2021, Number 1
Experience in the management of coledochal cyst in a general surgery service. Report of four cases
Full text
10.35366/103913
Language: English/Spanish [Versi?n en espa?ol]
References: 15
Page: 40-46
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ABSTRACT
The choledochal cysts are dilatations of the bile duct that occur mainly in females during childhood, in the adult stage a high index of suspicion is required, especially in rural hospitals where there is no technology to diagnose them. The management can be by endoscopic or surgical approach depending on the classification of the cyst. It is well established that, at an older age during diagnosis, the chances of malignancy are also higher, with up to 30% being reported. Four female patients between 14 and 43 years were included. Three patients were not diagnosed with choledochal cyst of first intention, being diagnosed as hepatic cyst, acute cholecystitis and stenosis of the bile duct. The main symptom was abdominal pain and only one of them had jaundice and palpable mass. Three had type I cyst and one type IV A. All underwent open surgery with resection of the cyst and hepatojejun anastomosis in Roux's Y. There were no postoperative complications. The histopathological study was negative to malignancy in all cases.INTRODUCTION
Choledochal cysts (CC) are congenital cystic dilatations of the biliary tree. The incidence is 1:100,000 to 1:150,000 in populations of Western countries. They mainly affect women and although sometimes they are diagnosed in childhood, up to 20% of patients are adults when the diagnosis is done. The risk of developing cholangiocarcinoma in choledochal cysts increases with age, and they occur more frequently in types I (68%) and IV (21%),1,2 which supports their complete removal when diagnosed. Several theories have been proposed to explain the pathogenesis of common bile duct cysts. The current and most accepted is the anomalous union of the pancreatic and biliary ducts outside the duodenal wall, forming a single channel, which can reach a length between 10 and 45 mm. This anomaly causes reflux of pancreatic secretions into the biliary tree and because the pressure of the pancreatic duct is greater than the pressure of the biliary duct, it results in activation of pancreatic enzymes, alteration of the biliary composition, local inflammation, and damage to the biliary epithelium, causing weakness and dilatation of the biliary duct wall.3,4
The most used classification is that made by Todani and collaborators, who classify the common bile duct cyst in five types:3
Type I: saccular or cystic dilatation of the extrahepatic bile duct.
Type II: common bile duct diverticulum.
Type III: dilatation of the intraduodenal portion of the common bile duct (choledococele).
Type IV A: multiple intrahepatic and extrahepatic cysts.
Type IV B: multiple extrahepatic cysts.
Type V: intrahepatic cysts (Caroli's disease).
Less than half of patients present with the typical clinical triad of abdominal pain, jaundice, and an abdominal mass. In more than half of the cases the symptoms are nonspecific.3,4 Since incomplete excision of the cyst can lead to recurrence, symptoms and malignant transformation within the remnant tissue, current surgery involves removal of the entire cyst (including the gallbladder) and restoration of biliary-enteric continuity.5
PRESENTATION OF CLINICAL CASES
Case report 1: a 14-year-old female with four-year evolution of colicky pain in the right hypochondrium. Four months prior to her admission she presented more frequent episodes, adding jaundice, coluria, acholia, nausea, and vomiting. She was admitted with jaundice, pain on palpation in the right hypochondrium and epigastrium, and an abdominal mass measuring approximately 15 × 15 cm. Liver function tests showed mixed hyperbilirubinemia (total bilirubin: 7.1 mg/dl, direct bilirubin: 3.7 mg/dl, increased alkaline phosphatase: 651 U/l). The ultrasonography scan revealed calculous cholecystitis and choledocholithiasis versus common bile duct cyst. The computed tomography (CT) scan showed a calculous cholecystitis and cystic dilatation of the bile duct (Figure 1). The cholangial-resonance imaging showed a 72 mm choledochal cyst with intrahepatic bile duct dilatation (Figure 2). Elective surgery was performed where a gallbladder with lithiasis and a type I choledochal cyst according to Todani's classification attached to duodenum measuring 17 × 10 × 10 cm was found (Figure 3).
