Anaesthetic Management of Patient with Ellis Van Creveld Syndrome

Ellis van Creveld syndrome (EVC syndrome) also known as chondroectodermal or mesoectodermal dysplasia was first described by Richard W.B. Ellisand Simon Van Creveld in 1940. The name chondroectodermal is used because it affects the skeleton (chondro) and skin (ectoderm). In general population the incidence is reported as 7 per 1,000,000 live births. Due to multisystem involvement anaesthesia care is challenging during intraoperative as well as post-operative period. Here we describe the perioperative care of a 14 year old female with EVC syndrome who underwent corrective osteotomy of tibia for genu valgum. Patient was given anaesthesia fitness under American Society of Anesthesiologists as grade 1. We planned to give subarachnoid block to the patient as she had no anomalies involving any other system of body except skeletal system, moreover our patient had normal lumbosacral spine study but thoracic spine scoliosis. Perioperative period was without any adverse events. Postoperative management constitutes adequate analgesia and prevention of adverse cardiorespiratory events.