Secondary excision of choledochal cysts after previous cyst-enterostomies

BACKGROUND/AIMS: Patients with a choledochal cyst previously treated by internal drainage should undergo secondary cyst excision. The results of such secondary excisions have not yet been reported. METHODOLOGY: Over a 27-year period, 121 patients underwent excision of a choledochal cyst at our hospitals. Of these, 14 patients underwent secondary cyst excision following internal drainage. These patients were compared retrospectively with the remaining 107 patients who underwent primary cyst excision. RESULTS: Blood loss and operative time were greater, and early and late post-operative complications were significantly more frequent in the secondary excision group. Wound infection (early complication), pancreatic stones (late complication), and hepatolithiasis (late complication) were significantly more common in the secondary excision group. Histologically, inflammation and biliary glands were more frequently seen in the resected bile ducts in the secondary excision group. CONCLUSIONS: The long-term results of secondary excision of choledochal cysts are worse than after primary excision. The frequent late complications may be related to the development of biliary glands as a consequence of cyst-enterostomy.     

囊肿切除肝总管空肠Roux-en-Y吻合术治疗先天性胆管囊肿

目的 观察囊肿切除肝总管空肠Roux-en-Y吻合术治疗小儿先天性胆管囊肿的疗效。方法 采用囊肿切除肝总管空肠Roux-en-Y吻合术治疗小儿先天性胆管囊肿56例。结果 56例患儿手术全部成功。术后患儿胆管囊肿症状消失,肝胆系统结构和功能均恢复正常。术后并发胆道感染1例,腹膜炎1例,肺炎3例,无胆瘘和伤口感染者,无死亡病例。结论 囊肿切除肝总管空肠Roux-en-Y吻合术是治疗小儿先天性胆管囊肿的有效术式。     

A case of intrahepatic cholangiocarcinoma associated with Type IV choledochal cyst

Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.     

Endoscopic biliary drainage for children with persistent or exacerbated symptoms of choledochal cysts

Background

Symptoms of choledochal cysts sometimes persist or become exacerbated. As preoperative management for patients with these cysts, we prospectively employed endoscopic drainage, based on the theory that protein plugs cause symptoms by obstructing the pancreatobiliary ducts.

Methods

Children with choledochal cysts underwent endoscopic retrograde cholangiopancreatography (ERCP). When ERCP showed compaction with filling defects in patients with persistent or worsening symptoms (study patients), the placement of a short biliary stent tube was attempted for drainage. The clinical and ERCP findings of the study patients were compared with those of patients who were asymptomatic at ERCP (asymptomatic patients).

Results

There were 13 study patients (median age 2.9 years) and 41 asymptomatic patients (4.7 years) enrolled in the study between August 2005 and February 2011. Study patients more frequently had jaundice and elevated transaminase levels. ERCP showed that all study patients had obstruction or compacted filling defects in the common channel or the narrow segment distal to the cyst. Insertion of a stent tube was successful in 11 patients. Symptoms were relieved soon after biliary drainage. Surgery revealed that the obstructing materials were protein plugs, except in one case, which involved fatty acid calcium stones.

Conclusions

These results support the protein plug theory. Endoscopic short-tube stenting is adequate and effective as preoperative management.     

Carcinoma of the head of the pancreas after excision of a choledochal cyst

We here report a 53-year-old woman who had undergone resection of a choledochal cyst and hepaticojejunostomy three years before. She was readmitted because of intermittent fever, and abdominal computed tomography revealed a 4-cm tumor in the head of the pancreas. We performed pancreatoduodenectomy, and examination of the resected specimen showed well-differentiated papillary adenocarcinoma. Only 5 cases of carcinoma occurring after the resection of a choledochal cyst have been reported, and to our knowledge, this is the second case of carcinoma of the head of the pancreas.     

Surgical treatment of choledochal cysts in children

Choledochal cyst is often associated with pancreatobiliary malunion, and pancreatic juice usually refluxes into the bile duct via the malunion. Various pathological conditions develop in the biliary tract, pancreas, and liver: cholangitis, biliary dilatation, biliary perforation, biliary cancer, acute pancreatitis, and/or biliary cirrhosis. The performance of cystenterostomy has recently been abandoned because of high morbidity after surgery. Accurate delineation of the biliary tree and the pancreatobiliary junction obtained by endoscopic retrograde cholangiopancreatography or operative cholangiography is necessary. Cyst excision should be performed to prevent ascending cholangitis and biliary cancer. Many types of procedures are employed in biliary reconstruction, but free drainage of bile is imperative for preventing cholangitis and stone formation. This can be achieved only by a wide anastomosis performed at the hilum after ductoplasty.     

