Semin Liver Dis 2000; Volume 20(Number 03): 391-396
DOI: 10.1055/s-2000-9392
Copyright © 2000 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

DIAGNOSTIC PROBLEMS IN HEPATOLOGY

Portal Hypertension and Hepatopulmonary Syndrome in a Middle-Aged Man with Hepatitis B InfectionM. ISABEL. FIEL, THOMAS. D. SCHIANO1,2 , ARIEF. SURIAWINATA, SUKRU. EMRE2
  • 1Lillian and Henry M. Stratton-Hans Popper Department of Pathology; Division of Liver Diseases, Department of Medicine Mount Sinai School of Medicine of the City University of New York, New York
  • 2Recanati-Miller Transplant Institute, Mount Sinai School of Medicine of the City University of New York, New York
Further Information

Publication History

Publication Date:
31 December 2000 (online)

CASE REPORT

A 55-year-old Turkish man was evaluated for orthotopic liver transplantation (OLT) because of advanced liver disease due to chronic hepatitis B viral infection (HBV), which he contracted in Turkey 35 years previously. Chest X-ray and echocardiogram were unremarkable at the time.

More recently, the patient suffered from incapacitating fatigue, generalized weakness, memory loss, pedal edema, and worsening shortness of breath. He was hospitalized several times due to hepatic encephalopathy. Physical examination demonstrated 4+ clubbing of the fingers, splenomegaly, peripheral edema, asterixis, and psychomotor retardation. He had proximal muscle wasting, but there was no jaundice or ascites. There was orthodeoxia and platypnea.

Laboratory testing demonstrated a total serum bilirubin of 2.8 mg/dL (nl = 1-1.5), prothrombin time of 16.2 seconds (nl = 10.3-13), and a serum albumin level of 2.6 g/dL (nl = 3.3-5.3). Four months earlier, the prothrombin time was 15.7 seconds and the albumin was 3.3 g/L. The alkaline phosphatase level was 104 U/L (nl = 32-115), ALT 32 U/L (nl = 2-50), AST 50 U/L (nl = 2-40). Serologic testing showed reactivity for HBsAg. Anti-HBe, anti-HBs, HBeAg, HBV-DNA, anti-HDV, and anti-HCV were all non-reactive. Autoantibodies were not detectable and serum iron indices were normal.

Chest x-ray showed a fine nodular reticular pattern in the lungs. Pulmonary function testing demonstrated a moderate restrictive defect with a diffusion capacity of 57%. Kveim test was inconclusive. Doppler echocardiogram showed left ventricular hypertrophy and, when agitated saline was infused, a right to left shunt was demonstrated consistent with pulmonary arteriovenous malformation and hepatopulmonary syndrome. CT scan of the chest demonstrated cardiomegaly and enlargement of both hila. The azygous vein was prominent with no lymphadenopathy noted. CT scan of the abdomen showed a small liver, estimated to be 700 mL in size with diffuse heterogenous echogenicity, and splenomegaly.

The clinical diagnosis was Child's C cirrhosis related to chronic hepatitis B, complicated by hepatopulmonary syndrome. The pulmonary disease was considered to be reversible with liver transplantation. Orthotopic liver transplantation was performed.

