Craniofacial Anomalies, Part 2 - Plastic Surgery Internal

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
SRPS Volume 10, Number 17, Part 2
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
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