Efficacy of ERCP via the accessory papilla in children with choledochal cysts

BACKGROUND: ERCP via the major duodenal papilla in children with choledochal cyst may not clearly visualize the entire pancreatic duct and the junction of the pancreatic and biliary ducts. This may be caused by obstruction of the pancreatic duct by a dilated common bile duct. METHODS: Patients with choledochal cysts who underwent ERCP with injection of contrast medium at the major duodenal papilla were classified as either belonging to a MP group, in which the entire pancreatic duct and junction of the pancreatic and biliary ducts were visualized, or to a M/AP group, in which these structures were either partially visualized or not visualized. ERCP via the accessory papilla subsequently was performed in the M/AP group by using 0.6- or 0.8-mm metal-tip catheters. The efficacy of ERCP via the accessory papilla in children with choledochal cysts was evaluated. OBSERVATIONS: ERCP was performed in 13 patients. Seven were assigned to the MP group and 6 to the M/AP group. ERCP via the accessory papilla in the M/AP group was successful in 5 of the 6 patients; pancreas divisum was demonstrated in one, a protein plug at the main pancreatic duct in another, and the entire pancreatic duct and junction of the pancreatic and biliary ducts in the remaining 3 patients. No patient developed pancreatitis as a result of ERCP, including ERCP via the accessory papilla. CONCLUSIONS: In children with choledochal cyst, ERCP via the accessory papilla is an effective method for visualization of the detailed structure of the entire pancreatic ductal system and junction of the pancreatic and biliary ducts when ERCP via the major duodenal papilla is unsuccessful.     

Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst

A 56-year-old woman who had undergone excision of the gallbladder because of a choledochal cyst had a tumorous lesion of the pancreas identified by upper abdominal ultrasonography, but an operation was not carried out, because there was no apparent increase in the cystic mass and no elevation of serum tumor markers. In October 2001, she was admitted to our hospital to check for malignancy because of elevated levels of the tumor marker Dupan-2. Abdominal enhanced computed tomography and upper abdominal ultrasonography revealed a large multilocular cystic mass in the body to tail of the pancreas. Endoscopic retrograde cholangiopancreatography showed elongation of the common duct that communicates with the common bile duct and the main pancreatic duct, indicating an anomalous arrangement of the biliary and pancreatic duct system. No apparent communications between the cystic mass and the main pancreatic duct were observed. In January 2002, the patient underwent a spleen-preserving distal pancreatectomy, and histopathological and immunohistochemical examinations led to the diagnosis of pancreatic mucinous cystadenoma with ovarian-like stroma. The mucinous cystadenoma was detected 17 years after the operation for the choledochal cyst. To the best of our knowledge, no documented case reports of mucinous cystadenoma of the pancreas associated with a choledocal cyst have been reported to date. We present here the first case report of pancreatic mucinous cystadenoma occurring in the body to tail of the pancreas, associated with a choledocal cyst.     

Congenital choledochal cyst: Occurrence of primary intrahepatic stones and prevention of carcinogenesis

Intrahepatic biliary stones and carcinogenesis are two major complications seen after corrective surgery for congenital choledochal cyst. The incidence of these two complications was reviewed in patients seen at out-patient clinics at a mean follow-up time of 13 years and 6 months. Intrahepatic biliary stones developed in 5 of 56 patients who had undergone a standard excisional operation; in 4 of the 5 patients, the stones were found in the preexisting intrahepatic biliary dilatation (with or without a downstream stenosis), 7–15 years postoperatively. Biliary stricture with dilatation is a putative cause of intrahepatic bile duct stones, and this hypothesis was supported here, since stones developed in all 3 of the patients who had the peculiar configuration of intrahepatic biliary dilatation and stenosis. Biliary carcinoma was not observed in any of the 56 patients who had undergone standard excision of a choledochal cyst followed by hepatico-jejunostomy, but developed in 1 of 12 patients in whom complete excisional resection of the dilated extrahepatic bile duct had not been performed. The occurrence of carcinoma in choledochal cysts is reviewed.     

Amylase level in extrahepatic bile duct in adult patients with choledochal cyst plus anomalous pancreatico-biliary ductal union

AIM: To investigate the relationship between pancreatic amylase in bile duct and the clinico-pathological features in adult patients with choledochal cyst and anomalous pancreatico-biliary ductal union (APBDU). METHODS: From 39 patients who underwent surgery for choledochal cyst between March 1995 and March 2003, we selected 15 adult patients who had some symptoms and were radiologically diagnosed as APBDU, and their clinico-pathological features were subsequently evaluated retrospectively. However, we could not obtain biliary amylase in all the patients because of the surgeon's slip. Therefore, we measured the amylase level in gall bladder of 10 patients and in common bile duct of 11 patients. RESULTS: Levels of amylase in common bile duct and gall bladder ranged from 11 500 to 212 000 IU/L, and the younger the patients, the higher the biliary amylase level (r= -0.982, P<0.01). Pathologically, significant correlation was found between the size of choledochal cyst and the grade of inflammation (r= 0.798, P<0.01). And, significant correlation was found between the level of amylase in gall bladder and the grade of hyperplasia. On the other hand, there was no correlation to the age of symptomatic onset or inflammatory grade (r = 0.743, P<0.05). Level of lipase was elevated from 6 000 to 159 000 IU/L in bile duct and from 14 400 to 117 000 IU/L in the gall bladder; however, there was no significant correlation with age or clinico-pathological features. CONCLUSION: The results support the notion that amylase has a particular role in the onset of symptoms, and suggest that a large amount of biliary amylase induces early onset of symptom, thereby making early diagnosis possible.     

