Choledochal cyst is solitary or several dilatations of a part of biliary channel, intra hepatic, extra hepatic, or intra and extra hepatic duct (1). Choledochal cyst is common in children, especially in females (2), three to four times higher in females (1). Incidence differs in communities, common in Asians (3).

Presentation of choledochal cyst varies in children and adults (4), children usually present with jaundice, abdominal pain and palpable mass (1, 5). In Adults obvious mass is unusual (1). The Majority of patients have history of vague right sided or upper abdominal pain, sometimes jaundice, cholangitis or pancreatitis (1). Choledochal cyst is characterized into five types according to Todani classification system. Type l is more common 80-90%, Type ll is 2%, Type lll 4%, Type lV 15-20% (6, 7). CT scan, MRI/MPCP, CTC are commonly used for diagnosis of choledochal cyst, with sensitivity of 90 – 100% (5). For prevention of complications in future like liver failure and carcinoma, proper treatment is crucial (6). Supportive care, complex endoscopic procedures, resections, even liver transplant are treatment options depending upon type of cyst (2). Excision of cyst and Roux en Y hepaticojejunostomy is ideal treatment (8), for extra hepatic type of cyst, type1 and type lV (9). Liver resection is performed in type V cyst and is customized to the limit of intra hepatic disease, considering the presence and severity of underling chronic liver disease and relevant kidney disease (9). As published data shows higher Prevalence of Choledochal cyst in Asia and women, most commonly presents in children. That’s why higher volume of data is needed about common types of choledochal cyst, proper management and post operative complications. Current study is coined to evaluate common types, their management and postoperative complications of choledochal cyst in a hepato-pancreato-biliary unit.

This retrospective cohort type of study was conducted in Gambat institute of medical sciences, Gambat, Sindh after approval from ethical committee of the hospital. we included all patients who underwent surgical treatment for choledochal cyst from January 2019 to December 2024 in HPB and liver transplant department, Gambat institute of medical sciences regardless of age and gender. Those patients were excluded from study whose medical record were incomplete or had lost follow up. Main presentations of patients were abdominal pain, jaundice, palpable abdominal mass, fever, and cholangitis. In six patient surgery was attempted before referral to our center. in three among these patients CBD exploration was performed and T tube was placed. and in three patients hepaticojejunostomy were attempted. Two patients among those in whom hepaticojejunostomy was performed, presented with anastomotic stricture and one patient presented with bile leak. Diagnosis and type of choledochal cyst was made with MRCP and CT scan, and type of cyst was confirmed by intra operative findings. Type of cyst characterized according to Todani classification. Routine investigations such as CBC, UCE, LFT, RBS, were performed in all patients. Patients who had presented with fever and cholangitis were treated first with antibiotics, initially antibiotics was given empirically, later on tailored according to culture and sensitivity. in those patients who did not respond to antibiotics, image guided drainage or ERCP was done, and surgery performed when fever settled down. Table 1: Details of demography, presentations, and treatment modalities Variables N (%) Gender Male Female 27 (33.3%) 54 (66.7%) Clinical Presentation Abdominal pain Jaundice Fever Cholangitis Palpable mass 80 (98.8%) 27 (33.3%) 12(14.8%) 14 (17.3%) 3 (3.7%) `` Type of cyst Type 1 Type 4 Type 5 50 (61.7%) 30 (37%) 1 (1.2%) Intervention ERCP Image guided drainage ERCP+IGD 11 (13.6%) 8 (9.9%) 4 (4.9%) Previous surgery Hepaticojejunostomy CBD Exploration and T Tube drainage 3 (3.7%) 3 (3.7%) Surgery: Surgery was the main treatment modality performed. Surgery was performed by senior consultant of our department. All pre operative and intra-operative aseptic measures were taken. Abdomen was opened via Right subcostal incision with midline upward extension. Excision of cyst done and reconstruction with Roux En Y hepaticojejunostomy was performed in all patients except one, where only anterior segmentectomy was done, in those cases where there was localized dilatation of intra hepatic biliary ducts, liver resection was performed and hepaticojejunostomy was done with right or left hepatic duct. bilioenteric anastomosis was carried out with 4 -5/0 proline or PDS in interrupted fashion. Entero-enterostomy anastomosis was done in continuous manner in two layers, inner layer with 3-4/0 PDS, and outer layer with 4-5/0 proline. Single drain was placed in right subhepatic region. Post-operative care: After surgery patients were normally admitted in HDU for 5 to 6 days, and followed for any post-operative complication such as fever, respiratory tract infection, jaundice, cholangitis, wound infection, bile leak, bleeding, reoperations, biliary stricture, malignancy and mortality. After discharge from the ward, follow up was done in OPD weakly for one month, then at 2nd 3rd and 6th and 12th month than annually. Data collection and Analysis: data was collected from electronic data entry system of the hospital and from review of files. Demographic information like age and gender, presentation, most common types, treatment modalities and postoperative complications of all those patients were collected who had undergone follow up for at least 1 year. Analysis of data was performed using SSPS, and results were expressed in frequencies and percentages. P value less than 0.05 was considered significant. Table 2: surgical procedures Surgery N (%) Roux En Y hepaticojejunostomy 78 (96.3%) Right Hepatectomy and Roux En Y hepaticojejunostomy 2 (2.5%) Anterior segmentectomy 1 (1.2%)

