Abernethy Malformation Type II and Concurrent Nodular Hyperplasia in a Rare Female Case

Case Reports in Radiology
17 May, 2019 ,

This is a rare female case of Abernethy malformation type II with concurrent occupying lesion in the right liver, which was successfully transplanted; the occupying lesion was pathologically proven to be nodular hyperplasia. The case was successfully transplanted by piggyback method.

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A 10-year-old female visited the emergency room of our hospital with right upper quadrant abdominal pain for 8 days that was not associated with eating. No fever, jaundice, emesis, diarrhea, or melena was reported. Initial blood tests and liver function test revealed no abnormalities other than mild elevation of cytomegalovirus IgG antibody (23.7 U/ml). A routine urine test was positive for red blood cells (3+, 294.8/μl) and white blood cells (3+, 422.9/μl). Alpha-fetoprotein and cancer embryonic antigen were negative, whereas cancer antigen 19-9 was elevated (247.25 U/ml, normal level 2-37 U/ml).

Ultrasound examination exhibited a mass measuring 78 x 71 mm in the right liver with abundant blood signals. No obvious portal venous trunk and branches were noted in the liver but the superior mesenteric vein and splenic veins drained directly into the inferior vena cava. The left hepatic vein and middle hepatic vein conjoined together and then drained to the inferior vena cava.

Abdominal enhanced CT with multiplanar reconstruction revealed that the splenic vein and superior mesenteric vein drained directly into the inferior vena cava after confluence, and only two branches of hepatic veins drained into the inferior vena cava. A barely perceptible small branch of the portal vein measuring 3 mm supplied the left lobe of the liver. In addition, a mass was noted in hepatic segment 5, approximately 55 mm in size and poorly demarcated. Arterial phase of enhancement showed mildly heterogeneous enhancement, while portal venous and delayed phases demonstrated iso- to hypoattenuating. Abernethy malformation (type II) and concurrent hepatocellular carcinoma or hepatoblastoma were suspected.

The liver donor from a close relative was examined comprehensively and the transplantation for this suffered child was conducted 1 month after diagnosis. Surgical findings demonstrated a mass occupying the right liver measuring 60 mm in diameter. No remarkable liver cirrhosis was noted. The superior mesenteric vein and splenic vein merged together and drained directly to the inferior vena cava.

A faintly visible branch vessel measuring 3 mm dominated the left lobe of the liver; no veins supplied the left medial lobe and the right lobe of the liver. A left lobe of liver was donated from a close relative. Considering that the donor liver weighed only 250g and the ratio of donor liver to recipient weight was 0.83, an auxiliary piggyback liver transplantation method was conducted. The allogeneic iliac vein was adopted to reconstruct the left branch of the recipient portal vein and the left hepatic vein.

Then, the hepatic vein was anastomosed to the recipient vena cava. The right hepatic artery of the recipient and the left lobe of the donor liver were anastomosed with the allogeneic iliac artery. The left hepatic duct, the ductus cysticus, and the biliary tree of the donor liver were also reconstructed by end-to-end anastomosis. Meanwhile, the diseased right lobe of the liver was resected. Two years after the transplantation, physical examination as well as CT and MR imaging showed the patient was in a satisfactory condition; the transplanted liver was practically normal in contour and signal intensity. The child is still alive at present, 4 years after surgery.

Microscopically, the lesion was in nodular appearance without feeding hepatic veins. Abnormal hepatic lobules and distorted bile ducts scattered throughout the mass, with increased amount of fibrous connective tissues and chronic inflammatory cells. Some hepatocytes were characterized by hydropic degeneration, cholestasis, and regeneration.

Portal spaces exhibited an absence of veins and were replaced by chronic inflammatory cell infiltration, with 3 lymph nodes featuring reactive hyperplasia. The final pathological diagnosis was congenital extrahepatic portal-systemic shunt and coexisting nodular hyperplasia.