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Indian Pediatr 2017;54: 690 |
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Outcome of Biliary Atresia After Kasai’s
Portoenterostomy: Few Concerns
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Vikrant Sood and *Seema
Alam
Department of Pediatric Hepatology, Institute of Liver
and Biliary Sciences, Vasant Kunj, New Delhi, India.
Email: [email protected]
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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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