In September of 2003, a 32-year-old Caucasian female presented to an outside institution with a chief complaint of increasing abdominal fullness for 2 months. The patient reported mild abdominal discomfort during her daily activities, as well as decreased appetite and early satiety. Her past medical history was significant for an incidentally diagnosed liver mass in 1994 when she had undergone an abdominal computed tomographic (CT) scan as part of a workup for amenorrhea. At that time, the 10-cm well-marginated hypodense mass in the right lobe of the liver was confirmed to be a hemangioma by subsequent nuclear scan, and no further treatment was initiated. On her subsequent presentation in 2003, the patient was found to have massive hepatomegaly and thus underwent laparoscopic biopsy of the hepatic lesion. Pathology revealed a benign cavernous hemangioma, and the patient was then transferred to our institution for further evaluation.
On admission, the patient's laboratory values were notable for a total bilirubin of 2.1 mg/dL (normal range, 0.3–1.3) and an international normalized ratio (INR) of 1.4. Serum tumor markers were all within normal limits. CT scan of the abdomen and chest showed numerous, large hypervascular liver masses, the largest measuring 18
23 cm (Fig. ), splenic hemangiomas, as well as innumerable bilateral pulmonary nodules (Fig. ). Given the multiorgan presentation raising concern for a metastatic neoplasm, the patient underwent video-assisted thoracoscopic surgery which demonstrated diffuse, purple, raised, subpleural nodules (Fig. ). The lung biopsy demonstrated pulmonary cavernous hemangiomas (Fig. ). Additional stains for both CD34 and CD31 highlighted the benign endothelial cell lining of the vascular spaces. In addition, HMB-45 staining was negative, ruling out lymphangioleiomyomatosis. The patient was discharged from the hospital and started on interferon alpha-2b in an attempt to shrink her hepatic and pulmonary lesions; this resulted in mild radiological and symptomatic improvement. Given the size and number of her lesions, it was felt that curative liver resection was not technically possible and that ultimately she might require liver transplantation. The patient was listed for transplantation, but over the ensuing years she remained stable.
In January of 2010, the patient developed severe mid-abdominal pain while riding a horse. She subsequently suffered a syncopal episode and was taken to the emergency room where she was found to be hypotensive with a hematocrit of 22%. She was transfused 4 units of blood and transferred to our facility. On arrival, she was in no distress and hemodynamically stable. Her exam was significant for markedly increased abdominal distension (Fig. ). Her admission weight was 84 kg. Laboratory values demonstrated a hematocrit of 24.6%, INR of 2.1, fibrinogen of 78 mg/dL, platelets of 99,000
/L (decrease from her baseline of 180,000
/L), creatinine of 0.61 mg/dL, and a total bilirubin of 4.4 mg/dL. Her admission Model for End-Stage Liver Disease (MELD) score was 20. A CT scan demonstrated a large amount of intra-peritoneal blood in addition to the known hepatic hemagiomas. Over the course of the subsequent 48 h, her abdominal distension worsened, and she developed progressive lower extremity edema. Despite resuscitation with blood products, as well as the use of aminocaproic acid, she continued to demonstrate a consumptive coagulopathy with evidence of ongoing bleeding. Her abdominal compartment syndrome worsened, with evidence of decreased urinary output and rising creatinine levels. A petition to the United Network for Organ Sharing Regional Review Board requesting a high initial MELD to ensure life-saving emergent transplantation was submitted and approved.
Fig. 2 a Intra-operative photo prior to start of liver transplantation demonstrating the patient's massive abdominal distension. b Giant cavernous hepatic hemangioma occupying the entire abdominal cavity. c Posterior aspect of gross liver specimen following (more ...)
On hospital day 14, a suitable donor became available. The recipient was brought to the operating room and explored. Four liters of old blood, with no evidence of clot, were evacuated. There were no immediate signs of active bleeding. The liver occupied her entire abdomen, the majority of which showed the appearance of a massive cavernous hemangioma (Fig. ). The omentum contained innumerable berry-sized hemangiomas. The patient had a conventional main hepatic artery, as well as replaced right and left hepatic arteries. These were individually ligated and divided. Clamps were then placed on the supra-hepatic inferior vena cava (IVC), infra-hepatic IVC, and portal vein. The liver was then drained of a large volume of blood to facilitate the completion of the posterior dissection under improved visualization. The liver was excised (Fig. ), and the donor allograft was brought to the operative field. A bicaval anastomosis, followed by a portal vein anastomosis, was performed. Given the small caliber size of each of the recipient's hepatic arteries, the arterial anastomosis was done directly to the supraceliac aorta. Biliary drainage was achieved by choledochocholedochostomy. Following completion of the liver transplant (Fig. ), the patient was extubated in the operating room and transferred to the intensive care unit. She was subsequently transferred to the transplant ward on post-operative day 1 and discharged home on post-operative day 11 following an uneventful post-operative course. On outpatient follow-up, the patient remains well and has begun to resume a normal level of activity.
The patient's weight at the time of discharge was 46 kg, a net loss of 38 kg from her pre-transplant weight. Histological examination of the liver revealed multiple areas of dilated blood-filled spaces lined by a layer of flattened endothelial cells, thus confirming the diagnosis of cavernous hepatic hemangioma. These changes occupied the entire right lobe and the majority of the left lobe of the liver.