Anaesthetic Management of Paediatric Patient with Klippel-Trenaunay Syndrome undergoing Excision of Scalp Cyst: A Case Report

Abstract

Klippel-Trenaunay Syndrome (KTS) is a sporadic condition characterised by the clinical triad of capillary malformation, atypical varicosity, and soft-tissue and bony hypertrophy. This triad was observed in the current case. Despite being initially described over a century ago, the exact incidence of this syndrome remains unestimated. The clinical presentation of KTS is highly diverse, ranging from asymptomatic cases to severe instances involving life-threatening bleeding and embolism. The authors present a case report of a 10-year-old girl with complaints of a cystic swelling in her left temporal region. She exhibited distinct features such as facial asymmetry, a high-arched palate, a prominent mandible, a port-wine stain on her cheeks, nasal bridge depression, and right-sided body hypertrophy. The child showed delayed developmental milestones, including holding her neck at 1.5 months, a bidextrous approach at seven months, and social smiling at six months, indicating global developmental delay. The patient was scheduled for excision of the swelling. Following the successful excision of the cyst, the child was extubated. The extubation was uneventful. In the postoperative period, the patient remained haemodynamically stable. Managing such a patient for a surgical procedure requires a meticulous approach involving accurate anaesthetic preparation and the prevention of complications.