Early Open Extensive Suturectomy for the Severe Multisuture Synostosis

Abstract

Neonates born with severe multisuture synostosis can present as an emergency. The severe craniocerebral disproportion with or without underlying hydrocephalus and retruded midface can result in raised intracranial pressure and airway compromise within the first few days or weeks of life. This presents a challenging multidisciplinary condition. There is no international consensus on management. There are limited publications available describing the approach to treatment. In our unit, children who present in the neonatal period with severe multisuture synostosis are offered early open extensive suturectomy within the first few months of life. The goals are; reduction in raised intracranial pressure, improvement in head shape and bone formation, and avoidance of a ventriculoperitoneal shunt. This is performed as an adjunct, not a replacement of other traditional skull vault procedures. We describe the technique and postoperative care without the need for a helmet that leads to excellent skull-shape outcomes and avoidance of a ventriculoperitoneal shunt.