Markedly Elevated Intracranial Pressure Treated With Cranial Vault Expansion, Instead of CSF Shunting, in a Child With Hurler-Scheie Syndrome and Multiple Suture Craniosynostosis.

Despite a known association of mucopolysaccharidoses (MPS) and craniosynostosis, treatment of elevated intracranial pressure (ICP) in these patients is primarily cerebrospinal fluid (CSF) shunting. We present a unique case of Hurler-Scheie syndrome with multisuture craniosynostosis and elevated ICP, without ventriculomegaly, where elevated ICP was successfully treated with extensive cranial vault expansion and shunt placement was avoided. Patients with MPS should be evaluated for craniosynostosis, and calvarial vault expansion may be considered as a viable treatment alternative to CSF shunting for elevated ICP in select patients.

A Simple Mandibular Distraction Protocol to Avoid Tracheostomy in Patients With Pierre Robin Sequence.

Objective: Recent treatment goals for Pierre Robin sequence (PRS) focus on avoiding tracheostomy through modalities such as mandibular distraction osteogenesis (MDO). We primarily evaluated the efficacy of our straightforward MDO treatment protocol for resolution of PRS-associated airway obstruction while secondarily analyzing patient characteristics associated with success or failure of MDO.

Design: A retrospective chart review before and after treatment.