Spontaneous liver rupture in London Lucien P. J. Ooi, FRCS(Edin & Glasg), Stephen V. Lynch, FRACS, David A. Graham, FRACS, and Russell W. Strong, FRACS, Brisbane and Cairns, Australia amyloidosis From the Hepatobiliary Unit, Department of Surgery, Princess Alexandra Hospital, Brisbane, and Cairns Base Hospital, Cairns, Australia SPONTANEOUS HEPATIC RUPTURE is a n u n c o m m o n prob- lem most often seen with large hepatocellular carcino- ma, 1 malaria, preeclampsia of pregnancy, 2 and some benign liver tumors. 3 Hepatic amyloidosis as a cause for spontaneous rupture of the liver is rare, with only seven cases reported. 4-9 Death has been inevitable within hours to days of presentation. We report a case of a pa- dent with spontaneous rupture of an amyloid liver who survived the immediate event, and we review the obser- vations of the other reported cases for a better under- standing of this condition. CASE REPORT A previously well, 41-year-old man with no history of trauma had sudden upper abdominal and back pain. He was admitted to a regional hospital with right upper quadrant tenderness and an ambiguous ultrasonographic finding suggestive of a thickened gallbladder. He had a leukocytosis of 16.2 • 109/L, bilirubin of 24 ~tmol/L (normal, 3 to 22 pmol/L), alkaline phosphatase of 264 units/L (normal, 30 to 121 units/L), y-glutamyl transpeptidase of 150 IU/L (normal, 0 to 50 IU/ L), aspartate aminotransferase of 72 IU/L (normal, 2 to 42 IU/L), and alanine aminotransferase of 82 IU/L (normal, 10 to 60 IU/L). The abdominal signs progressed to generalized peritonism during a period of 24 hours. Laparotomy was per- formed, and more than 1 L of old blood was evacuated from the peritoneal cavity. The liver was tense and engorged, with rupture of the right lobe. The spleen was enlarged and rock hard. An operative diagnosis of possible ruptured hepatocel- lular carcinoma or lymphoma or a hemangioma was made. Biopsy specimens of the liver and spleen were taken, hemo- static packs were placed, and the ventilated patient was trans- ferred by air transport the next day to our institution for fur- ther management. On arrival he was tachycardic and had a blood pressure of 108/50 mm Hg mad a central venous pressure of 4 mm Hg. Hematologic profile was similar to that at the regional hospi- tal, and blood coagulation studies and fibrinogen levels were Accepted for publication Jan. 10, 1996. Reprint requests: Dr. Stephen V. Lynch, FRACS, Department of Sur- gery, Princess Alexandra Hospital, Ipswich Road, Woolloongabba, Brisbane, Q4102, Australia. Surgery 1996;120:117-9. Copyright 9 1996 by Mosby-Year Book, Inc. 0039-6060/96/$5.00+ O 11/57/72202 normal. An ultrasonographic examination did not show a mass lesion or vascular anomalies, and selective arteriography disclosed no aneurysm or tumor blush. The patient underwent another laparotomy to remove the packs and achieve definitive control of the problem. At oper- ation the liver was tense and diffusely enlarged with a rupture on the anterior surface of the right lobe over segments V and VI. The diffusely tense liver was suggestive of an acute Budd- Chiari syndrome, but the absence of operative evidence of portal hypertension, the involvement of the caudate lobe by the same process, and the identification of normal hepatic veins by using intraoperative ultrasonography excluded thiLs diagnosis. Edema was present in the porta hepatis region, and the spleen was mildly enlarged and hard. No lymphadenopa- thy was noted within the abdomen to suggest a lymphoprofif- erative disorder. Control of hemostasis was achieved with a combination of electrocantery, argon beam coagulation, and suture ligation. Core biopsy specimens from the liver, stained with Congo red, showed amyloid deposits in a diffuse pattern, causing at- rophy and disappearance of hepatocyte cords. Areas of infarc- tion of the liver and hemorrhage were obvious. The biopsy specimens from the spleen taken earlier at the regional hos- pital showed similar amyloid infiltration. After operation a diagnosis of plasma cell dyscrasia with amyloidosis was established by finding Bence-Jones protein in the urine, an immunoglobulin G (IgG)h M band on plasma electrophoresis, and 15% plasma cells from a bone marrow as- pirate. Recovery was uneventful, with a short stay in the inten- sive care unit and discharge 3 weeks later to receive chemo- therapy in his own city. CLINICAL PROFII,E Seven cases of spontaneous rupture from amyloid disease of the liver have been reported. Available data for the six cases 46' 8, 9 in the English-language literature are presented in the Table. The five women and two men ranged between 37 to 73 years of age. Whereas most patients had symptoms and a diagnosis of amyloid disease established by liver biopsy months to year's before the acute episode of liver rupture, our patient had no prior symptoms that led to amyloid disease be- ing suspected or diagnosed before his acute presenta- tion. Herald right upper quadrant pain was the initial presenting problem in only two of the patients and was presumably due to gross hepatomegaly or liver infarc- tion. Subcapsular hematoma was found in five padent& SURGERY 117