De Simone Belinda1**, Del Rio Paolo2, Napoli Josephine Andrea3 and Sianesi Mario2
1Department of Emergency and General Surgery, Tourcoing’s Hospital, Tourcoing, France
2Dpt of General Surgery and Organ Trasplantation, Parma University Hospital, Parma, Italy
3Department of Medical Sciences, University of Hawaii, Hawaii, United States of America
Received: 22 April, 2016; Accepted: 06 June, 2016; Published: 08 June, 2016
Dr. De Simone Belinda, Department of Emergency and General Surgery, Surgical Sciences, Tourcoing’ Hospital, Tourcoing, France, E-mail:
de Simone B, del Rio P, Napoli AJ, Sianesi M (2016) Choledochal Cyst in a Female Adult Asymptomatic Patient: Review of the Literature. Global J Med Clin Case Reports 3(1): 008-011. DOI: 10.17352/2455-5282.000024
© 2016 de Simone B, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Choledochal cyst; Todani’s Classification; Cholangiocarcinoma; Roux en Y hepatico-jejunostomy
Cystic disease of the bile duct is a congenital abnormality of the biliary tree that most commonly affects the neonatal population, and is uncommon after childhood, it is rarely diagnosed in adults. We report the case of a 42-year-old woman affected by choledochal cyst type I, according to Todani’s classification, found unintentionally. The patient was submitted to the complete surgical excision of choledochal cyst, and discharged on the 11th postoperative day without complications.
Cystic disease of the bile duct (CDBD) is a congenital abnormality of the biliary tree that occurs most commonly in the neonatal population (1 / 150000 live births in North America) , with a high prevalence in Asian countries, and increased female prevalence relative to males (F : M = 3:1) ; it occurs very rarely in adults in Europe.
CDBD is characterized by a cystic dilatation of intra- and/or extra- hepatic bile ducts, with a multifactorial etiology: an anatomic abnormality of the biliary-pancreatic junction (Babbit’s “common channel” theory, 1969) , allows the reflux of the pancreatic juice into the biliary tree, which is associated with a functional obstruction (biliary stasis and lithiasis) to the normal outflow of bile, which leads to chronic inflammation of the epithelial lining of the bile ducts. Chronic inflammation results in a cystic dilatation of the wall of the ducts, and is associated with an increased risk of developing cholangiocarcinoma in youth [2,3]. The risk of developing cholangiocarcinoma in young patients affected is increased 20 -30 times compared to in the general population .
The only curative treatment of CDBD is surgery, considering the significant association between the disease and the development of cholangiocarcinoma.
We report the case of a 42-year-old female patient, affected by a type I choledochal cyst, according to Todani’s classification (Tdc).
A 42-year-old woman was admitted in our Operative Unit of General Surgery and Organ Transplantation because of an incidental radiological diagnosis of choledochal cysts type I, according to Tdc. At the admission, she presented with nausea and unspecified gastritis. The patient was affected by Factor VII Deficiency; she had no family history of neoplastic diseases, cardiovascular and pulmonary diseases; she was not diabetic. The patient was not a smoker, she did not take drugs, and she never presented allergic reactions to medications or foods; she reported episodes of tachycardia and palpitations without any pathological significance in accordance with the cardiological evaluation.
At physical examination, the patient did not present jaundice or abdominal pain; no abdominal masses were palpable. The patient had laboratory blood tests that revealed a Protrombin. Time ratio of 1.16, and International National Ratio (INR) of 1.15; neoplastic markers (CEA, Ca 19-9) were normal.
The preoperative anesthetist evaluation has been assigned an American Society of Anesthesiologists (ASA) score 2.
She received the diagnosis of choledochal cyst type I when, because of urinary disorders, she had abdominal ultrasound evaluation that showed her gallbladder was not completely extended, with contracted walls. The gallbladder contained microlithiasis and gallstones of 6mm in diameter; no dilatation of intrahepatic biliary ducts was present; and, a 79x21 mm type 1 choledochal cyst, according to Tdc, was detected.
After this primary diagnosis, she was underwent an abdominal CT, which showed a fluid formation of about 50mm in diameter, extending for about 70mm along the choledochus, morphologically compatible with type I b choledochal cyst. CT revealed no gallstones within the common bile duct and no thickening wall of the common bile duct; the pancreas had regular morphology, and no dilatation of the Wirsung duct. A 60x40mm cyst was detected on the right ovary.
