Congenital choledochal cysts in adults

Brendan C. Visser, Insoo Suh, Lawrence W. Way, Sang Mo Kang, Clifford Deveney, Susan Orloff, Eric W. Fonkalsrud, Theodore X. O'Connell, Yeu Tsu M Lee, Kimberly S. Kirkwood, Bruce E. Stabile

Research output: Contribution to journalArticle

103 Citations (Scopus)

Abstract

Hypothesis: Excision of the extrahepatic portion of congenital choledochal cysts (CCs) avoids the risk of cancer. The standard classification scheme is out of date. Design: Retrospective case series and literature review. Setting: Tertiary care university hospital. Patients: Thirty-eight adult patients diagnosed as having CC from 1990 to 2004. Main Outcome Measures: Clinical and radiographic imaging findings, operative treatment, pathologic features, and clinical outcome. Results: Thirty-nine adult patients were treated for CCs (mean [SD] age at diagnosis, 31 [17] years, and mean [SD] age at surgery 37 [14] years). The primary report' was abdominal pain (36 of 39 patients). Eight patients had cholangitis, 5 had jaundice, and 6 had pancreatitis. Radiographic imaging studies and operative findings showed that the abnormality predominantly involved the extrahepatic bile duct in 30 patients, the intrahepatic and extrahepatic bile ducts in 7 patients; and 2 were diverticula attached to the common bile duct. Surgical treatment in 29 (90%) of 31 patients with benign cysts (regardless of intrahepatic changes) consisted of resection of the enlarged extrahepatic bile duct and gallbladder and Roux-en-Y hepaticojejunostomy. Eight patients (21%) were initially seen with associated cancer (cholangiocarcinoma of the extrahepatic duct in 6; gallbladder cancer in 2). Seven of 8 patients had a prior diagnosis of CC but had undergone a drainage operation (3 patients), expectant treatment (3 patients), or incomplete excision (1 patient). In none of the patients with cancer was surgery not curative. Nine patients had previously undergone a cystoduodenostomy and/or cystojejunostomy as children. Four of them had cancer on presentation as adults. There were no postoperative deaths. Cancer subsequently developed in no patient whose benign extrahepatic cyst was excised, regardless of the extent of enlargement of the intrahepatic bile duct. Conclusions: Congenital CCs consist principally of congenital dilation of the extrahepatic bile duct with a variable amount of intrahepatic involvement. We believe that the standard classification scheme is confusing, unsupported by evidence, misleading, and serves no purpose. The distinction between type I and type IV CCs has to be arbitrary, for the intrahepatic ducts were never completely normal. Although Caroli disease may resemble CCs morphologically, with respect to cause and clinical course, the 2 are unrelated. The other rare anomalies (gallbladderlike diverticula; choledochocele) are also unrelated to CC. Therefore, the term "congential choledochal cyst" should be exclusively reserved for congenital dilation of the extrahepatic and intrahepatic bile ducts apart from Caroli disease, and the other conditions should be referred to by their names, for example, choledochocele, and should no longer be thought of as subtypes of CC. Our data demonstrate once again a persistent tendency to recommend expectant treatment in patients without symptoms and the extreme risk of nonexcisional treatment. The entire extrahepatic biliary tree should be removed when CC is diagnosed whether or not symptoms are present. The outcome of that approach was excellent.

Original languageEnglish (US)
Pages (from-to)855-862
Number of pages8
JournalArchives of Surgery
Volume139
Issue number8
DOIs
StatePublished - Aug 2004
Externally publishedYes

Fingerprint

Choledochal Cyst
Extrahepatic Bile Ducts
Intrahepatic Bile Ducts
Caroli Disease
Diverticulum
Neoplasms
Cysts
Dilatation
Gallbladder Neoplasms
Therapeutics
Cholangitis
Cholangiocarcinoma

ASJC Scopus subject areas

  • Surgery

Cite this

Visser, B. C., Suh, I., Way, L. W., Kang, S. M., Deveney, C., Orloff, S., ... Stabile, B. E. (2004). Congenital choledochal cysts in adults. Archives of Surgery, 139(8), 855-862. https://doi.org/10.1001/archsurg.139.8.855

Congenital choledochal cysts in adults. / Visser, Brendan C.; Suh, Insoo; Way, Lawrence W.; Kang, Sang Mo; Deveney, Clifford; Orloff, Susan; Fonkalsrud, Eric W.; O'Connell, Theodore X.; Lee, Yeu Tsu M; Kirkwood, Kimberly S.; Stabile, Bruce E.

