Saudi Surgical Journal

: 2019  |  Volume : 7  |  Issue : 4  |  Page : 138--142

Experiences of choledochal cyst in pediatric and adult population: A case series

AK Roy1, Sanjay Kumar Bhat2, Rahul Bhushan1, Nisar Ahmed Ansari1, PK Mishra3, Maham Ahmad1, Nikhil Mehrotra1, CS Rawat1,  
1 Department of Surgery, Era Medical College, Lucknow, Uttar Pradesh, India
2 Department of General Surgery, Dr. Ram Manohar Lohia Institute of Medical Science, Lucknow, Uttar Pradesh, India
3 Department of Health and Bio Statistics, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Correspondence Address:
Sanjay Kumar Bhat
Associate Professor, Department of General Surgery, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow


Introduction: Choledochal cysts are a relatively uncommon disease of the bile duct which is the cystic dilatation of the intra- or extrahepatic bile ducts. Literature is scarce on its clinicopathological behavior among children and adults and its comparison. Methods: The retrospective study was aimed to study the clinical characteristics, management, and complications between pediatric and adult patients with choledochal cysts. Results: There was higher female preponderance with male to female ratio of 3:17 in pediatric age group while 1:4 in adult age. Abdominal pain was more common among adults, while palpable mass was more common among pediatrics population (P < 0.05). Jaundice was more evident in the pediatric age group, yet the classic triad of choledochal cyst (abdominal pain, jaundice, and a palpable mass) was not observed in any age group. About 76% of the cysts were type 1 cysts, which was more common among pediatric age group (40% vs. 90% P < 0.05) while with adults presented more with a type IVA cyst (60% vs. 10% P > 0.05). No patients with type II, type III, type IVB cysts, or type V were found. Sixteen patients underwent Lilly technique, with resection of the choledochal mucosa and Roux-en-Y hepaticojejunostomy, while nine patients underwent resection of the choledochal cyst and Roux-en-Y hepaticojejunostomy. Patients who received total excision had fewer surgical complications in both groups. Conclusion: Although there is a significant difference in the clinical characteristics of choledochal cysts between children and adults, yet early detection and necessary surgery is essential for patients with choledochal cysts.

How to cite this article:
Roy A K, Bhat SK, Bhushan R, Ansari NA, Mishra P K, Ahmad M, Mehrotra N, Rawat C S. Experiences of choledochal cyst in pediatric and adult population: A case series.Saudi Surg J 2019;7:138-142

How to cite this URL:
Roy A K, Bhat SK, Bhushan R, Ansari NA, Mishra P K, Ahmad M, Mehrotra N, Rawat C S. Experiences of choledochal cyst in pediatric and adult population: A case series. Saudi Surg J [serial online] 2019 [cited 2020 Aug 15 ];7:138-142
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Full Text


Choledochal cysts are congenital conditions involving cystic dilatation of bile ducts. It is a fairly uncommon anomaly of the biliary system.[1]

Its incidence is 1:100,000–1:150,000 live births in the West while it is 1:1000 in Asia with two-third of cases reported from Japan[2] with female preponderance.[3]

This surgical illness usually manifests with classic triad of intermittent abdominal pain, jaundice, and a right upper quadrant abdominal mass. It usually leads to obstruction of the bile ducts and retention of bile. Although it is usually found in infants and children yet can go undiagnosed and may manifest in adulthood, about 60% of cases diagnosed in the first decade of life.

The importance of choledochal cysts lies in the lethal complications such as biliary stasis, cholangitis, cholelithiasis, pancreatitis,[3],[4],[5],[6] and malignant transformation.[7],[8],[9],[10],[11] Even with adequate surgical treatment, long-term complications develop and include anastomotic stricture, cholangitis, biliary cirrhosis, and biliary tract malignancy.[4],[12],[13]

To date, complete excision of the cyst and bilioenteric anastomosis is the treatment of choice.[14] with various types of drainage procedures such as Roux-en-Y hepaticojejunostomy, hepaticoduodenostomy, and jejunal interposition hepaticoduodenostomy.[15] However, laparoscopic excision of choledochal cyst and hepaticoduodenostomy is advised.

