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Laparoscopic excision of a rare type II choledochal cyst: Case report and review of the literature
Laparoscopic Excision of a Rare Type II Choledochal Cyst: Case Report and Review of the Literature By Donald C. Liu, Jaime A. Rodriguez, Funda Meric, and James L. Geiger New Orleans, Louisiana and Ann Arbor, Michigan
Although once considered rare, choledochal cysts now are extensively reported on and have an official anatomic classification. The authors report a case of a 4-year-old girl whom on routine abdominal ultrasound as follow-up for vesicoureteral reflux was found to have a choledochal cyst. Follow-up hepatobiliary scan together with the ultrasound findings was consistent with a rare type II choledochal cyst. On laparoscopy, a type II choledochal cyst was confirmed on dissection and amenable to laparoscopic excision. Intraoperative cholangiogram after excision showed normal remaining biliary
anatomy. The patient was discharged the following day without delayed complications on follow-up visits. This report suggests a role for laparoscopic surgery in the definitive management of selected cases of choledochal cystic disease. (Included is relevant radiological documentation, illustration of technique, and review of the literature.) J Pediatr Surg 35:1117-1119. Copyright r 2000 by W.B. Saunders Company.
CASE REPORT
der generally enters the choledochal cyst, and the right and left hepatic ducts as well as the intrahepatic ducts are normal in size. Type II choledochal cyst, the subject of this report, is the rarest of all choledochal cysts and commonly described as a diverticular malformation of the common duct with an otherwise normal intra and extrahepatic biliary tree. Such diverticular cysts may be either small or very large and occur in less than 2% of reported cases. Type III choledochal cyst or choledochocele usually is intraduodenal, but occasionally intrapancreatic and is slightly more common than the type II variety.5 Type IV choledochal cysts occur in approximately 10% of cases and are multicystic structures, usually with both intra- and extrahepatic components at presentation. Finally, type V forms of the anomaly are single or multiple intrahepatic cysts. When these intrahepatic cysts are associated with hepatic fibrosis, they are referred to as Caroli’s disease.6 Whether type II choledochal cysts are congenital or acquired remains speculative in nature, as is the etiology of choledochal cysts. The common channel theory proposed by Babbit7 in 1969 is the most commonly accepted theory. This theory suggests that a high insertion of the bile duct into the pancreatic duct allow pancreatic enzymes to reflux into the bile duct, causing inflammation, weakening, and eventual fibrosis of the bile duct. Distal obstruction then leads to progressive dilatation of
An asymptomatic 4-year-old girl with normal liver function tests underwent an abdominal ultrasound scan as follow-up for vesiculoureteral reflux. She was incidentally found to have a 1.8-cm round hypoechoic structure in the porta hepatis region. This structure was adjacent to, but clearly separate from the gallbladder neck. The common bile duct also was identified separately adjacent to the mass and appeared to be normal in caliber without dilatation. A hepatobiliary scan showed a separate mass with isotope uptake near the gallbladder. Together with the above ultrasound findings, we suspected a type II choledochal cyst. Because intraoperative cholangiogram was to be performed, preoperative endoscopic retrograde cholangio-pancreatography (ERCP) was felt unnecessary. The patient was taken to the operating room with a preoperative diagnosis of type II choledochal cyst. A standard 4-port technique for laparoscopic cholecystectomy was utilized. On laparoscopy, a 2-cm cystic mass was found infero-posterior to the gallbladder and partially embedded in the subhepatic fossa. The cystic mass was bluntly teased away from its hepatic attachments and excised endoscopically (Fig 1). No connection, however, between the cyst and the biliary tract was identified clearly during our dissection precluding safe definitive surgical ligation with either endoclip or endoloop. On gross examination, the cystic mass was found intact, unilocular, filled with bile, but without a clear stalk. A cholangiogram was then performed through the gallbladder showing normal remaining biliary tract anatomy without obvious leakage of bile. A drain left next to the common bile duct was absent of bile drainage and removed on postoperative day 1. Recovery was uneventful, and the patient was discharged home on postoperative day 1 without delayed complications on follow-up visits. Histopathologic findings were consistent with a choledochal cyst.
DISCUSSION
The first description of a choledochal cyst was by Vater and Ezler in 1723,1 and of cyst resection by McWhorter in 1924.2 In 1959, Alonzo-Lej et al,3 and, in 1977, Todani et al4 classified choledochal cysts based on the location of the cyst. Type I or cystic dilatation of the common duct constitutes approximately 85% to 90% of the cases in all reported series. In this form of the anomaly, the gallbladJournal of Pediatric Surgery, Vol 35, No 7 (July), 2000: pp 1117-1119
INDEX WORDS: Laparoscopy, choledochal cyst.
