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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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ORIGINAL ARTICLE
Year : 2005  |  Volume : 10  |  Issue : 1  |  Page : 48-49
 

Choledochal cyst associated with extrahepatic bile duct atresia


1 Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi - 110029, India
2 Department of Radiology, All India Institute of Medical Sciences, New Delhi - 110029, India

Correspondence Address:
V Bhatnagar
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi - 110029
India
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DOI: 10.4103/0971-9261.16077

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   Abstract 

Three infants who presented with clinical features of neonatal cholestasis and clinically suspected to have biliary atresia were found on imaging studies and surgery to have extrahepatic bile duct atresia in association with choledochal cyst. All patients were treated by bilio-enteric bypass procedure. Post operatively jaundice cleared in only two patients. The prognosis of this association depends on the pre-existent liver damage due to biliary atresia.


Keywords: Biliary atresia, Choledochal cyst


How to cite this article:
Kumar A, Gupta A K, Bhatnagar V. Choledochal cyst associated with extrahepatic bile duct atresia . J Indian Assoc Pediatr Surg 2005;10:48-9

How to cite this URL:
Kumar A, Gupta A K, Bhatnagar V. Choledochal cyst associated with extrahepatic bile duct atresia . J Indian Assoc Pediatr Surg [serial online] 2005 [cited 2014 Oct 31];10:48-9. Available from: http://www.jiaps.com/text.asp?2005/10/1/48/16077


Choledochal cyst is localized dilatation of the extrahepatic and/or intrahepatic biliary tree while biliary atresia is a type of obstructive cholangiopathy in which there is obliteration/sclerosis of the extrahepatic bile ducts. Choledochal cyst is a rare congenital anomaly with a reported incidence of 1 in 100,000-150,000 live births.[1] The incidence of biliary atresia is about 1 in 10,000-12,000 live births.[2] Hence, choledochal cyst and extrahepatic biliary atresia are individually rare congenital malformations and their association in a single case is still rarer. Very few cases have been reported in the literature.3],4]


   Materials and Methods Top


We report three infants, aged 2, 2.5 and 3 months, with association of choledochal cyst and extrahepatic biliary atresia who were treated at our institute. The clinical presentation was similar to that of biliary atresia in all the three infants - clay colored stool, high colored urine and icterus since birth. Imaging evaluation was done with ultrasonography (USG), magnetic resonance cholangio-pancreatography (MRCP), 99mTc iminodiacetic acid hepatic scan (HIDA) and peroperative cholangiography (POC). Biopsy of the liver was taken in all the three cases.


   Results Top


Dilatation of the extrahepatic bile ducts was evident on USG and MRCP preoperatively and HIDA scan did not show gut activity in all the three cases [Figure - 1] and [Figure - 2]. POC failed to demonstrate patency of the distal common bile duct while liver biopsy showed classical features of biliary atresia in each case i.e. cholestasis, lobular disarray, bile duct proliferation, portal tract inflammation and extensive fibrosis bordering on cirrhosis. All the three infants were treated surgically with bilio-enteric bypass procedures. Three months post operatively jaundice cleared in only two cases.


   Comments Top


Biliary atresia is a type of obstructive cholangiopathy in which there is obliteration/sclerosis of the extrahepatic ducts. Pathologically, the liver shows periportal inflammation and fibrosis, bile-duct proliferation, with variable absence of the extrahepatic bile ducts. Choledochal cyst is a localized dilatation of the common bile duct with obstruction to flow of bile into the duodenum and occasionally hepatocellular damage. The association of biliary atresia and choledochal cyst is extremely rare with clinical presentation mimicking that of biliary atresia. Diagnosis can be suspected on preoperative imaging and investigations. Liver histology is that of biliary atresia. Prognosis after surgical treatment is poor because of the pre-existing hepatic damage.

 
   References Top

1.Howard ER. Choledochal cysts. Surgery of liver disese in children. Howard ER, editor. Oxford: Butterworth-Heineman; 1991. p. 78-90.  Back to cited text no. 1    
2.Ohi R, Nio M. The jaundiced infant: Biliary atresia and other obstructions. Pediatric Surgery, 5th Ed. Mosby 1998. p. 1465-81.  Back to cited text no. 2    
3.Lam AH, Lam VK. Choledochal cyst with biliary atresia in an infant. Australas Radiol 1987;31:384-5.  Back to cited text no. 3  [PUBMED]  
4.Landing BH. Considerations of pathogenesis of neonatal hepatitis, biliary atresia and choledochal cyst - the concept of infantile obstructive cholangiopathy. Progr Pediatr Surg 1974;6:133-9.  Back to cited text no. 4    


Figures

[Figure - 1], [Figure - 2]


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