Choledochal cyst is a cystic dilatation of the bile ducts, both intrahepatic and extrahepatic, which causes biliary obstruction and progressive biliary cirrhosis. Cystic dilatation of the bile ducts, especially choledochal cysts, is a rare disorder in the Western world, but in East Asia and Southeast Asia it is relatively more common [1].

Cases of choledochal cysts are relatively rare in Western countries, around 1 cases in 100,000-150,000 to 1 case in 2 million live births. The prevalence of choledochal cysts is more common in Asian countries, where 33-50% of reported cases occur in Japan, reaching 1 case in 1000 population [2]. Choledochal cysts are more common in women than men, with a comparison female and male is 3:1 to 4:1. These cases can be found at any age, but almost 67% of cases with these signs are found before the age of 10 years. In this study, we will discuss the surgical management of choledochal cysts [3].

Case Report

A 9 year old boy came to General Hospital Dr. Moewardi with the chief complaint of a lump in the abdomen. The patient was a referral from the Karanganyar Regional General Hospital, with complaints of a lump in the patient's abdomen. Patient complaints are also accompanied by yellow eyes. History of vomiting (+) containing white mucus, fever (+). Urination is thick yellow like tea, pale white stools. Because the complaint did not go away, the patient's family was taken to the Karanganyar Hospital, the patient was treated by the Pediatrics Department. However, due to limited facilities, the patient was referred to the Dr. Moewardi General Hospital, the patient was given initial treatment in the form of O2 and infusion. The patient was then examined for blood, and a chest X-ray by the pediatrics department. From the pediatrics department, the patient was consulted to the pediatric surgery Department.

From the results of physical examination found blood pressure 125/75 mm Hg, heart rate 134x / minute, temperature 37.8 ^°C, respiration rate 30 times / min and SpO2 100%. Based on the results of the blood examination, it was found that Hb 10.5 g/dL, Hematocrit 33%, thrombosis 114 thousand/uL, MCV 73.3, MCH 23.4, SGOT 248 u/L, SGPT 193 u/L, total bilirubin 6.96 mg/dL, indirect bilirubin 1.64 mg /dL. The results of the ultrasound examination revealed a dilated intrahepatic bile duct. From the results of the CT scan, it was found hepatomegaly, inhomogeneous liver parenchyma with nodular lesions in the 6th segment of the right lobe of the liver and cysts in the head of the pancreas with suspected liver malignancy. The results of MRCP showed multiple occlusions in the cholecoccal duct and ampulla of vater, intra and extra hepatic biliary dilatation and cysts in the head of the pancreas.

Based on the results of physical examination and support, a diagnosis of choledochal duct cyst was obtained. The patient was then indicated for an exploratory laparotomy. The patient then underwent biliodigest RouX-en-Y laparotomy and biopsy. The procedure was carried out with the first procedure, the patient was positioned in a supine position, then after being anesthetized using general anesthesia, the patient was incised supra-umbilically about 8 cm and deepened to the peritoneum. The peritoneum is opened and the gallbladder, ductus choledocus and hepatomegaly are identified. The ductus choledocus was opened and a cyst was found, then cystectomy and liver biopsy were performed. RouX-en-Y anastomoses were performed, the jejunum with the common hepatic ductus end to side, and the jejunum with the jejunum end to side. After that, wash the abdominal cavity with 0.9% NaCl, suture the lining of the peritoneum and the operation is complete.

There is no strong theory that states the pathophysiology of choledochal cysts. Pathogenesis is likely multifactorial. In some patients with choledochal cysts, there is an anomalous association between the common bile duct and the pancreatic duct. This occurs when the pancreatic duct drains fluid into the common bile duct more than 1 cm proximal to the ampulla. This abnormal fusion causes pancreatic secretions to enter the common bile duct, where pancreatic proenzymes become active, which can damage and weaken thewall bile duct. In addition, other causes are defects in epithelialization and recanalization of development bile duct and congenital weakness of the duct wall. This also causes the occurrence of choledochal cysts [4]. 

The triad of pain, intra-abdominal mass, and obstructive jaundice indicates the possibility of choledochal cysts. Laboratory tests show abnormalities due to bile duct obstruction, especially elevated alkaline phosphatase levels. Regardless of the shape of the anatomic abnormality, radiological examination is the key in establishing the diagnosis. Computed tomography (CT) cholangiography, formerly used as an adjunct in the diagnosis of common bile duct cysts, is now being replaced by a more accurate examination [3].

