Adult Noonan’s syndrome and associated severe scoliosis. A case report and discussion of the anaesthetic implications.

Abstract

Noonan’s syndrome is a relatively rare genetic disorder with distinct clinical manifestations. Surgery is usually required early on in life however many of these patients can present for surgery later in life. Specific challenges to the anaesthesia can include, coexistent cardiac abnormalities, risk of difficult intubation, bleeding abnormalities, scoliosis and the potential for triggering Malignant Hyperthermia. Although characteristic phenotypical appearances can become less distinct with age, many abnormalities persist along with new potential problems. We report a case of an adult male with Noonan’s syndrome who developed severe scoliosis and presented for elective hemorrhoid repair. The procedure was safely performed under low dose spinal saddle anaesthesia. We also review the relevant perioperative issues and the challenges that can be encountered when deciding on the most appropriate mode of anaesthesia.