Craniotomy in Klippel-Trenaunay syndrome: Concerns and challenges

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder characterised by cutaneous capillary malformations, soft tissue and bone hypertrophy and venous varicosities. The coexistence of Chiari 1 malformation and an intracranial tumour has been rarely reported in the literature. Multisystem involvement of this syndrome mandates adequate preparation and planning, with meticulous conduct of anaesthesia to achieve favourable outcomes. We report a case of KTS syndrome with Chiari 1 malformation who had presented for craniotomy, and thereby discuss the challenges faced during anaesthetic management of these patients for major surgeries.