The preferred treatment for the treatment of bile duct cysts is complete excision with cholecystectomy and reconstruction with Roux-en-Y hepaticojejunostomy. In the previous year, the patient was often treated without excision with anastomoses of the cyst to the jejunum, duodenum or stomach. Internal drainage procedures result in high rates of stenosis, lithiasis, cholangitis, and reoperation and fail to address the premalignant nature of these lesions. Currently, cyst excision can be performed with a lower morbidity and mortality rate compared to previous operations with internal drainage. Unfortunately, when the cystic process involves multiple intrahepatic and extrahepatic, complete excision may not be feasible. In these circumstances, partial excision combined with drainage of the remaining abnormal ducts may be the only solution [1].

Type 1 cysts are exposed by mobilizing the hepatic flexure from the colon down and Kocherize the duodenum. The locations of the hepatic artery and of each right hepatic artery originating from the superior mesenteric artery were identified. Intrahepatic fluorocholangiography was performed to confirm the anatomy of the proximal duct and pancreaticobiliary junction. Cholangiography can be obtained by means of a cystic duct or direct cyst puncture, or, if the cyst is large, opened by placing an appropriately sized balloon catheter for injection of the proximal and distal ducts. Intraoperative endoscopy can be used to examine the proximal biliary tract to look for stenosis or debris (Koshinaga, Ohashi, Ono, Kaneda, & Furuya, 2018).

Type 1 cysts must be completely excised. The surgeon must resist the temptation to leave too much of the proximal and distal ductus remaining. Distally, the resection is carried down into the pancreas and there are two notes to be noted. First, if the resection is taken too far, the main pancreatic duct may be affected. It is usually not possible to see the pancreatic duct and the cyst is often very narrow near its border. Second, the distal bile duct must be monitored to prevent postoperative pancreatic fistula, which is prone to occur if the patient has previously had an abnormal pancreaticobiliary junction. The distal duct may be small and inappropriate suturing may occlude the pancreatic duct [4]. 

The proximal resection should be extensive until the mucosa is normal. An anastomosis of granulation tissue or ulcerated mucosa will produce stricture. Leaving the proximal periphery of the rest of the cyst so that the anastomosis will be wider or easier to form is a wrong concept. The right hepatic duct and especially the left hepatic duct can be incised (after the hilar plate has been opened) to provide the perfect length for the anastomosis. The standard reconstruction after cyst excision is a Roux-en-Y hepaticojejunostomy with 40-60 cm Roux branches. Roux branches are shorter for babies (15-20 cm) or children (30-40 cm). Techniques have included the creation of a valve in the branch of the small intestine and placement of a duct between the bile duct and the duodenum [5].

Type 2 cysts are rare. When encountered, the treatment is by excision of the cyst. If an anomaly of the pancreaticobiliary junction is present, biliary diversion with a Roux-en-Y hepaticojejunostomy may be necessary to prevent the continuation of pathogenic pancreaticobiliary reflux. Type 3 cysts (choledochoceles) are also rare and near the trans duodenum. Because there is no uniformity regarding the pathogenesis, classification, anatomy, and clinical, treatment is individualized. Endoscopy and sphincterotomy may be sufficient for patients who have small cysts in the absence of duodenal obstruction. In other circumstances, trans-excision of the duodenum with sphincteroplasty or ductal reimplantation has been performed. Type 4 cysts involve several parts of the duct. For cysts confined to the extrahepatic ducts are treated by complete excision, similar to type 1 cysts. For cysts involving both the intrahepatic and extrahepatic ducts which are problematic because complete excision may not be short of total hepatectomy. This condition is usually treated by resection of the extrahepatic component with a Roux-en-Y hepaticojejunostomy at the hilum of the liver. Intrahepatic strictures can be dilated. If intrahepatic disease is limited to one lobe, hepatic resection can be performed [6].

Surgical management of patients with type 5 disease involving the intrahepatic bile ducts should depend on the individual's extent of the anatomy and function of the liver. One lobe involvement is effectively treated with hepatic resection. Liver transplantation is the definitive therapy for patients who have diffuse disease, liver cirrhosis, or associated malignancy. For patients who do not have cirrhosis, drainage with biliary anastomoses, transhepatic stent placement and their combination may help control symptoms [7].

Outcomes of cyst excision and hepatic enterotomy in children can be excellent. In a series of 180 cases of children followed for a median of 11 years, only 2.3% developed complications of cholangitis and duct stones. Cyst excision in adult patients can be performed with low mortality, although the morbidity rate is 20% or more. After complete excision, approximately 10% of adult patients develop recurrent cholangitis, pancreatitis, or chronic liver disease, and there is a small but limited risk of malignancy. For this reason, long-term follow-up is highly recommended [8].