There were eighty-one patients in our study, 27 (33.3%) male and 54 (66.7%) female, with median age of 16 years ranging from 1 – 52 years and SD± 13.956. Pain was the only presentation in 54 (66.7%) patients. Second most common presentation was pain along with jaundice present in 12 (14.8%) patients, typical presentation of choledochal cyst that is pain, jaundice and mass was present in 3 (3.7%) patients. Demographics, presentations, types, and treatment modalities are given in table 1. Most common type in our study was type 1 choledochal cyst present in 50 (61.7%) patients, followed by type 4 was found in 30 (37%) patients, type 5 was found in a single patient (1.2%). Surgery was the main treatment modality in current study. Excision of cyst and Roux En Y hepaticojejunostomy was performed in all patients except one, where only anterior segmentectomy performed due to localized dilatation of anterior segment. in two (2.5%) patients right hepatectomy was carried out and hepaticojejunostomy was performed with left hepatic duct, in these two patients intrahepatic dilatation was limited to right lobe of liver. Surgical procedures are given in table 2. Duration of surgery, blood loss, and hospital stay are given in table 3. In our study 9 (11.1%) patients developed grade 2 or above complications, based on Clavien-Dindo classification system (10). 2 (2.5%) patients developed respiratory tract infection; these patients were treated with antibiotics. 3 (3.7%) patients developed wound infection, in these patients’ skin stiches opened, wound irrigation with normal saline and dressing were done daily, and antibiotic given according to pus culture and sensitivity. biliary leak occurred in 4 (4.9%) patients, these patients were treated with antibiotics and observation of drain, 2 (2.5%) of them responded to conservative treatment and bile leak stopped spontaneously, and in two patients re exploration was done and hepaticojejunostomy was revised, one of them expired due to sepsis from re leak, and in one of them patient duodenal perforation occurred, patient re explored second time and Graham omentopexy done and patient recovered. In 3 (3.7%) patients bleeding occurred, two (2.5%) patients presented with Malena and third patient presented with bleeding from draining tube. In all of these three patients re exploration was done, in two patients bleeding was from entero-enterostomy, so jejunojejunostomy anastomosis was revised, and in third patient bleeding was from hepaticojejunostomy site, so hepaticojejunostomy was revised. biliary stricture developed in 2 (2.5%) patients, and were treated with PTBD and dilatation. Single patient in our study expired. Complications are given in table 4.

