Evaluation and Management of Patients with Hydrocephalus in Craniosynostosis

Hydrocephalus in presence of craniosynostosis, though relatively rare, occurs in 4%-10% cases, with an increased incidence in syndromic craniosynostosis. The optimum management in these patients is unknown.

A search was performed on the departmental craniofacial database to identify all patients with craniosynostosis and hydrocephalus from January 2000 to December 2020. Diagnosis was confirmed by a meticulous review of the notes and previous imaging. These patients were grouped into two groups based on the primary treatment they received: either a cerebrospinal fluid (CSF) diversion procedure or a calvarial remodeling procedure. By analyzing the outcomes for each group, we endeavor to rationalize and outline our management strategy for this complex cohort of patients.

Sixty-four of 989 patients were confirmed to have hydrocephalus. Of these, 55 patients underwent calvarial expansion while nine had CSF diversion as a primary procedure. Our study demonstrates that the complication rate is lower in the primary calvarial expansion group. Furthermore, the need for a CSF diversion procedure was avoided in a significant number of these patients as a direct result.

In the vast majority of patients with craniosynostosis and hydrocephalus, calvarial expansion surgery should be the preferred primary management option.