In: Archives of Surgery, Vol. 139, No. 8, 08.2004, p. 855-862.

Research output: Contribution to journalArticle

Visser, BC, Suh, I, Way, LW, Kang, SM, Deveney, C, Orloff, S, Fonkalsrud, EW, O'Connell, TX, Lee, YTM, Kirkwood, KS & Stabile, BE 2004, 'Congenital choledochal cysts in adults', Archives of Surgery, vol. 139, no. 8, pp. 855-862. https://doi.org/10.1001/archsurg.139.8.855
Visser, Brendan C. ; Suh, Insoo ; Way, Lawrence W. ; Kang, Sang Mo ; Deveney, Clifford ; Orloff, Susan ; Fonkalsrud, Eric W. ; O'Connell, Theodore X. ; Lee, Yeu Tsu M ; Kirkwood, Kimberly S. ; Stabile, Bruce E. / Congenital choledochal cysts in adults. In: Archives of Surgery. 2004 ; Vol. 139, No. 8. pp. 855-862.
@article{63c07e78248649a8ac6d89f1a490bfa8,
title = "Congenital choledochal cysts in adults",
abstract = "Hypothesis: Excision of the extrahepatic portion of congenital choledochal cysts (CCs) avoids the risk of cancer. The standard classification scheme is out of date. Design: Retrospective case series and literature review. Setting: Tertiary care university hospital. Patients: Thirty-eight adult patients diagnosed as having CC from 1990 to 2004. Main Outcome Measures: Clinical and radiographic imaging findings, operative treatment, pathologic features, and clinical outcome. Results: Thirty-nine adult patients were treated for CCs (mean [SD] age at diagnosis, 31 [17] years, and mean [SD] age at surgery 37 [14] years). The primary report' was abdominal pain (36 of 39 patients). Eight patients had cholangitis, 5 had jaundice, and 6 had pancreatitis. Radiographic imaging studies and operative findings showed that the abnormality predominantly involved the extrahepatic bile duct in 30 patients, the intrahepatic and extrahepatic bile ducts in 7 patients; and 2 were diverticula attached to the common bile duct. Surgical treatment in 29 (90{\%}) of 31 patients with benign cysts (regardless of intrahepatic changes) consisted of resection of the enlarged extrahepatic bile duct and gallbladder and Roux-en-Y hepaticojejunostomy. Eight patients (21{\%}) were initially seen with associated cancer (cholangiocarcinoma of the extrahepatic duct in 6; gallbladder cancer in 2). Seven of 8 patients had a prior diagnosis of CC but had undergone a drainage operation (3 patients), expectant treatment (3 patients), or incomplete excision (1 patient). In none of the patients with cancer was surgery not curative. Nine patients had previously undergone a cystoduodenostomy and/or cystojejunostomy as children. Four of them had cancer on presentation as adults. There were no postoperative deaths. Cancer subsequently developed in no patient whose benign extrahepatic cyst was excised, regardless of the extent of enlargement of the intrahepatic bile duct. Conclusions: Congenital CCs consist principally of congenital dilation of the extrahepatic bile duct with a variable amount of intrahepatic involvement. We believe that the standard classification scheme is confusing, unsupported by evidence, misleading, and serves no purpose. The distinction between type I and type IV CCs has to be arbitrary, for the intrahepatic ducts were never completely normal. Although Caroli disease may resemble CCs morphologically, with respect to cause and clinical course, the 2 are unrelated. The other rare anomalies (gallbladderlike diverticula; choledochocele) are also unrelated to CC. Therefore, the term {"}congential choledochal cyst{"} should be exclusively reserved for congenital dilation of the extrahepatic and intrahepatic bile ducts apart from Caroli disease, and the other conditions should be referred to by their names, for example, choledochocele, and should no longer be thought of as subtypes of CC. Our data demonstrate once again a persistent tendency to recommend expectant treatment in patients without symptoms and the extreme risk of nonexcisional treatment. The entire extrahepatic biliary tree should be removed when CC is diagnosed whether or not symptoms are present. The outcome of that approach was excellent.",
author = "Visser, {Brendan C.} and Insoo Suh and Way, {Lawrence W.} and Kang, {Sang Mo} and Clifford Deveney and Susan Orloff and Fonkalsrud, {Eric W.} and O'Connell, {Theodore X.} and Lee, {Yeu Tsu M} and Kirkwood, {Kimberly S.} and Stabile, {Bruce E.}",
year = "2004",
month = "8",
doi = "10.1001/archsurg.139.8.855",
language = "English (US)",
volume = "139",
pages = "855--862",
journal = "JAMA Surgery",
issn = "2168-6254",
publisher = "American Medical Association",
number = "8",