From the Children’s Hospital of New Orleans, New Orleans, LA. Address reprint requests to Donald C. Liu, MD, PhD, Suite 224, Children’s Hospital of New Orleans, 200 Henry Clay Ave, New Orleans, LA 70118. Copyright r 2000 by W.B. Saunders Company 0022-3468/00/3507-0023$03.00/0 doi:10.1053/js.2000.7840 1117
1118
LIU ET AL
Fig 1. Illustration of laparoscopic excision of a type II choledochal cyst. Sketch represents likely cyst insertion site on common bile duct, because this was not clearly identified during dissection.
the biliary tree. Although this theory most likely explains the occurrence of fusiform dilatation seen in type I cysts, it may not explain adequately the anatomy of type II (diverticular), or even type III cysts. With regard to type II cysts, Hayes et al8 suggested that the diverticulum is a remnant of an earlier stage of bile duct development when it represents more a network of cells than tubular ducts. This is in line with the theory of Yotsuyanagi.9 However, clinical experience indicates that it is just as likely that the large diverticular forms of choledochal cyst represent end-stage healing of prenatal rupture of the common duct.10 Lending support to this line of thinking, choledochal cysts have been identified in the fetus during prenatal ultrasound studies.11 Earlier promising reports of internal drainage by cyst enterostomy have been replaced by documented occurrence of late complications such as suppurative cholangitis, lithiasis, pancreatitis, cirrhosis, portal hypertension, intrahepatic abscess, and cholangio-carcinoma.12 As a result, the treatment of choice today for choledochal cysts is surgical excision whenever possible. Laparoscopic surgery is a primary component in the
armamentarium of today’s pediatric general surgeon. Laparoscopic surgery in children has been known to afford several advantages over traditional open techniques for a variety of common abdominal surgical procedures, such as esophageal-gastric fundoplication for gastroesophageal reflux disease and splenectomy for a variety of hematologic disorders.13,14 Benefits include reduced hospital stay, faster return to unrestricted activity, and improved cosmetic results. Complications of the open technique, such as postoperative ileus and atelectasis, even pneumonia, associated with splinting also appear to be lower.15,16 Because children make up a sizable portion of patients with choledochal cyst (as high as 40%).17 The application of minimally invasive techniques is potentially beneficial for all the reasons described above. Laparoscopic surgery for biliary tract disease has been reported extensively in the literature. Recent advances have extended the spectrum from simple excision (ie, cholecystectomy) at 1 end to radical excision plus complicated biliary tract reconstruction at the other. Shimura et al18 reported recently on a type I choledochal cyst successfully treated completely by laparoscopy. The case presented in this report lies more toward the beginning of the spectrum and represented a unique case of a type II cyst amenable to simple laparoscopic excision. Although in this case report the presence of a common channel was not entirely excluded by intraoperative cholangiography, the presence of an otherwise normalappearing remaining common bile duct along with a clinically asymptomatic child prompted the authors to choose simple cyst excision as the treatment of choice. This was a unique case and the treatment appropriate for this special circumstance, albeit a clear exception to standard excision and hepatico-jejunostomy for the usual type II cyst with a broad connection to the common duct. Although the child was well nearly 3 years after surgery, long-term follow-up is needed to truly assess surgical outcome, notably complications such as stricture formation or the development of malignancy. Importantly, this report shows that laparoscopy may have an important place in the algorithm for surgical management of choledochal cysts, especially if a preoperative diagnosis of a rare type II choledochal cyst is entertained. Large abdominal incisions and longer hospital stays necessitated by conventional open surgery thus could potentially be avoided.