The lower incidence of this disease among the adults in western literature and variability in the clinical presentation and outcomes between pediatrics and adult population,[4],[5] the direct comparison between the two is lacking in literature.[16] Thus, the study was aimed to identify the differences in presentation, types, management, and outcomes in both the groups.


We conducted a retrospective analysis of 25 patients with choledochal cyst operated at Era's Lucknow Medical College and Hospital (ELMCH) from January 2011 to December 2016. The study was ethically approved by institutional review board in accordance with the ethical standards of the responsible committee on human experimentation (institutional or regional) and with the Helsinki Declaration of 1975, as revised in 2000.

For inclusion in the study, we considered patients of both genders, aged between 6 months and 60 years, whose surgery was performed at the Department of Surgery of ELMCH and attending at least three visits postoperatively.

We analyzed the demographic data, presenting complaints, age at which the diagnosis was made, applied surgical procedures, complications, anatomical classification of cysts, and postoperative outcomes.

Of the 25 cases identified, all patients had complete treatment and follow-up records for analysis. All patients received surgical treatment, Lilly technique, with resection of the choledochal mucosa and Roux-en-Y hepaticojejunostomy in 16 while in another 9 patients, resection of the choledochal cyst and Roux-en-Y hepaticojejunostomy was carried.

Statistical analysis of data was performed using Statistical package for social sciences, version-23 (SPSS-23, IBM, Chicago, USA) and MedCalc statistical software were used for data analysis. Statistical significance was set at P < 0.05.


Among the 25 patients, male to female ratio was 3:17 in the pediatric age group, whereas 1:4 in adult age group with higher female predominance in both the groups. Majority of the cases were found among females <18 years of age with 75% of patients being <10 years.

Clinical symptoms, operative findings, and the Todani classification are shwon in [Table 1] and [Table 2].{Table 1}{Table 2}

Clinical symptoms

In the pediatric age group, the most common clinical manifestation was abdominal pain in 95% of cases (19 patients), followed by jaundice, present in 30% of patients (6 cases). Five patients had a palpable abdominal mass (25%).

In adults, pain abdomen was common presentation among all the patients (100%). Only one patient had palpable abdominal lump, while none had jaundice. Thus, abdominal pain was more common among adults while palpable mass was more common among pediatrics population (P < 0.05).

Acute cholangitis was present in 1 child (5%).

The classic triad of choledochal cyst (abdominal pain, jaundice, and a palpable mass) was not observed in any age group.

Ultrasonography (USG) of the abdomen was done in all patients, followed by contrast-enhanced computed tomography (CT).

The mean interval between diagnosis and surgery was 3 weeks.

Cyst type

About 76% of the cysts were type 1 cysts with adults presented with type l less commonly (40% vs. 90% P < 0.05). No patients with Type II, Type III, Type IVB cysts, or Type V were found. Adults were more likely to present with a Type IVA cyst (60% vs. 10% P > 0.05) [Table 2].

Surgical treatment

In 16 patients, we applied the Lilly technique, with resection of the choledochal mucosa and Roux-en-Y hepaticojejunostomy. In nine patients, we performed resection of the choledochal cyst and Roux-en-Y hepaticojejunostomy. All adult patients underwent choledochal cyst resection and Roux-en-Y hepaticojejunostomy without exception.


Two patients (10%) had immediate postoperative complications, one in pediatric age group and one in adult age group, both having a biliary leak from the bilioenteric anastomosis, which were managed successfully by conservative means.

Two patients (10%) both in adult age group developed acute cholangitis out of which one was managed successfully by IV antibiotics and supportive care while other patients expired.

One patient died of postoperative complication after developing acute severe cholangitis.

All patients had a grossly normal-appearing liver at laparotomy.

None of the excised cysts had malignant degeneration or metaplasia at histopathology.

All patients received antibiotic cover for 1 month and ursodeoxycholic acid for 3 months.

The mean duration of postoperative follow-up was 30 months.

All patients showed clinical improvement, with no evidence of jaundice, biliary stricture, or any malignant transformation.


