Successful surgical management of multiple hepatocellular adenomas in a young female patient with glycogen storage disease type Ia: a case report.

BACKGROUND: Glycogen storage disease type Ia is a rare inherited metabolic disorder characterized by glucose-6-phosphatase deficiency. Hepatocellular adenoma development is a frequent complication in patients with this condition, with prevalence rates reaching up to 75%. The management of hepatic adenomas in these patients remains challenging due to risks of hemorrhage, malignant transformation, and surgical complications. Case Presentation We report a 22-year-old Chinese female with glycogen storage disease type Ia who presented with acute metabolic decompensation, including hypoglycemia (2.8 mmol/L), severe hyperlactatemia (20.0 mmol/L), and marked hypertriglyceridemia (31.78 mmol/L). The patient had been diagnosed with glycogen storage disease type Ia at age 5 and maintained on dietary therapy. At age 18, multiple hepatic adenomas were detected. Following intensive preoperative metabolic optimization and a comprehensive perioperative management protocol, she underwent successful indocyanine green fluorescence-guided left hepatectomy at age 22 with excellent postoperative recovery and no evidence of malignant transformation.

CONCLUSION: This case demonstrates that hepatic adenomas in patients with glycogen storage disease type Ia can be successfully managed surgically with appropriate perioperative metabolic management. Early diagnosis, regular surveillance, and timely surgical intervention are crucial for preventing life-threatening complications in this patient population.