European Journal of Cardiovascular Medicine

Choledochal cyst (CC) is congenital dilatation of bile ducts. CC is rare in Western countries. The estimated incidence varies between 1 in 100,000 and 1 in150,000. The incidence is higher in Asia and occurs more in women, with a male to female ratio of 1:3–4.^1,2. Cystic or fusiform dilatation of the common bile duct is the most common form. The pathogenesis of choledochal cyst is difficult to explain. The common channel theory is more widely accepted but it doesn’t explain segmental nature or development of intra hepatic disease. So we can conclude that it has a spectrum of embryonic and pathologic origin. The treatment of CC is excision of cyst and establishing biliary enteric continuity. Traditionally excision and Roux-en-Y hepatico jejunostomy(HJ) has been gold standard in the treatment of choledochal cyst^{3, 4}. Other drainage procedures like hepaticoduodenostomy⁵ (HD), jejunal interposition⁶ have been described. Recently laparoscopic excision and hepaticoduodenostomy/Roux-en Y hepatico jejunostomy has become increasingly popular^7,8. Hepaticoduodenostomy believed to be more physiological, simpler to perform and associated with fewer complications like adhesive obstruction, leak and stricture etc.⁵. Recently some other authors have come up with contradicting results, who reported higher incidence of post operative complications with HD as compared to HJ ³.

 The purpose of this study is to share our experience with HD and HJ in 65 children with choledochal cyst operated in our institute over a period of 17 years and analyze the outcome and complications..

This study was conducted in department of Paediatric surgery in kurnool medical college, Kurnool. The outcome of 65 children with choledochal cyst who were operated during the period of 5 years by a single surgeon in two referral hospitals, (Government General hospital and Rainbow children hospitals, Kurnool) in southern India was done.

Patients with uncomplicated type1 and type IV choledochal cyst were included in this study. Complicated choledochal cyst like rupture, pancreatitis and type II, III and type V choledochal cysts were not included in the study. Children with CC who underwent procedures like lillys procedure, jejunal conduit were also excluded from the study.

 Study population is divided in to 3 Cohort groups. Group I includes children with CC who underwent excision and conventional HJ, Group II excision of CC and HD, Group III Laparoscopic excision of CC with HD. Initially total of 25 children underwent excision of CC and conventional Roux-en Y hepatico Jejunostomy and later on words we changed our technique to open hepatico duodenostomy in 20 patients. From then onwords we have shifted focus from open to laparoscopy. Laparoscopic excision of choledochal cyst and hepatico duodenostomy  was carried out in another 20 patients.

The mean operation time, post operative stay, early post operative complications like bleeding, biliary leak and late complication like biliary reflux, cholangitis, stricture, need for reoperation were analyzed. All 65 patients have come for follow up at least 3to4 times after surgery. The follow up period was ranging from 1year to 17 years. Upper GI endoscopy was done in all patients with HD, one year after surgery during follow up.

A total of 65 children were operated during the study period. Males were12 and females were 53. Male to female ratio was 1:4. Mean age was 4 years, ranging from 6months to 12 years. The commonest presentation was pain in epigastrium and right hypochondrium. Four children presented with pancreatitis before surgery. Classic triad of pain abdomen, jaundice and lump was present in only eight patients (16%). Two patients presented with ruptured cyst with biliary peritonitis, both of them underwent laparotomy, excision of cyst and HD. Twenty five children underwent open cyst excision and Roux-en Y hepatico jejunostomy. In another twenty open cyst excision and hepatico duodenostomy was done. Between 2017-2022 twenty children underwent laparoscopic cyst excision and hepatico duodenostomy. The mean operation time in HD group was 150mts, in HJ group 180 min. where as in laparoscopic group it was 240 min. Mean post operative stay in HD group was 6 ±2.5 days and in laparoscopic group 5±2.5 days, where as it was 8±2.5 in HJ group. One patient had post operative biliary leak in open HD group which subsided spontaneously by 4^th post operative day(complication rate 5%).One patient in HJ group (complication rate 5%) was re operated for adhesive obstruction. None of the laparoscopy patients had complications. Two patients in laparoscopy group required conversion to open surgery due to anomalous biliary tract. Both children had abnormal, long, dilated, tortuous cystic duct with very low insertion into common bile duct (just above duodenum), and it was mimicking choledochal cyst. Both underwent laparotomy, cyst excision and HD.  One child who was planned for HD was converted into HJ due to profuse, uncontrolled bleeding from duodenal incision due to ?AV malformations, so incision closed and HJ was  performed. One child had CC involving only rt hepatic duct. Laparoscopic duodeno rt hepatic duct anastomosis was done, (Fig 1-4) and left hepatic and common bile ducts were normal and left undisturbed. No problems during follow up after one year after surgery.Upper GI endoscopy showed asymptomatic biliary reflux into stomach in 2 patients of HD group. None of our patients in HD group suffered with biliary gastritis. There was no mortality. None of our patients had post operative cholangitis, bleeding, stricture   in either group.

