Extrahepatic biliary atresia in Saudi Arabia: the importance of early diagnosis and referral.

OBJECTIVE: A retrospective analysis of the medical records of children who were managed for extrahepatic biliary atresia (EHBA) over a 6-year period. MATERIALS AND METHODS: Twelve children were managed at King Fahad National Guard Hospital with a diagnosis of extrahepatic biliary atresia from January 1993 through December 1998. The data obtained included age, sex, clinical presentation, age at referral, investigations, management, complications and follow-up. RESULTS: There were 7 males and 5 females with age of onset of jaundice ranging from 3 days to 42 days (mean 10 days). The mean age at referral was 17 weeks (range 6-49 weeks). Kasai Portoenterostomy (PE) was performed in 7 infants and the mean age at surgery was 8 weeks (range 7 to 10.5 weeks). Four children could not undergo Kasai PE because they were more than 20 weeks old and 1 child although 10 weeks old had extensive liver cirrhosis and was considered not suitable for the procedure. One child who had primary liver transplant outside the Kingdom and 4 children who had Kasai PE are still alive. Seven children are lost to follow-up and are probably dead. CONCLUSION: The incidence of EHBA in Saudi Arabia is unknown and the disease is probably rare based on our review. Although jaundice, pale stools and dark urine were observed in early infancy, referral was always late. Public and health professional's awareness of the clinical features of EHBA is important in early identification of infants and early referral. Starting paediatric hepatobiliary centers in Saudi Arabia will improve the management of these cases.

Turcot's syndrome in an Arab child.

A 12 year old female child of Arab origin presented with chronic bloody diarrhoea and growth failure. Physical examination confirmed a growth failure and cafe-au-lait patches. Colonoscopy and colon biopsies showed adenocarcinoma of the colon and laparotomy confirmed metastases to the liver. A diagnosis of Turcot's Syndrome was entertained in this child.