General anesthesia for a spinal muscular atrophy type I patientundergoing feeding gastrostomy: A case report

Jang, Eun Ho; Cho, Kwang Rae; Kim, Hyun Tae; Lim, Se Hun; Lee, Jeong Han; Lee, Kun Moo; Cheong, Soon Ho; Kim, Young Jae; Shin, Chee Mahn

Case Report

Neuromuscular Physiology and Pharmacology

Anesthesia and Pain Medicine 2010;5(4):329-332.
Published online October 31, 2010.

General anesthesia for a spinal muscular atrophy type I patientundergoing feeding gastrostomy: A case report

Eun Ho Jang, Kwang Rae Cho, Hyun Tae Kim, Se Hun Lim, Jeong Han Lee, Kun Moo Lee, Soon Ho Cheong, Young Jae Kim, Chee Mahn Shin

Department of Anesthesiology and Pain Medicine, Paik Hospital, College of Medicine, Inje University, Busan, Korea. maeteo@hanmir.com

Abstract

Spinal muscular atrophy (SMA) in children leads to progressive muscle weakness, dysphagia, aspiration, and death. The most common and severe form of SMA is designated as type I, also known as Werdnig-Hoffman Disease or Floppy Baby syndrome. We anesthetized an 8 month-old female infant with SMA type I undergoing feeding gastrostomy. We planned to use inhalational anesthesia without muscle relaxants. Anesthesia and surgery were uneventful. We herein report a case of successful peri-operative anesthetic management for SMA type I infant with aspiration pneumonia.

Key Words: Gastrostomy, Neuromuscular disease, Spinal muscular atrophy type I