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Correspondence

Indian Pediatr 2017;54: 690-691

Outcome of Biliary Atresia After Kasai’s Portoenterostomy: Few Concerns: Authors Reply

 

Rajeev Gurunath Redkar

Department of Pediatric Surgery, Lilawati Hospital, Bandra (West) Mumbai, Maharashtra, India.
Email: [email protected]

  


The points raised by these readers include utilization of liver biopsy rather than HIDA scan for diagnosis of biliary atresia. We would like to clarify that as mentioned in our methods, all patients underwent clinical examination, stool color examination, liver function tests and ultrasonography of abdomen. While TORCH serology and a HIDA scan were done in most patients [1], we have relied on intra-operative cholangiogram as the diagnostic test for BA, which is still considered the gold-standard for its diagnosis [2]. In addition, the interpretation of a biopsy can be difficult and needs an experienced pathologist as there is a lot of overlap in the histological findings of biliary atresia and neonatal hepatitis [3]. Infact, lack of expertise in histopathology has also refrained us from incorporating histological findings in our study report. We have accepted that as one of the limitations in our study [1].

The reviewer also pointed on testing for TORCH serology and treatment for the same. As mentioned above, TORCH serology was done in most (n=78/121) patients as a part of workup for neonatal cholestasis. Ganciclovir was started after a CMV-PCR confirmation (not mentioned in the article), without alteration or delay in the usual workup and management of biliary atresia. CMV positive biliary atresia patients have poorer outcome with reduced jaundice clearance, native liver survival and increased mortality [4]. We usually treat patients of BA associated with CMV with ganciclovir.

In our study, although 14% of patients presented with clinical ascites, most of them (n= 11/14, 78.6%) had normal or borderline albumin levels with normal bleeding parameters. Out of these, four patients had jaundice clearance, and were alive at 1 year. This cannot be considered as a poor outcome, especially considering the non-feasibility of primary liver transplantation in our country. Factors like scarcity of organs and centers offering pediatric liver transplant programs, financial constraints and need for lifelong immunosuppression, make Kasai’s Portoenterostomy as the initial procedure of choice, even for patients with late presentations [1,5].

References

1. Redkar R, Karkera PJ, Raj V, Bangar A, Hathiramani V, Krishnan J. Outcome of biliary atresia after Kasai’s portoenterostomy: A 15-year experience. Indian Pediatr. 2017;54:291-4.  

2. Wildhaber BE. Biliary Atresia: 50 Years after the First Kasai. ISRN Surgery. 2012;2012:132089. doi:10.5402/2012/132089.

3. Sinha CK, Davenport M. Biliary atresia . J Indian Assoc Pediatr Surg 2008; 13:49-56.

4. Zani A, Quaglia A, Hadziæ N, Zuckerman M, Davenport M. Cytomegalovirus-associated biliary atresia: An aetiological and prognostic subgroup. J Pediatr Surg. 2015;50:1739-45.

5. Govindrajan KK. Biliary Atresia: current trends in outcome and management. Indian Pediatr. 2017;54:277.


 

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