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Perioperative management of benign hepatic tumors in patients with glycogen storage disease type Ia
Author(s) -
Oshita Akihiko,
Itamoto Toshiyuki,
Amano Hironobu,
Ohdan Hideki,
Tashiro Hirotaka,
Asahara Toshimasa
Publication year - 2008
Publication title -
journal of hepato‐biliary‐pancreatic surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.63
H-Index - 60
eISSN - 1868-6982
pISSN - 0944-1166
DOI - 10.1007/s00534-007-1244-3
Subject(s) - perioperative , medicine , lactic acidosis , hypoglycemia , metabolic acidosis , glycogen storage disease type i , gastroenterology , acidosis , glycogen storage disease , disease , surgery , endocrinology , insulin , anesthesia
Glycogen storage disease type Ia (GSD‐Ia; von Gierke disease) is an inherited disorder caused by glucose‐6‐phosphatase deficiency, and there have been some reports of hepatic tumors in patients with this disease. We report two patients with benign hepatic tumors with GSD‐Ia. One is a 19‐year‐old man who underwent segmentectomy 4 for a focal nodular hyperplasia, and the other is a 31‐year‐old woman who underwent segmentectomies 3, 5, and 6 for hepatic adenomas. Two significant perioperative complications, resulting from the carbohydrate metabolic disorders, hypoglycemia and metabolic acidosis, occurred in both patients. We managed the metabolic complications successfully by administering a sufficient volume of glucose intravenously. Close perioperative monitoring of blood glucose and lactate concentrations is essential in the perioperative management of patients with GSD‐Ia. The intravenous administration of glucose, starting with a smaller dose and then increasing the dose, is adequate management for lactic acidosis with or without hypoglycemia during the perioperative period.