Case report 2: a 43-year-old woman presented with colicky pain in the right hypochondrium, without accompanying symptoms; she attended a rural hospital, where she underwent open cholecystectomy during which an increase in the diameter of the common bile duct was observed. It was decided to perform a choledochotomy with T-tube placement. In the outpatient clinic, a T-tube cholangiography was requested, which showed a common bile duct cyst, so she was referred to our hospital. She was admitted without jaundice and an open Kerr type tube with biliary output. Liver function tests were within normal parameters and only shoed hypoalbuminemia. Surgery was performed with findings of a common bile duct cyst measuring 15 × 8 × 7 cm attached to the duodenum, stomach, pancreas, portal vein and vena cava. A right and left hepatic duct with a diameter of 1 cm was found.
Clinical case 3: a 15-year-old female, with a history of right hypochondrium pain and fever. The ultrasonography scan revealed a hepatic abscess. She underwent exploratory laparotomy where a giant tension common bile duct cyst with necrosis and adhesions to the gallbladder was found. Choledochotomy, aspiration and primary closure with placement of a Penrose drainage was performed. On the second postoperative day, 400 cm3 of bile came out through the drainage and, by ultrasound, free liquid in the cavity was seen, for which reason she underwent a new laparotomy. In this second surgery drainage, cholecystectomy, and placement of a Kerr probe in the cyst bag were performed. She was referred to our hospital, where she was maintained with medical treatment and later sent to outpatient care. A T-tube cholangiography revealed a cystic pocket compatible with a common bile duct cyst. A scheduled surgery was performed, and a common bile duct cyst of approximately 8 × 5 cm with a T-tube inside and multiple adhesions was detected (Figure 4).
Case report 4: a 43-year-old woman presented with two-year evolution of colicky pain in the right hypochondrium. Two months prior to her referral to our hospital she presented with increasing pain intensity, accompanied by nausea, vomiting and fever. An endoscopic retrograde cholangiopancreatography (ERCP) was performed, which reported common bile duct dilatation measuring 25 mm of the intrahepatic and extrahepatic bile duct. A sphincterotomy and stent placement were performed. The cytology brushing was negative for malignancy. She was sent to our hospital, where a cholangial-resonance imaging was performed, which showed fusiform dilatation of the main biliary tract, and two more smaller dilatations in the right and left hepatic duct. A diagnosis of type IV A choledochal cyst was made. Liver function tests showed a serum alkaline phosphatase level of 595 U/l, an aspartate aminotransferase (TGO) level of 139 U/l, and alanine aminotransferase (TGP) level of 181 U/l and a total bilirubin level of 1.2 mg/dl.
All patients underwent cyst resection and Roux-en-Y choledochal-duodenal-anastomosis, and two of them also underwent cholecystectomy. They were discharged without complications (Table 1).
DISCUSSION
The choledochal cyst (CC), although rare, is a well-described clinical entity, affecting women more frequently. Even though choledochal cysts are diagnosed in infancy or childhood, up to 20% of patients are adults when the diagnosis is made. The risk of a choledochal cyst transforming into a cholangiocarcinoma increases with age and occurs more frequently in types I (68%) and IV (21%),1,2 which justifies its complete removal when diagnosed. The most accepted theory is the anomalous union of the pancreatic and biliary ducts outside the duodenal wall, forming a single duct, which can reach a length between 10-45 mm.6 The most frequent symptoms are right upper abdominal pain and jaundice. Fever may also be detected when associated with cholangitis. On physical examination, an abdominal mass may be palpated in the right upper quadrant.6,7 In our patients, all of them started with pain and two of them (50%) had fever as a symptom of cholangitis. The classic triad of abdominal pain, jaundice and an abdominal mass is present in less than 15% of adult cases. We saw it in only one patient. A study of 20 patients revealed that 100% were female, 19 (95%) had a type I choledochal cyst, while one had a choledochal cyst type IV. The main symptom was abdominal pain in 15 women (75%) and the other five (25%) had jaundice and/or cholangitis, which coincides with the findings seen in our patients. The diagnostic suspicion was made by ultrasonography scan in 50% of the cases and 18 (90%) were diagnosed by magnetic resonance cholangiopancreatography (MRCP).8 In our