Hemobilia in Hereditary Hemorrhagic Telangiectasia: An Unusual Complication of Endoscopic Retrograde Cholangiopancreatography

Hepatobiliary manifestations of hereditary hemorrhagic telangiectasia (HHT) are rare, but often involve cholestasis. We report here a case of HHT associated with cholestasis due to common bile duct stenosis. Attempted balloon dilation of the stenosis during endoscopic retrograde cholangiopancreatography (ERCP) resulted in hemobilia. Hemostasis was achieved by adjusting the nasobiliary drain. The aim of this report is to highlight the biliary manifestations of HHT and draw attention to an unusual complication of ERCP in this setting.

References

• 1 Plauchu H, de Chadarevian J P, Bideau A, Robert J M. Age-related clinical profile of hereditary hemorrhagic telangiectasia in an epidemiologically recruited population.  Am J Med Genet. 1989;  32 291-297
• 2 Reilly P J, Nostrant T T. Clinical manifestation of hereditary hemorrhagic telangiectasia.  Am J Gastroenterol. 1984;  79 363-367
• 3 Trell E, Johansson B W, Linell F, Ripa J. Familial pulmonary hypertension and multiple abnormalities of large systemic arteries in Osler’s disease.  Am J Med. 1972;  53 50-63
• 4 Wanless I R, Gryfe A. Nodular transformation of the liver in hereditary hemorrhagic telangiectasia.  Arch Pathol Lab Med. 1986;  110 331-335
• 5 Garcia-Tsao G, Korzenik J, Young L. et al . Liver disease in patients with hereditary hemorrhagic telangiectasia.  N Engl J Med. 2000;  343 931-936
• 6 Cooney T, Sweeney E C, Coll R, Greally M. “Pseudocirrhosis” in hereditary hemorrhagic telangiectasia.  J Clin Pathol. 1977;  30 1134-1141
• 7 Wanless I R. Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2 500 autopsies and a new classification of benign hepatocellular nodules.  Hepatology. 1990;  11 787-797
• 8 Shimamatsu K, Wanless I R. Role of ischemia in causing apoptosis, atrophy, and nodular hyperplasia in human liver.  Hepatology. 1997;  26 343-350
• 9 Bernard G, Mion F, Henry L. et al . Hepatic involvement in hereditary hemorrhagic telangiectasia: clinical, radiological, and hemodynamic studies of 11 cases.  Gastroenterology. 1993;  105 482-487