tessier 0 and 14 

Treacher Collins = 6, 7, and 8 clefts 

Temporoauromandibular dysplasia = Hemifacial 

microsomia (craniofacial microsomia) 

Pathogenesis of Clefts 

There are two leading theories of facial cleft formation. 

The classic theory holds that clefts are caused 

by failure of fusion of the facial processes. 17-19 In this 

theory the medial face unites by fusion of the paired 

facial processes beneath the nasal pits. Epithelial 

contact is established and mesenchymal penetration 

completes the fusion of the lip and palate. If the 

sequence is disturbed, a cleft forms. 

3


The mesodermal penetration theory states that 

the edges of the facial processes consist of a bilaminar 

membrane of ectoderm with epithelial seams 

demarcating the major process. 20-23 Mesenchymal 

cells then penetrate the layers and smooth out the 

seams. If the mesenchymal penetration fails, the 

epithelium dehisces and a cleft results. The severity 

of the cleft is proportional to the amount of mesodermal 

penetration. 

Management of Facial Clefts 

By far the most common craniofacial anomaly is 

cleft of the lip/palate, followed by isolated cleft palate 

as a distant second. These topics have been 

discussed in detail in Selected Readings in Plastic 

Surgery volume 10, number 16, 24 and will not be 

addressed further here. 

The incidence of rare clefts is estimated at 1.4–4.9 

per 100,000 live births. 25 Reconstruction focuses 

initially on soft-tissue closure25 with excision of the 

free borders of the cleft to normal tissue, followed by 

meticulous layered soft-tissue closure. 

Van der Meulen26 offers a thorough review of the 

pathology, etiology, and reconstruction of oblique 

facial clefts. He agrees with Tessier’s principle of 

combining skeletal and soft-tissue realignment in one 

major surgical procedure. Resnick and Kawamoto, 27 

Galante and Dado, 28 and Fuente del Campo29 give 

specific recommendations for the treatment of 

unusual facial clefts on the basis of their respective 

experiences with Tessier’s Nos. 4, 5, and 8 clefts. 

Menard30 describes the application of tissue 

expansion in the soft-tissue closure of facial clefts in 8 

patients. Due to underlying bony hypoplasia, skeletal 

reconstruction is often necessary when the child 

is older. 25 

There are multiple approaches to cleft repair, timing 

of repair, and technique of reconstruction. 

Although several protocols have been suggested, these 

should be viewed as guidelines, not absolute directives 

for care. The variability in approaches mirrors 

the extreme variability in congenital clefts, dysplasias, 

and dysostoses. A few in-depth details and reconstructive 

algorithms will be presented for the more 

common abnormalities, including craniofacial 

microsomia, Goldenhar syndrome, Treacher Collins 

syndrome, Nager syndrome, Binder syndrome, Pierre 

Robin sequence, and encephaloceles. 

4 

CRANIOFACIAL MICROSOMIA 

Craniofacial or hemifacial microsomia is the term 

most frequently used to describe the first and second 

branchial arch syndrome, which is defined as a Tessier 

7 atypical facial cleft. Thomson31 was the first to 

suggest in 1843 that the malformation was due to 

imperfect development of the first two anterior branchial 

arches. Clinical manifestations are underdevelopment 

of the external and middle ear, mandible, 

zygoma, maxilla, temporal bone, facial muscles, 

muscles of mastication, palatal muscles, tongue, and 

parotid gland; macrostomia; a first branchial cleft 

sinus; 31–33 and possible involvement of any or all 

cranial nerves34,35 (Fig 3). 

Fig 3. Craniofacial microsomia in a 7-year-old child. (Reprinted 

with permission from McCarthy JG, Grayson BH: Reconstruction: 

Craniofacial Microsomia. In Mathis SJ (ed), Plastic 

Surgery, 2nd ed. Philadelphia, Elsevier, 2006. Vol IV, Ch 103, 

pp 521-554.) 

The birth incidence of craniofacial microsomia is 

approximately 1 in 4000. 35 Approximately 10% of 

cases are bilateral. 31,36 The etiology is thought to 

relate to occlusion or thrombosis of the stapedial 

artery with injury to the developing first and second 

branchial arches. 35,37 In its fullest expression, the 

craniofacial microsomia syndrome is made up of a 

constellation of congenitally malformed facial structures 

arising from the embryonic first and second

visceral arches, the intervening first pharyngeal pouch 

and first branchial cleft, and the primordia of the 

temporal bone. 38 

Originally craniofacial microsomia was thought to 

represent a progressive skeletal and soft-tissue deformity 

that worsens over time, 39 but subsequently 

Polley 40 assessed longitudinal cephalometric data from 

26 patients with unoperated hemifacial microsomia 

and demonstrated that the condition is not 

progressive. These findings were further confirmed 

by Kearns et al 41 in 67 subjects. The disorder varies 

widely in presentation and may range from simple 

preauricular skin tags to composite mandibular and 

maxillary hypoplasia. Its management depends on 

the severity of the defect and the functional and 

aesthetic reconstructive needs. 42-46 

Pruzansky 44 described three types of mandibular 

deficiency in craniofacial microsomia according to 

the anatomical area affected (Table 2). 

This classification was modified by Mulliken and 

Kaban, 47 who subdivided Type II into Type IIA, in 

which the glenoid fossa-condyle relationship is maintained 

and the TMJ is functional, and Type IIB, in 

which the glenoid fossa-condyle relationship is not 

maintained and the TMJ is nonfunctional. 

Munro’s 42,44 classification extended the skeletal 

anomaly to include the orbit. This system aims at 

providing a basis for surgical reconstruction and consists 

of five types denoting increasingly severe hypoplasia 

of the facial bones (Table 3). Isolated microtia 

is considered to be a microform of craniofacial 

microsomia. 48 

Meurman 46 recognizes three grades of auricular 

deformity, as follows: Grade I: distinctly smaller, 


malformed auricle but all components are present; 

Grade II: only a vertical remnant of cartilage and 

skin is present, with atresia of the external meatus; 

Grade III: complete or nearly complete absence of 

the auricle. 

David and colleagues 49 proposed a multisystem 

classification of hemifacial microsomia in the TNM 

style. The physical manifestations of hemifacial 

microsomia are graded according to five levels of 

skeletal deformity (S1–S5) equivalent to the Pruzansky 

classification for S1-S3, plus S4 representing orbital 

involvement and S5 representing orbital dystopia. 

Auricular deformity (A0–A3) is similar to the classification 

described by Meurman. Tissue deficiency 

(T1–T3) is graded as mild, moderate, or severe. The 

SAT sytem allows a comprehensive and staged 

approach to skeletal and soft-tissue reconstruction. 

Early macrostomia repair (1–2 months of age) 

yields excellent functional and cosmetic results. 50 

More extensive reconstruction, including composite 

correction in moderate to severe deformity, is reserved 

for early childhood (age 5–6) but should not wait 

until facial growth is complete. 39,42–44,51–54 The mandible 

is usually corrected first in the hope that repositioning 

the jaw will unlock the growth potential of 

the functional matrix to allow normal growth of the 

mandible 55,56 and release abnormal growth tendencies 

of the maxilla. 

In mild cases, Posnick prefers to wait for skeletal 

maturity, and employs traditional orthognathic 

surgery to achieve favorable aesthetic results. 50 Distraction 

osteogenesis provides excellent correction 

in cases of mandibular deformity up to Type IIB. 57,58 

In cases of Type III deformity, a costochondral 

TABLE 2 

Mandibular Deficiency in Craniofacial Microsomia (Pruzansky) 

5


graft is usually indicated, though many authors have 

noted unpredictable overgrowth of costochondral 

grafts. 34,42–44,53,59 

Ross 60 reviewed 55 cases of severe craniofacial 

microsomia treated with costochondral grafts at 

The Hospital for Sick Children. The success rates 

were higher when the children were operated earlier 

(85% for age 3–7y vs 50% for age >14y). 

Growth equal to the other side was seen in 46%, 

undergrowth in 15%, and overgrowth in 39%. 

Given the option of early or delayed surgery, the 

author favors early surgery at age 4–5, citing a 

higher graft success rate, the psychosocial advantage 

of attaining facial symmetry at a younger age, 

and the additional benefit that erupting teeth will 

assume a more normal position, making future 

orthodontic treatment less difficult. 

Munro 42,44,53 usually advocates much more 

extensive surgery, operating on the maxilla at the 

same time as the mandible. For mild cases, mandibular 

surgery may involve contour surgery with or 

without genioplasty once skeletal maturity is 

reached. In case of orbitozygomatic hypoplasia, 

Posnick 50 uses split cranial bone grafts at age 5–7, 

stating that at age 7 the cranioorbitozygomatic complex 

is nearly mature so that an adult-size vault, 

orbit, and cheekbone can be fashioned. Also the 

thickness of the calvarium at that age makes for an 

easier harvest of split calvarial bone grafts. Refinements 

to the mature skeleton may be needed eventually 

to achieve a symmetrical and aesthetic 

result, 50 and may take the form of sagittal split 

osteotomy, Le Fort I osteotomy, or genioplasty. 

At times the soft-tissue deformity in craniofacial 

microsomia must also be addressed, and usually follows 

skeletal reconstruction. Various methods of 

6 

TABLE 3 

Mandibular and Orbital Deficiency in Craniofacial Microsomia (Munro) 

soft-tissue augmentation with microvascular free transfers 

are described by La Rossa 61 and Upton. 62 This 

subject is further discussed in Selected Readings in 

Plastic Surgery volume 10, number 5, part 1. 63 

GOLDENHAR SYNDROME 

(Oculoauriculovertebral Dysplasia) 

Goldenhar syndrome is characteristically bilateral. 

Features include prominent frontal bossing, 

a low hairline, mandibular hypoplasia, low-set ears, 

colobomas of the upper eyelid, epibulbar dermoids, 

accessory auricular appendages that are bilateral 

and anterior to the ears, and vertebral anomalies. 5 

Occurrence is believed to be sporadic, with only a 

weak genetic component. The presence of 

epibulbar dermoid is required for a diagnosis of 

Goldenhar syndrome. The reconstruction follows 

the principles of craniofacial microsomia presented 

above. 

TREACHER COLLINS SYNDROME 

(Mandibulofacial Dysostosis) 

The first reference to mandibulofacial dysostosis 

in the medical literature was made by Berry in 1889. 

Berry described the physical symptoms and speculated 

about the inherited character of the deformity. 

His treatise was certainly much more detailed than 

the two cases reported by E Treacher Collins 11 

years later, after whom the syndrome was named. 

In Europe the deformity is known as the Franceschetti- 

Zwahlen-Klein syndrome on the basis of their 1949 

monograph summarizing the world literature. 64 The 

incomplete form should be designated as Treacher 

Collins syndrome65 and the complete form as 

Franceschetti’s syndrome.

The Treacher Collins syndrome66 represents a 

manifestation of the Tessier Nos. 6, 7, and 8 cleft. It 

is inherited as an autosomal dominant trait with an 

incidence of 1/10000 live births. 67 Bilaterality is the 

norm and phenotypic expression is variable. The 

gene for Treacher Collins syndrome was identified 

in 1991, when it was mapped to chromosome 5 by 

Dixon and colleagues68 from a study of 12 families. 

Later that year the genetic locus was refined to bands 

5q31.3?q33.3. 69 In 1996, transcription mapping 

localized the critical region to a specific locus, TCOF1, 

which produces an abnormal protein named Treacle. 

This nucleolar protein is under intense study and 

appears to function in microtubule formation and 

cell migration. 70,71 

The pathogenesis of Treacher Collins syndrome 

remains unknown. Sulik and colleagues72 were able 

to produce the facial abnormalities of Treacher Collins 

in mice by administering isotretinoin, suggesting that 

the syndrome can be triggered by disruption of vitamin 

A metabolism. There is an apparent correlation 

between frequency of mutation and advanced 

paternal age. 

The typical features of the Treacher Collins syndrome 

include the following (Fig 4): 

• palpebral fissures sloping downward laterally 

(antimongoloid slant), with coloboma of the outer 

portion of the lower lid and rarely the upper lid 

• hypoplasia (aplasia) of the facial bones, especially 

the malar bones and mandible 

• malformation of the external ear and occasionally 

the middle and inner ear 

Fig 4. Treacher-Collins Syndrome: Softtissue 

and skeletal deformities. (Reprinted 

with permission from Bartlett SP, Losee JE, 

Baker SB: Reconstruction: Craniofacial Syndromes. 

In Mathis SJ (ed), Plastic Surgery, 

2nd ed. Philadelphia, Elsevier, 2006. Vol IV, 

Ch 102, pp 495-519.) 


• macrostomia, high palate, abnormal position and 

malocclusion of the teeth 

• blind fistula between the angles of the mouth 

and the ears 

• atypical hair growth in the form of tongue-shaped 

processes of the hairline extending toward the 

cheeks 

• absence of eyelashes in at least the medial third 

of the lower eyelid 

In affected newborns the first priority is airway 

management. Shprintzen et al 73 noted that some 

patients have marked narrowing of the airway (pharyngeal 

diameter


traction osteogenesis in children

Fig 5. (Above) The nose and upper lip in maxillonasal dysplasia. 

1 - Retracted columella-lip junction and lack of triangular flare at 

the base. 2 - Perpendicular alar-cheek junction. 3 - Upper lip 

convex with wide, shallow philtrum. 4 - Crescent-shaped nostril 

without nostril sill. 5 - Low set and flat nasal tip. 6 - Cupid’s bow 

stretched and shallow. (Below) Surgical correction is by medial 

rotation of tissues on either side of the nasal midline. (Reprinted 

with permission from Holmstrom H: Clinical and pathologic 

features of maxillonasal dysplasia (Binder’s syndrome): significance 

of the prenasal fossa on etiology. Plast Reconstr Surg 

78:559, 1986.) 

a coronal incision and reaching the nasal floor through 

an incision in the upper buccal sulcus. The nasal soft 

tissues and alar cartilages are mobilized. The nose is 

lengthened and tip projection is achieved with a cantilever 

graft of lyophilized cartilage. 

Wolfe 107 describes a technique of nasofrontal 

osteotomy to lengthen the nose in cases of posttraumatic 

shortening and Binder syndrome. 

McCollum 108 reviews the literature and provides long 

term follow up of 2 patients, one treated with traditional 

orthognathic surgery and the other with a 

growth center implant to the nose. 

PIERRE ROBIN SEQUENCE 

In 1923 Pierre Robin, a French stomatologist, noted 

a triad of characteristics of the upper airway which is 

now known as the Pierre Robin sequence. 109 The 

characteristic features consist of micrognathia, 

glossoptosis, and airway obstruction. 8,109 An associated 

high arched midline cleft of the soft palate and occasionally 

of the hard palate is present in approximately 

50% of cases. 110,111 The sequence shows great etiologic 

heterogeneity, with as many as 18 associated syndromes. 

The glossoptosis in Pierre Robin can begin a vicious 

sequence of events: airway obstruction, increased 


Fig 6. Patient with Binder syndrome. A, B, at age 10. C, E, 

preoperatively at age 17. D, F, after orthodontic treatment (maxillary 

first bicuspid extractions), orthognathic surgery (Le Fort I osteotomy 

with horizontal advancement), and nasal reconstruction 

(corticocancellous iliac graft). (Reprinted with permission from 

Posnick JC, Tompson B: Binder syndrome: staging of reconstruction 

and skeletal stability and relapse patterns after Le Fort I osteotomy 

using miniplate fixation. Plast Reconstr Surg 99:967, 1997.) 

energy expenditure, and decreased caloric intake 

from impaired feeding. Afflicted infants typically fail 

to thrive because of respiratory and feeding difficulties. 

If these problems are ignored, respiratory failure, 

cardiac failure, and death may result. 

9


In 1946 Douglas 112 reported >50% mortality with 

conservative treatment of Pierre Robin. It is now 

clear that the key to successful medical treatment of 

infants with Pierre Robin is to hold the infant prone 

to relieve the glossoptosis and open the airway. In 

some cases this position must be maintained for 24 

hours a day, even during feeding, baths, and diaper 

changing. 113 

While most infants can be successfully managed 

conservatively, a few will require surgical intervention. 

If medical treatment fails to relieve the symptoms 

of airway obstruction, the baby would formerly 

be considered for tongue-lip adhesion or tracheostomy. 

112,114,115 The advent of distraction osteogenesis 

provides an effective alternative for addressing 

airway obstruction. Denny 116 describes a series of 

10 patients with airway obstruction from severe craniofacial 

syndromes who were treated with distraction 

osteogenesis of the mandible. All children were clinically 

improved, and 2 of 3 patients with tracheostomies 

were successfully decannulated within 6 weeks. 

The mean functional airway increase after distraction 

was 67.5% (Fig 7). 

ENCEPHALOCELES 

An encephalocele is a protrusion of part of the 

cranial contents through a defect in the skull. The 

mass may contain meninges (meningocele), meninges 

and brain (meningoencephalocele), or meninges, 

brain, and ventricle (meningoencephalocystocele). 117 

Encephaloceles categorized by their position in the 

skull can be basal, sincipital, or convexity. 118 The 

sincipital group can be further divided into 

frontoethmoidal, interfrontal, and those associated 

with clefts. The frontoethmoidal group can be subdivided 

into nasofrontal, nosoethmoidal and 

nasoorbital types. 119 

The presence of an encephalocele may be detected 

on fetal ultrasound or by an elevated alpha fetoprotein 

level. 118 The differential diagnosis of frontal midline 

masses includes encephaloceles, teratomas, gliomas, 

and dermoids. 120,121 High-resolution CT scans 

can establish the intracranial component of 

encephaloceles. 121 In a frontoethmoidal or nasal 

encephalocele, the cranial defect is in the anterior 

midline between the frontal bone preformed in membrane 

and the ethmoid preformed in cartilage. The 

craniofacial deformity consists of hypertelorism, orbital 

dystopia, elongation of the face, and dental maloc- 

10 

Fig 7. Two patients with Nager syndrome and tracheostomy before 

(left) and after (right) mandibular distraction. (Reprinted with 

permission from Denny AD, Talisman R, Hanson PR, Recinos RF: 

Mandibular distraction osteogenesis in very young patients to 

correct airway obstruction. Plast Reconstr Surg 108:302, 2001.) 

clusion, reflecting the distorting influences on facial 

bone growth by the extruded intracranial contents 

(Fig 8). 