}

TY - JOUR

T1 - Congenital choledochal cysts in adults

AU - Visser, Brendan C.

AU - Suh, Insoo

AU - Way, Lawrence W.

AU - Kang, Sang Mo

AU - Deveney, Clifford

AU - Orloff, Susan

AU - Fonkalsrud, Eric W.

AU - O'Connell, Theodore X.

AU - Lee, Yeu Tsu M

AU - Kirkwood, Kimberly S.

AU - Stabile, Bruce E.

PY - 2004/8

Y1 - 2004/8

N2 - Hypothesis: Excision of the extrahepatic portion of congenital choledochal cysts (CCs) avoids the risk of cancer. The standard classification scheme is out of date. Design: Retrospective case series and literature review. Setting: Tertiary care university hospital. Patients: Thirty-eight adult patients diagnosed as having CC from 1990 to 2004. Main Outcome Measures: Clinical and radiographic imaging findings, operative treatment, pathologic features, and clinical outcome. Results: Thirty-nine adult patients were treated for CCs (mean [SD] age at diagnosis, 31 [17] years, and mean [SD] age at surgery 37 [14] years). The primary report' was abdominal pain (36 of 39 patients). Eight patients had cholangitis, 5 had jaundice, and 6 had pancreatitis. Radiographic imaging studies and operative findings showed that the abnormality predominantly involved the extrahepatic bile duct in 30 patients, the intrahepatic and extrahepatic bile ducts in 7 patients; and 2 were diverticula attached to the common bile duct. Surgical treatment in 29 (90%) of 31 patients with benign cysts (regardless of intrahepatic changes) consisted of resection of the enlarged extrahepatic bile duct and gallbladder and Roux-en-Y hepaticojejunostomy. Eight patients (21%) were initially seen with associated cancer (cholangiocarcinoma of the extrahepatic duct in 6; gallbladder cancer in 2). Seven of 8 patients had a prior diagnosis of CC but had undergone a drainage operation (3 patients), expectant treatment (3 patients), or incomplete excision (1 patient). In none of the patients with cancer was surgery not curative. Nine patients had previously undergone a cystoduodenostomy and/or cystojejunostomy as children. Four of them had cancer on presentation as adults. There were no postoperative deaths. Cancer subsequently developed in no patient whose benign extrahepatic cyst was excised, regardless of the extent of enlargement of the intrahepatic bile duct. Conclusions: Congenital CCs consist principally of congenital dilation of the extrahepatic bile duct with a variable amount of intrahepatic involvement. We believe that the standard classification scheme is confusing, unsupported by evidence, misleading, and serves no purpose. The distinction between type I and type IV CCs has to be arbitrary, for the intrahepatic ducts were never completely normal. Although Caroli disease may resemble CCs morphologically, with respect to cause and clinical course, the 2 are unrelated. The other rare anomalies (gallbladderlike diverticula; choledochocele) are also unrelated to CC. Therefore, the term "congential choledochal cyst" should be exclusively reserved for congenital dilation of the extrahepatic and intrahepatic bile ducts apart from Caroli disease, and the other conditions should be referred to by their names, for example, choledochocele, and should no longer be thought of as subtypes of CC. Our data demonstrate once again a persistent tendency to recommend expectant treatment in patients without symptoms and the extreme risk of nonexcisional treatment. The entire extrahepatic biliary tree should be removed when CC is diagnosed whether or not symptoms are present. The outcome of that approach was excellent.

