Expansion cranioplasty with jackscrew distracters for craniosynostosis and intracranial hypertension in transplanted osteopetrosis

Abstract

Background: An 11-month-old boy with autosomal recessive infantile osteopetrosis presented, 7 months after bone marrow transplantation, with normal ventricular size and life-threatening intracranial hypertension due to pansynostosis.

Methods: The cranial vault was expanded by using jackscrew distracters to upwardly advance the upper part of the calvarium.

Results: The procedure achieved a 15-mm upward expansion of the cranial vault over a 15-day period, and the volume of the cranial vault was increased by 139 ml. All clinical manifestations of intracranial hypertension resolved.

Conclusion: Cranial vault expansion with jackscrew distracters was successful in relieving intracranial hypertension in an infant with pancraniosynostosis complicating bone marrow transplanted malignant infantile osteopetrosis.