REFERENCES 1. Vater A, Ezler CS: Dissertatio de scirrhis viscerum occasione sectionis viri tympanite defunte. Wittenburgae Pamphlers 881:22, 1724 2. McWhorter GL: Congenital cyst dilatation of the common bile duct. Arch Surg 8:604-626, 1924
3. Alonzo-Lej F, Rever WB Jr, Pessagno DJ: Congenital choledochal cysts, with a report of 2, and an analysis of 94 cases. Surg Gynecol Obstet 108:1-30, 1959 4. Todani T, Watanabe Y, Narusue M, et al: Congenital bile duct
LAPAROSCOPIC EXCISION OF CHOLEDOCHAL CYST
cysts. Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134:263-269, 1977 5. Kaytal D, Lees GM: Choledochal cysts: A retrospective review of 28 patients and a review of the literature. Can J Surg 35:584-588, 1992 6. Caroli J, Soupalt R, Kossakowski J, et al: La dilatation polykystique congenitale des voies biliaires intrahepatiques. Essar de classification. Semin Hop Paris 34:488-495, 1958 7. Babitt DP: Congenital choledochal cysts: New etiological concept based on anomalus relationships of the common bile duct and pancreatic bulb. Ann Radiol 12:231-240, 1969 8. Hayes MA, Goldenberg IS, Bishop CS: The developmental basis for bile duct anomalies. Surg Gynecol Obstet 107:446-456, 1958 9. Yotsuyanagi S: Contribution to etiology and pathology of idiopathic cystic dilatation of the common bile duct with report of three cases. Gann 30:601-752, 1936 10. Lilly JR, Weintraub WH, Altman RP: Spontaneous perforation of the extrahepatic bile ducts and bile peritonitis in infancy. Surgery 75:664-673, 1974 11. Frank JL, Hill MC, Chirathivat S, et al: Antenatal observation of
1119
a choledochal cyst by ultrasound. AJR Am J Roentgenol 137:166-168, 1981 12. O’Neill JA: Choledochal cyst. Curr Probl Surg 29:365-410, 1992 13. Georgeson KE: Laparoscopic gastrostomy and fundoplication. Pediatr Ann 18:192-196, 1993 14. Janu GJ, Rogers DA, Lobe TE: A comparison of laparoscopic and traditional open splenectomy in childhood. J Pediatr Surg 26:407412, 1991 15. Shippers E, Oettinger AP, Anurov M, et al: Intestinale motilitaet nach laparoskopischer vs. konventioneller cholecystektomy. Lagenbecks Arch Chir 377:14-18, 1992 16. Frazee RC, Roberts JW, Okeson GC, et al: Open versus laparoscopic cholecystectomy: A comparison of post operative pulmonary function. Ann Surg 213:651-654, 1991 17. Grosfeld JL, Rescorla FJ, Skinner MA, et al: The spectrum of biliary tract disorders in infants and children: Experience with 300 cases. Arch Surg 129:513-520, 1994 18. Shimura H, Tanaka M, Shimizu S, et al: Laparoscopic treatment of congenital choledochal cyst. Surg Endosc 12:1268-1271, 1998
Although once considered rare, choledochal cysts now are extensively reported on and have an official anatomic classification. The authors report a case of a 4-year-old girl whom on routine abdominal ultrasound as follow-up for vesicoureteral reflux was found to have a choledochal cyst. Follow-up hepatobiliary scan together with the ultrasound findings was consistent with a rare type II choledochal cyst. On laparoscopy, a type II choledochal cyst was confirmed on dissection and amenable to laparoscopic excision. Intraoperative cholangiogram after excision showed normal remaining biliary
anatomy. The patient was discharged the following day without delayed complications on follow-up visits. This report suggests a role for laparoscopic surgery in the definitive management of selected cases of choledochal cystic disease. (Included is relevant radiological documentation, illustration of technique, and review of the literature.) J Pediatr Surg 35:1117-1119. Copyright r 2000 by W.B. Saunders Company.
