Anaesthetic management of an obstetric patient with Pompe disease

• Published in: International journal of obstetric anesthesia Vol. 17; no. 2; pp. 170 - 173
• Main Authors: Cilliers, H.J, Yeo, S.T, Salmon, N.P
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ltd 01-04-2008
• Subjects: Adult; Anaesthesia: spinal; Anesthesia & Perioperative Care; Anesthesia, Epidural; Anesthesia, Obstetrical - methods; Anesthesia, Spinal; Caesarean section; Cesarean Section, Repeat; Female; Glycogen Storage Disease Type II - complications; Humans; Obstetrics and Gynecology; Pompe disease; Postoperative Care - methods; Pre-Eclampsia - drug therapy; Pregnancy; Pregnancy Complications; Prenatal Care - methods; Spirometry; Treatment Outcome; Pompe disease; Pregnancy; Caesarean section; Anaesthesia: spinal
• ISSN: 0959-289X; 1532-3374
• DOI: 10.1016/j.ijoa.2007.11.001

Abstract Pompe disease (Glycogen storage disease type II) leads to abnormal glycogen deposition in various vital organs resulting in multiple systemic sequelae. We present the anaesthetic management for caesarean section of a 31-year-old parturient with known Pompe disease. The parturient had symptoms and signs of respiratory dysfunction and the pregnancy was complicated by preeclampsia. She underwent urgent caesarean section under regional anaesthesia resulting in the birth of a healthy baby girl. To our knowledge, this is the first reported case of both spinal anaesthesia for caesarean section and successful live birth in a patient with Pompe disease.