TABLE 1

choledochal cysts are rare congenital malformations. The estimated incidence in Western countries varies between 1 in 100,000 and 1 in 1500001 where as the incidence is higher in Asia with reported incidence of I in 1000.9 .It is more common in female children with M: F ratio 1:4. The commonest presentation in our study was pain abdomen and the classic triad of symptoms were seen only in 16% of children and two of our children presented with rupture of cyst and biliary peritonitis. Similar observations were reported by Juan I. Menchaca”etal 10 who found that the triad of symptoms jaundice, pain, lump were present in 14.5% only and pain was the commonest symptom seen in 85.7%.

Open CC excision and HD was done in a total of 20 patient with mean operation time 150mts and mean post operative stay was 6 days. One patient had biliary leak which was subsided spontaneously by 4th post operative day. The complication rate was 5 %. If we also include 10 patients from lap group the complication rate comes down to 3.3%. Vijay patil etal11 analyzed  56 of their patients who underwent hepatico duodenostomy in their institute during the period between 1982 to 2007 and reported that 3 children had biliary leak in the early postoperative period and subsided spontaneously and only one child required  redo surgery for stricture 18 years after HD. They concludes that HD is simple to perform, with single anastomosis and it is safe with very low complication rate.

Open CC excision and Roux-en Y HJ was done during the period between January 2005 to December 2012 in 25 patients. The mean operation time was 180 min and mean post operative stay was 8 days. One patient required redo surgery for adhesive obstruction. The complication rate was 5 %. The mean post operative time was 150 min and mean postoperative stay 6 days in open HD group and in lap HD group it was 240 min and 5 days respectively (TABLE 1).  There was increased operative time and post operative stay in HJ group and no difference in the rate of complications when compared to open HD group. This is in comparison with Matthew T Santore etal 12 who have done retrospective analysis in 59 children with choledochal cyst.Thirty nine patients  underwent excision of CC and HD where as in 20 HJ was done. They have reported that children with HD required less operative time, tolerated diet faster, discharged early and associated with fewer number of complications when compared to HJ group.

Similarly Juan I. Menchaca”etal 10 reported hepaticojejunostomy(HJ) with 25% rate of complications versus hepatoduodenal (HD) anastomosis with 16.6%  rate of complications. The average hospital length of stay in the group of HD vs. HJ was 14 ± 1.6-days vs. 19 ± 8.2-days respectively.

Sarath Kumar Narayan etal13 in their large series of 412 patients with HD and 267 patient with HJ, showed higher incidence of biliary reflux/gastritis in HD group. We have done Upper G I endoscopy in all patient with HD, only 2 children showed asymptomatic reflux and none of our patients suffered with biliary gastritis. This low incidence in our children is due to the fact that we do kocherization of the duodenum and  hepatoduodenal anastomosis was done near the angle between 1st and 2nd part of duodenum so that hepatic duct is in direct continuity with vrtical  part of duodenum, and the bile is directed directly downward  rather than towards stomach.

Laparoscopic excision of CC and HD was done in 20 of our patients, the mean operation time was 240 min and average post operative stay was significantly low(P 0.0002) when compared to other groups. None of our patients in this group had biliary leak. There was no perioperative mortality. All patients underwent upper GI endoscopy during follow up two patients in laparoscopy group required conversion due to anomalous biliary tract. Both children had abnormal, long, dilated, tortuous cystic duct with very low insertion into common bile duct (just above duodenum), and it was mimicking choledochal cyst. Both underwent laparotomy, cyst excision and HD. In one patient HD was converted into HJ due to profuse, uncontrolled bleeding from duodenal incision may be due to? AV malformations, so incision in the duodenum was closed and open Roux-en Y hepatico jejuenostomy was  performed.   In another patient the choledochal cyst was present posterior to gall bladder fossa involving rt hepatic duct only rest of extra hepatic ducts were normal. Laparoscopic excision and duodeno rt hepatic duct anastomosis was done near hilum after kocherization of duodenum. The child did well postoperatively and is under regular follow up. This is in comparision with Fanny Yeung etal 14 who performed retrospective analysis of their 54 patient and reported that laparoscopic excision of CC with HD reconstruction is safe and feasible with better short-term outcomes and comparable long-term outcomes when compared to Roux-en-Y HJ reconstruction.

