Anaesthetic Concerns in a Child with Lesch-Nyhan Syndrome

Abstract

Lesch-Nyhan syndrome is a rare X-linked recessive disorder caused by a deficiency of Hypoxanthine-Guanine Phosphoribosyl Transferase (HGPRT) enzyme. The patients with Lesch-Nyhan Syndrome will have spasticity due to which there could be several problems such as positioning, difficult intravenous cannulation and difficult airway. And these patients will have self-mutilating behavior and lack of pain sensation. Bradycardia, pulmonary aspiration, convulsion are the important causes of death. Anesthetic management of these patients in pediatric age group is not well described. Here, we report our anesthesia experience in a 3 years-old child who was posted for debridement of right great toe with Lesch-Nyhan syndrome. Patients with Lesch-Nyhan syndrome should be induced in titrated doses and no analgesia is required because of lack of sensation. Careful monitoring is required during perioperative and post-operative period as they are more prone for bradycardia, apnea and aspiration.