Intrahepatic Lymphedema Presenting With Abnormal Bilirubin Metabolism as the Initial Manifestation

Intrahepatic lymphedema is a rare condition usually associated with hepatobiliary diseases, cardiogenic factors, pancreatic disorders, or malignant lymph node lesions. This report describes a unique case of intrahepatic lymphedema presenting with abnormal bilirubin metabolism as the initial manifestation, with no identical cases identified in the China National Knowledge Infrastructure (CNKI), Wanfang Database, or PubMed. The patient was a 30-year-old Chinese man admitted due to fatigue, anorexia, and yellowish urine for 3 days. Physical examination revealed mild generalized jaundice without hepatosplenomegaly or signs of chronic liver disease. Laboratory tests showed marked elevation of total bilirubin (347.1 μmol/L), direct bilirubin (241.7 μmol/L), and indirect bilirubin (105.4 μmol/L), while viral hepatitis markers (Hepatitis B Virus DNA, Hepatitis C Virus RNA, Human Immunodeficiency Virus RNA) and autoimmune antibody panels were negative. Abdominal computed tomography (CT) demonstrated dilated lymphatic vessels around the intrahepatic portal vein without extrahepatic biliary obstruction or cardiac dysfunction. Intravenous methylprednisolone sodium succinate (40 mg/d) was initiated as exploratory treatment since autoimmune hepatitis could not be excluded. Jaundice improved significantly after 4 days, with the dose reduced to 20 mg/d. By day 8 of treatment, total bilirubin decreased to 112 μmol/L and liver CT showed regression of lymphatic dilation. At the 14-day follow-up, all biochemical indicators had normalized and CT abnormalities resolved; liver biopsy was not performed due to the patient's refusal and rapid clinical response to glucocorticoids. This case suggests that exploratory treatment with glucocorticoids (e.g., methylprednisolone sodium succinate) may be effective for patients with unexplained intrahepatic lymphedema and abnormal bilirubin metabolism, especially when an immune-mediated etiology is suspected.