7242 Primary sclerosing cholangitis in a patient with abdominal situs inversus.

Abstract

INTRODUCTION: We present a case of primary sclerosis Cholangitis (PSC) in a patient with abdominal situs inversus (ASI). PSC is a disease of unknown etiology which results in progressive fibrosis and obliteration of the biliary sistem. The disease typically affects middle-aged adults and has a strong association with inflamatory bowel disease. ASI is a rare congenital anomaly occurring once in every 10000 to 50000 people. In this anatomic alteration a successful canulation and ERCP is technically difficult.We have not seen so far any description of the association of ASI with PSC in the literature. Given the rarely of this entity, we find interesting report this case. Case Report: A 22-year-old white woman presents with ASI and intermitent abdominal pain, jaundice, pruritus and abnormal liver function tests: bilirubin 27 mg/dl (19.7 DB), alkaline phosphatase 1003 U/liter (normal 85 to 115), SGOT 60 IU/liter (normal 0 to 40), SGPT 40 IU/liter (normal 0 to 40). Hemoglobin 10.4 g/dl. ANA, AMA, AML, Anti- LKM, ANCA, HCV and HBV markers negatives. Abdominal ultrasound and CT scan whitin normal limits without biliary tree dilatation. Hepatic biopsy showed tipically patologic changes compatible with PSC. Normal colonoscopy. ERCP may be technically difficult in such patients, but this problem can be solved by altering the position of the patient and endoscopist. In our case the ERCP was performed in traditional position without any special problems. Cholangiographic findings were : Diffuse, multifocal strictures involving both the intrahepatic and extrahepatic ducts. Strictures were tipically short and annular, alternating with normal or minimally dilated segments to produce a characteristic beaded appearance. The serious and progressive nature of the disease in the majority of cases can be surmised from the estimated median survival of 12 years. The most effective treatment is the liver transplant when the effectivity of drug treatment fails. In this moment our patient is asymtomatic with supportive medical care (ursodeoxycholic acid).