Craniofacial Anomalies, Part 2 - Plastic Surgery Internal

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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 SRPS Volume 10, Number 17, <strong>Part</strong> 2 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
SRPS Volume 10, Number 17, <strong>Part</strong> 2 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
Page 2 and 3: CRANIOFACIAL EMBRYOLOGY To understa
Page 4 and 5: CENTRIC Corresponding Cranial Facia
Page 6 and 7: visceral arches, the intervening fi
Page 8 and 9: The Treacher Collins syndrome66 rep
Page 12 and 13: Fig 8. Left, child with asymmetrica
Page 14 and 15: Fig 10. Cranial sutures in the huma
Page 16 and 17: Approximately 2% of cases of isolat
Page 18 and 19: tosis usually have normal mental de
Page 20 and 21: Fig 14. Technique for correction of
Page 22 and 23: SRPS Volume 10, Number 17, Part 2 F
Page 24 and 25: mutations implicated in the cranios
Page 26 and 27: Pfeiffer syndrome is inherited as a
Page 28 and 29: Associated Anomalies Although cervi
Page 30 and 31: • The transfacial approach330 may
Page 32 and 33: Relative indications for decompress
Page 34 and 35: traces the patients’ course over
Page 36 and 37: tomical correction of the deformiti
Page 38 and 39: TABLE 7 Brain Growth During the Fir
Page 40 and 41: Fig 24. Interocular distance measur
Page 42 and 43: Le Fort II Osteotomy The Le Fort II
Page 44 and 45: 0 and 7 days. This would suggest th
Page 46 and 47: inconvenience to the patient and th
Page 48 and 49: SRPS Volume 10, Number 17, Part 2 F
Page 50 and 51: lengthening was 16.8mm in the mandi
Page 52 and 53: 1. Sperber GH: Craniofacial Embryol
Page 54 and 55: 85. Dufresne C: Treacher Collins Sy
Page 56 and 57: 169. Opperman LA, Chhabra A, Cho RW
Page 58 and 59: 247. Sutton LN, Barlett SP, Duhaine
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334. Colen SR: Selective microvascu
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420. Tessier P: Dysostoses cranio-f
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503. Kunz C, Hammer B, Prein J: Man
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Craniofacial Anomalies, Part 2 - Plastic Surgery Internal

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