A Case of Glycogen Storage Disease Type Ia with Multiple Hepatocellular Adenomas that were Resected Twice

Naoya Ikeda, Kiyoshi Fukunaga, Shinji Hashimoto, Tadashi Kondo, Tatsuya Oda, Ryoko Sasaki and Nobuhiro Ohkohchi

Department of Surgery, University of Tsukuba

Glycogen storage disease type Ia (GSDIa) is a congenital metabolic disorder often associated with multiple hepatocellular adenomas. We report a case of adenomas with internal bleeding requiring hepatectomy twice. A woman diagnosed with GSDIa when one year old was later found to have multiple hepatocellular adenomas and followed up thereafter. At age 18, she was seen for sudden upper left abdominal quadrant pain and fever and diagnosed with adenoma with internal bleeding in the left lateral segment. Hepatic selective transcatheter arterial embolization (TAE) for preventing rupture failed to relieve pain and fever, so we conducted lateral segmentectomy to alleviate symptoms and due to the possibility of malignancy. Three years later, we partially resected her liver due to growing adenomas with internal bleeding. Liver transplantation is a radical treatment for multiple hepatic adenomas in GSDIa. We found adenoma resection useful because liver transplantation was hardly a possibility due to Japan's dire donor shortage. Careful follow-up is especially important to subjects in their 30s due to potential malignancy.

Key words
glycogen storage disease type Ia, hepatocellular adenoma, hepatectomy

Reprint requests
Naoya Ikeda Department of Surgery, University of Tsukuba
1-1-1 Tennoudai, Tsukuba, 305-8575 JAPAN

Accepted
March 24, 2010

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