study we observed that with ultrasonography scan, being an operator-dependent study, only one patient had a choledochal cyst suspected, and the diagnosis was attained by means of a Kerr probe cholangiography and two of them by MRCP. Two patients were operated on under suspicion of another diagnosis. The Todani classification is widely adopted for the classification of choledochal cysts and the choice of surgical management. The concept of complete excision is the ideal treatment. In proximal cyst removal the procedure must be performed carefully to protect the portal vein and hepatic artery. In distal cysts, the cyst usually extends into the pancreas, so excision may be difficult due to surrounding adhesion to the pancreas. Sometimes complete excision is not possible due to postoperative risk, pancreatic leakage, bleeding, and peritoneal infection.9,10 However, the unresected intrapancreatic choledochal cyst gives rise to the formation of a dead space within the pancreas. Reflux of intestinal secretions results in activation of pancreatic enzymes in the remaining cyst, which can lead to infection, stone formation, and increased risk of malignancy.11 Type I choledochal cyst, which has no obvious relation to the pancreatic duct, intrapancreatic cyst or apparently normal bile duct, shall be completely excised and the stump is sutured. In our study, three patients were carriers of type I choledochal cysts, so total resection of the cyst was performed, taking special care in the intrapancreatic portion and in the dissection of the portal adhesions. In type IV choledochal cysts according to Todani's classification with extrahepatic and intrahepatic cysts, the extrahepatic cyst must be completely excised. If the intrahepatic cyst is limited, a partial hepatectomy with biliary-enteric reconstruction is performed.12 In Mexico, Martínez-Ordaz carried out a study that analyzed 23 patients in 17 years that were operated on. Of these, 70% were women, 87% presented with pain and only four of them had the classic clinical triad; 74% had a type I choledochal cyst. Patients with type I and IV A cysts underwent resection of the cyst and Roux-en-Y choledochal-jejunal-anastomosis by open surgery. Three patients were reoperated for biliary leakage. There were no deaths.13
The laparoscopic approach is gradually replacing open surgical treatment of choledochal cysts in pediatric patients.14 In contrast, laparoscopically choledochal cyst excision in adults is technically difficult and associated with a high rate of complications and conversion into open surgery; however, some authors have reported results like those obtained in children. In a study of 20 patients, Hirdaya found that laparoscopic excision could be completed in 16 cases (80%), while the other four (20%) required conversion to the open approach. The main reason for conversion was technical difficulty due to the initial learning curve, the presence of adhesions, and inflammation of the cyst and/or duct wall. There were no deaths.8 In a study of 110 patients, where half were under 16 years of age, Palanisamy et. al. observed that type I cysts were the most common (71.82%) with an average size of 4.67 ± 1.59 cm. They were operated under laparoscopic approach. They compared the results obtained in the group of children versus adults and demonstrated that minimally invasive surgery had better results in pediatric patients with a lower conversion to open approach rate, a shorter hospital stay and a lower rate of complications. The reported hospital mortality after laparoscopic choledochal cyst excision is 1.8% and that of open choledochal cysts excision is 3 to 4%.15 Altered anatomy, presence of adhesion and lack of palpatory sensation during the laparoscopic excision make it technically difficult and increase the risk of injury. In our hospital unit the patients had a good postoperative evolution, with no morbidity or mortality. In our population there is still no experience in the laparoscopic approach due to the low number of patients with choledochal cysts.
REFERENCES
AFFILIATIONS
1 Assigned to the General Surgery Service of the General Hospital of Acapulco, Ministry of Health. Guerrero, Mexico.
Ethical considerations and responsibility: The authors declare that they followed their center\'s protocols on the publication of patient data and safeguarded their right to privacy through the confidentiality of their data.
Funding: No financial support was received for this work.
Disclosure: The authors declare that there was no conflict of interest in carrying out this work.
CORRESPONDENCE
María Azucena Reyes-García. E-mail: drareyesgar@gmail.comReceived: 03/26/2019. Accepted: 10/10/2019