A 30-year-old female with a medical history of an omphalocele with displacement of her liver into the pelvic cavity presented to the emergency department with several days of fever, lower abdominal pain, and distension. Physical examination revealed lower abdominal tenderness without peritoneal signs. The liver enzymes were mildly elevated and her hemoglobin dropped 1.7 g while in the emergency room.
Abdominal computed tomography (CT) scan showed that the liver was absent from the right upper quadrant and revealed a pelvic “upside-down” liver with a dominant lesion in the right lobe with hemorrhage suggestive of hepatic adenoma with acute bleed (Fig. 1A). A stalk of liver tissue containing vascular structures and the common bile duct was visualized extending upward from the pelvis.
The patient was explored in the operating room through a lower midline incision. She underwent a right hepatic lobectomy with removal of the hemorrhagic adenoma (Fig. 1B). Care was taken to avoid injury to the elongated portal triad, which extended from the pelvic liver to the right upper quadrant (Fig. 1C). Histologic specimen demonstrated a mass lesion composed of cells resembling normal hepatocytes arranged in trabecular pattern with areas of hemorrhage surrounded by focal inflammation and granulation (Fig. 1D). Final pathology diagnosis was hepatocellular adenoma with focal hemorrhage and necrosis. The patient had an uncomplicated postoperative course and was discharged in good condition.
Although benign lesions have been reported in ectopic liver tissue,1 an important distinction between these cases and the current report is that ectopic liver consists of patches of normal liver tissue outside the primary liver, which often do not receive any arterial supply from the hepatic artery and lack a portal system and ductal connection to the biliary tract. This patient had her entire liver congenitally displaced into the pelvic cavity, and it was actually flipped upside-down. This is the first case reported of a ruptured hepatic adenoma in a congenital pelvic liver resulting from an abdominal wall defect. Interestingly, an “upside-down” gallbladder has been reported in a case where the ectopic gallbladder initially mimicked a possible neoplasm on CT scan.2 The discovery of a hepatic adenoma should almost always result in surgical resection due to the risk of life-threatening hemorrhage or progression to hepatocellular carcinoma.3 Laparoscopic resection may be an alternative to open surgery for selected patients.4