Combined type IV A and type III choledochal cysts associated with autosomal dominant polycystic kidney disease in an adult


  • Cyriac Abby Philips Department of Hepatology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi- 110070, India
  • Chetan Ramesh Kalal Department of Hepatology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi- 110070, India
  • Amrish Sahney Department of Hepatology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi- 110070, India


Choledochal cyst, Biliary tract, Polycystic kidney disease, Cholangitis, Choledochocele


Combination of different types of choledochal cysts are very rare, seldom reported in literature. Their association with polycystic kidney disease has been never reported before. Caroli’s disease is the most commonly associated fibrocystic disease with polycystic kidney disease. Here, we present an a middle aged woman, in whom, evaluation for chronic dyspepsia  led to diagnosis of Type IVa and Type III (Choledochocele) associated with polycystic kidney disease, a combination that has never been reported before.


Komi N, Takehara H, Kunitomo K, Miyoshi Y, Yagi T. Does the type of anomalous arrangement of pancreaticobiliary ducts influence the surgery and prognosis of choledochal cyst? J Pediatr Surg. 1992;27(6):728-31.

Alonso-Lej F, Rever WB Jr, Pessagno DJ. Congenital choledochal cyst, with a report of 2, and an analysis of 94, cases. Int Abstr Surg. 1959;108(1):1-30.

Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital Bile duct cyst: Classification, operative procedures and review of 37 cases including cancer arising from choledochal cyst. Am J Surg. 1977;134(4):263-9.

Todani T, Watanabe Y, Toki A, Ogura K, Wang ZQ. Co-existing biliary anomalies and anatomical variants in choledochocal cyst. Br J Surg. 1998;85(6):760–3.

Sarris GE, Tsang D. Choledochocele: Case report, literature review, and a proposed classification. Surgery. 1989;105(3):408-14.

Kagiyama S, Okazaki K, Yamamoto Y. Anatomic variants of choledochocele and manometric measurements of pressure in the cele and the orifice zone. Am J Gastroenterol 1987;82(7):641-9.

Hateboer N, Dijk MA, Bogdanova N, Coto E, Saggar-Malik AK, San Millan JL, et al. Comparison of phenotypes of polycystic kidney disease types 1 and 2. Lancet. 1999;353(9147): 103-7.

Sharif-Naeini R, Folgering JH, Bichet D, Duprat F, Lauritzen I, Arhatte M et al. Polycystin-1 and -2 dosage regulates pressure sensing. Cell. 2009;139(3):587-96.

Babbit DP. Congenital choledochal cysts: new etiological concept based on anomalous relationships of the common bile duct and pancreatic bulb. Ann Radiol. 1969;12(3):231-40.

Ladas SD, Katsogridakis I, Tassios P, Tastemiroglou T, Vrachliotis T, Raptis SA et al. Choledochocele, an overlooked diagnosis: report of 15 cases and review of 56 published reports from 1984 to 1992. Endoscopy. 1995;27(3):233-9.

Hung-Da Tung, Chung-Huang Kuo, Chi-Sin. A Rare Combination of Choledochal Cyst Type III and IV A —a case Report. J Int Med Taiwan. 1999;12(6):19 -27.

Kimura K, Ohto M, Ono T, Tsuchiya Y, Saisho H, Kawamura K, et al. Congenital cystic dilatation of the common bile duct: relationship to anomalous pancreaticobiliary ductal union. AJR AMJ Roentgenol. 1977;128(4): 571-7.

Gupta R, Pruthi HS, Baijal SS, Saraswat VA.. An unusual choledochal cyst. J Gastroenterol Hepatol 1997;12(11): 771-4.

Kaneyama K1, Yamataka A, Kobayashi H, Lane GJ, Miyano T. Mixed type I and II choledochal cyst: a new clinical subtype? Pediatr Surg Int. 2005; 21(11):911-3.

Loke TK, Lam SH, Chan CS. Choledochal cyst: an unusual type of cystic dilatation of the cystic duct. AJR AM J Roentgenol. 1999;173(3):619-20.

Torra R, Badenas C, Darnell A, Brú C, Escorsell A, Estivill X. Autosomal dominant polycystic kidney disease with anticipation and Caroli's disease associated with a PKD1 mutation: Rapid communication. Kidney Int. 1997;52(1):33-8.

Hasegawa T, Kim M, Kitayama Y, Kitamura K, Hiranaka T. Choledochal cyst associated with polycystic kidney disease: report of a case. HPB Surg. 1999;11(3):185-9.

Kim YJ, Kim JH, Park SM. Type I choledochal cyst combined with polycystic kidney disease. Korean J Pancreatobiliary 2011;16:131-4.

Ryu SJ, Kim KH, Kim TW, Ban WH, Lee SL, Kim SS, Kim YO. Autosomal Dominant Polycystic Kidney Disease with an Asymptomatic Type I Choledochal Cyst. Korean J Med. 2013;85(2):205-9.

Law R, Topazian M. Diagnosis and treatment of choledochoceles. Clin Gastroenterol Hepatol. 2014;12(2):196-203.

Lal R, Agarwal S, Shivhare R, Kumar A, Sikora S S, Saxena R, Kapoor VK. Type IV-A choledochal cysts: a challenge. J Hepatobiliary Pancreat Surg.2005;12(2):129-34.






Case Reports