The pathogenesis of frontoethmoidoencephaloceles 

is as follows. Early in embryogenesis, diverticula 

of dura project anteriorly through the fonticulus 

nasofrontalis (a small fontanelle between the developing 

nasal and frontal bones) or inferiorly through 

the developing frontal bone into the prenasal space. 

These diverticula may come in contact with skin and 

adhere to it. Normally the diverticulum regresses 

and the bone closes, creating both the normal 

nasofrontal suture anteriorly and, passing through 

the skull base just anteriorly to the crista galli, the

Fig 8. Left, child with asymmetrical nasoethmoidal, nasoorbital 

encephalocele. Right, same child at age 5, after removal of the 

encephalocele and correction of the trigonocephaly, orbital 

dystopia, and hypertelorism in one procedure at 8 months of age. 

(Reprinted with permission from Holmes AD, Meara JG, Kolker 

AR, et al: Frontoethmoidal encephaloceles: reconstruction and 

refinements. J Craniofac Surg 12:6, 2001.) 

foramen cecum. In a frontoethmoidal encephalocele 

the diverticulum does not recede and the bone 

does not close. The etiology of encephalocele is 

unknown but includes racial, genetic, environmental, 

and paternal factors. 122 

Encephaloceles occur with a number of craniofacial 

syndromes. 123 The world wide incidence of 

encephalocele is 1/5000. 124 In Western Europe, North 

America, Australia, and Japan, occipital encephaloceles 

predominate. In Southeast Asia and Russia, 

anterior encephaloceles outnumber posterior ones 

by a 9.5:1 ratio. 124,125 The reason for this discrepancy 

is unknown. 

The principles of treatment are incision of the sac, 

amputation of excess tissue to the level of the surrounding 

skull, closure of the dura, and closure of 

the skin. 126 David, 127 Forcada et al, 128 and Smit and 

colleagues129 review the spectrum of cranial and 

cerebral malformations that may be present in 

frontoethmoidal encephaloceles and discuss the 

diagnosis and management of these deformities. 

David127 analyzed the experience with frontoethmoidal 

encephaloceles by the Australian Craniofacial 

Unit from 1975 to 1993 and reached the following 

conclusions: 

• Early complete surgery is indicated to allow the 

developing brain and eyes to remodel the facial 

deformity. 

• Intracranial abnormalities are common. 


• Frontoethmoidal encephaloceles differ from other 

neural tube defects in their lack of a familial pattern 

and peculiar geographic distribution. 

• Treatment by craniofacial technique is best. 

• The established deformity can be effectively managed 

by craniofacial osteotomies. 

• Most patients have abnormal intercanthal distances 

but normal interpupillary and lateral canthal 

measurements. 

• The frontal sinus region often needs repeat bone 

grafting, and nasal bone grafts commonly need 

to be replaced as patients age. 

• Treatment for craniofacial clefts should be postponed 

until after growth is complete. 

• Early treatment of patients with basal encephaloceles 

is indicated to prevent further damage and 

infection. 

• Surgery for extensive basal encephaloceles is complex 

and probably should be done through a 

facial hemisection approach. 

Holmes et al130 offer their experience with 35 

cases of frontoethmoidal encephalocele. The goals 

of treatment are as follows (Fig 9): 

• urgent closure of open skin defects to prevent 

infection and desiccation of brain 

• removal or invagination of nonfunctional extracranial 

tissue 

• watertight dural closure 

• total craniofacial reconstruction with special care 

to avoid the “long nose deformity” 

To correct the deformity caused by hypertelorism 

and a long midface, Holmes and coworkers130 lower 

the supraorbital bar by rotating it medially, posteriorly 

and downward in the midline, while laterally it is 

widened to correct the trigonocephalic deformity. 

Successful correction depends on an understanding 

of the pathologic anatomy; careful planning of 

osteotomies and bone movements to correct the 

whole deformity, including trigonocephaly and the 

long nose deformity; nasal reconstruction with cantilever 

graft to avoid the long nose deformity; skin 

closure removing abnormal skin and careful placing 

of scars; transnasal canthoplasty to reposition the 

11


Fig 9. Three-dimensional models of a frontoethmoidal encephalocele and its correction. A, B, note the external cranial opening, interorbital 

hypertelorism, and depressed cribriform plate. The supraorbital osteotomies and central metopic area of bone are marked, as well as the 

bony Z-plasties on the lateral orbital rims. C, diagram of the proposed osteotomies and bone movements. D, after repositioning the medial 

and lateral walls of the orbit and supraorbital bar and fixation of the bone graft for nasal reconstruction. (Reprinted with permission from 

Holmes AD, Meara JG, Kolker AR, et al: Frontoethmoidal encephaloceles: reconstruction and refinements. J Craniofac Surg 12:6, 2001.) 

medial canthi; and single-stage surgery displaying craniofacial 

and neurosurgical expertise. 

II — CRANIOSYNOSTOSIS 

Virchow (1851) was the first to use the term craniosynostosis, 

although abnormal head shapes related 

to cranial sutures were noted by Hippocrates as early 

as 100 BC. Virchow noted that growth restriction 

occurred perpendicular to the fused cranial suture 

and compensatory growth occurred parallel to the 

affected suture 131 (Fig 10). This combination of growth 

restriction in one dimension and compensatory 

growth at right angles results in consistent patterns of 

abnormal head shape. 132 

12 

The incidence of craniosynostosis is estimated at 1 

in 2000 live births. 133 Retrospective studies using 

radiographic criteria alone underestimate the occurrence 

of craniosynostosis. 134,135 Craniosynostosis may 

occur as a sporadic, isolated abnormality or as a feature 

of a congenital syndrome. It can be isolated 

nonsyndromic or syndromal. The best way to classify 

isolated craniosynostosis is to name the suture(s) 

involved—eg, left unicoronal synostosis, metopic synostosis, 

bicoronal synostosis—but it is also common to 

refer to the resulting head shape 123,136–138 (Fig 11). 

Trigonocephaly results from premature fusion of 

the metopic suture. The spectrum of deformities 

includes a vertical midline forehead ridge, triangular 

or “keel-shaped” contour, bitemporal narrowing, and 

hypotelorism.

Fig 10. Cranial sutures in the human fetus. Premature closure 

produces growth restriction perpendicular to the line of the 

suture and compensatory overgrowth parallel to it. 

Scaphocephaly (dolichocephaly) is due to premature 

fusion of the sagittal suture. Features of sagittal 

synostosis include a palpable ridge overlying the sagittal 

suture, decreased biparietal diameter, and elongation 

of the skull in the anteroposterior dimension. 

Significant frontal and occipital bossing are commonly 

noted. The appearance resembles a boat or “scaphe.” 

Plagiocephaly stems from the Greek word meaning 

“crooked head”, and is an asymmetrical deformity. 

It may be anterior or posterior. Two etiological 

variants must be distinguished – deformational 

and synostotic. 

Posterior plagiocephaly is usually positional and a 

result of the child lying predominately on his/her 

back. It produces a classic parallelogram skull 

deformity. 139 The incidence has increased with the 

recommendation by the American Academy of 

Pediatrics to lay infants on their backs to reduce the 

risk of sudden infant death syndrome (SIDS). 140,141 

Treatment of positional plagiocephaly depends on 


the severity of the deformity and often requires helmet 

therapy for correction in severe cases. However, 

large studies have shown little difference in 

outcome between helmet therapy and consistent 

repositioning of the infant off the flat spot in mild to 

moderately severe cases. 141,142 

Posterior synostotic plagiocephaly can also result 

from unilambdoid synostosis. Unilambdoid synostosis 

is a very rare entity. 

Brachycephaly is the result of bicoronal synostosis 

and is characterized by anteroposterior shortening 

of the skull. The lower part of the forehead and 

supraorbital bar are retropositioned. This is the 

characteristic head shape deformity that accompanies 

Apert and Crouzon syndrome, and in these 

cases it is believed to be due to abnormalities that 

extend into the cranial base, causing the associated 

facial deformities of exorbitism and maxillary retrusion. 

Posterior brachycephaly is unusual but can 

be the external manifestation of bilateral lambdoid 

suture synostosis. 

Turricephaly (towering head deformity) is characterized 

by excessive skull height and a vertical forehead. 

This deformity is typically an untreated 

brachycephaly where compensatory expansion leads 

to an increasing vertical height to the cranium. Children 

with Apert syndrome have a particular tendency 

toward turricephaly. 

Oxycephaly is a pointed head. The forehead is 

retroverted and tilted back in continuity with the 

nasal dorsum. Oxycephaly is usually due to fusion of 

multiple sutures. 

When multiple sutures are involved, head shapes 

are more variable and the distinctions blur. In these 

cases the specific sutures involved should be named 

when describing the deformity. The kleeblattschadel 

or cloverleaf skull deformity results from pansutural 

synostosis, and usually requires early, aggressive treatment 

to prevent cerebral compromise. 

Craniofacial Growth 

The cranium is made up of the neurocranium, 

which includes the chondrocranium of the skull base 

and the membranous bone of the calvarium, and 

the viscerocranium, which forms the membranous 

bones of the face. The various areas of the 

craniomaxillofacial skeleton grow by very different 

methods. The cranial sutures are skeletal joints of 

the syndesmosis type as well as being sites of osteo- 

13


Fig 11. Skull shapes and affected sutures in craniosynostosis. See text for details. (Reprinted with permission from Cohen MM Jr, MacLean 

RE: Anatomic, Genetic, Nosologic, Diagnostic, and Psychosocial Considerations. In: Cohen MM Jr, MacLean RE (eds), Craniosynostosis. 

Diagnosis, Evaluation, and Management, 2nd ed. New York, Oxford Univ Press, 2000; Ch 11, pp 119-143.) 

genesis and skeletal adjustments. The interlocking, 

peg-and-socket arrangement, allows osteogenesis to 

occur mainly at the bottom of the socket and point 

of the peg, for simultaneous jointing and growth of 

the bone against the suture. In contrast, the facial 

sutures, especially the zygomatic, maxillary, and 

palatine, are simply overlapping or sliding joints where 

the direction of bone growth tends to parallel the 

plane of the suture. This arrangement provides for 

adaptive adjustments to pressure in utero and early 

infancy. 

The intrinsic maxillary growth concept recognizes 

specific bone growth sites in the maxilla, with growth 

occurring mainly in a backward direction by osteogenesis 

on the retromaxillary surface. The nasal septum 

exerts forward pull on the maxilla at the insertion 

of the septopremaxillary ligament. 143,144 Mandibular 

growth is primarily through growth and elongation at 

the level of the ramus and subcondylar segments. 

Experimental studies indicate that condylar cartilage 

14 

growth is probably secondary to traction by the soft 

tissues within and attached to the mandible, such as 

the tongue and muscles of mastication. 55 

Pathogenesis of Craniosynostosis 

The pathogenesis of craniosynostosis is complex 

and probably multifactorial. Moss145 theorized that 

abnormal tensile forces are transmitted to the dura 

covering the brain from an anomalous cranial base 

through key ligamentous attachments, and this leads 

to craniosynostosis. This hypothesis fails to explain 

the coexistence of nonsyndromic craniosynostosis with 

a normal cranial base configuration. 

Cohen123,146 suggests craniosynostosis is either primary 

due to suture biology147 or secondary to 

another event such as in-utero compression of the 

cranium, 148,149 decompression of a hydrocephalus, 

150–152 inadequate intrinsic growth forces of the 

brain (microcephaly), hyperthyroidism, 153 ricketts, 

or shunted hydrocephalus.

Approximately 2% of cases of isolated craniosynostosis 

are inherited. 154 Cohen, Dauser, and Gorski 155 

documented three close relatives with delayed onset 

of exorbitism and midfacial retrusion thought to be 

consistent with a diagnosis of familial, nonsyndromic 

craniosynostosis. In contrast, a hereditary component 

has been identified in as many as 50% of 

syndromal craniosynostosis patients. 154 

The Role of the Dura in Craniosynostosis 

In cases of primary craniosynostosis the underlying 

dura mater acts locally to supply the overlying 

suture with osteogenic growth factors. Opperman in 

1993 showed the role of the dura in determining the 

fate of suture fusion, 156 and that these dural factors 

were soluble. 157 Hobar158,159 noted the importance 

of the dura in the regeneration of cranial bones in 

infants. Greenwald160,161 went further, finding that 

immature dura mater contained a subpopulation of 

osteoblast-like cells. Levine162 showed that the region 

of the dura was as important as the interaction 

between the suture and the dura. The overlying 

pericranium does not play a role in suture biology, 

according to Opperman. 163 Clearly the dura underlying 

the suture drives the timing of closure through 

osteoinductive growth factors 

Molecular Genetics in Craniosynostosis 

Elevated FGF-R2 was identified by Delezoide164 as the first real marker of prechondrogenic condensations. 

Mangasarian165 identified a tyrosine for cysteine 

substitution on the mutated FGF-R2 that creates 

an activated form, resulting in uncontrolled FGF 

signaling and premature suture closure. 

Most166 and Mehrara167 found that expression 

of basic fibroblast growth factor (bFGF) was 

increased in the dura beneath the suture prior to 

fusion and increased in the osteoblasts at the suture 

during fusion. Other growth factors have been 

found to be increased in prematurely fusing 

sutures: transforming growth factor B (TGF- 

B) 166,168–171 and bone morphogenic proteins 

(BMPs). 171 Research is now directed at inducing 

craniosynostosis in animal models by the application 

of exogenous FGFs to confirm their role in 

suture closure. 172 Genes whose mutations result 

in craniosynostosis syndromes are listed in Table 

4. 173,174 


TABLE 4 

Genes Bearing Known Mutations 

for Craniosynostosis 

(Reprinted with permission from Cohen MM Jr: Discussion of 

“Differential expression of fibroblast growth factor receptors in 

human digital development suggests common pathogenesis in 

complex acrosyndactyly and craniosynostosis”, by Britto JA, 

Chan JCT, Evans RD, et al. Plast Reconstr Surg 107:1339, 2001.) 

In 1993 a mutation in the homeobox gene MSX2 

was identified in a single family with autosomal dominant 

craniosynostosis, now known as Boston-type 

craniosynostosis. 175 Recent evidence suggests that 

this mutation may exert its effect through BMP pathways 

176,177 and that MSX2 mutations may function 

together with FGF-R2. 178 

Crouzon syndrome has been extensively studied 

genetically and a mapped abnormality is noted on 

the long arm of chromosome 10. 179 Further analysis 

localized an FGF-R2 genetic mutation within this 

chromosome in subjects with Crouzon. 180 

In 1994 Muenke and colleagues 181 described a 

common mutation in the FGF-R1 gene as the cause 

of Pfeiffer’s syndrome. In 1995 Apert and Pfeiffer 

syndromes were also found to be related to FGF-R2 

mutations. 182–184 Mutations in the FGF-R2 gene now 

having been shown to be the cause of Jackson-Weiss 

syndrome, Crouzon syndrome, Pfeiffer syndrome, and 

Apert syndrome, these conditions should be seen as 

defined points along a spectrum of disease (Fig 12). 

FGF-R3 mutations are also implicated in isolated 

unicoronal craniosynostosis. 185 Current understanding 

allows a molecular diagnosis in all phenotypical 

cases of bicoronal synostosis. 186 Other genetic mutations 

have been associated with synostosis. The 

hedgehog family of homologs, including sonic hedgehog, 

play a role in vertebral embryogenesis. Recently 

15


Fig 12. Fibroblast growth factor 

mutations and their associated craniofacial 

anomalies. (Reprinted with 

permission from Cohen MM Jr: Discussion 

of “Differential expression of 

fibroblast growth factor receptors in 

human digital development suggests 

common pathogenesis in complex 

acrosyndactyly and craniosynostosis”, 

by Britto JA, Chan JCT, Evans 

RD, et al. Plast Reconstr Surg 

107:1339, 2001.) 

an association was made between craniofacial anomaly 

and sonic hedgehog target genes. 187–189 The TWIST 

gene locus regulates osteoblast differentiation190 and 

mutations are associated with Saethre-Chotzen syndrome, 

191–193 causing an up-regulation in FGF-R 

expression leading to premature oseoblast differentiation 

and cranial suture fusion. Rice194 proposes an 

integration between FGF and TWIST mutations in suture 

development and suggests that different mutations may 

work on the same pathway at different stages. 

Ting195,196 examined the molecular difference 

between fused and nonfused human coronal sutures 

and identified overexpresion of the Nell-1 gene, again 

related to premature osteoblast differentiation. 

It is not adherent tensile forces or an intrinsic property 

of the suture that determines suture biology, but 

rather a combination of dura-related genetic, 

molecular, and cellular factors that act individually 

or jointly in the development of craniosynostosis. 

Longaker197 provides a comprehensive review of current 

cranial suture research, concluding that gene 

therapy may offer targeted interventions that may 

alter synostosis onset and progression: 

Looking forward into the new millennium, one 

can imagine a time when major reconstructive 

surgery for craniosynostosis will no longer be 

necessary. 

Longaker (2001) 

16 

Indications for Surgery 

Indications to proceed with surgical reconstruction 

of craniosynostosis include significant cranial and 

facial asymmetry, elevated intracranial pressure (ICP), 

and neuropsychologic disorders. In most cases of 

single suture craniosynostosis, “functional” indications 

(elevated ICP and treatable neuropsychologic disturbance) 

are not present, and the indication for surgery 

is the cranial/facial asymmetry, its degree and 

severity. 

The clinical symptoms of intracranial hypertension 

include headaches, irritability, and difficulty 

sleeping. Radiographic signs may include cortical 

thinning or a luckenschadel (beaten metal) appearance 

of the inner table of the skull. Unfortunately, 

clinical and radiographic signs are relatively late 

developments, and increased intracranial pressure 

may be present for some time before these signs 

arise. Because of this, most craniofacial surgeons 

advocate early treatment, especially of infants with 

multiple suture synostoses. 198 

Marchac and Renier136,199 found that intracranial 

pressure may be elevated in those with single suture 

synostosis, although it is more common with multiple 

suture involvement (13% and 42%, respectively). The 

mean ICP has been shown to decrease after cranial 

vault remodeling. Young children with craniosynos-

tosis usually have normal mental development, but 

the proportion of normal children decreases with 

age, particularly for children with multisuture synostosis 

displaying brachycephaly and oxycephaly. 200 On 

the basis of their studies, the authors recommend 

early surgery, as there is no reliable way to distinguish 

which infants will not have problems from craniosynostosis. 

