THE DETECTION AND MANAGEMENT OF INTRACRANIAL HYPERTENSION AFTER INITIAL SUTURE RELEASE AND DECOMPRESSION FOR CRANIOFACIAL DYSOSTOSIS SYNDROMES

WE PERFORMED A retrospective study of 107 consecutive patients with sy ndromic forms of craniosynostosis (craniofacial dysostosis) seen by th e craniofacial team at the Hospital for Sick Children between 1986 and 1992. There were 51 patients with Crouzon's syndrome, 33 with Apert's syndrome, 8 with Pfeiffer syndrome, 11 with Saethre-Chotzen syndrome, and 4 with kleeblattschadel anomaly. Six patients developed raised in tracranial pressure (ICP) after initial suture release and decompressi on (Apert's syndrome, three patients; Pfeiffer syndrome, one patient; Saethre-Chotzen syndrome, two patients). Raised ICP was considered in those children who returned with a bulging fontanelle, progressive fro ntal bone protrusion, intermittent headaches, irritability, and vomiti ng. The diagnosis of raised ICP was based on papilledema (four patient s), progressive macrocephaly (one patient), and ICP monitoring (one pa tient). No child in this group had hydrocephalus requiring cerebrospin al fluid diversion. Once raised ICP was detected in these children, a second operation was immediately performed to reduce the ICP with the intention of expanding the volume of the cranial cavity. The second pr ocedures included: anterior cranial vault and upper orbital reshaping (four patients), posterior cranial vault reshaping (one patient), and total cranial vault reshaping (one patient). There were no perioperati ve complications in these patients, although one patient showed subseq uent recurrence of raised ICP requiring further cranial vault re-expan sion. At follow-up, ranging from 3 to 7 years, all six patients were a symptomatic without evidence of raised ICP. In our series, raised ICP occurred in 6% of the children with a craniofacial dysostosis syndrome after initial suture release and decompression, Accordingly, all chil dren undergoing an early suture release and reshaping procedure for a craniofacial dysostosis syndrome require serial neurosurgical, craniof acial, and neuro-opthalmological examinations postoperatively. Despite an initial suture release and reshaping procedure in craniofacial dys ostosis syndrome patients, the cranial vault may lack adequate growth potential to accommodate the growing brain. Appropriate timing of the revision craniotomy and reshaping procedure can prevent irreversible c omplications.