Shunt-induced craniosynostosis with craniocerebral disproportion: a new way of thinking about an old problem.

Objective

Shunting during infancy can precipitate premature closure of one or more cranial suture. Shunt-induced craniosynostosis (SIC) may in turn result in craniocerebral disproportion (CCD). The authors present a single-institution series and offer a pragmatic diagnostic and treatment framework.

Methods

This study was a retrospective case series of patients with SIC-CCD who underwent surgical correction between 2018 and 2025 at a single institution.

Results

Eighteen patients were identified. The mean (range) age at initial shunt placement was 2 (0-9) months, and the mean age at CCD corrective surgery was 5.6 (1.1-18.4) years. Eleven patients (61%) presented with headaches and 14 (78%) had one or more symptoms attributable to CCD. All patients underwent surgery for CCD: 16 underwent cranial vault expansion (12 with distractors), and 2 underwent suturectomy. Three patients required a delayed secondary salvage expansion. The mean follow-up was 3.47 (0.56-8.28) years. At the last follow-up, more than two-thirds of patients reported symptom improvement and all had significantly less radiographic intracranial crowding. Emergency department visits for shunt concerns and shunt revisions decreased after vault expansion.

Conclusions

Pediatric neurosurgeons, craniofacial plastic surgeons, and neuroradiologists must maintain a high suspicion for SIC-CCD in every child that was shunted early in life. Once CCD is diagnosed, early surgical expansion is recommended. Contemporary craniofacial techniques, aided by computer-assisted surgical planning (CAS), can safely achieve meaningful intracranial volume expansion with symptomatic improvement for most patients.