Neuropediatrics 2009; 40(1): 28-31
DOI: 10.1055/s-0029-1231066
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

Treatment of Gastroesophageal Reflux with Nissen Fundoplication and Gastrostomy Tube Insertion in Infantile Pompe's Disease

M. Hirschburger 1 , 6 , A. Hecker 1 , 6 , W. Padberg 1 , B. A. Neubauer 2 , R. Motz 3 , C. Haase 4 , T. Marquardt 5 , A. Hahn 2
  • 1Department of General and Thoracic Surgery, University of Giessen, Giessen, Germany
  • 2Department of Neuropediatrics, University of Giessen, Giessen, Germany
  • 3Department of Pediatric Cardiology, Elisabeth Hospital, Oldenburg, Germany
  • 4Department of Pediatrics, University of Jena, Jena, Germany
  • 5Department of Pediatrics, University of Münster, Münster, Germany
  • 6These authors contributed equally to this work
Further Information

Publication History

received 11.04.2009

accepted 19.06.2009

Publication Date:
28 July 2009 (online)


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.



A. Hahn, MD 

Department of Neuropediatrics

University of Giessen


35385 Giessen


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