Choledochal cysts are congenital malformations of bile ducts that represent a major diagnostic and therapeutic challenge for the surgeon, demanding a high degree of suspicion and efficient etiological investigation for the correct diagnosis and institution of appropriate treatment.[17]

The treatment should consist of resection of the dilated portion of the extrahepatic biliary tree with reconstruction with a Roux-en-Y hepaticojejunostomy. When intrahepatic dilatation is very extensive, liver transplantation is an alternative.

We report our experience about the clinicopathological differences between pediatric and adult patients with choledochal cysts in our institute. The classic triad of jaundice, abdominal pain, and abdominal mass was often seen in pediatric patients than in adults in the previous study[14],[15] was not evident in our study.

There is no consensus in the literature about the most common clinical presentation. Some authors believe abdominal pain to be the main signal, similar to the result obtained in this sample. Other studies, however, report that jaundice is the most prevalent symptom.[18],[19]

We observed more of abdominal pain as the chief complaints in children and adults while abdominal mass of classical presentation more among children. Jaundice was more evident among children, as was the observation from others in literature.[16] Lilly argued that jaundice should be more commonly seen in infants and abdominal pain in older patients, probably due to the better capacity to verbalize.[19]

The observation of the predominance of female patients in this sample, consisting of 85%, is consistent with other studies on choledochal cysts.

The age at which the diagnosis was made, with the majority of patients in the first decade of life, agrees with other reports, where 80% of cases are diagnosed before 10 years of life, whereas only 20% of common bile duct cysts are seen in adulthood.

Although abdominal pain, jaundice, and abdominal mass are described as the classic triad of choledochal cyst, their association was not observed in this study, in disagreement with data reported by other authors.[10]

Although the concept that this presentation would be more frequent in children than in adults, it is believed that early diagnosis in the current era would prevent the choledochal cyst from greatly increasing in volume. None of our patients had any associated complications like stricture or stones except 1 having cholangitis. This largely depends on the type of cyst and to the duration and severity of biliary stasis.

Currently, the preoperative diagnosis of choledochal cyst is mostly done by the USG, which has high sensitivity for the diagnosis of biliary tract disease. It was the first complementary method used in all our patients and in other studies as well.[20] In all seven patients who underwent magnetic resonance imaging (MRI) the diagnosis was correctly achieved, demonstrating that this method is a more sensitive diagnostic tool for the evaluation of diseases of the biliary tract than CT [Figure 1].{Figure 1}

Three-dimensional reconstruction of MRI cholangiopancreatography allows better anatomical evaluation of biliary tract and pancreas. Another innovation is the virtual cholangioscopy, which allows preoperative radiological exploration of the bile ducts.[12]

According to medical literature, choledochal cysts type I correspond to up to 90% of all cases, confirming the demonstrated in this series, where the majority of cysts (85%) represented a fusiform dilatation of the common bile duct. Choledochal cysts in children were predominantly type I cystic lesions (18 out of 20), whereas type IV cysts were more common in adult patients (3 out of 5).

The current treatment is total resection of the cyst since 1980s; however, associated complications and accompanying malignant disease alter the further management in terms of extension of resection, reconstruction method, and need for hepatic resection. Thus, the type of choledochal cyst becomes the most critical variable. Fortunately, our patient population were of Type 1 and Type 4 and underwent bilioenteric anastomosis associated with resection of the mucosal lining of the cyst, the Lilly technique, in 16 patients and Roux-en-Y hepaticojejunostomy was performed in 4 patients with low incidence of postoperative complication [Figure 2], [Figure 3], [Figure 4].{Figure 2}{Figure 3}{Figure 4}

In this series, the low incidence of postoperative complications and good clinical outcome suggests that it is technically possible to safely perform bilioenteric anastomosis, as previously described.


Given all these data, we conclude that abdominal pain in childhood must remain a warning sign for congenital malformations of the biliary tract. Choledochal cysts in children were predominantly type I cystic lesions, whereas type IV cysts were more common in adult patients. The surgical treatment of choledochal cysts, with resection and hepaticojejunostomy, is the treatment of choice and is safe even in young children even when symptoms are minimal. Patients who received total excision had fewer surgical complications in both groups. Diagnosis and treatment should be early to avoid a greater involvement of the hepatic parenchyma, whose severity depends on the degree of obstruction and time course.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


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