REFERENCES

  • 1 Mikkelsen W P, Edmondson H A, Peters R L. Extra- and intrahepatic portal hypertension without cirrhosis (hepatoportal sclerosis).  Ann Surg . 1965;  162 602-620
  • 2 Aikat B K, Bhusnumath S K, Chuttani P N. The pathology of noncirrhotic portal fibrosis.  Hum Pathol . 1979;  10 405-418
  • 3 Vakili C, Farahvash M J, Bynum T E. ``Endemic'' idiopathic portal hypertension: Report on 32 patients with noncirrhotic portal fibrosis.  World J Surg . 1992;  16 118-125
  • 4 Boyer J L, Hales M R, Klatskin G. ``Idiopathic'' portal hypertension due to occlusion of intrahepatic portal veins by organized thrombi.  Medicine . 1974;  53 77-91
  • 5 Nayak N C, Ramalingaswami V. Obliterative portal venopathy of the liver.  Arch Pathol . 1969;  87 359-369
  • 6 Okuda K, Obata H. Idiopathic portal hypertension (hepatoportal sclerosis). In: Okuda K, Benhamou JP, eds. Portal Hypertension: Clinical and Physiological Aspects Tokyo: Springer-Verlag 1991: 271-287
  • 7 Nakanuma Y, Ohta G, Kurumaya H. Pathological study on livers with noncirrhotic portal hypertension and portal venous thromboembolic occlusion: Report of seven autopsy cases.  Am J Gastroenterol . 1984;  79 782-789
  • 8 Ludwig J, Hashimoto E, Obata H, Baldus W P. Idiopathic portal hypertension: A histopathological study of 26 Japanese cases.  Histopathology . 1993;  22 227-234
  • 9 Nakanuma Y, Hoso M, Sasaki M. Histopathology of the liver in noncirrhotic portal hypertension of unknown aetiology.  Histopathology . 1996;  28 195-204
  • 10 Sciot R, Staessen D, Van Damme B, et a l. Incomplete septal cirrhosis: Histopathological aspects.  Histopathology . 1988;  13 593-603
  • 11 Wanless I R, Godwin T A, Allen F, Feder A. Nodular regenerative hyperplasia of the liver in hematologic disorders: A possible response to obliterative portal venopathy.  Medicine . 1980;  59 367-379
  • 12 Bioulac-Sage P, Le Bail B, Bemard P-H, Balabaud C. Hepatoportal sclerosis.  Semin Liv Dis . 1995;  15 329-339
  • 13 Oikawa K, Masuda T, Sato S-I. Changes in lymph vessels and portal veins in the portal tract of patients with idiopathic portal hypertension: A morphometric study.  Hepatology . 1998;  27 1607-1610
  • 14 Ohashi K, Kojima K, Fukazawa M. Long-term prognosis of non-shunt operation for idiopathic portal hypertension.  J Gastroenterol . 1998;  33 241-246
  • 15 Kilpe V E, Krakauer F L, Wren R E. An analysis of liver transplant experience from 37 transplant centers as reported to Medicare.  Transplantation . 1993;  56 554-561
  • 16 Loinaz C, Colina F, Musella M. Orthotopic liver transplantation in 4 patients with portal hypertension and non-cirrhotic nodular liver.  Hepato-Gastroenterology . 1998;  45 1787-1794
  • 17 Elariny H A, Mizrahi S S, Hayes D H. Nodular regenerative hyperplasia: A controversial indication for orthotopic liver transplantation.  Transplant Int . 1994;  7 309-313
  • 18 Bernard P-H, Le Bail B, Cransac M. Progression from idiopathic portal hypertension to incomplete septal cirrhosis with liver failure requiring liver transplantation.  J Hepatol . 1995;  22 495-499
  • 19 Fiel M I, Schiano T D, Bodenheimer Jr HC, Miller C M, Thung S N. Hepatoportal sclerosis incidentally found after liver transplantation.  Hepatology . 1997;  26 506A
  • 20 Sugimura T, Tsuji Y, Ibayashi H. Portal-systemic shunting in a patient with normal portal vein pressures and histological evidence of idiopathic portal hypertension.  J Gastroenterol Hepatol . 1996;  11 301-304
  • 21 Terano A, Honda Z, Shiga J. Nodular regenerative hyperplasia of the liver diagnosed by needle biopsy of the liver: Report of a case and review of the literature.  J Gastroenterol Hepatol . 1998;  3 657-661
  • 22 Thung S N, Gerber M A. Differential Diagnosis in Pathology: Liver Disorders.  Tokyo: Igaku-Shoin; 1995
  • 23 Desmet V. What is congenital hepatic fibrosis?.  Histopathology . 1992;  20 465-477
  • 24 Krowka M J, Dickson E R, Cortese D A. Hepatopulmonary syndrome: Clinical observations and lack of therapeutic response to somatostatin analogue.  Chest . 1993;  104 515-521
  • 25 Babbs C, Warnes T W, Haboub N Y. Noncirrhotic portal hypertension with hypoxaemia.  Gut . 1998;  29 129-131