Treatment strategies and prognostic outcomes in craniosynostosis with concurrent hydrocephalus: a case series

Background: Craniosynostosis complicated with hydrocephalus is a rare pediatric condition with complex and debated treatment protocols. The optimal sequence and combination of surgical interventions remain controversial, particularly in cases where both conditions coexist. Understanding effective management strategies is crucial to improving outcomes in these patients.

Case Description: We analyzed nine pediatric patients treated at our institution for combined craniosynostosis and hydrocephalus. The cohort included seven males and two females, aged 8 to 45 months. Seven patients had syndromic craniosynostosis, while two had non-syndromic forms. All were admitted due to abnormal facial features. At admission, six had normal head circumference, two had reduced, and one had increased head circumference. Elevated intracranial pressure was observed in eight patients. All underwent cranioplasty, with cerebrospinal fluid leakage occurring in two cases. Five patients required ventriculoperitoneal (VP) shunt placement due to persistent hydrocephalus after surgery. Follow-up, averaging 53.6 months, showed improved cranial shape and symptom relief in eight patients, and resolution or significant mitigation of hydrocephalus in all cases.

Conclusions: Craniosynostosis frequently coexists with hydrocephalus, particularly in syndromic cases with normal head circumference. Based on our institutional experience, initial cranioplasty was commonly performed to address cranial deformities and was associated with improvement in some patients. However, persistent hydrocephalus in certain cases required additional intervention, such as VP shunting.