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Conventional and unconventional surgical modalities for choledochal cyst: Long term follow up

Anshuman Sharma, Anand Pandey, Jiledar Rawat, Intezar Ahmed , Ashish Wakhlu, Shiv Narain Kureel
Department of Pediatric Surgery CSM Medical University Lucknow, India

 

Correspondence

Dr Anand Pandey
Department of Pediatric Surgery
CSMMU (erstwhile King George’s Medical University).
Lucknow 226003, U.P. India.
Phone- +91522 2257825
Email: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Background: This paper presents our experience of evaluation of various modes of presentation, various surgical methods both conventional and unconventional applied and their outcome on the patients of choledochal cyst.

Method: All patients of choledochal cyst were studied over a period of 10 years (1997-2007). These patients were analysed retrospectively. Data included age, sex, modes of presentation, various surgical techniques, outcome and follow up.

Results: Total number of patients was 35. The most common mode of presentation was recurrent abdominal pain in 20 (57%) patients. It was followed by jaundice in 16 (45%), fever, nausea, vomiting in 10 (28.5%), and others. Roux en Y hepaticojejunostomy was performed in 26 (74%) patients, hepaticoduodenostomy in five (14%) patients, external T-tube drainage in three (8%) patients and cystoduodenostomy in two (5%) patients. Two patients expired in this series. The follow up loss was 20%. Three patients who suffered from cholangitis were managed conservatively. No patient has shown cirrhosis and cholangiocarcinoma up till now.

Conclusion: Choledochal cyst is an important entity in Indian setup. The presentation has a wide spectrum; therefore, different types of surgical intervention would play a significant role in various situations.

Key words: choledochal cyst, surgical techniques, follow up of choledochal cyst.

 

Introduction

Choledochal cyst (CC) is a congenital biliary tract anomaly that may involve extrahepatic, intrahepatic or even both biliary radicals [1]. The disease is characterized by various types of abnormal biliary tract dilatations, which formed the basis of its classification. Initially given by Alonso-Lej et al., and subsequently modified by Todani, this disease is more prevalent in Asian countries [1, 2].

The females, according to literature, show eight times more predominance of the disease than males [1]. Even though prenatal ultrasonography (US) of fetal choledochal cyst has been reported by a number of investigators, the majority of these patients present during childhood, which makes this disease crucial to be diagnosed and managed promptly [1].

This paper shares our experience of various modes of presentation, the different surgical modalities both conventional and unconventional applied and their effect on the overall survival of these patients.

Material and methods

In this retrospective study, all patients of CC admitted from January 1997 to Dec 2007 in the department were included. The patients were analysed on the basis of age at presentation, sex, mode of presentation, diagnostic modalities utilised, types of surgical intervention applied and their outcome on follow up.

All patients were evaluated clinically, supplemented by radiological interventions like US abdomen, computerised tomography (CT) abdomen, MRCP and HIDA scan. They were followed up in the same manner on their every visit.

The various procedures used in our series were Roux-en-Y hepaticojejunostomy, hepaticoduodenostomy, cystoduodenostomy and external T-Tube drainage. Cystoduodenostomy was done in those patients who had evidence of cirrhotic changes of liver at the time of surgery. If common hepatic duct was long enough to be anastomosed with the duodenum, hepaticoduodenostomy was performed. The external T-Tube drainage was performed in patients of ruptured choledochal cyst. In the postoperative period, the patients were kept nil per os (NPO) for five to seven days. After discharge, the patients were called up in the out patient department (OPD) after 15 days. Liver functions were assessed at that time and after two months. HIDA scan was performed on any evidence of obstruction such a raised bilirubin etc. If the patients had no problems they were called after six months or they themselves consulted for any problem, if it arose. Cholangitis was suspected on clinical evidence of fever and jaundice, supplemented by the liver functions. It was treated by antibiotics.

Results

The total duration of this study was 10 years. The total number (n) of patients was 35. CC was found more commonly in the age group of 1-5 years (n=23), followed by 6-10 years of age (n=9), with only 3 cases (n=3) recorded in less than six months of age. Mean age was 4.39 ± 2.58 years. Male to female ratio was approximately 2:1.

Clinically, recurrent abdominal pain was present in 20 (57.14%) patients. Other complaints noticed were jaundice, fever, nausea and vomiting, palpable lump in abdomen, peritonitis, and ascitis (table 1). Todani types 1, 2 and 4a were encountered in our series. Out of these, type 1 was found to have the maximum occurrence in our series (table 2).