Hongeun Lee etal 15 compared laparoscopic with Robotic surgery in children with choledochal cyst and conclude that Robotic surgery allow more precise and secure sutures during anastomosis thereby reducing biliary complications. With expanding knowledge and expertise, robotic surgery may offer more advantages over laparoscopy in the era of minimally invasive surgery. None of our 20 patients with laparoscopy had biliary leak. We agree with Hongeun Lee etal that robo assisted surgery is more precise, technically easy but it is more expensive and available only in limited centers in India.

Our early results showed HD is good alternative to HJ and is associated with less operative time, short hospital stay, a smaller number of complications, with no significant biliary gastritis. Large prospective studies and longer follow up is required to support the above results

1. Lu S. Biliary cysts and strictures. In: Kaplowitz N, editor. Liver and Biliary Diseases. Baltimore, MD: Williams and Wilkins; 1996. pp. 739–753.
2. Lipsett P. Biliary atresia and cysts. In: Pitt H, editor. The Biliary Tract (Part of Clinical Gastroenterology). Vol. 11. London, UK: Balliere Kindall; 1997. pp. 626–641.
3. Shimotakahara A, Yamataka A, Yanai T, Kobayashi H, Okazaki T, Lane GJ, et al. Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy for biliary reconstruction during the surgical treatment of choledochal cyst: Which is better? Pediatr Surg Int. 2005;21:5–7.
4. Stringer MD. Wide hilar hepatico-jejunostomy: The optimum method of reconstruction after choledochal cyst excision. Pediatr Surg Int. 2007;23:529–32.
5. Todani T, Watanabe Y, Mizuguchi T, Fujii T, Toki A. Hepaticoduodenostomy at the hepatic hilum after excision of choledochal cyst. Am J Surg. 1981;142:584–7.
6. Narasimha Rao KL, Mitra SK, Kochher R, Thapa BR, Nagi B, Katariya S, et al. Jejunal interposition hepaticoduodenostomy for choledochal cyst. Am J Gastroenterol. 1987;82:1042–5.
7. Liem NT, Dung LA, Son TN. Laparoscopic complete cyst excision and hepaticoduodenostomy for choledochal cyst: Early results in 74 cases. J Laparoendosc Adv Surg Tech. 2009;19:87–90.
8. Srimurthy KR, Ramesh S. Laparoscopic management of pediatric choledochal cysts in developing countries: A review of 10 cases. Pediatr Surg Int. 2006;22:144–9.
9. Bhavsar MS, Vora HB, Giriyappa VH. Choledochal cyst: A review of literature. Saudi J Gastroenterol. 2012;18:230–6.
10. Menchaca JI, Guadalajara Jalisco, et al. Treatment of choledochal cyst in a pediatric population: A single institution experience of 15 years. Case series. Ann Med Surg (Lond). 2016;5:81–5.
11. Patil V, Kanetkar V, Talpallikar MC. Hepaticoduodenostomy for biliary reconstruction after surgical resection of choledochal cyst: A 25-year experience. Indian J Surg. 2015;77:240–4.
12. Santore MT, Behar BJ, et al. Hepaticoduodenostomy vs hepaticojejunostomy for reconstruction after resection of choledochal cyst. J Pediatr Surg. 2011;46:209–13.
13. Narayan SK, Chen Y, Narasimhan L, et al. Hepaticoduodenostomy versus hepaticojejunostomy after resection of choledochal cyst: A systematic review and meta-analysis. J Pediatr Surg. 2013;48(11):2336–42.
14. Yeung F, Fung ACH, Chung PHY. Short-term and long-term outcomes of Roux-en-Y hepaticojejunostomy versus hepaticoduodenostomy following laparoscopic excision of choledochal cyst in children. Surg Endosc. 2020;34(5):2172–7.
15. Lee H, Kwon W, Han Y, Kim JR, Kim SW, Jang JY. Comparison of surgical outcomes of intracorporeal hepaticojejunostomy in the excision of choledochal cysts using laparoscopic versus robot techniques. Ann Surg. 2018;94(4):190–5.