201,202 

Kapp-Simon and colleagues, 203 on the other hand, 

longitudinally examined the mental development of 

infants before and after cranial release, and compared 

it with that of infants who were not surgically 

treated. The authors concluded that cranial release 

and reconstruction did not affect mental development 

either positively or negatively. Renier and 

Marchac, 204 in a commentary of the above paper, 

strongly dispute this finding and state that the number 

of patients in Kapp-Simon’s study was too small 

to warrant any conclusions. 

Subsequently Kapp-Simon 205 published her analysis 

of a series of 84 patients with single suture synostosis 

followed longitudinally for >1y and reported a mental 

retardation rate of 6.5%, which is 2–3X the normal. 

Almost half the children who were of school 

age displayed some type of learning disorder. More 

importantly, these results were independent of early 

surgical correction, discounting the hypothesis that 

early correction of craniosynostosis would improve 

mental function. Similarly, Virtanen 206 found that 

the neurocognitive performance of children with craniosynostosis 

did not reach that of matched normal 

controls, suggesting the impairment of brain function 

had already taken place in utero. 

Gault et al 207 attempted to correlate elevations of 

intracranial pressure with decreases in intracranial 

volume as measured by CT. They found no direct 

relationship between the two on measurement of 

104 children with craniosynostosis. 202 It appears that 

decreased intracranial volume alone is not adequate 

justification for surgery in cranisynostosis. 208 

Posnick and colleagues 209 demonstrated that true 

measurements of intracranial volume can be obtained 

indirectly using CT scans. Premature closure of either 

the sagittal or metopic suture does not result in 

diminished intracranial volume. This was confirmed 

in long-term follow-up by Polley, 210 who showed 

that craniofacial procedures can be relied upon to 

increase the intracranial volume. Polley also found 

that long-term normative intracranial volume was 

maintained postoperatively, and hypothesized that 


although normal volumes are seen pre- and postoperatively 

in craniosynostosis, reconfiguration of the 

skull dimensions in the region of the synostosed suture 

may be beneficial. 

David et al 211 adds further support to this theory 

in a study of cerebral perfusion pre- and postoperatively, 

where they found cerebral perfusion defects 

preoperatively in the area of the fused suture that 

were corrected following surgery. In cases of complex 

craniosynostosis, abnormalities of venous drainage 

at the level of the skull base produce venous 

hypertension and subsequent raised ICP. 212 Most 

surgeons therefore operate on infants at age 6–9 

months, and even earlier in severe cases. 

Cohen 213 reviews the evidence and concludes that 

differences in methods of pressure measurement, 

patient selection, and the lack of normative data 

make interpretation of existing studies difficult. He 

suggests that a clinical awareness of the signs of raised 

ICP is essential, though in cases of moderate deformity 

and no signs of raised ICP, close follow-up is 

applicable. 

Neuropsychiatric disorders range from mild 

behavioral disturbances to overt mental retardation 

possibly secondary to cerebral compression. The 

abnormally shaped skull also imposes psychological 

considerations that can be severe and should not be 

underestimated. Barritt and associates 214 report that 

children with untreated scaphocephaly are teased 

and taunted at school for their head shape, which 

compounds their slow learning and poor motor skills. 

Arndt and others 215 and Pertschuk and Whitaker 216 

studied the psychosocial adjustments of children to 

the correction of a deformity by craniofacial surgery. 

The authors noted increased self-esteem and adaptive 

functioning along with a decrease in hyperactive 

behavior and inhibited attitude, peaking at 1 year 

postoperatively. Despite cosmetic improvements and 

lower anxiety levels, however, social interactions were 

not helped by the surgery. Likewise, Ousterhout 

and Vargervik 217 noted normalization of anthropometric 

points on CT scan of children who underwent 

Le Fort III and genioplasty because of craniosynostosis, 

but the degree of postoperative change did not 

equate with attractiveness. 

In another study, Barden and colleagues 218,219 

looked at changes in physical attractiveness as well as 

emotional and behavioral reactions of children before 

and after craniofacial surgery. Their findings suggest 

17


a positive effect of the surgery in terms of social 

interactions and the development of cognitive and 

emotional competence. 

ISOLATED CRANIOSYNOSTOSES 

Trigonocephaly 

Trigonocephaly accounts for 10–20% of all craniosynostosis 

cases, occurring in 1 in 2500–15000 newborns. 

220 Lajeunie221 found 237 cases of metopic 

synostosis for an incidence of 1 in 15000. The 

male:female ratio was 3.3:1 and 5.6% were familial. 

Infants with trigonocephaly have an easily visible 

and palpable midline frontal ridge that extends from 

the anterior fontanelle to the glabella. When viewed 

from above, the forehead resembles the keel of a 

boat. Sadove and coworkers222 note that depending 

on the timing and extent of premature suture closure, 

metopic synostosis can manifest as a spectrum 

of deformities ranging from an isolated midline forehead 

ridge to a keel-shaped frontal bony protruberance. 

The forehead deformity is accompanied 

by variable degrees of orbital hypotelorism, ethmoidal 

hypoplasia, and bitemporal narrowing. The 

intracranial volume of both frontal areas is often 

decreased, which results in compensatory overgrowth 

of both parietal areas. The frequency of elevated 

intracranial pressure is estimated to be approximately 

10%, 223 though it is likely that pressure is only an 

issue in the frontal lobes of those with severe deformity. 

Cognitive impairment is some series reaches 

33% 224 and correlates with severity of deformity, 225 

though it is likely to be an associated factor rather 

than a causative one. 

Posnick and associates226 evaluated the results of 

surgical intervention in metopic synostosis. They used 

CT scans to quantitatively record the deformities of 

uncorrected and corrected metopic synostosis, 

namely orbital hypotelorism, retruded lateral orbital 

rims, and narrow bitemporal width. Postoperative 

assessment confirmed that the anterior cranial vault 

and lateral orbital wall positions, which were initially 

dysmorphic, were corrected successfully and 

remained in good position despite subsequent calvarial 

growth (Fig 13). The orbital hypotelorism was 

improved but not totally corrected. 

Havlik et al227 examined 10 patients with severe 

trigonocephaly as defined by the central angle—the 

convergence of the two hemisupraorbital segments. 

18 

Fig 13. Left, a 10-month old boy with metopic synostosis and 

trigonocephaly. Right, after surgical correction with cranial vault 

and three-quarter orbital osteotomies. (Reprinted with permission 

from Posnick JC, Lin KY, Chen P, Armstrong D: Metopic 

synostosis: quantitative assessment of presenting deformity and 

surgical results based on CT scans. Plast Reconstr Surg 93:16, 

1994.) 

Preoperative angles were consistently in the vicinity 

of 110 °. In these cases they recommend expanding 

the supraorbital bar with interpositional bone grafts 

measuring 10–20mm, expanding the angle to 

approximately 145°, and thus widening the bitemporal 

narrowing (Fig 14). In milder cases they suggest 

that more conventional methods such as contour 

reduction or the floating forehead technique 

will be adequate. 

McCarthy and others 228 reviewed a 20-year experience 

with early surgery for isolated craniosynostosis. 

Of the 104 patients in their study, 29 had metopic 

suture synostosis. Orbital hypotelorism, initially 

apparent in 19, was significantly reduced postoperatively 

in 17 patients. In general, surgical correction 

of metopic synostosis was associated with the best 

aesthetic results of all the isolated craniosynostoses; 

only one patient required a second craniofacial procedure. 

McCarthy also followed 24 patients with 

metopic synostosis for whom surgical correction was 

not recommended. None of these patients demon-

Fig 14. Technique for correction of trigonocephaly showing two 

perspectives on the osteotomy sites (A & B, left) and after 

anterolateral transposition of the hemisupraorbital segment and 

bone grafting (A & B, right). (Reprinted with permission from 

Havlik RJ, Azurin DJ, Bartlett SP, Whitaker LA: Analysis and 

treatment of severe trigonocephaly. Plast Reconstr Surg 103:381, 

1999.) 

strated progression of the deformity, and 15 showed 

significant improvement in frontoorbital form. The 

authors believe that surgical correction is not justified 

in cases of metopic synostosis associated with only 

mild change in morphology and without evidence of 

functional disturbance. The risk of surgically induced 

complications, including failure of frontal sinus 

development, 229 outweighs the potential benefits of 

surgery. 

Scaphocephaly 

Sagittal synostosis, with its characteristic oblong 

calvarial shape (scaphocephaly), is the most common 

type of craniosynostosis. 230 The severity of the 

calvarial deformity varies from slight cranial elongation 

with sagittal ridging to extreme elongation with a 

large occipital shelf and pronounced frontal bossing. 

231 Persing, Jane, and Edgerton232 suggested that 

the deformity is not limited to the sagittal suture but 

also involves the base of the skull, which in part 

contributes to the degree of cranial dysmorphology. 

The optimal operative management of scaphocephaly 

is still debated. Multiple procedures are 

described in the literature. Strip craniectomy (Fig 


15) as described by Ingraham 233 and its modifications 

234 are employed in cases of early diagnosis, but 

have produced unreliable results. 235–239 This may be 

due in part to recurrence of the synostosis 240 or 

deformation where rigid fixation is not employed. 

The “pi technique” as described by Jane 241 and its 

modifications 242 have been associated with elevation 

in intracranial pressure. 243 Techniques that employ 

wider stripping, such as total vertex craniectomy, 244 

produce a superior result 198 compared with strip 

craniectomy. 245 Kaiser 198 compared the postoperative 

results of midline craniectomy, bilateral 

parasagittal craniectomy, and total vertex craniectomy. 

Cases of vertex craniectomy of Epstein 244 all 

had normalization of the cephalic index, while the 

other two groups were successful only one-fourth to 

one-half of the time. 

The trend to more radical and extensive correction 

of the entire cranial vault 236,246–248 has led to the 

goal of actively correcting the cranial index and 

attaining an ideal cranial shape at the time of surgery. 

Postoperative passive correction is not expected. 

For severe cases of sagittal synostosis, Marchac and 

coworkers 249 perform frontocranial remodeling in one 

stage. The supraorbital bar is usually left in place 

and the upper forehead is reconstructed just above 

it. Posteriorly the occipital bone is moved forward 

and the vault is constructed of three or four bone 

segments cut transversely like parts of a barrel. These 

segments are rearranged to shorten the anteriorposterior 

distance, expand the transverse diameter, 

and lift up the retrocoronal depression. 

Older affected children who have not been operated 

on and children with failed previous attempts at 

scaphocephalic correction probably warrant a more 

aggressive approach consisting of total calvarial 

remodeling with anterior craniofacial reconstruction, 

temporoparietal widening, posterior remodeling, and 

anterior-posterior shortening if required. 250 

Plagiocephaly 

Deformational Plagiocephaly 

Mulliken251 and Huang and colleagues252 review 

the morphologic findings in posterior plagiocephaly 

and the differential diagnosis between positional and 

synostotic (Fig 16). In Huang’s prospective series of 

115 infants with posterior plagiocephaly, only one 

had lambdoid synostosis. 252 

19


Fig 15. Child with sagittal synostosis before (A-C) and after (D-F) surgical correction. (Reprinted with permission from Persing JA, Jane 

JA, Edgerton MT: Surgical Treatment of Craniosynostosis. In: Persing JA, Edgerton MT, Jane JA (eds), Scientific Foundations and 

Surgical Treatment of Craniosynostosis. Baltimore, Williams & Wilkins, 1989, p 191.) 

The importance of differentiating between 

deformational and synostotic plagiocephaly 251 is that 

conservative therapy will achieve results equivalent 

to those of surgery. 253,254 Deformational plagiocephaly 

is best treated with positioning, frequent head 

turning, and helmet therapy. 141,255 Vies, 142 reporting 

on a series of 105 patients treated with either head 

positioning or helmet therapy, noted faster and better 

results in the helmet group. 

Synostotic Plagiocephaly 

Posterior synostotic plagiocephaly is due to lambdoid 

synostosis and accounts for only 1% of occipital 

plagiocephaly cases. 256 In analyzing their series, 

Huang and colleagues139,252 found that in true lambdoid 

synostosis the contralateral posterior bossing 

occurred more laterally and superiorly in the parietal 

region; frontal bossing was not a striking feature, but 

20 

when it occurred it was contralateral rather than 

ipsilateral; and ipsilateral occipitomastoid bossing was 

consistently present in lambdoid synostosis, whereas 

it was conspicuously absent in deformational posterior 

plagiocephaly. 

The treatment implications are clear: Few patients 

with positional plagiocephaly actually require operative 

correction. Their skulls will remold satisfactorily 

with conservative measures (positioning of the child in 

the crib or helmet therapy). The surgical correction of 

posterior plagiocephaly caused by unilateral lambdoid 

synostosis is via posterior craniofacial reconstruction 

and remodeling, as recommended by Persing. 257 

Anterior synostostic plagiocephaly is due to unilateral 

coronal suture synostosis. Coronal synostosis 

may be either unilateral or bilateral. In a 21 year 

experience consisting of 116 patients with coronal 

synostosis, 47% were unicoronal, 9% were bicoronal 

without associated syndromes, 34% were bicoronal


Fig 16. Posterior plagiocephaly from positional molding (above) and unilambdoid synostosis (below). Arrows indicate vectors of 

compensatory growth. (Reprinted with permission from Huang MHS, Mouradian WE, Cohen SR, Gruss JS: The differential diagnosis of 

abnormal head shapes: separating craniosynostosis from positional deformities and normal variants. Cleft Palate Craniofac J 35:204, 1998.) 

associated with a syndrome, and the remaining 20% 

were associated with multiple suture synostosis. 258 

Unicoronal synostosis causes regional growth 

restriction and compensatory expansion of the neighboring 

tissues, producing overt frontoorbital 

dysmorphology. Characteristic deformities ipsilateral 

to the synostosis include flattening of the frontal bone 

and ipsilateral forehead, ipsilateral elevation-recession 

of the supraorbital rim, and narrowing and lateral 

deviation of the orbit, deviation of the nasal root 

towards the flattened side, and elevation of the ipsilateral 

ear. 259 On the contralateral side there is bulging 

of the frontal bone. 260,261 An AP radiograph usually 

demonstrates the characteristic harlequin eye 

deformity. Bruneteau and Mulliken 262 believe that 

physical examination focusing on the supraorbital 

rims, nasal root, ears, and malar eminences can easily 

distinguish between synostotic and deformational 

plagiocephaly. This distinction has obvious clinical 

implications, as synostotic plagiocephaly is the only 

group with strong surgical implications. 

Lo and colleagues 263 described the endocranial configuration 

in unilateral coronal synostosis as follows: 

• constriction of the ipsilateral anterior cranial fossa 

• deviation of the anterior fossa midline 

• elevation of the ipsilateral floor 

• straightening of the lesser sphenoid wing 

These features of the cranial base correlate with 

the orbital dysmorphology. The authors reviewed 

28 patients with unicoronal synostosis and noted more 

21


symmetrical bony orbits and orbital contents during 

the first year after cranioorbital surgery. They conclude 

that surgery for unicoronal synostosis (Fig 17) 

in infancy does not inhibit growth of orbital hard or 

soft tissues and seems to allow normalization of previously 

impaired growth. 265 

Fig 17. Anterior plagiocephaly and its surgical correction. A, site 

of anterior cranial vault and three-quarter orbital osteotomies. B, 

reshaping and fixation of bone segments after osteotomies. 

(Reprinted with permission from Posnick JC: Craniosynostosis: 

Surgical Management in Infancy. In: Bell WH (ed), Orthognathic 

and Reconstructive Surgery. Philadelphia, WB Saunders, 1992; 

vol 3, p 1837.) 

Bicoronal Synostosis 

Bicoronal synostosis produces an abnormal skull 

shape—brachycephaly. The calvarium is short 

anteroposteriorly, widened mediolaterally, and elongated 

vertically compared with normal subjects. The 

orbital rims are hypoplastic, the occipital region is flattened, 

the frontal and temporal bones are protruberant, 

and the anterior cranial base is foreshortened. 266 

Although the brachycephalic deformity is characteristic 

of bicoronal synostosis, morphologic variants have 

been recognized. 267 

22 

Marchac’s 268 series of 35 children included 20 

patients with ‘simple’ and ‘familial’ brachycephaly 

treated by his “floating forehead” technique. Only 

one patient (5%) required secondary frontal advancement. 

In his series of 22 consecutive patients with 

nonsyndromic bicoronal synostosis, Wagner 266 reports 

62% relapse in patients operated on at age 5 months 

or younger. These infants subsequently needed total 

reoperation. In contrast, only 11% of children operated 

on at age >6mo showed recurrence of the 

deformity. This observation is contrary to contemporary 

theory and the experimental and clinical 

impression of others, which indicate that normalization 

of calvarial shape is more likely after early surgery. 

269,270 

SYNDROMES WITH CRANIOSYNOSTOSIS 

Craniosynostosis is not a syndrome in itself but a 

sign of at least 150 different syndromes. 271 A 

multidisciplinary team involving craniofacial, ophthalmologic, 

and ear-nose-throat surgeons, geneticists, 

neuropsychologists, nutritionists, and social workers 

will allow total patient care. Priorities regarding 

intracranial pressure, airway obstruction, and globe 

exposure will dictate surgical priorities. Some of the 

more common syndromes with craniosynostosis as 

their predominant physical finding are described 

below (Table 5). 

Apert Syndrome 

Originally described by Wheaton in 1894, the 

condition is named after Apert, who in 1906 

described 4 cases. 272 The incidence of Apert syndrome 

is reported to be 1:100,000 to 1:160,000 

births. 273 Although transmission is autosomal dominant, 

occurrence is sporadic as new mutations with 

normal karyotype. 

The skull in Apert syndrome is brachycephalic. 274 

Facial features incude hypoplasia of the midface, 

hypertelorism, 275 strabismus, ocular muscle palsies, 276 

and slanting palpebral fissures. There is moderate to 

severe exorbitism, short zygomatic arches and, in the 

euryprosopic type of Apert, a prominent bregmatic 

bump. The fontanelles may be large and late in 

closing. The palate is narrow and either has a median 

groove or is cleft with a bifid uvula (Fig 18). The 

limbs show bony syndactyly (secondary to the FGF-R

mutations implicated in the craniosynostosis 173 ) with 

complete fusion of the fingers and a free thumb. 

The distal phalanx of the thumb is often broad. 

Cutaneous syndactyly of all toes may be either simple 

or complex. There is moderate to severe facial and 

upper extremity acne. 

Mental retardation is variously reported in association 

with Apert syndrome, but it is unclear how 

much of it is secondary to environmental influences. 

