title: Anaesthetic management of an obstetric patient with Pompe disease.
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.
authors.affiliation: Department of Anaesthesia, Hereford Hospitals NHS Trust, Hereford, UK. hjcilliersmail-medical@yahoo.co.uk
authors.forename: H J
authors.initials: HJ
authors.lastname: Cilliers
authors.forename: S T
authors.initials: ST
authors.lastname: Yeo
authors.forename: N P
authors.initials: NP
authors.lastname: Salmon
mesh_terms: D000328:Adult; D000767:Anesthesia, Epidural; D000773:Anesthesia, Obstetrical / Q000379:methods; D000775:Anesthesia, Spinal; D017604:Cesarean Section, Repeat; D005260:Female; D006009:Glycogen Storage Disease Type II / Q000150:complications*; D006801:Humans; D011182:Postoperative Care / Q000379:methods; D011225:Pre-Eclampsia / Q000188:drug therapy*; D011247:Pregnancy; D011248:Pregnancy Complications; D011295:Prenatal Care / Q000379:methods; D013147:Spirometry; D016896:Treatment Outcome