Sanghai SR, Shah I, Bhatnagar S, Murthy A

Language: English
References: 14
Page: 120-122
PDF size: 38.97 Kb.

Text Extraction

Aim: To estimate the incidence of Biliary Atresia(BA) amongst Neonatal Cholestatic Syndromes (NCS) and determine prognostic factors in BA patients who have undergone Kasai’s portoenterostomy. Study design- Retrospective analysis. Setting- Pediatric Hepatobiliary Clinic at B.J. Wadia Children’s Hospital, Mumbai. Methods and materials: 32 patients diagnosed with BA referred to the clinic from May 2005 to July 2007 were included in the study. All patients underwent a detailed history, clinical examination and were tested for Liver function tests (LFT), USG abdomen, Liver biopsy, intra-operative cholangiogram and CMV tests. Patients were followed up for a period of 1 month to 7 years post operatively and complications such as cholangitis, progress to liver cell failure and cirrhosis was noted. Results: Incidence of BA amongst NCS (n = 88) was 36.4%. 8 patients of BA (25%) were lost to follow up. Out of the remaining, 10 (41.7%) improved and 14 (58.3%) did not improve. The mean age of presentation was 89 ± 55.8days. 1 patient (25%) out of 4 with bile duct size of ‹ 100 microns showed an improvement whereas 3 (37.5%) out 8 patients with bile duct size 100-200 microns showed improvement and 4 (50%) with bile duct size of › 200 microns had improvement post Kasai surgery. Those with bile duct sizes › 200 microns had better prognosis than those with sizes 100-200 microns (Odd’s ratio = 1.8) and ‹ 100 microns (Odd’s ratio = 3). 12 patients (50%) were operated before 3 months of age and 50% of them responded to surgery. The remaining 12 patients were operated after 3 months of age and only 33% showed any improvement. (Odd’s ratio = 2). Other parameters like SGOT (P = 0.598), SGPT (p = 0.901), total Bilirubin (p = 0.349), Direct Bilirubin (p = 0.429), Alkaline Phosphatase (p = 0.605) and GGTP (p = 0.480), cirrhosis (p = 0.417), degree of fibrosis (p = 0.384), degree of inflammation (p = 0.964) and Cholangitis (P = 0.388) had no effect on the outcome. Conclusion: Biliary Atresia is a common cause of NCS in India. Children with Bile duct size › 200 microns have a good prognosis. Portoenterostomy before 3 months of age has a better outcome.

REFERENCES

1. Balistreri WF, Grand R, Hoofnagle JH, et al. Biliary atresia: current concepts and research directions. Summary of a symposium. Hepatology 1996; 23: 1682–92.

2. Narsimhan KL, et al. Outcome of biliary atresia from Chandigarh: Results of a prospective analysis. Indian Pediatrics 2001; 38: 1144-1148.

3. Bittmann S. Surgical experience in children with biliary atresia treated with portoenterostomy. Curr Surg 2005; 62(4): 439-43.

4. Chittmittrapap S, Chandrakamol B, Poorvorawan Y, Suwangool P. Factors influencing outcome after hepatic portoenterostomy for biliary atresia: a logistic regression analysis. J Med Assoc Thai 2005; 88(8): 1077-82.

5. Miyano T, Kobayashi H, Chen SC. Long term results of biliary atresia: Current point of view for research concerning prognostic factors for biliary atresia. J Indian Assoc Pediatr Surgery 1999; 4: 58-64.

6. Chang MH, Huang HH, Huang ES, et al. Polymerase chain reaction to detect human cytomegalovirus in livers of infants with neonatal hepatitis. Gastroenterology 1992; 103(3): 1022-5.

7. Tan CE, Davenport M, Driver M, Howard ER. Does the morphology of the extrahepatic biliary remnants in biliary atresia influence survival? A review of 205 cases. J Pediatr Surg 1994; 29(11): 1459-64.

8. Hung PY, et al. Long term prognosis of patients with biliary atresia: a 25 year summary. J Pediatric Gastroenterl Nutr 2006; 42(2): 190-5.

9. Langeburg SE, Poulik J, Gortesky M, Klein AA, Klein MD. Bile duct size does not predict success of portoenterostomy for biliary atresia. J Pediatric Surgery, 2000; 35(6): 1006-7.

10. Karrer FM, Lilly JR, Stewart BA, Hall RJ. Biliary atresia registry, 1976 to 1989. J Pediatr Surg 1990; 25(10): 1076-80.

11. McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 2000; 355: 25-29.

12. Tarr PI, Haas JE, Christie DL. Biliary atresia, cytomegalovirus, and age at referral. Pediatrics 1996; 97(6 Pt 1):828-31.

13. Bhatnagar V, Singh MK, Mitra DK. Bile-duct diameter at the porta hepatis and liver biopsies in Indian children with biliary atresia. Pediatr Surg Int 1994; 9: 558-560.

14. Gautier M, Jehan P, Odievre M. Histologic study of biliary fibrous remnants in 48 cases of extrahepatic biliary atresia: correlation with postoperative bile flow restoration. J Pediatr 1976; 89: 704-709.