Certainly many patients with Apert syndrome attain 

a high level of mental function. 

TABLE 5 

Syndromic Craniosynostoses 


(Reprinted with permission from Whitaker LA, Bartlett SP: Craniofacial Anomalies. In: Jurkiewicz MJ, Krizek TJ, Mathes SJ, Ariyan S (eds), 

Plastic Surgery. Principles and Practice. St Louis, CV Mosby, 1990. Vol 1, Ch 7.) 

Although head shape in Apert syndrome suggests 

bicoronal synostosis, a postmortem study of the craniofacial 

changes in the syndrome concluded that the 

pathology is not primarily due to craniosynostosis but 

rather stems from reduced growth potential of the 

cranial base, leading to premature fusion of the midline 

sutures from the occiput to the anterior nasal 

spine 273 and resulting in severe calvarial, maxillary, 

nasal, and mandibular abnormalities. Other autopsy 

reports 277 suggest that basisphenoid synchondrosis 

23


Fig 18. Child with Apert syndrome. A, B, clinical presentation. C, findings on 3-D reconstruction of CT scan. Note the synostotic coronal 

suture, the open fontanelle, and the midfacial hypoplasia. (Reprinted with permission from Buchman SR, Muraszko KM: Syndromic 

Craniosynostosis. In: Lin KY, Ogle RC, Jane JA (eds), Craniofacial Surgery. Science and Surgical Technique. Philadelphia, WB 

Saunders, 2002; Ch 18, pp 252-271.) 

may be the main factor in facial deformities through 

synostoses posteriorly and anteriorly to the vomer. 

For a comprehensive discussion of Apert syndrome, 

the reader is referred to the April 1991 issue of Clinics 

in Plastic Surgery, 278 which is devoted entirely to 

various aspects of the syndrome. 

Saethre-Chotzen Syndrome 

Saethre-Chotzen syndrome was first recognized 

as a discrete entity in 1931-32 by the physicians 

who gave their name to the syndrome. Saethre- 

Chotzen syndrome is characterized by a broad 

and variable pattern of craniofacial anomalies, frequently 

asymmetrical. Bicoronal synostosis, low 

hairline and upper eyelid ptosis characterize the 

syndrome. Intellect is normal. The midface is 

usually normal, which led Tessier273 to call it “upper 

Apert.” There is partial cutaneous or simple 

syndactyly, usually between the second and third 

fingers but sometimes involving the small finger 

too. Inheritance is autosomal dominant. 

Clauser279 reviewed the literature and found an 

incidence of 1 in 25000 to 1 in 50000. A mutation 

of the TWIST gene is identified in approximately 

68% of cases, and mutations of FGF-R2 and FGF-R3 

have been described. 

Pfeiffer Syndrome 

(Acrocephalosyndactyly Type V) 

Pfeiffer syndrome is characterized by broad thumbs 

and great toes. The severe midfacial hypoplasia 

24 

characteristic of Apert syndrome is present, but cranial 

vault malformations are not as extreme. Intellect 

is normal. Tessier 273 refers to Pfeiffer syndrome 

as a “low Apert.” 

A 1993 report 280 identified subgroups of Pfeiffer 

syndrome according to the apparently consistent patterns 

of phenotypic expression, including head shape. 

The classic syndrome (Cohen type I) consists of symmetrical 

bicoronal synostosis; all other sutures are 

normal. Cohen type II or cloverleaf skull is an 

expression of early, extensive multisuture synostosis. 

All affected subjects have multiple constricting rings 

of prematurely fused sutures, grossly foreshortened 

anterior and posterior cranial bases, and coincident 

hydrocephalus. Moore 281 states: “This early, extensive 

fusion of multiple suture rings in association with 

an intrinsic, or secondary, cranial base distortion promotes 

the development of hydrocephalus, raised 

intracranial pressure, calvarial vault thinning, and cranial 

lacunae. Despite early radical craniectomies 

and cranial vault reshaping in these patients, recurring 

fusion of the ‘neosutures’ is often rapid.” 

Cohen type III is intermediate in the pattern of 

extensive sutural involvement. In addition to the 

bicoronal synostosis, isolated fusion of other sutures 

is evident early. This becomes progressively more 

widespread with time. Cohen 280 describes patients 

with type III Pfeiffer syndrome as having severe ocular 

proptosis, an extremely short anterior cranial base, 

neurological compromise (hydrocephalus is common), 

and a poor prognosis with early death. All 

reported cases of type III Pfeiffer syndrome have 

been sporadic. 282

Pfeiffer syndrome is inherited as an autosomal 

dominant disorder. Muenke et al 283 and Kerr and 

colleagues 284 report an affected family in which some 

individuals have normal thumbs although several relatives 

do have hand anomalies (symphalangism). Not 

only is there phenotypic variability in Pfeiffer syndrome 

and other acrocephalic syndactylies, but recent 

molecular data indicate variable phenotypic expression 

for patients who have identical mutations. Point 

mutations in FGFR2 have been identified in Crouzon, 

Jackson-Weiss, and Apert syndromes in addition to 

sporadic cases of Pfeiffer syndrome. 285 In addition, a 

subset of Pfeiffer syndrome families have a common 

mutation in the FGFR1 gene. 

Carpenter Syndrome 

Carpenter syndrome was originally described in 

1901 by the man who lent it his name. Acrocephalopolysyndactyly 

or Carpenter syndrome consists 

of craniosynostosis, short fingers, soft tissue syndactyly, 

preaxial polydactyly, congenital heart disease, 

hypogenitalism, obesity, and umbilical hernia. 

As many as 75% of patients have some degree of 

intellectual impairment. 286 

The craniofacial anomalies are those of brachycephaly 

with variably severe synostosis of the coronal, 

sagittal, and lambdoid sutures, such that the 

calvarium is usually distorted and grossly asymmetrical, 

approaching the kleeblattschadel anomaly. 

Midfacial retrusion and dental malocclusion are less 

pronounced than in Apert, and syndactyly is only 

partial and usually simple. 273 This is one of the few 

craniofacial malformation syndromes that is inherited 

as an autosomal recessive. 

Poole287 cautions surgeons contemplating surgery 

on patients with Carpenter syndrome that they must 

beware of the venous and bony abnormalities attendant 

with this syndrome. The vascular anomalies 

may lead to excessive and rapid blood loss. 

Crouzon Syndrome 

The French neurosurgeon Crouzon described the 

disease that bears his name in 1912, at which time 

he listed four essential characteristics: exorbitism, 

retromaxillism, inframaxillism, and paradoxical 

retrogenia288 (Fig 19). The estimated incidence is 1 

in 25000 live births. 289 Inheritance is autosomal 


dominant and occurrence is both sporadic and 

familial. 

Fig 19. Clinical presentation of a child with Crouzon syndrome. 

Note exorbitism and hypertelorism, retromaxillism and midfacial 

hypoplasia, pseudoprognathic mandible, and paradoxical retrogenia. 

(Reprinted with permission from Buchman SR, Muraszko KM: 

Syndromic Craniosynostosis. In: Lin KY, Ogle RC, Jane JA (eds), 

Craniofacial Surgery. Science and Surgical Technique. Philadelphia, 

WB Saunders, 2002; Ch 18, pp 252-271.) 

There are many clinical variations of Crouzon disease, 

often with one or more of the “typical” signs 

missing. 290 The shape of the skull alone does not 

differentiate Apert syndrome from Crouzon disease, 

although Crouzon shows less severe deformity. 

Crouzon is characterized by recession of the frontal 

bone and supraorbital rim, retrusion of the facial 

mass, exorbitism with proptosis, and hypoplasia of 

the infraorbital rim. Unlike Apert syndrome, 

hypertelorism and a bregmatic bump may not be 

evident, the nose does not always deviate, and the 

orbital deformities tend to be symmetrical. The orbits 

are quite shallow, with hypoplastic and flat infraorbital 

rims, distention of the eyelids, palpebral fissures 

slanted downward, exophoria, and divergent strabismus; 

50% of patients with Crouzon disease have 

hyperactivity of the inferior oblique muscles. Crouzon 

patients usually develop normal intelligence and limb 

anomalies are absent. The mandible in Crouzon 

disease, as in Apert, is considerably shorter than normal, 

producing a distinctive ramus/body length ratio, 

particularly in older patients. 291 

25


Cephalometric analysis of patients with Crouzon 

disease shows a direct correlation between the altered 

facial morphology and abnormalities of the cranial 

base. The exorbitism is a reflection of vertical sloping 

of the anterior cranial fossa, while the midfacial retrusion 

can be traced to angulation of the cranial base 

flexure. 292 

Proudman and colleagues 293 reviewed 59 patients 

with Crouzon disease to determine the noncraniofacial 

manifestations. They conclude that 

Crouzon syndrome is not just a craniofacial deformity 

but “a dysplastic condition that can affect other 

areas of the bone and chondral growth, such as the 

spine and elbows. Ligaments and soft tissues may 

also be involved.” Cervical spine anomalies (40%), 

stylohyoid calcification (50%), elbow anomalies (18%), 

minor hand deformities (10%), other musculoskeletal 

anomalies (7%), and visceral anomalies (7%) were 

all present. 

Kreiborg 294 traces the craniofacial growth, clinical 

findings, craniofacial morphology, occlusion, and 

other aspects of dysmorphology in 61 patients with 

Crouzon syndrome before treatment. Extensive statistical 

data on adult skeletal dimensions are given, as 

well as a comprehensive bibliography. Later, Kreiborg 

and colleagues 295 used 3D CT scans to compare the 

anatomy of the calvarial cranial base in Apert and 

Crouzon syndromes. Their findings suggest that the 

conditions are very different in cranial development. 

Cartilage abnormalities, particularly those of the 

anterior cranial base, play a major role in cranial 

development in Apert syndrome, whereas the primary 

abnormality in Crouzon appears to be premature 

fusion of sutures and synchondrosis. The authors 

advocate early surgery in both groups of patients, 

but for different reasons: In Apert syndrome, early 

surgery is indicated to reduce further dysmorphic 

growth changes in the calvarial cranial base; in 

Crouzon syndrome, early surgery can prevent or 

relieve elevated intracranial pressure. 

Posnick and colleagues 296 reviewed their experience 

in 14 children with Crouzon syndrome who 

presented with bicoronal synostosis. Measurements 

of the cranioorbitozygomatic region taken from CT 

scans done pre- and postoperatively served to document 

the results of surgical correction. Early surgical 

attempts to decompress and reshape the cranioorbital 

regions may limit the effects of increased intracranial 

pressure but do not correct the deformity, as judged 

by CT scans. Although the Crouzon deformity did 

26 

not worsen after surgery, the measurements remained 

far from normal. Perhaps more importantly, the 

maxillary and mandibular deformities were not 

addressed and the vertical dimension of the face was 

unchanged. 

In their review of syndromic craniosynostoses 

treated at NYU, McCarthy and associates 269 note 

disappointing aesthetic and functional results of surgery 

compared with cases of isolated craniosynostosis. 

The incidence of major secondary procedures, 

peri- and postoperative complications, hydrocephalus, 

shunts, and seizures was significantly increased 

in syndromic patients. Frequent problems included 

increased intracranial pressure, airway obstruction, 

and recurrent turricephaly or cranial vault maldevelopment. 

Moreover, early frontoorbital advancementremodeling 

“failed to promote midface development.” 

Craniofrontonasal Dysplasia 

Craniofrontonasal dysplasia (CFND) is a rare 

familial craniofacial disorder first described by 

Cohen. 297 The syndrome combines coronal craniosynostosis 

and frontonasal dysplasia with a variety of 

extracranial abnormalities. The coronal synostosis 

results in brachycephaly (usually asymmetrical) and 

frontal bossing, while the frontonasal “dysplasia” 

manifests as hypertelorism and a broad nose, frequently 

with a bifid nasal tip. Other features are 

curly hair, grooved nails, cleft lip and palate, high 

arched palate, strabismus, shoulder and hip girdle 

anomalies, soft-tissue syndactyly of fingers and toes, 

and a broad great toe. 298 

Orr and colleagues299 reviewed their experience 

with craniofrontonasal dysplasia in 10 female patients. 

Male patients seem to have a milder phenotype. 

The precise mode of genetic transmission is unclear, 

but maternal transmission to daughters and sons has 

been reported. Affected males have passed the condition 

to 100% of daughters in some published pedigrees, 

but male to male transmission is not known to 

occur. Treatment is in two stages, one for correction 

of the craniosynostosis and the other to deal with the 

hypertelorism. Six patients had facial bipartition and 

3 patients had combined intra- and extracranial 

periorbital osteotomies, resection of excess midline 

or paramedian bony tissue and ethmoid sinuses, and 

medial translocation of the orbits.

Associated Anomalies 

Although cervical spine anomalies are not usually 

mentioned in association with craniosynostosis, 

intervertebral fusion has been documented in 71% 

of children with Apert syndrome, in 38% of those 

with Crouzon disease, and in 30% of those with 

Pfeiffer syndrome. 300 Fusions are most often isolated 

and involve complex C5-6 lesions in Apert syndrome 

and the upper-level cervical vertebrae in Pfeiffer and 

Crouzon patients. Affected children may have limited 

range of cervical motion, which has implications 

for airway management during surgery. 300 

III — ATROPHY/HYPOPLASIA 

Romberg Disease 

(Progressive Hemifacial Atrophy) 

Romberg disease was first described by Parry301 in 

1825 and later by Romberg302 in 1946. Eulenberg303 coined the term “progressive facial hemiatrophy” in 

1871. The disease commences usually in the first or 

second decade of life and is more common in girls 

than boys by a 1.5:1 ratio. 304 The atrophy is unilateral 

in 95% of cases and affects either side of the 

face with equal frequency. 

The etiology of the disorder is unknown, although 

many theories for its pathogenesis have been proposed. 

Foremost among these are infection, 305 

trigeminal peripheral neuritis, 306 scleroderma, 307 and 

cervical sympathetic loss. 308 

The condition manifests as progressive hemifacial 

atrophy of skin, soft tissue, and bone. Pensler and 

colleagues308 evaluated 41 patients and noted that all 

atrophic changes began in a localized area and progressed 

at a variable rate within the dermatome of 

one or more branches of the ipsilateral fifth cranial 

nerve. The average age at inception of the disease 

was 8.8 years. The main period of progression was 

8.9 ± 6 years. In 26 patients with skeletal involvement, 

the mean age of onset was 5.4 years vs 15.4 

years for 15 patients without skeletal involvement. 

No correlation could be established between the 

severity of soft-tissue deformity and the age of onset. 

Tissue from 6 patients who had ultrastructural 

analysis revealed a lymphocytic neurovasculitis with 

striking abnormalities of the vascular endothelium 

and basement membrane. The alterations of the 

vascular basal lamina in lymphocytic neurovasculitis 


appears to reflect chronic vascular damage with repeat 

attempts at endothelial cell regeneration. 

Moore and colleagues 309 noted 50% of patients 

with Romberg disease had the classic early sign of 

coup de sabre, reflecting soft-tissue involvement in 

the upper face (frontal and maxillary dermatomes). 

In the presence of prolonged active disease, the softtissue 

atrophy extended to involve the whole hemiface. 

Late-onset disease appears to be characterized by 

soft-tissue atrophy in the lower face. Bony hypoplasia 

in the mid and lower face was most common. 

Involvement of the frontal region was relatively infrequent. 

The derangement of the craniofacial skeleton is 

unlikely to be solely due to an isolated intrinsic process. 

Moore et al 309 surmise that restriction of the 

abnormal soft-tissue envelope undoubtedly compounds 

any primary skeletal growth disturbance. If 

the disease involves bone, it likely exerts its effect on 

the craniofacial skeleton only during periods of facial 

growth acceleration. 

Treatment involves 3D reconstruction of all softtissue 

and skeletal disturbances. Surgery is usually 

undertaken at least 1 year after photographic records 

show no further loss of volume. Greater omentum 

free flaps have been described by Jurkiewicz and 

Nahai, 310 who note problems with lack of structural 

strength and gravitational descent. 

Inigo and colleagues 311 review their experience 

with dermis-fat free flaps in 35 patients with Romberg 

disease. They used the groin free flap in 33 

patients and a scapular flap in 3 patients in a twostage 

procedure consisting of transfer of the free 

flap and defatting and repositioning 6 months later. 

Adjuvant procedures included temporal fascial flaps 

for the frontal regions and cartilage grafts in the 

piriform fossa. The chin was corrected by either 

sliding osteotomies for projections of >1cm and 

by alloplastic implants for projections of


placing the dermis outward. Harashina and Fujino314 argue that placing the dermis side down should reduce 

gravitational sagging, one of the major problems with 

the reconstruction in this situation. The abdominal 

flap, a variation of the groin flap based on the inferior 

epigastric vessels, has also been reported. 310,315 

Koshima et al316 describe their experience with the 

deep inferior epigastric perforator flap (DIEP). 

Mordick and colleagues317 compared the outcome 

of reconstruction with dermal fat grafts (8) vs vascularized 

tissue transfers (8) and and note that vascularized 

transfers provide better augmentation, 

although dermal fat grafts gave a satisfactory result in 

mild to moderate defects. Dermal fat grafting is a 

shorter procedure requiring less anesthetic time, less 

technical expertise and support, and shorter hospital 

stays than microsurgical transfers. Free flaps containing 

adipose tissue appear to increase in volume during 

growth and may also increase with weight gain. 

As their series progressed, the authors opted increasingly 

for augmentation with the scapular flap because 

of its large-caliber vessels and long pedicle. Their 

patients averaged 3.3 procedures for final correction. 

Upton and colleagues318 report their experience 

with scapular flaps for cheek reconstruction in 28 

patients, 5 of whom had Romberg disease. The 

major advantages of the scapular flap were a constant 

proximal vascular anatomy, long vascular pedicle 

with large-caliber vessels, large amount of available 

tissue (including bone), relatively hairless skin in most 

people, and minimal or no functional problems at 

the donor site. Disadvantages included the lack of 

sensation, poor external cheek skin color match, and 

a predictably widened scar at the donor site. The 

study produced the following observations: 

• deficiency of the malar prominence cannot always 

be corrected with soft tissue alone and often needs 

bone grafting or alloplastic implant; 

• when long fascial extensions are used for correction, 

they must be transferred across the midline 

of the upper and lower lips; 

• the area most consistently undercorrected is the 

medial portion of the deficient upper and lower lip; 

• the area most consistently overcorrected overlies 

the mandibular body and ramus. 

