Anaesthetic Management of Circumcision and PEG Tube Replacement in an Infant with Congenital Myasthenic Syndrome: A Case Report

Background: Congenital myasthenic syndrome (CMS) is a rare genetic disease of neuromuscular transmission with abnormal synaptic transmission, which causes weakness, hypotonia and risk of respiratory insufficiency. It is accompanied by enhanced sensitivity to anaesthetic agents and a significant risk of perioperative respiratory effects. There are further complications to anaesthetic management due to repeated surgical procedures. Case presentation: We present an infant with CMS, who had undergone percutaneous endoscopic gastrostomy (PEG) insertion earlier and again presented for circumcision and PEG tube replacement. Persistent hypotonia was found preoperatively while the respiratory status was stable. Sevoflurane and minimal opiate supplementation titrated with intravenous propofol were used for induction of anaesthesia. Neuromuscular blocking agents were not used. Sevoflurane in an oxygen–air mixture and remifentanil infusion were used with spontaneous ventilation for maintenance. Results: No complications during the procedure, both procedures completed successfully. Throughout, the patient was hemodynamically stable and had adequate spontaneous respiration. There were no complications in the treatment of the patient, and ventilatory support was not needed during the recovery period. Conclusion: It is safe to repeat anaesthetic exposure in infants with CMS provided that the exposure is carefully planned, neuromuscular blocking agents are avoided and ventilation remains spontaneous. To achieve the best results, individual anesthetic plans should be developed and carefully monitored.

Keywords: Congenital myasthenic syndrome, circumcision, PEG tube replacement, paediatric anaesthesia, spontaneous ventilation.