The MRCP confirmed the presence of a cyst of the bile duct, classified type I, sized 60x42mm; the choledochus was dilatated, without endoluminal gallstones; there was no expansion of the intrahepatic ducts; gallbladder sludge was present and microlithiasis. The main pancreatic duct was regular and no focal pancreatic lesions were detected (Figure 1).
- Wang QG, Zhang ST (2009) A rare case of bile duct cyst. World J Gastroenterol 15: 2550-2551 .
- Mabrut JY, Bozio G, Hubert C, Gigot JF (2010) Management of congenital bile duct cysts. Dig Surg 27: 12-18.
- Liu CL, Fan ST, Lo CM, Lam CM, Poon RT, et al. (2002) Choledochal cysts in adults. Arch Surg 137: 465-468 .
- Ziegler KM, Pitt HA, Zyromski NJ, Chauhan A, Sherman S, et al. (2010) Choledochoceles: are they choledochal cysts? Ann Surg 252: 683-690 .
- Gouda BP, Desai DC, Abraham P, Joshi A, Shah SR (2010) Choledochal cysts with chronic pancreatitis in adults: report of two cases with a review of the literature. JOP 11: 373-376 .
- Huang CS, Huang CC, Chen DF (2010) Choledochal cysts: differences between pediatric and adult patients.J Gastrointest Surg 14: 1105-1110 .
- She WH, Chung HY, Lan LC, Wong KK, Saing H, et al. (2009) Management of choledochal cyst: 30 years of experience and results in a single center. J Pediatr Surg 44: 2307-2311 .
- Benhidjeb T, Ridwelski K, Wolff H, Münster B, Naundorf M, et al. (2010) Anomaly of the pancreatico-biliary junction and etiology of choledochal cysts. Zentralbl Chir 116: 1195-1203.
- Haciyanli M, Genc H, Colakoglu O, Aksoz K, Unsal B, et al. (2010) An adult choledochal cyst--the magnetic resonance cholangiopancreatography findings: report of a case. Surg Today 38: 1056-1059.
- Sacher VY, Davis JS, Sleeman D, Casillas J (2013) Role of magnetic resonance cholamgiopancreatography in diagnosing choledochal cysts: case series and review. World J Radiol 5: 304-312.
- Komi N, Tamura T, Miyoshi Y, Kunitomo K, Udaka H, et al. (1984) Nationwide survey of cases of choledochal cyst. Analysis of coexistent anomalies, complications and surgical treatment in 645 cases. Surg Gastroenterol 3: 69-73 .
- Khan SA, Davidson BR, Goldin R, Pereira SP, Rosenberg WM, et al. (2002) British Society of Gastroenterology. Guidelines for the diagnosis and treatment of cholangiocarcinoma: consensus document. Gut 6: VI1-9 .
- Singham J, Yoshida EM, Scudamore CH (2009) Choledochal cysts: part 2 of 3: Diagnosis. Can J Surg 52: 506-511 .
- Tian Y, Wu SD, Zhu AD, Chen DX (2010) Management of type I choledochal cyst in adult: totally laparoscopic resection and Roux-en-Y hepaticoenterostomy. J Gastrointest Surg 14: 1381-1388 .
- Akaraviputh T, Trakarnsanga A, Suksamanapun N (2010) Robot-assisted complete excision of choledochal cyst type I, hepaticojejunostomy and extracorporeal Roux-en-y anastomosis: a case report and review literature. World J Surg Oncol 8: 87 .
- Koshinaga T, Inoue M, Ohashi K, Sugito K, Ikeda T, et al. (2010) Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst. J Hepatobiliary Pancreat Sci 18: 47-52 .
- Machado NO, Chopra PJ, Al-Zadjali A, Younas S (2015) Choledochal Cyst in Adults: Etiopathogenesis, Presentation, Management, and Outcome-Case Series and Review. Gastroenterol Res Pract 2015: 602591 .
- Jin LX, Fields RC, Hawkins WG, Linehan DC, Strasberg SM (2014) A New Operative Approach for Type I Choledochal Cysts. J Gastrointest Surg 18: 1049-1053 .
- Wani BN, Jajoo SN (2009) Obstructive jaundice in neonates. Trop Gastroenterol 30: 195-200 .