Longaker and Siebert 319 reported their experience 

with 15 cases of Romberg disease representing 16 

28 

free tissue transfers. Deepithelialized, extended 

parascapular flaps with large fascial extensions of the 

dorsal thoracic fascia were used for reconstruction. 

The fascia can be folded into variable thicknesses to 

correct subtle contour defects of the upper lip, medial 

canthus, eyelids, and other facial features traditionally 

difficult to reconstruct. These extensions can be placed 

easily across the midline to interdigitate with normal 

tissues at the boundary of the facial deformity. 

Past treatment methods have included silicone fluid 

injections, 320 which are contraindicated because of 

long term complications, and injections of lipoaspirated 

fat, which have met with mixed results. 321 

Autologous fat injections certainly have a place in 

the correction of mild contour irregularities. Muscular 

atrophy of pedicled or free muscle flaps present a 

problem in calculating the final volume needed for 

the repair. 322 

IV — NEOPLASIA/HYPERPLASIA 

Craniofacial Tumors 

Intracranial neoplasms may be of many different 

types, such as fibrous dysplasia, simple osteoma, 

benign angiofibroma, neural tumor—specifically 

meningioma with extracranial erosion, encapsulated 

neurofibroma, schwannoma, or neurilemmoma323–325 

—cutaneous carcinoma, osteogenic 

sarcoma, and rhabdomyosarcoma. 

The first major offshoot from craniofacial surgery 

was the application of craniofacial techniques to the 

ablation of intracranial tumors. As clinical experience 

mounted, the following principles for the surgical 

management of craniofacial neoplasms evolved: 

• It is now possible to resect lesions that were previously 

considered unresectable. 326 

• Tumor position should be related to the base of 

the skull. 327 

• Tumor resection demands strict adherence to 

guidelines designed for tumor location, cell type, 

and stage, as well as the concept of complete “en 

bloc” excision. This latter is possible through 

regional orbitotomies for confined orbital tumors, 

bifrontal craniotomy, and facial incisions (eg, 

Weber-Ferguson) where necessary. 

• There is significant risk of losing the frontal bone 

flap to infection. 328,329

• The transfacial approach330 may reduce the frequency 

of infectious complications. 

• It is essential to prevent communication between 

the sinuses or nasal cavity and the meninges or 

intracranial dead space. The best way to achieve 

this is by interposing thin, vascularized tissue, such 

as a pedicled paracranial flap for very small defects, 

a galea frontalis flap for anterior defects, 331 and a 

temporalis muscle332 or temporoparietalis fascial 

flap for lateral and central defects. 

• Free flaps may be used not only to isolate and 

protect vital structures, but also to bring large 

amounts of soft-tissue bulk for contouring. Free 

flaps may be transferred secondarily to deal with 

complications, but probably should be raised prophylactically 

at the time of the ablative procedure. 

FIBROUS DYSPLASIA 

The most common osseous craniofacial tumor 

encountered by plastic surgeons is fibrous dysplasia. 

335 Fibrous dysplasia is an uncommon, nonneoplastic, 

benign disease of bone that was was first 

described by von Recklinghausen in 1891. 336 The 

pathogenesis of fibrous dysplasia involves abnormal 

activity of the bone-forming mesenchyme with an 

arrest of bone maturation in the woven bone stage, 

forming irregularly shaped trabecula. Mutations of 

signaling protein and increased IL-6 levels have been 

implicated in the process. 337 The condition is usually 

progressive until age 30, and reports of progression 

well into adulthood are not uncommon. 338 

Fibrous dysplasia in the cranioorbital area tends to 

be more osseous than fibrous dysplasia of other sites. 

Two patterns of presentation predominate: the 

monostotic form, with single-bone involvement, and 

the polyostotic variety, which may be associated with 

abnormal skin pigmentation, premature sexual 

development, and hyperthyroidism (Albright syndrome). 

The monostotic form is approximately 4X 

more common than the polyostotic form and 

approximately 30X more frequent than the complete 

Albright syndrome. The monostotic form commonly 

involves the ribs, femur, tibia, cranium, maxilla, 

and mandible. The most commonly affected 

bones in the cranium are the frontal and sphenoid 

bone; in the face, the maxilla338,339 (Fig 20). 


Posnick339 lists the keys to management of fibrous 

dysplasia, namely 

• accurate diagnosis 

• radiographic assessment 

• clinical evaluation 

• planning of interventions 

• long term follow-up 

Deformation caused by fibrous dysplasia of the 

anterior skull base can affect the architecture of 

the orbits and paranasal sinus, causing orbital displacement 

and exophthalmos. 340,341 In craniofacial 

fibrous dysplasia, the symptoms are varied and 

related to tumor mass, and may include facial pain 

and swelling, headaches, anosmia, deafness, blindness, 

malocclusion with displacement of teeth, 

diplopia, proptosis, and orbital dystopia. Cranial 

nerve palsies including optic nerve compression 

are not uncommon. 342 Eye symptoms may include 

extraocular muscle palsy and trigeminal neuralgia 

secondary to compression of the third and fifth 

cranial nerves. Orbital apex compression can initiate 

the cascade of ophthalmic venous engorgement, 

optic nerve atrophy, and loss of vision. Sphenoid 

body involvement can cause blindness as a 

result of compression of the optic nerve between 

the chiasm and optic foramen. Other minor ophthalmic 

symptoms include epiphora produced by 

occlusion of the lacrimal duct and visible external 

lid deformities. 343 

Malignant degeneration occurs in approximately 

0.5% of cases. 344 Clinical signs of malignancy consist 

of a rapid increase in size, pain, intralesional necrosis, 

bleeding, and elevation of serum alkaline phosphatase 

levels. The most common transformation is 

to osteogenic sarcoma, but fibrosarcoma and chondrosarcoma 

are also seen. The mean interval from 

diagnosis of fibrous dysplasia to evidence of malignancy 

is 13.5 years. 343 The polyostotic form of the 

disease has a higher incidence of malignant degeneration, 

although in the craniofacial region the 

monostotic form is more common. Malignant 

degeneration can occur spontaneously or following 

radiotherapy. 

Cherubism is a genetic disorder of the mandible 

and maxilla of the giant-cell type. It is strictly a selflimiting 

disease of children that regresses without surgery 

and leaves no deformity. 345 

29


Fig 24. A 5-year-old girl with polyostotic fibrous dysplasia and massive involvement of the maxilla and mandible bilaterally. A, C, D, before 

operation. B, 10 days after radical maxillary and mandibular debulking. (Reprinted with permission from Posnick JC, Hughes CA, Milmoe 

G, et al: Polyostotic fibrous dysplasia: an unusual presentation in childhood. J Oral Maxillofac Surg 54:1458,1996.) 

In the past, conservative treatment of fibrous dysplasia 

was preferred, occasionally with bonecontouring 

procedures. Spontaneous involution will 

not occur, however, and early surgical intervention 

when symptoms are just beginning may avoid extensive 

resection later. 346 When clinically feasible, the 

mainstay of therapy is complete or near-complete 

resection and reconstruction with normal autogenous 

bone. 

In 1972 Derome 347 pioneered the concept of total 

excision and immediate reconstruction of bone 

tumors, including 4 cases of fibrous dysplasia. Chen 

and colleagues 348 discussed the benefits and risks of 

prophylactic optic nerve decompression before symptoms 

develop. The decision for surgery in these 

cases is made solely on the basis of CT findings of 

encroachment of the optic canal by fibrous dyspla- 

30 

sia. The primary justification for prophylactic 

decompression is the speed with which visual deterioration 

can occur and the brief interval before it 

becomes permanent. Among reports of sudden loss 

of vision there are several that attributed the cause of 

blindness to hemorrhage or mucocele secondary to 

fibrous dysplasia. 349 In their own study of 18 patients 

with clinical or radiological evidence of optic canal 

involvement, Chen and associates 348 report 6 patients 

(33%) had loss of effective vision in the involved eye. 

From this experience and a review of the literature, 

the authors developed an algorithm for decompression 

of the optic nerve in fibrous dysplasia. Absolute 

indications for decompression are 

• progressive gradual visual loss 

• within 1 week of sudden visual loss

Relative indications for decompression are 

• patients presenting within 2–3 weeks of rapid visual 

loss 

• children or adolescents with no visual loss but 

radiographic evidence of optic canal reduction, 

since they are likely to have progressive growth of 

fibrous dysplasia 

• patients with no visual loss who have radiographic 

evidence of optic canal reduction and continuing, 

active fibrous dysplasia. 

Reconstruction after resection is typically immediate. 

Edgerton and colleagues336 described the use of 

the dysplastic bone as grafts, which following resection 

were contoured, thinned, and replaced. These 

grafts of dysplastic bone seem to function similarly to 

normal autogenous grafts and lack the potential for 

gradual recurrence of bone thickening frequently seen 

with in situ bone contouring. Autoclaving350 and cryotherapy351,352 

have been proposed to destroy the cellular 

elements and yet preserve the mineral matrix 

prior to reinsertion. 

Chen and Noordhoff353 analyzed their experience 

with the treatment of 28 patients with fibrous dysplasia. 

The authors outline a protocol for surgery (Table 

6) that includes 

1. total excision of dysplastic bone of the frontoorbital, 

zygomatic, and upper maxillary region 

and primary reconstruction with bone grafts; 

2. conservative excision of bone from under the hairbearing 

skull, central cranial base, and toothbearing 

regions; and 


3. optic canal decompression in patients with orbital 

involvement and decreasing visual acuity. 

In a follow-up averaging 5.3 years, they find no 

recurrence or invasion of fibrous dysplasia into the 

grafted bone, but 5 of 19 patients treated with 

reduction of alveolar dysplasia had recurrence of the 

deformity that necessitated reshaping. One patient 

with recurrent mandibular fibrous dysplasia was successfully 

treated with hemimandibulectomy and mandibular 

reconstruction with vascularized bone graft. 

Hansen-Knarhoy and Poole 354 remark on the difficulty 

differentiating fibrous dysplasia from 

intraosseous meningioma around the orbital apex. 

From a review of their files, they were able to summarize 

the differences as follows: 

• Fibrous dysplasia commences in childhood and 

early adolescence while meningiomas are diseases 

of middleage. 

• Visual symptoms are prominent in meningioma 

cases and uncommon in fibrous dysplasia. 

• Proptosis is much more marked than orbital dystopia 

in the meningioma group while the reverse 

is true in fibrous dysplasia. 

• Fibrous dysplasia patients have extensive frontal 

bone involvement that is clinically obvious. There 

is no evidence of this in meningioma patients. 

• Pain is present in both groups and is not a useful 

discriminating feature. 

Most fibrous dysplasia lesions can be only partly 

excised and some are unresectable, arguing for treat- 

TABLE 6 

Treatment Protocol Based on Location, as Proposed by Chen and Noordhoff 

(Reprinted with permission from Chen Y-R, Noordhoff MS: Treatment of craniomaxillofacial fibrous dysplasia: How early and how 

extensive? Plast Reconstr Surg 86:835, 1990.) 

31


ment modalities other than surgery. No medical 

treatment is available to cure or definitively halt the 

progression of fibrous dysplasia. Reports of promising 

responses to systemic treatments with biphosphonate 

pamidronate await further studies before conclusions 

can be drawn. 355 Clark and Hobar 356 

implanted fibrous dysplasia cells in a nude mouse 

model and successfully decreased the size of the 

tumor with tamoxifen administration. 

NEUROFIBROMATOSIS 

Neurofibromatosis is a hereditary condition 

occurring in 1 in 3000 live births. Approximately 

50% of patients have a positive family history of neurofibromatosis. 

The term neurofibromatosis is applied 

to two clinical genetic disorders. The first, neurofibromatosis 

type 1 (NF1), also known as von 

Recklinghausen disease, is more common. The second, 

neurofibromatosis type 2 (NF2), is a rarer condition 

and is known as bilateral acoustic neurofibromatosis. 

The two disorders have very distinctive clinical 

manifestations and some overlapping features. 

Mutation of genes on two different chromosomes 

are at the origin of the two disorders. The gene for 

NF1 is located on the long arm of chromosome 17, 

while the gene for NF2 is located on the long arm of 

chromosome 22. 357 Transmission is autosomal dominant, 

with variable penetrance. 

Neurofibromatosis is a benign tumor of neuroectodermal 

origin with diffusion to skin, subcutaneous 

tissue, and bone (Fig 21). Tumor growth is slow and 

irregular and not accelerated by surgical intervention. 

Sarcomatous degeneration is rare. 

Surgical care often produces less than complete 

correction, with some further progression over 

time. 358,359 Poole, 358 in a series of 11 patients, stresses 

the high complication rate and modest improvement 

in appearance that should be expected. The best 

cosmetic results are obtained in patients whose eye 

can be removed and a satisfactory bed created for 

an orbital prosthesis. Patients who have a seeing eye 

and wish to retain it should undergo a two-stage 

procedure with conservative debulking of intraorbital 

soft-tissue. 

Krastanova-Lolov and Hamza357 reviewed their 

experience with 14 cases of cranioorbital neurofibromatosis. 

They note the partial or complete 

absence of the greater wing of the sphenoid is 

responsible for enlargement of the sphenoidal fis- 

32 

Fig 21. Patient showing clinical evidence of advanced neurofibroma. 

Photo courtesy of PC Hobar MD. 

sure, with a consequent defect in the posterior wall 

of the orbit. Brain tissue, generally the temporal 

lobe, may herniate into the orbit, further increasing 

exorbitism and causing pulsation of the eye. The 

orbit is enlarged, with hypoplasia of the supraorbital 

and infraorbital rims and the zygomatic arch. When 

the globe is involved with the neurofibroma there 

may be buphthalmos, severely diminished visual acuity, 

and even blindness. Associated symptoms are 

irritation, pain in the eye, and moderate to severe 

epiphora. Enophthalmos may occur from enlargement 

of the inferior orbital fissure, which allows the 

orbital contents to prolapse into the infratemporal 

fossa. 

Snyder 360 describes the experience of the Australian 

Craniofacial Unit in correcting 14 cases of orbital 

neurofibromatosis. Of note is the finding of resorption 

of the bone graft used to reconstruct the greater 

wing of the sphenoid in four cases, which led to recurrence 

of globe pulsation. Subsequently the author 

began using titanium mesh in the reconstruction. 

Jackson 361 presents his experience with orbitotemporal 

neurofibromatosis in 24 patients and provides 

a classification and treatment scheme. He 

groups the condition into three categories, each of 

which requires a different treatment: (1) orbital softtissue 

involvement with a seeing eye; (2) orbital softtissue 

and significant bone involvement with a seeing 

eye; and (3) orbital soft-tissue and significant bone 

involvement with a blind or absent eye. The author

traces the patients’ course over a maximum of 12 

years of recurrence-free follow-up. 

V — UNCLASSIFIED 

A few rare anomalies do not fit into the first four 

categories and are best placed in this last category. 

They should be described based an the organ or 

organs involved. Examples include aglossia or macroglossia, 

anotia,and ocular anomalies such as 

epicanthal folds. 10 

CRANIOMAXILLOFACIAL SURGERY 

HISTORY 

The origin of craniofacial surgery can be traced as 

far back as 1890, when Lannelongue and Lane performed 

the first craniotomies, 362,363 but it was not 

until the First and Second World Wars that numerous 

battle casualties stimulated the development of 

techniques for the replacement of missing bone and 

soft-tissue of the face, and set the stage for attempts 

in the 1940s and ’50s to correct congenital facial 

deformities. 

Waterhouse 364 reviews the history of craniofacial 

surgery with particular emphasis on the contributions 

by Le Fort, Virchow, Gillies, and Tessier. In 

1951 Gillies and Harrison 365 reported the first successful 

Le Fort III advancement osteotomy for correction 

of midfacial retrusion in a patient with 

Crouzon syndrome. The operation was technically 

very complex and only partially successful in correcting 

the exorbitism, because it carried the osteotomy 

in front of the orbital rim, lacrimal sac, and medial 

canthal ligament. In 1962 Converse and Smith 366 

described an operation to correct hypertelorism based 

on their experience with malunited nasoorbital fractures 

with telecanthus. 

Paul Tessier is regarded by most clinicians as the 

father of craniofacial surgery. After years of careful 

study with many of the most innovative maxillofacial 

surgeons of his time and numerous hours spent in 

the laboratory doing cadaver dissections, Tessier proposed 

a novel method of facial osteotomies and 

wholesale mobilization of bone for operating on 

patients with deformities of the craniofacial skeleton. 

Tessier pioneered the intracranial approach for the 

correction of hypertelorism, worked closely with 

neurosurgeons to resect difficult craniofacial tumors, 


was first to manipulate cranial bones in the treatment 

of patients with craniosynostoses, and performed 

successful Le Fort III operations in difficult 

cases of Apert and Crouzon syndrome. In 1967 

Tessier 367 presented this work at the Fourth International 

Congress of Plastic and Reconstructive Surgery 

in Rome, and the new field of craniofacial surgery 

was launched. 

Two principles fundamental to the practice of 

craniomaxillofacial surgery emerged from his discussion: 

(1) large segments of the facial and cranial skeleton 

can be completely denuded of their blood supply, 

repositioned, and yet survive completely; and 

(2) the eyes can be translocated horizontally or vertically 

over a considerable distance without impairing 

the vision. 

Tessier’s results in the late ’60s and early ’70s 

proved conclusively that most skeletal deformities of 

the face and calvarium can be corrected or at least 

significantly improved by appropriate surgical 

maneuvers. The importance of his work to the 

intracranial correction of hypertelorism 368–370 cannot 

be overemphasized. Along with Converse’s onestage 

procedure, it is the backbone of present-day 

techniques for hypertelorism correction. 

Basing his approach on Le Fort’s anatomic research 

with cadaver skulls, 371 Tessier developed the Le Fort 

III osteotomy for facial advancement and presented 

his results in 1971. His experience encompassed 

151 patients representing almost 500 individual malformations 

of the cranial and facial region. Tessier’s 

contributions to craniofacial surgery were reviewed 

in Rome in 1982 on the occasion of the 15th anniversary 

of Tessier’s original presentation. 372 

The advent of plate-and-screw fixation after 

maxillary and mandibular osteotomies has been 

of tremendous benefit to the management of congenital 

as well as traumatic cases of craniomaxillofacial 

deformity. Rigid fixation has dramatically 

enhanced the stability of bony fragments, 

improved primary bone healing, and eliminated 

the need for prolonged maxillomandibular fixation 

in the older patient. 373–378 

The fate of microfixation hardware during rapid 

calvarial growth in infants and young children has 

been questioned. Munro and colleagues 379 acknowledge 

intracranial “migration” of microplates and 

screws. Goldberg and colleagues 380 report cases of 

children undergoing revisional surgery who were 

found to have microscrews and plates lying beneath 

33


the inner calvarial lamina. Subsequently Goldberg 

381 sought to clarify the incidence of marker 

plate translocation and tried to identify potential 

clinical implications. In a retrospective review of 

27 pediatric patients, CT imaging demonstrated 

internalization of marker fixation in 14 children. 

