Craniofacial Anomalies, Part 2 - Plastic Surgery Internal

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.
SRPS Volume 10, Number 17, Part 2
• 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
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