CASE REPORT
der generally enters the choledochal cyst, and the right and left hepatic ducts as well as the intrahepatic ducts are normal in size. Type II choledochal cyst, the subject of this report, is the rarest of all choledochal cysts and commonly described as a diverticular malformation of the common duct with an otherwise normal intra and extrahepatic biliary tree. Such diverticular cysts may be either small or very large and occur in less than 2% of reported cases. Type III choledochal cyst or choledochocele usually is intraduodenal, but occasionally intrapancreatic and is slightly more common than the type II variety.5 Type IV choledochal cysts occur in approximately 10% of cases and are multicystic structures, usually with both intra- and extrahepatic components at presentation. Finally, type V forms of the anomaly are single or multiple intrahepatic cysts. When these intrahepatic cysts are associated with hepatic fibrosis, they are referred to as Caroli’s disease.6 Whether type II choledochal cysts are congenital or acquired remains speculative in nature, as is the etiology of choledochal cysts. The common channel theory proposed by Babbit7 in 1969 is the most commonly accepted theory. This theory suggests that a high insertion of the bile duct into the pancreatic duct allow pancreatic enzymes to reflux into the bile duct, causing inflammation, weakening, and eventual fibrosis of the bile duct. Distal obstruction then leads to progressive dilatation of
An asymptomatic 4-year-old girl with normal liver function tests underwent an abdominal ultrasound scan as follow-up for vesiculoureteral reflux. She was incidentally found to have a 1.8-cm round hypoechoic structure in the porta hepatis region. This structure was adjacent to, but clearly separate from the gallbladder neck. The common bile duct also was identified separately adjacent to the mass and appeared to be normal in caliber without dilatation. A hepatobiliary scan showed a separate mass with isotope uptake near the gallbladder. Together with the above ultrasound findings, we suspected a type II choledochal cyst. Because intraoperative cholangiogram was to be performed, preoperative endoscopic retrograde cholangio-pancreatography (ERCP) was felt unnecessary. The patient was taken to the operating room with a preoperative diagnosis of type II choledochal cyst. A standard 4-port technique for laparoscopic cholecystectomy was utilized. On laparoscopy, a 2-cm cystic mass was found infero-posterior to the gallbladder and partially embedded in the subhepatic fossa. The cystic mass was bluntly teased away from its hepatic attachments and excised endoscopically (Fig 1). No connection, however, between the cyst and the biliary tract was identified clearly during our dissection precluding safe definitive surgical ligation with either endoclip or endoloop. On gross examination, the cystic mass was found intact, unilocular, filled with bile, but without a clear stalk. A cholangiogram was then performed through the gallbladder showing normal remaining biliary tract anatomy without obvious leakage of bile. A drain left next to the common bile duct was absent of bile drainage and removed on postoperative day 1. Recovery was uneventful, and the patient was discharged home on postoperative day 1 without delayed complications on follow-up visits. Histopathologic findings were consistent with a choledochal cyst.
DISCUSSION
The first description of a choledochal cyst was by Vater and Ezler in 1723,1 and of cyst resection by McWhorter in 1924.2 In 1959, Alonzo-Lej et al,3 and, in 1977, Todani et al4 classified choledochal cysts based on the location of the cyst. Type I or cystic dilatation of the common duct constitutes approximately 85% to 90% of the cases in all reported series. In this form of the anomaly, the gallbladJournal of Pediatric Surgery, Vol 35, No 7 (July), 2000: pp 1117-1119
INDEX WORDS: Laparoscopy, choledochal cyst.
From the Children’s Hospital of New Orleans, New Orleans, LA. Address reprint requests to Donald C. Liu, MD, PhD, Suite 224, Children’s Hospital of New Orleans, 200 Henry Clay Ave, New Orleans, LA 70118. Copyright r 2000 by W.B. Saunders Company 0022-3468/00/3507-0023$03.00/0 doi:10.1053/js.2000.7840 1117
1118
LIU ET AL
Fig 1. Illustration of laparoscopic excision of a type II choledochal cyst. Sketch represents likely cyst insertion site on common bile duct, because this was not clearly identified during dissection.
the biliary tree. Although this theory most likely explains the occurrence of fusiform dilatation seen in type I cysts, it may not explain adequately the anatomy of type II (diverticular), or even type III cysts. With regard to type II cysts, Hayes et al8 suggested that the diverticulum is a remnant of an earlier stage of bile duct development when it represents more a network of cells than tubular ducts. This is in line with the theory of Yotsuyanagi.9 However, clinical experience indicates that it is just as likely that the large diverticular forms of choledochal cyst represent end-stage healing of prenatal rupture of the common duct.10 Lending support to this line of thinking, choledochal cysts have been identified in the fetus during prenatal ultrasound studies.11 Earlier promising reports of internal drainage by cyst enterostomy have been replaced by documented occurrence of late complications such as suppurative cholangitis, lithiasis, pancreatitis, cirrhosis, portal hypertension, intrahepatic abscess, and cholangio-carcinoma.12 As a result, the treatment of choice today for choledochal cysts is surgical excision whenever possible. Laparoscopic surgery is a primary component in the