The younger the patient at the time of surgery, the 

higher the likelihood of translocation. Patients 

with syndromic craniosynostosis seemed to have a 

greater risk of translocation than those with isolated 

synostosis. Longer plates had a higher propensity 

for movement than shorter plates. Despite 

these observations, the authors note no complications 

related to the translocation of marker plates. 

Papay et al 382 state that plating osteosynthesis provides 

a three-dimensional, stable fixation of the cranial 

skeleton and anatomical recontouring of the 

cranial vault. In their series of 20 patients who had 

secondary cranial remodeling within 2 years of the 

first surgery, “false migration” of microplates was 

noted in 7. Neither the sharp edges of the screws 

nor the ends of the stainless steel wires pierced the 

dura mater in any patient. Despite the lack of neurological 

sequelae from these fixation systems, it is 

their opinion that marker plate fixation for cranial 

vault reconstruction should be limited to those regions 

where additional 3D structural support is essential. 

Berryhill et al 383 reviewed the fate of rigid plate 

fixation in 96 children. There were 375 titanium 

plates and 1944 screws placed among the patients in 

the series. They found 5 cases of delayed growth, 

one instance of restricted growth, 9 palpable plates 

causing pain, 3 fluid accumulations over plates, and 

2 cases of meningitis. Plates were removed in 8 

patients. The overall complication rate was 23%. 

A series by Orringer reported 55 instances of plate 

removal. 384 The most common reason was palpable 

or prominent hardware (34.5%). Other reasons 

included loose plates (25.5%), infection (23.6%), and 

exposure of hardware (20%). 

Resorbable plating systems have enjoyed wide 

acceptance in craniofacial surgery. The Lactosorb 

(Poly-Medics, Warsaw IN) plating material is a 

copolymer of polylactic and polyglycolic acid. 

Polyglycolic acid biodegrades more rapidly than 

polylactic acid, and thus these combination plates 

resorb faster than plates made entirely of polylactic 

acid. 385 

Eppley and Sadove 386 reviewed the effects of biodegradable 

plates on 10 juvenile rabbits. The fixa- 

34 

tion was placed across the left coronal suture. Six 

months after implantation, the authors noted symmetrical 

frontal bone development, unaffected 

growth across the coronal suture, and a histologically 

normal underlying suture. Apparently the fixation 

plate stretches to accommodate growth along the 

underlying suture. The change in shape of the 

resorbable device seems to be more important than 

complete degradation of the material in rapidly growing 

bone sites. 

Subsequently Eppley and Sadove 387 analyzed 

their results with resorbable coupling fixation in 

20 infants with calvarial deformities. Thin, straight, 

resorbable plates were used for skeletal fixation 

after osteotomies and repositioning. A total of 

231 fixation devices were implanted without complications 

and remained trouble-free after 12 postoperative 

months. Wiltfang et al 388 noted that the 

resorbable plates were prone to intraosseous 

migration (as are titanium plates), but their resorption 

over 12–18 months obviates this problem. 

Multiple other clinical series with long follow-up 

show the Lactosorb plating system to be associated 

with minimal or no complications. 389–391 

Other areas of significant advance in the field of 

craniofacial surgery include radiographic improvements 

such as MRI scans and 3D CT scans, 392 distraction 

osteogenesis, increased use of microvascular 

techniques and tissue expansion, and better understanding 

of alloplastic materials. 

SURGERY ON THE CRANIAL VAULT AND FOREHEAD 

Early Treatment 

The rationale for early treatment in craniosynostosis 

is to minimize the extent of primary and secondary 

calvarial deformity, to allow cranial expansion 

and prevent the sequelae of raised intracranial 

pressure, and to minimize operative morbidity. 

The history of neurosurgical craniectomy for the 

correction of craniosynostosis can be divided into 

two basic operative approaches. In the past the 

standard treatment was a linear craniectomy along 

the course of the stenosed suture line. This treatment 

was associated with a high rate of recurrence 

of craniosynostosis, and attempts at preventing relapse 

by the use of polyethylene film placed around the 

bone edges met with little success. 393 More recently 

the trend has been towards a more active ana-

tomical correction of the deformitiy at the time of 

surgery, with less reliance on passive correction 

postoperatively, although many authors still advocate 

vertex craniectomy for young infants with sagittal 

synostosis. 394 

In 1971 Tessier 275 mobilized the lower part of the 

frontal bone in one piece while performing maxillary 

advancement in adult patients with Crouzon or Apert 

syndromes. Hoffmann and Mohr 395 adapted Tessier’s 

supraorbital advancement techniques to infants, performing 

a unilateral coronal supraorbital advancement. 

Marchac 396 later devised a technique that involved 

(1) rocking and advancement of the supraorbital bar 

with a lateral temporal spur in the form of a Z-plasty 

for stability and retention—the floating forehead— 

and (2) mobilization and rearrangement of the anterior 

cranial vault by means of bone grafts. Marchac 

originally used the floating forehead technique with 

frontocranial remodeling in the treatment of 

brachycephaly, Crouzon or Apert syndromes, and 

oxycephaly. 397 Marchac had originally hoped that 

the floating forehead technique would allow normalization 

of midfacial growth, but unfortunately this 

did not materialize. 

Other techniques utilize the mathematics of projection, 

geometry, and stress relief to produce an 

appropriate forehead contour. 398 Other methods 

treat the forehead as a totally misshapen unit that 

must be adjusted in all three dimensions with multiple 

osteotomies and cranial bone grafts to recreate 

the forehead/bandeau unit in jigsaw-puzzle fashion 

(Fig 22). 399 

Munro and coworkers 400 advocate 180° reversal 

of the entire upper cranium, while Jackson, Hide, 

and Barker 401 prefer frontal transposition cranioplasty 

for correction of frontal contour. Ortiz Monasterio 

and colleagues 402 combine frontal mobilization with 

a one-piece orbitofacial advancement in Crouzon 

syndrome. 

Operations for frontocranial remodeling have 

facilitated the approach to cranial as well as facial 

deformities and have considerably improved the overall 

surgical results. The vast number and variety of 

methods to reshape the orbital rim and cranial vault 

are proof that no technique is ideal in all cases. The 

basic concept should be to reshape all bone that is 

abnormal and to rearrange bone to arrive at the 


Fig 22. Bilateral coronal synostosis and its surgical correction. A, 

site of anterior cranial vault and three-quarter orbital osteotomies. 

B, after osteotomies and reshaping and fixation of the 

cranioorbital region. (Reprinted with permission from Posnick 

JC: Craniosynostosis: Surgical Management in Infancy. In: Bell 

WH (ed), Orthognathic and Reconstructive Surgery. Philadelphia, 

WB Saunders, 1992; vol 3, p 1859.) 

most aesthetic contour, and the degree to which 

each of these maneuvers is applied differs among 

patients. For example, at times it may be preferable 

to shape the forehead by recontouring the existing 

frontal bone, while at other times this goal is best 

accomplished by replacing the frontal bone with 

parietal bone. Early frontal remodeling is facilitated 

by easy malleability of bone; rapid reossification; 

outward push by the growing brain; and the beneficial 

effect on adjacent structures of releasing the 

stenosed areas. 136,199 On the other hand, the earlier 

35


in infancy the cranium is reshaped, the more it can 

be potentially affected by growth. 403,404 

McCarthy et al 405 reported an adverse effect on 

frontal sinus development and forehead aesthetics 

of early frontoorbital advancement. Of 8 patients 

who underwent bilateral frontoorbital advancement 

in infancy who had been followed for at least 10½ 

years, only one developed a frontal sinus and all had 

a flattened brow. Of 3 patients with unilateral 

advancement, one developed a frontal sinus on the 

operated side and the others had an obvious deformity 

due to discrepancy in brow projection between 

the operated and nonoperated sides. The authors 405 

advocate bilateral frontoorbital advancement for correction 

of plagiocephaly because of the possible asymmetry 

that may result from unilateral frontal sinus 

development. 

In contrast, Bartlett, Whitaker, and Marchac 406 

evaluated a study population of 48 children operated 

for plagiocephaly in infancy using both bilateral 

and unilateral approaches. After following the patients 

for a minimum of 3 years, the authors concluded 

that either unilateral or bilateral frontoorbital 

advancement produced good results, and there was 

no advantage to using one technique over the other. 

The study obviously did not follow all these infants to 

the time of frontal sinus development. 

David and Sheen 407 analyzed their results in the 

surgical treatment of 39 patients with Crouzon syndrome. 

They found that early frontoorbital advancement 

was universally successful in relieving elevated 

intracranial pressure and reducing ocular proptosis 

but did not prevent midfacial hypoplasia or provide 

definitive cosmetic correction of the deformity. 

Although frontofacial advancement in adults gave 

good long-term results, it was attended by more complications 

than other corrective procedures. 

Marchac and Renier 403,404,408 note normal facial 

growth after early craniofacial surgery in many 

nonsyndromic patients. McCarthy, 409 on the other 

hand, reports premature refusion of the sutures or 

development of turricephaly and other calvarial contour 

irregularities in 50 children who had early surgery 

for craniofacial synostosis. The mild cases 

received extensive strip craniectomy and the more 

severe or syndromic patients had strip craniectomy 

with frontal bone advancement. A mean 8 years 

after craniofacial remodeling, 20% had to be reoperated 

because of recurrent deformity. The authors 

36 

conclude that early surgery did not result in satisfactory 

occlusal relationships or midfacial form. 

Dufresne and associates 410 used 3D CT analysis to 

calculate the intracranial/ventricular increases in volume 

that were produced by craniofacial surgical procedures. 

Marsh 411 studied with 3D CT imaging the 

endo- and exocranial bases of patients who had early 

corrective surgery for craniosynostosis. He concluded 

that surgery during infancy induces normalization of 

endocranial symmetry over the first postoperative 

year in patients with nonsyndromic solitary and 

bicoronal synostosis, but not in those with multiple 

synostoses. Other analyses of long-term results 412 

find less improvement in endocranial base symmetry 

or normalization of intracranial growth after early 

surgery. 

The optimal age of the patient at surgery remains 

controversial. Some authors strongly favor early treatment 

of craniosynostosis during infancy (which is variously 

interpreted as the period between the first week 

of life up to 4–6mo 393 ) to allow brain growth to 

remodel the calvarium. 413 

Ocampo and Persing 237 feel that the quality of the 

result is inversely proportional to age of the child at 

operation. Most craniofacial surgeons would agree 

that early surgery is indicated in the event of multiple 

suture synostosis 414 and in sagittal synostosis when an 

extended vertex craniectomy is performed. Certainly 

surgery should be done as early as possible for the 

severe craniosynostosis syndromes such as kleeblattschadel. 

396,404 Depending on the number and location 

of prematurely fused sutures, therefore, early 

cranial vault decompression may be indicated to prevent 

restrictions on the developing brain. 403,411,415 It 

is impossible to know which infants will require early 

release to prevent neural complications. 

By age 18 months, children have attained 

approximately 75% of their brain growth 136,411 (Table 

7). Reossification is more extensive at a younger 

age 416 and can be reliable up until age 2. 

When single sutures are involved, many craniofacial 

surgeons delay surgery until the child is 6–12 

months, at which time the risk of intracranial hypertension 

is thought to be low. 404,411 Wall 417 notes a 

higher reoperation rate when patients are operated 

on at an early age (3mo) than if frontoorbital 

advancement is delayed (6–12mo). Because the 

changes brought about by surgery will be diluted by 

postoperative growth, others 415 wait even longer in 

cases of Apert syndrome and the more severe sym-

TABLE 7 

Brain Growth During the First 20 Years of Life 

(Data from Blinkov SM, Glezer II: The Human Brain in Figures 

and Tables: A Quantitative Handbook. New York, Plenum 

Press, 1968. Reprinted with permission from Marchac D, Renier 

D: Craniofacial Surgery for Craniosynostosis. Boston, Little 

Brown, 1982, p 36.) 

metrical deformities to lessen the need for major 

revisions at a later date. 404 

Certainly unilateral cases can be expected to 

achieve better results than bilateral cases. McCarthy 

demonstrated good to excellent results in 86% of 

patients undergoing unilateral procedures and in 49– 

70% of bilateral procedures. 418,419 

SURGERY FOR COMBINED CRANIOSYNOSTOSIS AND 

HYPOPLASIA OF THE MIDFACE 

There have been few changes in the treatment of 

Crouzon disease since Tessier first described his classic 

facial osteotomy technique. 275,288,367,420,421 Modifications 

have progressed from the Tessier I—a 

subcranial Le Fort III—in 1958, through the more 

radical Tessier VIII of 1976, which split and bent a 

frontofacial monobloc segment, derotated the orbits, 

and brought the maxilla forward. In Tessier’s hands 

this extensive procedure yielded nearly normal results. 

In 1976 and again in 1979, Van der Meulen 422,423 

described a similar operation that involves simultaneous 

total osteotomy of the two halves of the face 

and correction of the orbitofrontal and maxillary 

deformities. The coronal approach to osteotomy of 


the pterygomaxillary fissure obviates intraoral surgery 

with its potential contamination. 

Ortiz Monasterio and associates 402 first advocated 

monobloc advancement of the entire forehead and 

face (Fig 23) in 1977. Anderl and coworkers 424 proposed 

a modification of the frontoorbital bar with 

preservation of an intact anterior cranial base to separate 

the cranial and nasal spaces when indicated for 

relief of respiratory or orbital distress accompanying 

severe craniosynostosis. Although many surgeons 

believe that monobloc advancements are hazardous 

because of the high number of associated infectious 

sequelae, the authors feel their technique is safe even 

at a young age. 

Fig 23. Cranial vault reshaping for correction of Crouzon 

syndrome with bilateral coronal synostosis. The procedure 

involves osteotomies of the anterior cranial vault, monobloc, Le 

Fort I, and chin. (Reprinted with permission from Posnick JC: 

Craniosynostosis: Surgical Management of the Midface Deformity. 

In: Bell WH (ed), Orthognathic and Reconstructive 

Surgery. Philadelphia, WB Saunders, 1992; vol 3, p 1888.) 

Wolfe 425 reviewed his experience with 32 patients 

who underwent transcranial monobloc frontofacial 

advancement +/– simultaneous facial bipartition. 

The author cautions that the procedure “carries with 

it substantial risks, [but] with careful consideration of 

airway control, the anterior cranial base dura, and 

retrofrontal dead space, the procedure is recommended 

for carefully selected patients.” Wolfe lists 

his indications and contraindications for the procedure 

in various age groups. 

Treatment for combined deformities from craniosynostosis 

of the anterior cranial vault and 

37


midfacial retrusion typically involves two operative 

stages, initially advancing the forehead and subsequently 

the face. This results in fewer infections 

than monobloc advancement. 426 Marchac and 

Renier 397 advocate Tessier’s frontal horizontal 

advancement with lateral fixation by tongue-ingroove 

for intracranial forehead correction. The 

resultant exaggeration in facial retrusion is corrected 

at a later date. 397,404 

Fearon and Whitaker 426 reviewed their 15-year, 

29-patient experience with midfacial and 

frontofacial advancement for craniosynostosis. Of 

the 30 procedures performed, 20 were Le Fort IIIs 

and 10 were monobloc advancements. All major 

infections occurred in the monobloc group. The 

aesthetic results were the same regardless of the 

type of facial advancement performed. Given the 

high infection rate associated with monobloc 

advancement and the similar outcomes, the 

authors believe the surgical correction of craniosynostosis 

should be staged. 

SURGERY ON THE ORBITS 

Surgical techniques for the correction of exorbitism, 

ocular dystopia, enophthalmos and hypertelorism are 

based on principles of craniofacial surgery. 

Dystopia 

Ocular dystopia can be congenital or traumatic 

and is corrected by unilateral orbital repositioning. 427 

Edgerton and Jane428 review various eye-leveling techniques, 

ranging from simple bone grafts to complex 

C-type osteotomies429 and Tessier’s marginal 

osteotomy, which removes the orbital rim, levels it, 

and repositions it. 

The surgical treatment of ocular dystopia varies 

according to the age of the patient and the cause 

of the orbital anomaly. In young patients who are 

actively growing, it is preferable to use the 

frontoorbital bandeau technique so as not to damage 

the dental buds. In adult patients, Tessier’s430 orbital quadrant technique is used. At birth the 

orbital volume is about 70% of its adult volume. 

At 18 months, it is about 75–80%, and at 3 years 

of age, it is about 90%. The orbital volume is 

completely developed by age 10 to 11. 

38 

Hypertelorism 

The severity of hypertelorism is rated according to 

the interorbital distance (IOD), 370 as follows: 

1° — 30–35mm IOD 

2° — 35–39mm IOD with normal orientation 

and shape of the orbits 

3° — 40+mm IOD with defects in the 

cribriform plate, orbital region, and 

lateral canthus 

The normal adult IOD is 25mm for women and 

28mm for men. The usual growth progression 431 is 

as follows: 

age 1 year 18.5mm 

3 years 21mm 

7 years 23mm 

12 years 26mm 

The interpupillary distance (IPD) is not a true indication 

of hypertelorism because abnormal rotation or 

divergent strabismus of the eyes commonly accompanies 

the syndromic craniofacial anomalies. Similarly, the 

intercanthal distance or ICD can be skewed by excessive 

soft tissue bulk or traumatic telecanthus. 

Evereklioglu et al 432 propose a clinically applicable 

and practical definition of hypertelorism as an 

increase in the interpupillary distance in the absence 

of strabismus (Fig 24). An isolated measurement of 

hypertelorism is not clinically applicable unless it is 

noted in the context of overall head size. The authors 

propose an interpupillary index which they believe is 

a more accurate projection of hypo- and hypertelorism 

related to head size. The index is calculated 

by the formula 

The authors also provide normative values in healthy 

children. 

Hypertelorism is etiologically heterogeneous. 

