Cholangiocarcinoma in Nepal

ERCP showing total obstruction of the common hepatic duct by cholangiocarcinoma (red arrow).

A 56-year-old diabetic, chronic smoker and alcoholic male was admitted with a history of more than a month of progressive jaundice and right hypochondrium discomfort with loss of appetite and weight. There was no significant past and family history. On examination he had deep jaundice, a tender right hypochondrium, a palpable, smooth, liver edge four centimeters below the costal margin, and ascites.

Liver function tests were abnormal, with elevated bilirubin (total 15.5 mg/dl, direct 9.5mg/dl), alkaline phosphatase 6670 U/L, SGPT 320 U/L and SGOT 62 UL. Ultrasonography was inconclusive but a CT scan of the abdomen revealed hepatomegaly, dilated common hepatic and common bile ducts with an irregular area pf low attenuation and the presence of ascites. Endoscopic retrograde cholangiopancreatography (ERCP) revealed irregular narrowing of the proximal part of the common bile duct suspicious of cholangiocarcinoma with secondary spread to the porta hepatis. A sample sent to Cytology but was negative.

Laparotomy, performed on July 11, 2006, revealed a mass in the middle third of the common bile duct (3×5 cm) which was inoperable. A T-tube was placed proximal to the mass. Unfortunately, the patient expired on July 16, 2006.

Discussion
Cholangiocarcinoma is a rare disease in Nepal. Parasitic infections, (Opisthorchis sinensis and viverrini, types of liver fluke), a recognized cause of cholangiocarcinoma, do not occur - the eating of raw fish, the usual source of infection - is not customary. Primary sclerosing cholangitis, ulcerative colitis and choledochol cyst, also predisposing factors, are very rare entities in Nepal. It would appear that the most prevalent etiological factor is chronic infection of the biliary tract duct, although congenital biliary-pancreatic abnormalities cannot be excluded. In spite of its rarity, most cases of cholangiocarcinoma in Nepal can be diagnosed because of the availability of newer diagnostic modalities such as CT scan and ERCP. Indeed, these imaging tools have probably led to more cases being recognized. The main difference in the presenting features of the disease from reports in the published literature is that patients in Nepal present very late. Jaundice is a common presenting symptom, but in Nepal, this is almost always assumed to be due to hepatitis – which, is, in general, correct. However this means that no further action is taken; patients are sent home to recover or they seek help from traditional healers, known as vaidyas (ayurvedic medicine practitioners) or even from faith healers.

If the patient’s condition does not improve he will usually go to a medical assistant or non-specialist doctor with, again, limited, if any intervention. Finally, as the disease worsens – usually after a month or two has passed, patients are taken to tertiary level hospitals. By now, they will usually have developed ascites. Blood tests and ultrasonography, which are within the financial reach of everybody, are not sufficient to diagnose cholangiocarcinoma. Most ordinaryNepalis cannot afford the necessary diagnostic tests such as CT scan and ERCP. Tests for tumor markers such as carcinoembryonic antigen or carbohydrate antigen 19-9l, are not available in Nepal and it would be both expensive and difficult to arrange for such testing in other countries.

Most cases are palliated by percutaneous drainage of the bile duct (PTBD); a few are treated by internal stenting. Patients in whom surgery is performed are usually treated by biliary bypass or the insertion of a T tube into the common bile duct for external drainage. The mortality of the disease is very high and most patients die within six months.

Manohar Lal Shrestha
Nepal Medical College and Teaching Hospital Atterkhel
Kathmandu, Nepal

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