Neurofibromatosis: Challenge for Anaesthetist.

Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous syndrome which is characterized by formation of neurofibromas all over the skin and various other body systems. The anaesthetic management of these patients requires attention to all possible abnormalities and associated disturbances to prevent any peri-operative complication. NF-1-associated complications of the musculoskeletal, respiratory, cardiovascular, central nervous, and gastrointestinal and genitourinary systems present with various degrees of considerations for anaesthesiologists. While evaluating and managing these patients for surgical procedures. NF-1 or von Recklinghausen disease, is an autosomal dominant disease with incidence of approximately 1/3000 births. Gastrointestinal (GI) neoplasms are present in about 2 - 25% of patients of NF-1. In these patients, neurofibromas are the most frequently (52%) diagnosed benign neoplasms. Patients with NF-1 and GI symptoms are at risk for gastrointestinal neoplasms; symptomatic patients are likely to experience significant morbidity. This report describes the anaesthetic of a patient with NF-1 and gastric outlet obstruction.