Nontraumatic hypertelorism is always secondary to 

another deformity, either craniosynostosis or a median 

or paramedian facial cleft. Munro 427 notes four types 

of ethmoid and medial orbital wall deformity in 

hypertelorism (Fig 25). Types C and D are the rarest 

and most difficult to correct. 

Correction of hypertelorism can be subcranial or 

intracranial. The subcranial approach offers two 

alternatives: medial orbital wall migration or U-shaped

Fig 24. Interocular distance measured from inner canthus to 

inner canthus. A, normal. B, primary telecanthus. C, true ocular 

hypertelorism. D, ocular hypertelorism with secondary 

telecanthus. (Reprinted with permission from Cohen MM Jr, 

Richieri-Costa A, Guion-Almeida ML, Saavedra D: Hypertelorism: 

interorbital growth, measurements, and pathogenetic considerations. 

Int J Oral Maxillofac Surg 24:387, 1995.) 

osteotomy mobilizing the lateral, inferior, and medial 

orbital walls as a single bloc. The maximum correction 

that is possible with medial orbital wall mobilization 

is 10mm; with a U-shaped osteotomy, 15mm. 

The intracranial approach as described by 

Tessier 368,369 with Converse’s modification 433 is preferred 

in all severe cases of hypertelorism or when 

the cribriform plate lies below the level of the 

frontonasal suture (Fig 26). The entire orbit is mobilized 

as a box, providing total control of the intracranial 

contents. 

McCarthy and colleagues 434 report correction of 

orbital hypertelorism on 20 children age


Fig 26. Above, one-stage medial orbital osteotomy with preservation 

of the cribriform plate. Below, subtotal orbital osteotomy 

with preservation of the cribriform plate. (Reprinted with permission 

from Converse JM, Wood-Smith D: An atlas and classification 

of mid-facial and craniofacial osteotomies. In: Transactions 

of the Fifth International Congress of Plastic and 

Reconstructive Surgery. Melbourne, Feb 1971, p 931.) 

Anophthalmia and Microphthalmia 

Anophthalmos and microphthalmos represent failure 

of optic development. Absence or diminutive 

size of the eyes fails to stimulate orbital growth and 

results in orbital hypoplasia. Kennedy437 elicited 

hypoplasia of the orbit by ocular enucleation during 

embryogenesis in cats. 

The classic treatment for orbital hypoplasia secondary 

to anophthalmia or microphthalmos has been 

the insertion of progressively larger silicone orbital 

implants covered by scleral shells. This treatment 

has met with only moderate success due to difficulty 

in maintaining the implants and the need for frequent 

readjustments. 

Tessier438 enlarged the orbit by a lateral 

osteotomy that allowed expansion of the orbital 

volume inferiorly and laterally. Marchac439 later 

added a supraorbital craniectomy and frontal craniotomy 

that relocated the orbital roof and allowed 

expansion of the orbital volume in three directions. 

Elisevich and colleagues440 subsequently 

40 

described a technique for 3D expansion of the 

orbit without craniotomy using a single burr-hole 

craniectomy of the frontosphenoid bone. 

Working from the basis that orbital hypoplasia is 

the consequence of an inadequate stimulating matrix, 

Lo et al 441 demonstrated the effectiveness of subperiosteal 

tissue expanders in the bony orbit to substitute 

for the missing stimulus. They enucleated one eye in 

12 6-week-old kittens and placed subperiosteal tissue 

expanders in half of the orbits; the other 6 served 

as controls. All the orbits in which tissue expanders 

were placed demonstrated equal or greater orbital 

volumetric measurements than normal orbits, compared 

to enucleated orbits that did not receive tissue 

expanders, whose measurements were significantly 

lower than the normal. 

Rodallec and coworkers 442 report the use of silicone 

expanders in 17 patients. The expanders were 

placed within the intramuscular cone to expand not 

only the bony orbit but also the soft tissues. 

SURGERY ON THE MAXILLA 

Le Fort I Osteotomy 

The Le Fort I osteotomy is a horizontal osteotomy 

of the upper jaw at the level of the (Le Fort I) fracture 

line. It was initially described by von Langenbeck in 

1859 and used widely in the second half of the 19th 

century to temporarily mobilize the maxilla for better 

access to tumors and polyps. Not until the 1960s, 

however, did mobilization of the maxilla become 

generally accepted, due in large measure to the precise 

operative technique and superb results reported 

by Obwegeser, 373 who demonstrated that it was possible 

to fully mobilize the maxillary segments and 

bring them into the desired position without softtissue 

resistance for primary stabilization. 443 

Obwegeser also described a higher maxillary 

osteotomy (Le Fort I½) for use in patients with pronounced 

midfacial recession to advance the retruded 

portion of the maxilla lateral to the piriform aperture. 

Other modifications of the Le Fort I osteotomy 

have been described, including a self-stabilizing 

osteotomy suggested by Munro—a “self-retained Le 

Fort I”. 444 

Many authors have confirmed tooth viability subsequent 

to Le Fort I osteotomy, with reinnervation of 

the pulp over a period of several months. 445,446

Le Fort II Osteotomy 

The Le Fort II osteotomy was popularized by 

Henderson and Jackson447 in cleft patients with associated 

paranasal and nasal shortening. Several variations 

have been described, including the tripartite 

osteotomy of Converse. 448 

Le Fort III Osteotomy 

The mainstay of total maxillary advancement is 

the Le Fort III osteotomy. 421 The procedure can be 

combined with other osteotomies, specifically a Le 

Fort I, for definitive treatment of severe midfacial 

retrusion. 288 Interpositional bone grafts and a 

maxillomandibular fixation appliance373–377 are useful 

adjuncts. If decreasing the interorbital distance or 

widening of the palate is necessary, a facial bipartition 

is indicated (Fig 27). 

A long-term study by Bachmayer et al449 of maxillary 

growth after Le Fort III osteotomy in syndromic 

craniosynostosis showed minimal (mean 5%) vertical 

relapse with rigid stabilization of the midface, but 

severe anteroposterior growth retardation . Although 

the average forward movement of the maxilla at the 

time of surgery was 12.4mm, horizontal maxillary 

growth was minimal after surgery, and subsequent 

maxillary advancement was invariably required at 

the completion of growth. 

In a retrospective study of patients who underwent 

Le Fort III advancement for craniofacial dysostosis, 

Kaban and colleagues450 note early skeletal 

relapse in the sagittal plane in approximately 33% of 

patients. The magnitude of relapse was greater in 


patients with Apert syndrome than in those with 

Crouzon disease. The small amount of relapse during 

the first year was followed by progressive downward 

and forward movement of the midface until, 2 

years after surgery, the midface was either anterior 

or inferior to the immediate postoperative position 

in 15 of 19 patients. Three patients required an 

additional Le Fort I osteotomy to correct Class III 

malocclusion. 

A prospective analysis of 12 “growing” patients by 

McCarthy and colleagues 451 disclosed a remarkable 

degree of postoperative skeletal stability. They followed 

this study with a clinical and cephalometric 

longitudinal analysis of 12 children with craniofacial 

synostosis syndromes who underwent Le Fort III 

advancement at age


morbidity and improve results include hypotensive 

anesthesia, shorter operative time, rigid stabilization 

of the mobilized bones at the end of the operation, 

fewer incisions, extensive antibiotic therapy, and 

greater surgeon’s experience. 

Munro and Sabatier 454 analyzed the results of 

craniofacial surgery in 1092 patients representing 

2019 procedures. Complications of surgery 

accounted for 0.18% of deaths. Major complications 

occurred in 14.3% of patients but not all had 

permanent sequelae. Infection was the greatest 

problem, occurring in 5.3%. As the surgeons’ 

experience increased, the complication rate 

decreased. 

David 455 reviewed his 10-year experience with 

craniofacial surgery in Australia. The overall infection 

rate in 170 patients was 6.5%, with a marked 

age differential: 23.5% in adults and 2.2% in children. 

The highest infection rates were seen in 

patients who required tracheostomy and had longer 

operative times, and the most common pathogen 

was Pseudomonas. His recommendations to prevent 

infectious complications include operative 

intervention at an early age, short preoperative hospital 

stay, antibiotic prophylaxis to include gramnegative 

cover, isolating the dead space, and strict 

tracheostomy care. 

Poole 456 analyzed the postoperative course of 200 

consecutive patients treated at the Oxford Craniofacial 

Unit. Their overall complication rate was 22%, 

including 1% mortality and 1% infections. In this 

series as well as in those mentioned above, the frequency 

of infection was greater in adults than in 

children and intracranial procedures carried a higher 

risk than extracranial procedures. 

Whitaker and colleagues 415 retrospectively analyzed 

the records of 164 patients with craniosynostosis 

operated on over a 12-year period. The 

authors state that excellent results can be expected 

in the asymmetrical deformities treated in infancy 

by a unilateral approach. Similarly, early surgery in 

the form of bilateral orbital advancement gives satisfactory 

results in most patients with mild symmetrical 

deformities. In contrast, cases of severe bilateral 

deformity or Apert syndrome often required a second, 

frequently extensive, operation. There were 

no deaths, blindness, or brain dysfunctions as a 

result of surgery. 

42 

DISTRACTION OSTEOGENESIS 

History 

Distraction osteogenesis (DO) is the process by 

which new bone is generated in the gap between 

two bone segments in response to the application of 

graduated tensile stress across the gap. DO was first 

described by Codivilla in 1905. 457 Beginning in the 

early 1950s, Ilizarov458 introduced the concept of 

distraction osteogenesis for acquired and congenital 

deformities of enchondral bone. 459 In 1973 Snyder460 reported distraction of the mandible in dogs, and 20 

years later McCarthy et al57 described lengthening of 

the mandible in 4 patients with hemifacial microsomia 

by application of DO. 

In 1998 Molina and Ortiz-Monasterio published 

their series of mandibular distraction in 274 patients, 58 

and the following year McCarthy reviewed his 10year 

experience with distraction of the mandible. 461 

Habal462 reported midfacial distraction using an 

external distractor in 1995. That same year Polley463 described monobloc craniomaxillofacial distraction. 

Chin and Toth464 developed an internal device for 

midface advancement at the Le Fort III level in 1997. 

Biology of Distraction 

The process of distraction allows for the generation 

of bone—osteogenesis—as well as soft-tissue— 

histogenesis. In a gap between two bone segments 

initially a hematoma forms, which then develops into 

a soft callus. The application of tensile stress prevents 

progression to hard callus and subsequent fracture 

union. Instead, intramembranous bone formation 

occurs within the ever increasing gap. Within 

the bony gap there are three zones. The fibrous 

interzone intervenes between the two primary mineralizing 

fronts adjacent to the osteotomy. At cessation 

of distraction the mineralizing fronts meet and 

unite to create union during the consolidation phase. 

The resultant bony union is dependent on the latency, 

rate, rhythm, and consolidation period of the distraction 

process. 

Latency. Latency is the time period after 

osteotomy/corticotomy and before commencing distraction. 

In an ovine model Tavakoli et al 465 found 

no difference in mechanical strength or bone density 

of the callus when the latency period varied between

0 and 7 days. This would suggest that the traditional 

practice of allowing a nontreatment interval may be 

unnecessary and may only prolong treatment time. 

Cedars 466 uses no latency period during Le Fort III 

distraction. 

Rate. Rate is the number of millimeters the bone 

gap is widened each day. Farhadieh and 

Gianoutsos 467 used a sheep model comparing distraction 

rates of 1mm, 2mm, 3mm, and 4mm per 

day. They then measured biomechanical properties, 

mineralization, and histology of the regenerate. 

They found a distraction rate of 1mm per day 

was superior to all others. A rate of 1mm/d also 

gave the most uniform bone formation radiologically 

and histologically in a porcine model studied 

by Troulis. 468 When distracting at the Le Fort III 

level, Cedars et al 466 uses a torque wrench to determine 

the rate of distraction, believing this reflects 

the tolerance of the soft-tissue envelope and is more 

appropriate than an arbitrary measurement. 

Rhythm. Rhythm is the number of times daily the 

distractor is activated. Rhythm varies from once per 

day to 4 times per day. 

Consolidation. A long consolidation period will 

lead to weakening of the regenerate as a result of 

disuse atrophy, 469 whereas too short a consolidation 

phase will lead to fibrous nonunion, buckling or fracture 

of the regenerate. 470 Rachmiel et al 471 showed 

24% mineralization after 3 weeks of consolidation 

and 77.8% by the end of 6 weeks. 

To date little clinical research has been done into 

the appropriate length of the consolidation period. 

Smith et al 472 used computed tomography to suggest 

6 weeks was the minimum amount of time the 

regenerate should be allowed to mineralize prior to 

device removal. Felemovicius 473 used bone scintigraphy 

with technetium-99 diphosphonate to assess the 

duration of the consolidation period. They found 

consolidation underway at 5 weeks in infants under 

12 months old, while in older children it was ongoing 

at 10 weeks and in adolescents and adults, at 10- 

14 weeks. 

Distraction Treatment Planning 

Compared with the relatively simple distraction of 

long bones, distraction of the mandible in the hypo- 


plastic deformity is decidedly a challenge. A treatment 

plan is essential to design the 3D vectors necessary 

to correct mandibular deficiency. Molina 474 states 

that the most critical factor in mandibular DO is 

proper planning of the distraction vector on the 

cephalogram and its replication in the operating room. 

The hypoplastic ramus must be elongated, bringing 

the affected side of the jaw downward, forward, and 

toward the midline. 

Tharanon and Sinn 475 describe 3D treatment 

planning of mandibular hypoplasia using a panoramic, 

postero-anterior (PA), and lateral 

cephalogram. From the panoramic cephalogram, 

the site for the osteotomy is chosen to avoid damage 

to the inferior alvelolar nerve and the tooth 

buds. The necessary vertical lengthening is determined 

from the PA cephalogram, to bring the 

angles of the mandible onto an equal plane. From 

the lateral cephalogram, the required horizontal 

lengthening is determined based on correction of 

the central incisors and chin to the desired position 

(Fig 28). The authors advocate this method 

on the basis that operative time is saved, the 

patient’s family can be informed as to the duration 

of the distraction phase, and the technique is 

simple and inexpensive. Orientation of the vector 

of distraction parallel to the sagittal plane avoids 

lateral displacement and bending forces on the 

bone. 472 

Gateno et al 476 described a technique of 3D modeling 

and animation they employed in 7 patients to 

simulate distraction osteogenesis in virtual reality. The 

technique requires familiarity with advanced animation 

software, is time consuming, and a surgical template 

must be constructed and used intraoperatively 

to ensure correct pin placement. 477 

Distraction Devices 

The initial devices developed for distraction 

osteogenesis were external and unidirectional. More 

recent devices operate in multiple planes and multiple 

vectors, from Pensler’s 478 ball and socket joint 

to McCarthy’s 479 multiplanar distractor that works 

in the sagittal, vertical, and coronal planes simultaneously 

(Fig 29). McCarthy 479 emphasizes the following 

technical points in the application of 

multiplanar distraction: 

43


Fig 28. Mandibular distraction with an extraoral device in hemifacial microsomia. A, location of the osteotomy. B, C, pin positions. D, 

amount of vertical mandibular distraction on the affected side. E, overbite relationship between the maxillary and mandibular central 

incisors. F, center of rotation. The distal segment is moved horizontally until the overjet and chin are in the best position. The horizontal 

movement determines the actual distance of AP distraction. (Reprinted with permission from Tharanon W, Sinn DP: Mandibular 

distraction osteogenesis with multidirectional extraoral distraction device in hemifacial microsomia patients: three-dimensional 

treatment planning, prediction tracings, and case outcomes. J Craniofac Surg 10:202, 1999.) 

• linear distraction continues at all times to avoid 

premature consolidation 

• linear distraction is begun first to generate 

adequate bony regenerate 

• both limbs of the device are available for linear 

distraction and can be varied appropriately 

• linear distraction is by far the most important and 

often the only vector required 

The development of intraoral devices was spurred 

by a desire to minimize external scarring. The intraoral 

44 

devices can be tooth/bone borne 480 and located 

intraorally (Guerrero 481 reports more than 200 distractions 

using this type of device), or bone borne 

and located submucosally or buried. 482 

Rachmiel et al 483 compared the results of internal 

and external devices in mandibular DO. In a 

poulation of 22 patients with hemifacial microsomia, 

12 had DO by an external device and 10 by 

an intraoral device. The external device allowed 

greater elongation and better extraoral vector control, 

and conserved the gonial angle. The main 

disadvantages of the external device were the 

Fig 29. Multiplanar mandibular distraction device to achieve simultaneous yet independent linear distraction (sagittal plane), angular 

distraction (vertical plane), and transverse distraction (coronal plane). (Reprinted with permission from McCarthy JG, Williams JK, 

Grayson BH, Crombie JS: Controlled multiplanar distraction of the mandible: device development and clinical application. J Craniofac 

Surg 9:322, 1998.)

inconvenience to the patient and the cutaneous scars. 

The intraoral device was more socially acceptable 

and left no visible scars, but there was loss of vector 

control and a second procedure was required to 

remove the device. 

Polley 484 reported on an external halo-mounted 

device for midfacial monobloc DO in a newborn. 

Chin and Toth 464 and Cohen 486 developed internal 

devices for midface advancement (Fig 30), citing 

the advantage of patient convenience; 

Cohen’s 486,487 device was resorbable to eliminate 

the need for a removal procedure. Motor-driven 

distraction devices have also been reported. 488 

External devices are most commonly used in the 

midface. 489 

Fig 30. Patient with repaired cleft lip and severe midfacial 

hypoplasia. A, B, before distraction. Middle, patient fitted with 

rigid external distraction apparatus for correction of midfacial 

deficiency. C, D, after maxillary distraction. (Reprinted with 

permission from Polley JW, Figueroa AA, Kidd M: Priciples of 

Distraction Osteogenesis in Craniofacial Surgery. In: Lin KY, 

Ogle RC, Jane JA (eds), Craniofacial Surgery. Science and 

Surgical Technique. Philadelphia, WB Saunders, 2002; Ch 11, 

pp 163-171.) 

