Remodelling the cranial vault in an attempt to increase the intracrani
al volume and thus control intracranial hypertension, whilst at the sa
me time improving the patient's appearance, has been the mainstay of s
urgery for syndromic craniosynostosis, We report a case of craniosynos
tosis in whom cranial vault expansion was followed by the development
of hind-brain herniation and hydrocephalus, This prompted a review of
our other cases of craniosynostosis who had been evaluated by magnetic
resonance imaging following surgery in order to assess the frequency
of hind-brain herniation and hydrocephalus in these children, Magnetic
resonance imaging had been performed in the postoperative evaluation
of 34 cases of craniosynostosis who had undergone procedures intended
to increase the intracranial volume, The position of the cerebellar to
nsils and the presence or otherwise of hydrocephalus was recorded for
all cases, The effectiveness of surgery in treating raised intracrania
l pressure (ICP) was evaluated by means of postoperative ICP monitorin
g and had been performed in 22 cases, Herniation of the hind-brain bel
ow the level of the foramen magnum was observed in 18 cases (53%), Hyd
rocephalus, requiring the insertion of a ventriculoperitoneal shunt, w
as present in 14 cases (41%) and had developed after the cranial vault
procedure in 9, The mean sleeping ICP measured postoperatively was no
rmal (<10 mm Hg) in 5, borderline (10-15) in 7, and raised (>15 mm Hg)
in 10 cases. Cranial vault expansion in complex craniosynostosis may
fail to address the underlying aetiology of intracranial hypertension,
Furthermore, both hydrocephalus and hind-brain herniation may develop
following such surgery. Neither the increase in intracranial volume a
fforded by cranial vault expansion nor the shunting of hydrocephalus p
recludes the persistence of abnormal ICP. These findings are discussed
in the light of possible mechanisms, in addition to cephalocranial di
sproportion responsible for intracranial hypertension in complex crani
osynostosis. The implications for the surgical management of complex c
raniosynostosis are reviewed.