Table 1: Modes of Presentation of patients of choledochal cyst

Signs and Symptoms

No. of Patients n (%)

Recurrent Abdominal Pain

20 (57.14%)

Jaundice

16 (45.7%)

Fever, Nausea, Vomiting

10 (28.57%)

Lump in abdomen

5 (14.28%)

Peritonitis

3 (8.57%)

Ascitis

2 (5.71%)

Table 2: Types of Choledochal cyst:

todani types

No. of patients n (%)

Type 1

28 (80%)

Type 2

4 (11.42%)

Type 3

0

Type 4a

3 (8.57%)

Type4b

0

Type5

0

Roux en Y hepaticojejunostomy was the most common operation performed. It was followed by hepaticoduodenostomy, cystoduodenostomy and external T-tube drainage (table 3). Follow up loss was approximately 20%. Two patients (5.71%) expired in our series.

Table 3: Surgical Modalities utilised for treatment of Choledochal cyst

type of Surgery

No. of Patients n (%)

Roux en Y Hepaticojejunostomy

26 (74.28%)

Hepaticoduodenostomy

5 (14.28%)

Cystoduodenostomy

2 (5.71%)

External T-Tube drainage

3 (8.57%)

Complications noticed were cholangitis in three patients, two of them in Roux en Y hepaticojejunostomy and one in cystoduodenostomy. All of these were managed conservatively. Another single case of type 1 Choledochal cyst, after undergoing Roux en Y hepaticojejunostomy, had presented in emergency, 1 week after discharge, with sub acute intestinal obstruction, for which exploratory laparotomy was performed and was found to have terminal ileal gangrene, distal ileum was exteriorised as ileostomy, which was later repaired with resection and anastomosis. Etiology for such an event could not be found as the histopathology report came out to be inconclusive. Mean follow up period was 2.83 ± 1.78 years.

Discussion

Vater had first described choledochal cyst with studies of normal and abnormal anatomy of biliary tree [1]. Later, Alonso-Lej et al. had revolutionised this entity by classifying it into three types, which was subsequently modified by Todani [1].

In our study, most common age group was 1-5 years, which corresponds with other studies. Males predominated in our series, while in other studies females had accounted for 80% of all cases [1, 3, 4]. The reason for this observation could not be ascertained. It may be due to the more care given to male children in this part of northern India. Though pain was the most common presentation in our series, jaundice has prevailed in other studies [1, 3]. The classical triad of pain, jaundice and lump is an uncommon entity, and was found in three patients, which corresponds with other studies [3, 5, 6]. Biliary peritonitis due to cyst rupture, although a rare entity in some studies [7, 8] needs emergency treatment. It can be diagnosed by US-abdomen [8] or DISIDA scan [7]. Todani type 1 was found in 28 patients, in which cystic and fusiform varieties occurred with equal frequencies in both sexes and all age groups unlike other studies where cystic varieties predominated [9]. Roux–en-Y hepaticojejunostomy was the most commonly performed operation in our series just like other studies [1, 3, 4, 6, 10-13]. Sometimes, there is laxity of the fascial planes created due to the space occupied by the lump [5]. In these cases, hepaticoduodenostomy can be easily performed as duodenum can be mobilised. There can be concerns of cholangitis in these patients but it has not been noticed up till now.

Biliary peritonitis due to ruptured choledochal cyst can be diagnosed on US abdomen on a basis of high suspicion. These patients were initially managed by External T tube drainage and later repaired with Roux-en-Y hepaticojejunostomy as a definitive procedure. We did not do Percutaneous Transhepatic Cyst (PTC) drainage, which was used as a bridge procedure [14]. We believe it to be invasive and was not attempted in our patients. Cystoduodenostomy, which has not been used much in the past, was also performed on two patients similar to other studies [5] that had presented with ascitis and on US were found to have some cirrhotic changes in the liver. Surprisingly, one patient is still following up and is doing well while the other was lost to follow up. Importantly, there has not been a single follow up evidence of cirrhosis or cholangicarcinoma noted till date [12].

By our study, we emphasise that, although, Roux-en-Y hepaticojejunostomy is the gold standard procedure, there are some patients, which would present to us in other ways and make the other procedures significant to provide a better management and outcome of CC. To conclude, CC is an important entity in Indian setup. Mostly, it presents in age group of 1-10 years. The presentation has a wide spectrum; therefore, different types of surgical intervention would play a significant role in various situations. Follow up forms an important part to look for and treat the possible complications.

 

 

 

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