Craniofacial Distraction 


Mandible 

McCarthy’s protocol for mandibular distraction is 

based on patient age and type of mandibular deformity, 

as follows: 461 

Under 2 years – DO of the mandible is not indicated 

Age 2-6 years – 

Pruzansky Type I – orthognathic surgery including 

DO are deferred until adolescence490,491 Pruzansky Type I (severe) and Type II – 

DO is indicated 

Pruzansky Type III – Stage I: costochondral rib 

graft at age 3-4 yrs; if absent, the glenoid is 

reconstructed with a rib graft and fixed to 

the zygoma. Stage II: DO of the rib graft at 

6 months postinsertion of costochondral rib 

graft 

Age 6-teenager – primary DO is considered if no 

previous treatment. Secondary DO is 

considered in patients with significant deformity 

after rib grafting. 

Teenager – surgery is postponed until skeletal maturity 

is reached. 

Post skeletal maturation – minimal deformities are 

best treated with classic orthognathic surgery. 492 

Mandibular distraction should be considered in 

patients with moderate to severe deformity and 

bilateral disease. 493 

McCormick and colleagues 494,495 investigated the 

effect of osteodistraction on the temporomandibular 

joint (TMJ), first in a canine model and then in human 

subjects. Compression on the cartilaginous portion 

of the condylar head has been shown to be detrimental 

to subsequent TMJ morphology and function, 

496–498 yet in this series initial condylar flattening 

was transient and completely reversible. In fact, bone 

distraction appears to improve the temporomandibular 

joint. 499 When properly applied, the distraction 

forces pull the mandible in a downward and forward 

direction, leading to new bone deposition along 

the posterior aspect of the mandible and resorption 

along the anterior ramus region. 500 The overall conclusion 

of these studies was that distraction was beneficial 

to the TMJ complex. 

Speech before and after mandibular DO was 

assessed by Guyette et al, 501 who report worse 

articulation and nasal resonance post-distraction. 

These changes were temporary, however, and in 

45


time all patients returned to their pre-distraction 

speech levels. 

The actual vector of distraction achieved may vary 

from that planned. This may be due to technical 

error or to the deforming forces of the soft-tissue 

envelope. Maloclusion, an anterior open bite, or an 

unaesthetic result can be adjusted by manipulation 

of the callus prior to mineralization. Hoffmeister et 

al 502 introduced the “floating bone” concept of elastics 

to reshape the regenerate and improve occlusion. 

Kunz 503 uses elastics during and after distraction 

to correct minor deviations of the distraction 

vector, a technique he calls “a lifeboat.” Pensler 478 

employs direct manual reshaping of the callus to 

achieve the same end. Early removal of the device 

(at 3 weeks) and orthodontic elastics can close an 

open lateral bite when an undesirable vector is 

obtained. 483 

In the neonatal population, mandibular distraction 

has been used to help manage the airway. 

Denny 116 reports a series of 10 patients with mandibular 

hypoplasia who were successfully treated with 

DO. After distraction was completed, 2 of 3 patients 

who had tracheostomies were successfully decannulated. 

Denny 504 later demonstrated that mandibular 

advancement via distraction osteogenesis could 

also be used to avert tracheostomy in neonates with 

craniofacial anomalies that impinge on the airway. 

Le Fort I 

Before the pubertal growth spurt, DO of the mandible 

will allow descent of the maxilla orthodontically 

to a more normal position. After the pubertal growth 

spurt, combined DO of the maxilla and mandible 

will be necessary because of the cessation of maxillary 

growth. Molina and Ortiz Monasterio58,505 describe a technique involving Le Fort I corticotomy 

at the time of mandibular osteotomy and distractor 

placement. After a 5-day latency, intermaxillary wiring 

of the jaws is performed and distraction begun. 

Consolidation extends for 8 weeks. 

Molina and Ortiz Monasterio506 also report their 

experience with distraction of the maxilla in isolation 

in 36 patients, 18 with unilateral cleft lip and palate, 

12 with bilateral cleft lip and palate, 2 with 

nasomaxillary dysplasias, and 4 with mandibular 

prognathism. All patients were between the ages of 

3 and 11 years at the time of operation. The distraction 

technique involved a vestibular incision, subpe- 

46 

riosteal dissection of the maxillae, and incomplete 

osteotomy above the canines and molar roots. The 

osteotomy must respect the integrity of the maxillary 

antral mucoperiosteum and should extend into the 

maxillary buttress. Pterygomaxillary dysjunction and 

dissection along the nasal floor and base of the septum 

are not done. On the fifth postoperative day, 

the authors hook a maxillary orthopedic appliance 

to a facial mask with rubber bands and 950g of 

mechanical force is applied to each side. Each week 

the maxilla is advanced about 3-4mm. Once half of 

the total projected maxillary advancement (8-12mm) 

and a Class II molar relationship have been obtained, 

the force is decreased to one rubber band on each 

side to promote new bone formation at the osteotomy 

site and pterygomaxillary junction. The overall 

improvement was reported as excellent. 

Cohen 485 applied the MIDS internal device for Le 

Fort I advancement in 2 patients and reports 15mm 

and 17mm of advancement (Fig 31). 

Ko et al 507 examined the soft-tissue response to 

DO of the maxilla in 16 patients. They found that 

the concave facial profile became convex, the 

advancement of soft-tissue to hard-tissue ratio at A 

point was 0.96:1, and the soft-tissue to hard-tissue 

ratio for the nasal tip was 0.53:1. In a separate 

study, Ko et al 508 reported on the effects of maxillary 

DO on the speech of 22 patients. The average 

maxillary advancement was 8.9mm and resulted in 

14% worsening of hypernasality. The hypernasality 

was apparently related to the degree of advancement 

and unaffected by the presence of a pharyngeal 

flap. Despite worsening of nasal air escape, 

57% of patients had improved articulation. 

Le Fort III/Monobloc 

Midface distraction was first performed in 1993. 509 

In 1995 Polley484 used an external distraction device 

to perform a monobloc distraction in a newborn, 

and in 1997 the same author described applications 

of the external device for midface distraction in childhood 

and adolescence. In 1996 Chin and Toth464 reported on Le Fort III distraction with an internal 

buried device. Distraction advancement of up to 

25mm has been described. 510 

In younger patients who require frontoorbital 

advancement as well as advancement of the midface, 

monobloc DO is appropriate. In older patients who 

have undergone previous frontorbital advancement


Fig 31. Modular internal distraction system applied in left, monobloc osteotomy and right, Le Fort III osteotomy. (Reprinted with 

permission from Cohen SR: Craniofacial distraction with a modular internal distraction system: evolution of design and surgical 

techniques. Plast Reconstr Surg 103:1592, 1999.) 

DO at the Le Fort III level, this may be all that is 

required. Both internal and external devices exist for 

midfacial DO. In younger patients the hypoplastic 

malar complex may not withstand the forces of distraction 

511 and an external appliance attached to the 

maxillary dental arch may be more appropriate. 

Cedars et al 466 reported a series of 14 patients 

who underwent Le Fort III DO with the internal 

device developed by Chin and Toth. 464 Following 

osteotomy and application of the device, the distraction 

pins exit percutaneously through the cheek, and 

a torque wrench is used to begin distraction intraoperatively. 

Distraction is continued immediately postoperatively 

using the torque wrench twice a day to a 

load of 14–18N-cm. DO is continued until the 

porion–orbitale distance is achieved as planned 

cephalometrically. The average distraction was 18mm 

(range 12.5–27mm; 11mm achieved intraoperatively). 

Cephalometric follow-up for more than 1 

year in 6 patients showed excellent stability of the 

advancement at the occlusal level, with some relapse 

at the level of the orbitale. All patients had improvement 

in symptoms of sleep apnea. One of 2 patients 

with tracheostomy was decannulated. The authors 

now concentrate the distraction process on achieving 

superior maxillary position, reserving height 

increase for Le Fort I osteotomy to be performed 

subsequently. 

Fearon 512 compares the results of Le Fort III 

advancement in a pediatric series of 22 patients. Ten 

patients underwent standard Le Fort III advancement 

and 12 underwent advancement by DO (2 

internal buried device, 10 external halo device). In 

the standard Le Fort III group, the average advancement 

was 6mm; in the distracted group, it was 19mm. 

Complications were evenly distributed between the 

two groups. The conclusion was that the advancement 

achieved with distraction is superior to that of 

standard Le Fort III. A halo distractor was preferred 

over the internal device because of better vector 

control. Focusing the vector on the facial midline 

allowed better correction of the concave facial profile 

to a convex profile. 

Cohen’s 485 series of 11 patients treated with the 

Modular Internal Distraction System (MIDS) 

(Howmedica-Leibinger) included 4 Le Fort III cases 

47


and 3 monobloc advancements. Midface retrusion 

and exorbitism were corrected in all patients and 

there were no infections in the monobloc group. 

Nadal 513 reported similar results in 8 patients who 

had monobloc advancement by distraction. Cohen 514 

also published a case report of a patient with facial 

bipartition treated with the MIDS. 

To avoid extensive surgery for removal of the 

device, Cohen and Holmes 487 used resorbable 

Macropore mesh as a substitute for the metallic fixation 

plate and achieved 20mm of distraction at the 

Le Fort III level in a 4-year-old girl with Crouzon 

syndrome. 

Distraction has also been described in Le Fort II 

advancement, 515 nasal bone lengthening, 516 

zygoma, 487,517 alveolar clefts, 518 and craniosynostosis. 

463,519,520 

Reviews and Long-Term Follow-Up 

Swennen and colleagues, 489 in an attempt to quantify 

clinical indications and treatment protocols, 

reviewed the literature on DO from 1966 to 

December 1999. They found 285 articles, including 

109 clinical studies involving treatment of 828 

patients. This impressive review provides an insight 

into the most widely employed indications and protocols. 

Mandibular distraction: Of 579 patients, 74.3% 

underwent mandibular lengthening. A latency period 

of 5–7 days was most common, as were a distraction 

rate of 1mm/day, with rhythms between 1 and 4 

times per day and a 6–8 week consolidation period. 

Unidirectional devices were used in 80.2% of cases, 

bidirectional in 15.6%, and multidirectional in 4.2%. 

External devices were used in 72% of cases and 

internal in 18%. Lengthening varied from 1–95mm. 

Maxillary distraction: Of 129 patients, 122 

underwent maxillary advancement. An external 

device was used in 95% of cases and an internal 

device in 5%. The average latency was 4–5 days, the 

rate was 1mm/day, and consolidation of 2–3 months 

was most common. Distraction lengths were 1– 

17mm. 

Simultaneous mandibular and maxillary distraction: 

This group included 24 patients who underwent 

mandibular DO with simultaneous maxillary 

48 

DO though IMF, most often though unilateral mandibular 

DO and an associated Le Fort I osteotomy. 

Mandibular distraction was accomplished with an 

external device in 87.5%, an internal device in the 

remaining 12.5%. Mandibular lengthening varied 

from 12–18mm. 

Midface and cranium: Of 96 cases, 64.6% had 

Le Fort III advancement and 35.4% had monobloc 

advancement. In patients younger than 4 years, 

monobloc was the most common procedure. 

The researchers’ conclusions were as follows: 

1. The issue of overcorrection remains controversial. 

2. A rate of 1mm/day is average. 

3. The standard latency period of 5–7 days could 

possibly be shortened, given the difference 

between the long bones and the membranous 

bones of the cranium and face. 

4. The complication rate was 22%, though most of 

these were mechanical problems associated with 

the device or minor pin site infections. 

5. There is a lack of long term data, especially 

regarding relapses. 

6. More objective data are necessary to refine protocols. 

7. The future of DO probably involves the development 

of miniaturized, multidirectional, and 

motorized devices. 

Mofid and colleagues 521 reviewed 3278 cases of 

craniofacial DO by means of a 4-page survey sent to 

2476 craniofacial and oral/maxillofacial surgeons 

worldwide. The response rate was 11.4%. Relapse 

was recognized by 50.4% of respondents; of these, 

64.8% had mandibular relapse and 60.6% had 

midface relapse. Relapse occurred within 6 months 

of completion of distraction in more than 66%. In 

cases of mandibular distraction, a higher use of internal 

devices (24.3%) and a higher rate (33.9%) of 

multivector devices was seen than reported by 

Swennen. 489 This most likely reflects advances in 

device design and surgeon experience. As noted by 

Swennen, the average latency was 4.9 days, the commonest 

rate was 1mm/day, and the average consolidation 

period was 7 weeks: 6 days in the mandible 

and 8 weeks and 6 days in the midface. Average

lengthening was 16.8mm in the mandible and 

14.5mm in the midface. 

Other complications of craniofacial distraction 

osteogenesis are tabulated in Table 8. 521 

AUGMENTATION AND CONTOURING PROCEDURES 

There are many indications for autogenous bone 

grafts in craniofacial surgery. 522 Most craniofacial 

surgeons believe that autogenous grafts of bone or 

cartilage are the materials of choice in craniofacial 

reconstruction because of their resistance to infection 

and low morbidity compared with alloplastic 

implants. 523 For an in-depth review of the subject, 

the reader is referred to Selected Readings in Plastic 

Surgery volume 10, number 2. 524 

The calvarium is usually the preferred source of 

donor autogenous bone for grafting in the craniofacial 

skeleton. 79,80,525,526 Cutting 525 delineated the vascular 

supply to the cranium and introduced the concept 

of using vascularized calvarial bone in craniofa- 

TABLE 8 

Complication Rates 


cial procedures. He determined that the most 

important source of perfusion to the cranium is the 

middle meningeal artery, which of course is not useful 

as a pedicle for bone transfer. Branches of the 

anterior and posterior deep temporal arteries, which 

also supply the temporalis muscle, constitute a lesser 

vascular supply to the cranium. Finally there is the 

vascular plexus fed by the supraorbital, supratrochlear, 

superficial temporal, and occipital arteries. The 

temporoparietalis fascia (superficial temporal fascia) 

contains the superficial temporal artery and provides 

a clinically useful pedicle to support a vascularized 

calvarial bone graft. 525,527 

McCarthy and colleagues 79,80 suggest leaving the 

galea and overlying vascular network broadly 

attached to the bone when transferring a vascularized 

calvarium bone flap because of the poor vascular 

connections within the periosteum. 

A review of vascularized calvarial inlay grafts based 

on a temporalis myoosseous design 528 notes preservation 

of the growth potential and a patent suture. 

(Reprinted with permission from Mofid MM, Manson PN, Robertson BC, et al: Craniofacial distraction osteogenesis: a review of 3278 

cases. Plast Reconstr Surg 108:1103, 2001.) 

49


Transsutural growth in the vascularized grafts continues 

for the duration of maxillary development despite cessation 

of skeletal growth at the calvarial donor site. 

Byrd and Hobar 529 described the use of porous 

hydroxyapatite (HA) granules in craniofacial augmentation 

procedures when structural support is 

not the main consideration. The HA granules contain 

pores approximately 200µ in diameter which 

allow fibrovascular ingrowth, so the implants are 

vascularized within a very few weeks. The HA 

granules are mixed with blood and microfibrillar 

collagen to make them easier to work with, and 

are then injected into a subperiosteal pocket wherever 

augmentation is desired. Onlays of hydroxyapatite 

do not resorb over time, are very seldom 

associated with infection, and seem to provoke no 

tissue reaction. The authors’ experience now 

extends to more than 200 patients operated on 

over an 8-year period, and they remain enthusiastic 

about the technique. 

A powdered form of hydroxyapatite marketed 

under the name of Bone Source (Leibinger Corp, 

Carrollton, Texas) has found application in craniofacial 

contour refinement. Burnstein and coworkers 530 

describe their experience with 61 patients, 56 of 

them children, treated with Bone Source for secondary 

craniofacial reconstruction over a 3- year period. 

The authors note excellent contour and volume 

retention and no interference with craniofacial 

growth. 

Jackson 531 remarks that the smaller pore size of 

the powdered HA compared with HA granules lends 

itself to a higher rate of bony replacement. They 

achieved excellent results in 20 patients, yet caution 

that long-term follow-up is necessary to establish the 

safety and reliability of its use. 

SURGERY FOR RADIATION-INDUCED DEFORMITY 

Jackson and colleagues 532 studied 14 children who 

received therapeutic radiation for malignant tumors 

in the orbital area and who subsequently showed 

widespread craniofacial deformities. The original 

tumors were retinoblastoma, rhabdomyosarcoma, 

and embryonic cell carcinoma. Years later, maldevelopment 

of the skull, orbit, maxilla, and mandible 

became apparent. The authors recommend correction 

of four levels of skeletal deformity in a single 

procedure, as follows: 

50 

1. frontotemporal expansion with repositioning of 

the cranial base 

2. orbital expansion and repositioning 

3. maxillary surgery with bone grafts to the zygoma 

as required 

4. mandibular lengthening and repositioning 

Bone grafts should be inlay rather than onlay, and 

soft tissue should be supplied by free-tissue transfer. 

At a second operation, the eye socket and eyelids 

are reconstructed to allow more satisfactory rehabilitation 

with an ocular prosthesis. On the basis of 

observations made during extensive skull base and 

orbital dissections, Jackson and colleagues 532 believe 

the radiation impaired growth of the sphenoid, which 

in turn locked the upper face and prevented normal 

development of the facial skeleton. In addition, the 

frontal, ethmoid, and maxillary sinuses failed to 

expand, resulting in further decrease of craniofacial 

dimensions. Craniotomy is essential to position the 

skull base correctly in order to accurately seat and 

remodel the orbit and maxilla. For further reading 

on this subject, one should refer to the articles by 

Nwoku, 533 Larson, 534 Marx, 535 Guyuron, 536,537 and 

Kawamoto. 538 

Cohen, Bartlett, and Whitaker 335 described their 

experience with reconstruction of late craniofacial 

deformities after therapeutic radiation of the head 

and face during childhood. High-dose radiation is a 

standard form of treatment for some pediatric craniofacial 

tumors, including retinoblastoma, rhabdomyosarcoma, 

Ewing’s sarcoma, and neurofibrosarcoma. 

The patients subsequently develop soft-tissue and 

bony hypoplasia of the irradiated areas, which can 

be partially corrected by onlay bone grafting and 

soft-tissue reconstruction with local pedicled flaps and 

dermal-fat grafts in multiple stages. 335 Forty percent 

of patients in their series required secondary procedures 

for improvement. The authors anticipate 

increasing usage of free-tissue transfer in these cases. 

Individuals who as children were irradiated for 

hemangiomas of the face are also presenting to plastic 

surgery clinics with severe craniofacial deformities. 

At one time high-beam radiotherapy was advocated 

for capillary hemangioma of the cheek, and 

the sequelae of this treatment in the growing child is 

now painfully evident as terrible facial deformities in 

the adult. Reconstruction should follow the principles 

outlined by Jackson and colleagues 532 above.

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