armamentarium of today’s pediatric general surgeon. Laparoscopic surgery in children has been known to afford several advantages over traditional open techniques for a variety of common abdominal surgical procedures, such as esophageal-gastric fundoplication for gastroesophageal reflux disease and splenectomy for a variety of hematologic disorders.13,14 Benefits include reduced hospital stay, faster return to unrestricted activity, and improved cosmetic results. Complications of the open technique, such as postoperative ileus and atelectasis, even pneumonia, associated with splinting also appear to be lower.15,16 Because children make up a sizable portion of patients with choledochal cyst (as high as 40%).17 The application of minimally invasive techniques is potentially beneficial for all the reasons described above. Laparoscopic surgery for biliary tract disease has been reported extensively in the literature. Recent advances have extended the spectrum from simple excision (ie, cholecystectomy) at 1 end to radical excision plus complicated biliary tract reconstruction at the other. Shimura et al18 reported recently on a type I choledochal cyst successfully treated completely by laparoscopy. The case presented in this report lies more toward the beginning of the spectrum and represented a unique case of a type II cyst amenable to simple laparoscopic excision. Although in this case report the presence of a common channel was not entirely excluded by intraoperative cholangiography, the presence of an otherwise normalappearing remaining common bile duct along with a clinically asymptomatic child prompted the authors to choose simple cyst excision as the treatment of choice. This was a unique case and the treatment appropriate for this special circumstance, albeit a clear exception to standard excision and hepatico-jejunostomy for the usual type II cyst with a broad connection to the common duct. Although the child was well nearly 3 years after surgery, long-term follow-up is needed to truly assess surgical outcome, notably complications such as stricture formation or the development of malignancy. Importantly, this report shows that laparoscopy may have an important place in the algorithm for surgical management of choledochal cysts, especially if a preoperative diagnosis of a rare type II choledochal cyst is entertained. Large abdominal incisions and longer hospital stays necessitated by conventional open surgery thus could potentially be avoided.
REFERENCES 1. Vater A, Ezler CS: Dissertatio de scirrhis viscerum occasione sectionis viri tympanite defunte. Wittenburgae Pamphlers 881:22, 1724 2. McWhorter GL: Congenital cyst dilatation of the common bile duct. Arch Surg 8:604-626, 1924
3. Alonzo-Lej F, Rever WB Jr, Pessagno DJ: Congenital choledochal cysts, with a report of 2, and an analysis of 94 cases. Surg Gynecol Obstet 108:1-30, 1959 4. Todani T, Watanabe Y, Narusue M, et al: Congenital bile duct
LAPAROSCOPIC EXCISION OF CHOLEDOCHAL CYST
cysts. Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134:263-269, 1977 5. Kaytal D, Lees GM: Choledochal cysts: A retrospective review of 28 patients and a review of the literature. Can J Surg 35:584-588, 1992 6. Caroli J, Soupalt R, Kossakowski J, et al: La dilatation polykystique congenitale des voies biliaires intrahepatiques. Essar de classification. Semin Hop Paris 34:488-495, 1958 7. Babitt DP: Congenital choledochal cysts: New etiological concept based on anomalus relationships of the common bile duct and pancreatic bulb. Ann Radiol 12:231-240, 1969 8. Hayes MA, Goldenberg IS, Bishop CS: The developmental basis for bile duct anomalies. Surg Gynecol Obstet 107:446-456, 1958 9. Yotsuyanagi S: Contribution to etiology and pathology of idiopathic cystic dilatation of the common bile duct with report of three cases. Gann 30:601-752, 1936 10. Lilly JR, Weintraub WH, Altman RP: Spontaneous perforation of the extrahepatic bile ducts and bile peritonitis in infancy. Surgery 75:664-673, 1974 11. Frank JL, Hill MC, Chirathivat S, et al: Antenatal observation of
1119
a choledochal cyst by ultrasound. AJR Am J Roentgenol 137:166-168, 1981 12. O’Neill JA: Choledochal cyst. Curr Probl Surg 29:365-410, 1992 13. Georgeson KE: Laparoscopic gastrostomy and fundoplication. Pediatr Ann 18:192-196, 1993 14. Janu GJ, Rogers DA, Lobe TE: A comparison of laparoscopic and traditional open splenectomy in childhood. J Pediatr Surg 26:407412, 1991 15. Shippers E, Oettinger AP, Anurov M, et al: Intestinale motilitaet nach laparoskopischer vs. konventioneller cholecystektomy. Lagenbecks Arch Chir 377:14-18, 1992 16. Frazee RC, Roberts JW, Okeson GC, et al: Open versus laparoscopic cholecystectomy: A comparison of post operative pulmonary function. Ann Surg 213:651-654, 1991 17. Grosfeld JL, Rescorla FJ, Skinner MA, et al: The spectrum of biliary tract disorders in infants and children: Experience with 300 cases. Arch Surg 129:513-520, 1994 18. Shimura H, Tanaka M, Shimizu S, et al: Laparoscopic treatment of congenital choledochal cyst. Surg Endosc 12:1268-1271, 1998