Treatment of gastroesophageal reflux with nissen fundoplication and gastrostomy tube insertion in infantile pompe's disease.

Hirschburger M, Hecker A, Padberg W, Neubauer BA, Motz R, Haase C, Marquardt T, Hahn A

Research article (journal)

Abstract

In infantile Pompe's disease, enzyme replacement therapy (ERT) has been shown to reverse cardiomyopathy, improve skeletal muscle strength, and prolong survival. We report on five patients in whom complications related to gastroesophageal reflux (GER) resulted in deterioration of their clinical status despite initial improvement under ERT. Surgical antireflux therapy, performed in four, yielded positive results in two. Three patients experienced severe aspirations related to GER and underwent fundoplication and gastrostomy subsequently. Two did not regain former motor functions and deceased shortly thereafter, while one slowly recuperated and is in a stable state at age 53 months. In a further patient, severe GER prompted fundoplication at age 17 months. No aspirations occurred until the girl deceased probably due to cardiac arrest 20 months later. These cases suggest that infants with Pompe's disease under ERT may benefit from timely performed fundoplication and gastric tube placement.

Details about the publication

JournalNeuropediatrics
Volume40
Issue1
Page range28-31
StatusPublished
Release year2009
Language in which the publication is writtenEnglish
DOI10.1055/s-0029-1231066
KeywordsGlycogen Storage Disease Type II. Postoperative Complications; Fundoplication; Humans; Intubation Gastrointestinal; Retrospective Studies; Follow-Up Studies; Infant; Female; Gastroesophageal Reflux; Treatment Outcome; Male; Gastrostomy; Glycogen Storage Disease Type II. Postoperative Complications; Fundoplication; Humans; Intubation Gastrointestinal; Retrospective Studies; Follow-Up Studies; Infant; Female; Gastroesophageal Reflux; Treatment Outcome; Male; Gastrostomy

Authors from the University of Münster

Hahn, Ann-Katrin
Institute of Art History
Marquardt, Thorsten
Center of Child and Adolescent Medicine