Naresh P Shanmugam and V Jayanthi

We read with interest "Evolving biliary atresia with cytomegalovirus" in the August issue [1], as the etiology and pathogenesis of biliary atresia (BA) still remains as an enigma. Instead of answers, we are left with several questions in our mind. It was not clear whether the author is implementing CMV as a cause for biliary atresia in these two cases or as a red herring? Though, there are several speculations about CMV causing biliary atresia, so far there is no hard evidence implementing the virus as a causative agent [2,3]. In the first case early liver decompensation and low gamma-glutamyl transferase (GGT of 10 IU/L) in the presence of severe cholestasis raise the suspicion of an underlying metabolic problem such as Progressive familial intrahepatic cholestasis (PFIC). A combination of PFIC and biliary atresia is unusual, but worthwhile considering as a possibility.

The most important question in both the cases is about timing of portoenterostomy. Surgery was undertaken when the children were more than 6 months old. Chardot, et al. showed that, the success of portoenterostomy is practically nil after 141 days [4]. In the absence of liver transplantation facility surgery one could argue for late surgery, as it might work in the absence of severe cirrhosis with stable synthetic liver function and give patients some survival advantage. The author could put forward the above mentioned argument for the second case, but for the first case, we are intrigued by the fact that the child underwent portoenterostomy when he/she was in frank liver failure, where Kasai’s procedure is contraindicated [5].

1. Mohanty S, Shah I, Bhatnagar S. Evoloving biliary atresia with cytomegalovirus. Indian Pediatr. 2011;48:644-6.

2. Rauschenfels S, Krassmann M, Al-Masri AN, Verhagen W, Leonhardt J, Kuebler JF, et al. Incidence of hepatotropic viruses in biliary atresia. Eur J Pediatr. 2009;4:469-76.

3. Hartley JL, Davenport M, Kelly DA. Biliary atresia. Lancet. 2009;14:1704-13.

4. Chardot C, Carton M, Spire-Bendelac N, Le Pommelet C, Golmard J, Reding R, et al. Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia? J Pediatr. 2001;2:224-8.

5. Engelmann G, Schmidt J, Oh J, Lenhartz H, Wenning D, Teufel U, et al. Indications for pediatric liver transplantation. Data from the Heidelberg pediatric liver transplantation program. Nephrol Dial Transplant. 2007;22:viii23-8.