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2016 ; 59
(3
): 204-13
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Physiological Changes and Clinical Implications of Syndromic Craniosynostosis
#MMPMID27226850
Sakamoto H
; Matsusaka Y
; Kunihiro N
; Imai K
J Korean Neurosurg Soc
2016[May]; 59
(3
): 204-13
PMID27226850
show ga
Syndromic craniosynostosis has severe cranial stenosis and deformity, combined
with hypoplastic maxillary bone and other developmental skeletal lesions. Among
these various lesions, upper air way obstruction by hypoplastic maxillary bone
could be the first life-threatening condition after birth. Aggressive cranial
vault expansion for severely deformed cranial vaults due to multiple synostoses
is necessary even in infancy, to normalize the intracranial pressure.
Fronto-orbital advancement (FOA) is recommended for patients with hypoplastic
anterior part of cranium induced by bicoronal and/or metopic synostoses, and
posterior cranial vault expansion is recommended for those with flattening of the
posterior part of the cranium by lambdoid synostosis. Although sufficient
spontaneous reshaping of the cranium can be expected by expansive cranioplasty,
keeping the cranial bone flap expanded sufficiently is often difficult when the
initial expansion is performed during infancy. So far distraction osteogenesis
(DO) is the only method to make it possible and to provide low rates of
re-expansion of the cranial vault. DO is quite beneficial for both FOA and
posterior cranial vault expansion, compared with the conventional methods.
Associated hydrocephalus and chronic tonsillar herniation due to lambdoid
synostosis can be surgically treatable. Abnormal venous drainages from the
intracranial space and air way obstruction should be always considered at any
surgical procedures. Neurosurgeons have to know well about the managements not
only of the deformed cranial vault and the associated brain lesions but also of
other multiple skeletal lesions associated with syndromic craniosynostosis, to
improve treatment outcome.