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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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 CASE REPORT
Year : 2023  |  Volume : 28  |  Issue : 6  |  Page : 520-522

Bardet–Biedl syndrome with choledochal cyst: Rare association with a novel variant


1 Department of Surgical Gastroenterology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
2 Department of Anesthesiology, BIG Apollo Spectra Hospitals, Agamkuan, Patna, Bihar, India
3 Department of Medical Genetics, Post Graduate Institute of Child Health, Noida, Uttar Pradesh, India

Correspondence Address:
Saket Kumar
Department of Surgical Gastroenterology, Indira Gandhi Institute of Medical Sciences, Sheikhpura, Patna - 800 014, Bihar
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_124_23

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Bardet–Biedl syndrome is an autosomal-recessive ciliopathic disorder affecting multiple organ systems. Characteristic features include progressive retinal dystrophy, obesity, polydactyly hypogonadism, mental retardation, and renal disorders. Other manifestations include congenital heart diseases, hepatic fibrosis, ataxia, and diabetes. Approximately 30% of patients with Biedl–Bardet syndrome (BBS) have hepatobiliary disorders such as periportal fibrosis, nonalcoholic fatty liver disease, and cystic dilation of the bile ducts. The association of BBS with choledochal cysts (CDC) is extremely rare. Here, we report a case of a 14-year-old boy with a novel variant of BBS and associated type IV CDC. The patient was managed surgically with CDC excision and Roux-en-Y hepaticojejunostomy.






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