Craniosynostosis; a Rare Complication of Endoscopic Third Ventriculostomy for Complex Hydrocephalus Associated with MAN2B1 Gene Mutations (Alpha-mannosidosis)

Due to lower risk of postoperative complications and shunt-independence, endoscopic third ventriculostomy (ETV) is a compelling alternative to CSF-shunt implantation in the management of hydrocephalus. While craniosynostosis is an uncommon complication of CSFshunt- based procedures, to our knowledge, no reports on post-ETV craniosynostosis, exist. The authors describe a case of a 2-year, 4-month-old female presented with pancraniosynostosis after endoscopic treatment of complex hydrocephalus performed at the age of 2 months. A 3-Dimentional CT-scan of the cranium revealed craniosynostosis involving all skull sutures, which was treated with fronto-orbital advancement and cranial vault reconstruction. Genetic study revealed two pathogenic copies of MAN2B1 gene (alphamannosidosis), which had probably contributed to the development of craniosynostosis in this patient. To the best of our knowledge, this is the first case of craniosynostosis occurring after ETV procedure. A close follow-up of this patient group allows early detection and management of rare yet correctable complication.