Complications of choledochal cysts are biliary obstruction, cholangitis, liver abscess, rupture and malignant changes. The probability of malignant change is 20 times and the risk of malignancy increases with age. When the patient is pregnant, the cyst may rupture. It is not uncommon for gallstones to be present in the cyst [8].

From the literature, it is stated that complications after cyst excision, both early and late, can occur, such as cholangitis, stone formation, anastomotic stricture, pancreatitis, liver dysfunction and malignancy. The phenomenon of stone formation after surgery was first described by Tsuchida et al. Uno and colleagues, in their research on intrahepatic stones that occur after cyst excision, explained that strictures always occur as an initial event. Cetta also reported that stasis of bile due to stricture of the duct is a precursor to, not following, formation of intrahepatic stones. There have been many reports of malignant degeneration either due to retained cysts or as a result of chronic inflammation that occurs due to reflux of pancreatic enzymes due to weakness of the sphincter of Oddi function which causes histological changes and progression towards malignancy. Acute pancreatitis is a complication that occurs in 20% of cases at long-term follow-up due to protein plug formation [9]. The prognosis after choledochal cyst excision is usually good. Patients require long-term monitoring due to an increased risk of cholangiosarcoma, even after complete excision has been completed [2].

Choledochal cyst is a cystic dilatation of the bile ducts, both intrahepatic and extrahepatic, which causes biliary obstruction and progressive biliary cirrhosis, with clinical symptoms such as jaundice, pain and fever. Choledochal cysts are more common in women than men. The diagnosis of choledochal duct cyst can be seen from the symptoms and also radiological examination in the form of ERCP and MRCP. A Roux-en-Y hepaticojejunostomy is needed to manage patient with choledochal cyst. Complications of choledochal cysts are biliary obstruction, cholangitis, liver abscess, rupture and malignant changes. The probability of malignant change is 20 times and the risk of malignancy increases with age.

Acknowledgment

The authors of this case report would like to thank Department of Surgery Dr. Moewardi General Hospital Surakarta for providing support and information for this study.

Conflict of Interest

No potential conflict of interest relevant to this study was reported

1. Kazaryan, A.M., et al. “Does Tumor Size Influence the Outcome of Laparoscopic Distal Pancreatectomy?” HPB, vol. 22, no. 9, 2020, pp. 1280–1287. https://doi.org/10.1016/j.hpb.2019.11.014.

2. Hurník, P., et al. “Another Case of Coincidental Giardia Infection and Pancreatic Cancer.” Parasitology International, vol. 71, 2019, pp. 160–162. https://doi.org/10.1016/j.parint.2019.04.013.

3. Akaydin, M., et al. “Importance of Spontaneous Choledochoduodenal Fistulas Detected During ERCP Procedure.” The Indian Journal of Surgery, vol. 80, no. 3, 2018, pp. 216–220. https://doi.org/10.1007/s12262-016-1569-8.

4. Bouvry, M., et al. “Acute Pancreatitis Complicated with Choledochal Duct Rupture.” Case Reports in Gastrointestinal Medicine, 2011, article 413268. https://doi.org/10.1155/2011/413268.

5. Pinon, M., et al. “Not Only Alagille Syndrome: Syndromic Paucity of Interlobular Bile Ducts Secondary to HNF1β Deficiency.” Italian Journal of Pediatrics, vol. 45, no. 1, 2019, p. 27. https://doi.org/10.1186/s13052-019-0617-y.

6. Gerosa, M., et al. “Wirsung Atraumatic Rupture in a Patient with Pancreatic Pseudocysts: A Case Presentation.” BMC Gastroenterology, vol. 18, no. 1, 2018, p. 52. https://doi.org/10.1186/s12876-018-0781-3.

7. Xia, H.-T., et al. “Sphincter of Oddi Dysfunction and the Formation of Adult Choledochal Cyst Following Cholecystectomy: A Retrospective Cohort Study.” Medicine, vol. 94, no. 47, 2015, e2088. https://doi.org/10.1097/MD.0000000000002088

8. Layfield, L.J., et al. “Utilization of Ancillary Studies in the Cytologic Diagnosis of Biliary and Pancreatic Lesions: The Papanicolaou Society of Cytopathology Guidelines for Pancreatobiliary Cytology.” Diagnostic Cytopathology, vol. 42, no. 4, 2014, pp. 351–362. https://doi.org/10.1002/dc.23093.

9. Koshinaga, T., et al. “Obliterative Cholangiopathy in Acquired Cystic Biliary Atresia Type III after Cyst Perforation: A Case Report.” BMC Pediatrics, vol. 18, no. 1, 2018, p. 158. https://doi.org/10.1186/s12887-018-1125-8.