Table 3:  duration of surgery, blood loss, hospital stay

Table 4:   Morbidity and Mortality

Multiple authors have reported the choledochal cyst more common in female with 4:1 female to male ratio (4, 11), some have reported 3:1 ratio (4). while we found predominance in female with almost 2:1 ratio, 66.7% females and 33.3% in males. Commonly presents with abdominal pain, obstructive jaundice and cholangitis (7), Pain was described most common presentation (11), we found same results, pain was present in almost all our patients 98.8%, Followed by jaundice (33.3%), fever (14.8%), and cholangitis (17.3%). Rarely presents with triad of pain, jaundice and abdominal mass in children (7), Omar j shah et al (12) observed it in 3.7%. while in our study 3 (3.7%) patients presented with typical triad of disease abdominal pain, jaundice and palpable mass that is almost equal to the above result. Commonest type of choledochal cyst is type 1 comprises 50-90% followed by type 4 comprising 30-40% according to todani classification (13). our study has same distribution, type l CC 61.7% and type lV 37%, and type 1 was 1.2%. There was not a single case of type ll, and type lll, choledochal cyst in our study. Our center is specialized HPB and liver transplant center and selected patients are referred to us, perhaps this is the reason for absence of other types. Treatment with antimicrobials is required in patients with cholangitis before surgery (14). we optimized the patients who presented with fever and cholangitis, using antimicrobial before definitive surgery. Roux En Y hepaticojejunostomy is procedure of choice for treatment of choledochal cyst (9, 15), we performed excision of cyst and reconstruction with roux en Y hepaticojejunostomy in 98.8% patients. Some authors favor liver resection in patients with type lV and type V cyst with segmental involvement to reduce complications of dilated intrahepatic ducts (14, 16). In our study two patients had dilatation of right intrahepatic duct along with extrahepatic duct involvement, in these patients we performed right hepatectomy in addition. In one patient there was dilatation of intrahepatic ducts of anterior sector alone, we carried out anterior segmentectomy alone.  Results were excellent in both of these patients. Risk of malignancy increases with choledochal cyst as compared with common people (17, 18). Incidence of malignancy increases with age, and is more common in type lV and V choledochal cyst (19, 20). Patients with malignancy due to choledochal cyst have poor prognosis (19, 21). Incidence of malignancy in histopathology report has been reported 4.3 – 9.2%  (18, 22, 23). While in our cohort malignancy was found in a single (1.2%) histopathology report. We referred the patient to oncology further management. post-operative complications have been reported in different literatures, 25% over all complication (24), Kevin C. Soares et al (11) in his research has narrated over all complication rate 28.7%, pulmonary complication 2.5%, wound infection 6.9%, cholangitis 4.3%, bile leak 5.6%, anastomotic leak 1%. Biliary stricture 0.8%, bleeding 0.8%, %, pulmonary 2.5%. in our study over all complication rate was 11.1%. comparatively better than the result of mention literatures. In our study Respiratory tract infection was noted in 2 (2.5%) patients, however this is more or less equal to the quoted result. We managed these patients with antibiotic.  Wound infection in our study was found in 3 (3.7%) patients, this is comparatively better than the result 6.9%. we irrigated the wound with normal saline before closure of skin, which resulted in decrease wound infection rate. Patients who developed wound infection were managed with opening of skin sutures, daily irrigation and dressing of wound as well as added antibiotics. In our study bile leak was noted in 4 (4.9%) patients, that is comparable to the quoted result of 5.6%. two out these 4 patients responded to conservative treatment. Two out of 4 patients with bile leak required re operation, hepaticojejunostomy was revised in these patients. In one from these two patients, duodenal perforation occurred, once again re explored and Graham’s omentopexy done, and patient improved. in second patient again bile leak occurred and expired from sepsis. In our study bleeding occurred in 3 (3.7%) patients, this is high than the result quoted, 0.8%. all of these patients were managed with reoperation, in two patients bleeding was from entero-enterostomy, anastomosis was revised in these patients, in third patient bleeding was from hepaticojejunostomy and anastomosis was refashioned. bleeding in this patient was periductal vessels. We perform entero-enterostomy anastomosis in continuous fashion with PDS 3 - 5/0, so giving up of anastomosis is possible. Break down or opening of notes may result in anastomotic break down and bleeding. we noted biliary stricture in 2 (2.5%) patients. In both of these patients’ surgery was attempted and hepaticojejunostomy was performed before referral to our center. Though after revisional surgery they developed anastomotic stricture. We could not find any reason for stricture formation. Although we managed these patients with PTBD and dilatation. Moslim et al (4) in his publication has shone re exploration rate of 16% and mortality rate 7.5%, whereas in current study re exploration was required in 5 (6.2%) patients, and mortality 1.2% recorded. We found association of overall complications with previous attempted surgery (p 0.O16). we reported association of stricture (p <001), respiratory tract infection (p 0.002) wound infection (p 0.021), bleeding (p 0.021) with previous attempted surgery.

choledochal cyst a complex disease, its presentations and treatment options vary, and malignant potential, surgery is recommended as early as diagnosis is confirmed, to reduce complication rate.

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