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Surgery for gastroesophageal reflux disease with Gaucher disease type 2
Author(s) -
Kubo Hiroyuki,
Shimono Ryuichi,
Tanaka Aya,
Fujii Takayuki,
Yasuda Saneyuki,
Koyano Kosuke,
Jinnai Wataru,
Kondo Sonoko,
Kondo Takeo,
Kusaka Takashi
Publication year - 2016
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12851
Subject(s) - medicine , hepatosplenomegaly , nissen fundoplication , enzyme replacement therapy , disease , gerd , reflux , surgery , gaucher's disease
Gaucher disease, the most common lysosomal storage disease, is sometimes complicated with gastroesophageal reflux disease (GERD). The present patient was a 136‐day‐old Japanese boy with Gaucher disease type 2. Enzyme replacement therapy and chemical chaperone therapy were successful for the skin disorders, joint contractures, hepatosplenomegaly and thrombocytopenia, but he also had GERD. Accordingly, a Nissen fundoplication with gastrostomy was performed. There was no vulnerability of organs, easy bleeding or difficulty of maintaining the visual field because of hepatosplenomegaly during operation. In the perioperative period, there was no prolonged wound healing or infection. GERD was improved. In the near future, the number of long‐term survivors of Gaucher disease will increase due to improvements in medical therapy. Therefore, it is expected that the number of patients requiring fundoplication will also increase. In patients with successful medical therapy, surgical fundoplication can be safely and effectively performed.