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Correspondence

Indian Pediatr 2017;54: 690

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

 

Vikrant Sood and *Seema Alam

Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences,  Vasant Kunj, New Delhi, India.
Email: [email protected]

 


We read with interest the recent article by Redkar, et al. [1] highlighting the surgical outcomes of biliary atresia after Kasai’s Portoenterostomy (KPE). Authors reported their experience from a large retrospective cohort reconfirming the utility of jaundice clearance at 3 months post-surgery as a valid indicator of long-term outcome.

There are several points that need to be clarified. In the ‘Methods’ section, authors mentioned utilizing Hepatobiliary iminodiacetic acid (HIDA) scan, rather than liver biopsy, for diagnostic purpose. All of their patients presented with pale stools. HIDA scan itself has limited use in patients presenting with pale stools considering its low specificity (as low as 45-70%) [2-4]. It adds little to diagnostic evaluation in a cholestatic infant, and is of value only in excluding (and not in diagnosing) billary atresia by documenting patency of biliary tree [1]. On the other hand, sensitivity, specificity and diagnostic accuracy of liver biopsy for diagnosis of billary atresia exceeds 90% [1,4]. Other causes of cholestasis such as bile duct paucity and idiopathic neonatal hepatitis can also have non-excretory HIDA scan, but can be diagnosed reliably on liver biopsy avoiding unnecessary exploratory laparatomy [2].

Authors also tested for ‘TORCH’ serology in all patients with suspected biliary atresia [1]. Out of the 78 patients tested for TORCH infection, 39 had CMV IgM positive and were treated with gancicyclovir. Routinely doing ‘TORCH’ serology in these patients is of very limited use as there is still no definite link between ‘TORCH’ infections and causation of billiary atresia. Investigation for ‘TORCH’ infections and their subsequent treatment based on only serology (rather than on confirmatory liver tissue histology and polymerase chain reaction based methods) only delays the optimum management, and may even adversely affect the outcomes [5].

In the present study, 14% patients had clinical ascites on admission. This suggests an already advanced liver disease. Though there is no definite upper age limit of KPE, attempting surgery in decompensated liver disease patients is unheard of in literature and is likely associated with extremely poor outcomes. Follow-up of only one year also limits drawing of any definite conclusions from the study, as biliary atresia is a progressive fibro-inflammatory disease even post-KPE.

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. Fawaz R, Baumann U, Ekong U, Fischler B, Hadzic N, Mack CL, et al. Guideline for the  Evaluation  of  Cholestatic  Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastro-enterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2017;64:154-168.

3. Kianifar HR, Tehranian S, Shojaei P, Adinehpoor Z, Sadeghi R, Kakhki VR et al. Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: Systematic review and meta-analysis of the literature. Pediatr Radiol. 2013;43:905–19.

4. Yang JG, Ma DQ, Peng Y, Song L, Li CL. Comparison of different diagnostic methods for differentiating biliary atresia from idiopathic neonatal hepatitis. Clin Imaging. 2009;33:439–46.

5. Tarr PI, Haas JE, Christie DL. Biliary atresia, cytomegalovirus, and age at referral. Pediatrics. 1996;97:828-31.

 

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