AB - Hypothesis: Excision of the extrahepatic portion of congenital choledochal cysts (CCs) avoids the risk of cancer. The standard classification scheme is out of date. Design: Retrospective case series and literature review. Setting: Tertiary care university hospital. Patients: Thirty-eight adult patients diagnosed as having CC from 1990 to 2004. Main Outcome Measures: Clinical and radiographic imaging findings, operative treatment, pathologic features, and clinical outcome. Results: Thirty-nine adult patients were treated for CCs (mean [SD] age at diagnosis, 31 [17] years, and mean [SD] age at surgery 37 [14] years). The primary report' was abdominal pain (36 of 39 patients). Eight patients had cholangitis, 5 had jaundice, and 6 had pancreatitis. Radiographic imaging studies and operative findings showed that the abnormality predominantly involved the extrahepatic bile duct in 30 patients, the intrahepatic and extrahepatic bile ducts in 7 patients; and 2 were diverticula attached to the common bile duct. Surgical treatment in 29 (90%) of 31 patients with benign cysts (regardless of intrahepatic changes) consisted of resection of the enlarged extrahepatic bile duct and gallbladder and Roux-en-Y hepaticojejunostomy. Eight patients (21%) were initially seen with associated cancer (cholangiocarcinoma of the extrahepatic duct in 6; gallbladder cancer in 2). Seven of 8 patients had a prior diagnosis of CC but had undergone a drainage operation (3 patients), expectant treatment (3 patients), or incomplete excision (1 patient). In none of the patients with cancer was surgery not curative. Nine patients had previously undergone a cystoduodenostomy and/or cystojejunostomy as children. Four of them had cancer on presentation as adults. There were no postoperative deaths. Cancer subsequently developed in no patient whose benign extrahepatic cyst was excised, regardless of the extent of enlargement of the intrahepatic bile duct. Conclusions: Congenital CCs consist principally of congenital dilation of the extrahepatic bile duct with a variable amount of intrahepatic involvement. We believe that the standard classification scheme is confusing, unsupported by evidence, misleading, and serves no purpose. The distinction between type I and type IV CCs has to be arbitrary, for the intrahepatic ducts were never completely normal. Although Caroli disease may resemble CCs morphologically, with respect to cause and clinical course, the 2 are unrelated. The other rare anomalies (gallbladderlike diverticula; choledochocele) are also unrelated to CC. Therefore, the term "congential choledochal cyst" should be exclusively reserved for congenital dilation of the extrahepatic and intrahepatic bile ducts apart from Caroli disease, and the other conditions should be referred to by their names, for example, choledochocele, and should no longer be thought of as subtypes of CC. Our data demonstrate once again a persistent tendency to recommend expectant treatment in patients without symptoms and the extreme risk of nonexcisional treatment. The entire extrahepatic biliary tree should be removed when CC is diagnosed whether or not symptoms are present. The outcome of that approach was excellent.

UR - http://www.scopus.com/inward/record.url?scp=3843137261&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=3843137261&partnerID=8YFLogxK

U2 - 10.1001/archsurg.139.8.855

DO - 10.1001/archsurg.139.8.855

M3 - Article

VL - 139

SP - 855

EP - 862

JO - JAMA Surgery

JF - JAMA Surgery

SN - 2168-6254

IS - 8

ER -