Partial Hepatectomy and Total Cyst Excision is Curative for Localized Type IV-A Biliary Duct Cysts – Report of Four Cases and Review of Management

Abstract

Purpose: With the increasing use of newer modalities such as CT cholangiography and MRCP offering an accurate delineation of the ductal anatomy of choledochal cysts, the incidence of type IV-A biliary duct cysts has increased. Although the management of the more common type I cysts is well established, that of type IV-A cysts is still controversial. The localized/unilobar variety of type IV-A cysts is a unique entity amenable to curative surgical management.

Methods: Between Jan 2000 and Jan 2005, 10 of 25 cases with choledochal cysts were diagnosed as having type IV- cysts. Four of these were localized or unilobar variants affecting only one lobe of the liver. Three cases had a left ductal involvement and one had a right ductal involvement of their intrahepatic component. We describe the presentation and curative surgical management of these patients with a unilobar variety of type IV-A cysts and discuss the management options for type IV-A cysts in the literature.

Results: One child with previous surgical treatment for presumed type 1 cyst, diagnosed as having a type IV-A right ductal cyst complicated with liver abscess, underwent right hepatectomy, two children had a left hepatectomy and one child had a left lateral lobectomy. All four children had uncomplicated intraoperative and postoperative courses. After 4-8 years’ follow-up, all are symptom-free. There were no biliary tract or hepatic abnormalities on radiological surveillance with MRCP.

Conclusions: In some series, the incidence of type IV-A choledochal cysts is equal to or higher than that of type 1 cysts. MRCP and intraoperative cholangiogram can help to confirm the anatomical varieties and associated ductal strictures. In the unilobar/uniductal varieties, complete cyst excision can be safely achieved in children through an additional hepatectomy/lobectomy. This can result in a curative status, leaving the child free from complications such as cholangitis, abscess, hepatolithiasis and the risk of biliary duct malignancy.

Key words

type IV-A choledochal cyst - uniductal/localized variety - hepatectomy - curative resection - children

References

• 1 Alonso-Lej F, Rever WB, Pessango D. Congenital choledochal cyst with a report of two and an analysis of 94 cases.  Int Abst Surg. 1959;  108 1-30
• 2 Benhidjeb B, Munster B, Ridwelski K, Rudolph B, Mau H, Lippert H. Cystic dilatation of the common bile ducts: surgical treatment and long-term results.  B J Surg. 1994;  81 433-436
• 3 Chijiiwa K, Komura M, Kameoka N. Postoperative follow-up of patients with type IVA choledochal cysts after excision of extrahepatic cyst.  J Am Coll Surg. 1994;  179 641-64
• 4 Ishibashi T, Kasahara K, Yasuda Y, Nagai H, Makino S, Kanazawa K. Malignant change in the biliary tract after excision of choledochal cyst.  Br J Surg. 1997;  84 ((12):) 1687
• 5 Kagawa Y, Kashihara S, Kuramoto S, Maetani S. Carcinoma arising in a congenitally dilated biliary tract. Report of a case and review of the literature.  Gastroenterology. 1978;  74 ((6)) 1286-1294
• 6 Kanoh K, Shimura T, Tsutsumi S, Suzuki H, Nagashima K, Kuwano H. Left hepatectomy for the choledochal cyst (type IV-A) with intrahepatic stenosis: report of a case.  Hepatogastroenterology. 2002;  49 ((43)) 144-147
• 7 Kaneko K, Ando H, Ito T, Watanabe Y, Seo T, Harada T. et al . Secondary excision of choledochal cyst after previous cyst-enterostomies.  Hepatogastroenterology. 1999;  46 2772-2775
• 8 Kobayashi S, Asano T, Yamasaki M, Kenmochi T, Nagagohri T, Ochioi T. Risk of bile duct carcinogenesis after excision of extrahepatic bileducts in pancreaticobiliary maljunction.  Surgery. 1999;  12 ((5)) 939-944
• 9 Lal R, Agarwal S, Shivhare R, Kumar A, Sikora S, Saxena R. et al . Type IVA choledochal cysts: a challenge.  J of Hep Bil Pancr Surg. 2005;  12 ((2)) 129-134
• 10 Nakayama H, Masuda H, Ugajin W, Koshinaga T, Fukuzawa M. Left hepatic lobectomy for type IV A choledochal cyst.  Am Surg. 2000;  66 ((11)) 1020-1022
• 11 O’Neill JA. Choledochal cyst. In: Grossfeld JL, O’Neill JA, Coran AG, Fronkalsrud EW, eds. Pediatric Surgery. 6th Edition Mosby Elsevier, Philadelphia 2006: 631-632
• 12 Strong R. Type IVA choledochal cyst: Is hepatic resection necessary?.  HPB Surg. 1996;  10 61-63
• 13 Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cyst: its classification, operative procedures, and review of our 37 cases including cancer arising from choledochal cyst.  Am J Surg. 1977;  134 263-269
• 14 Todani T, Narusue M, Watanabe Y, Tabuchi K, Okajima K. Management of congenital choledochal cyst with intrahepatic involvement.  Ann Surg. 1978;  187 ((3)) 272-280
• 15 Todani T, Watnabe Y, Urushihara N, Noda T, Morotomi Y. Biliary complications after excisional procedure for choledochal cyst.  J Pediatr Surg. 1995;  30 478-481
• 16 Todani T, Watanabe Y, Yoki A, Ogura K, Wang ZQ. Coexisting biliary anomalies and anatomical variants in choledochal cyst.  1998;  85 760-763
• 17 Watanabe Y, Toki A. Todani T. Bile duct cancer developed after cyst excision for choledochal cyst.  J Hepatobil Pancreat Surg. 1999;  6 207-212
• 18 Yilmaz S, Kirimlioglu H, Kirimlioglu V, Isik B, Coban S, Yildirim B. et al . Partial hepatectomy is curative for the localized type of Caroli's disease: a case report and review of the literature.  Surgeon. 2006;  4 ((2)) 101-105
• 19 Zheng LX, Jia HB, Wu D, Shang H, Zhong XY, Wang QS. et al . Experience of congenital choledochal cyst in adults: Treatment, surgical procedures and clinical outcome in the second Affiliated Hospital of Harbin Medical University.  J Korean Med Sci. 2004;  19 842-847

Correspondence