不同Todani分型小儿先天性胆总管囊肿CT表现

目的 探讨CT术前检查诊断不同Todani分型小儿先天性胆总管囊肿(CCC)效能.方法 2013年1月 ～2020年6月我院收治的CCC患儿126例,入院后接受CT平扫和增强扫描.所有患儿接受外科手术治疗,以术后组织病理学诊断为金标准,判断术前CT检查诊断Todani分型的效能.结果 在126例患儿中,术后组织病理学诊...     

Outcomes after clearance of pancreatic stones with or without pancreatic stenting

Background Extracorporeal shockwave lithotripsy (ESWL) and endoscopic lithotripsy are useful for the fragmentation and extraction of pancreatic stones. However, pancreatic stones often recur, for which an adequate strategy is needed. Treatment for stricture of the main pancreatic duct (MPD) with a pancreatic stent after clearance of pancreatic stones may reduce the recurrence of pancreatic symptoms and stones. Methods Forty patients with chronic pancreatitis with MPD stones were treated with ESWL in combination with endoscopic stone extraction. After clearance of the stones, a pancreatic stent was inserted when a stricture of MPD was observed on pancreatography. The stent was exchanged every 3 months and removed after a total of 1 year. We examined episodes of recurrent pain and pancreatitis in patients with and without stenting, as well as the MPD diameter, during follow-up. Results MPD stricture was seen in 27 patients, and a stent was successfully inserted in 24 of them. Pancreatic symptoms recurred in five patients (21%) in the stenting group and in three patients (23%) in the control group during a mean follow-up period of 1.5 and 1.2 years, respectively. The diameter of the MPD, before, just after, and 1 year after treatment, was 7.6, 5.4, and 5.8 mm, respectively. It was significantly decreased after 1 year of follow-up, as well as just after stent removal, compared with before treatment (P < 0.05). Conclusions Additional stenting for MPD after extraction of pancreatic stones may reduce the risk of recurrence of pancreatic symptoms.     

Role of endotoxaemia in hyperdynamic circulation in rats with extrahepatic or intrahepatic portal hypertension

This study investigated the role of endotoxaemia in the development of hyperdynamic circulation observed in rats with extrahepatic (high collateralization) or intrahepatic (low collateralization) portal hypertension. Compared with sham-operated rats, decreased mean arterial pressure and systemic vascular resistance were detected on days 1, 4 and 14 following partial portal vein ligation. By day 1, the cardiac index of portal vein-ligated rats was similar to that of sham-operated rats and progressively increased, thereafter, reaching statistically higher values on days 4 and 14. No differences in plasma endotoxin levels were found between portal vein-ligated and sham-operated rats throughout the observation period. Both carbon tetrachloride-induced cirrhotic rats with and without ascites had a higher cardiac index and lower systemic vascular resistance than those of control rats, and cirrhotic rats with ascites had the lowest systemic vascular resistance. Plasma endotoxin levels were higher in cirrhotic rats with ascites (8.6±2.0 pg/mL; P < 0.01) than those of control rats (2.2±0.3 pg/mL) and cirrhotic rats without ascites (2.4±0.6 pg/mL). These results suggest that factors other than endotoxaemia play a role in the development of hyperdynamic circulation observed in rats with extrahepatic portal hypertension and cirrhotic rats without ascites, but that endotoxaemia may contribute to the maintenance of hyperdynamic circulation found in cirrhotic rats with ascites. The severity of liver disease may be a more important factor than the presence of portosystemic shunting for the development of endotoxaemia in portal hypertensive states.     

A case of carcinoma arising in the intrapancreatic terminal choledochus 12 years after primary excision of a giant choledochal cyst

A 27-yr-old woman with carcinoma developing in the intrapancreatic terminal choledochus 12 yr after the initial excision of giant choledochal cyst with Roux-en-Y hepaticojejunostomy is reported. Despite the generally accepted concept that the presence of bile is associated with malignancy in the remnant biliary ducts after excision of a choledochal cyst, this is the first report of a malignant growth developing in the terminal choledochus which had been free from the bile exposure long after the excision of the choledochal cyst. Long-term follow-up of the patient is advisable even after operation for primary resection of the choledochal cyst.     

A case of carcinoma associated with the remained intrapancreatic biliary tract 17 years after the primary excision of a choledochal cyst.

Although the incidence of biliary carcinoma associated with choledochal cyst is high, there are extremely rare reports about cancer development in the remaining intrapancreatic biliary tract in patients who underwent primary excision of the choledochal cyst. The authors present a case of carcinoma associated with the remnant intrapancreatic biliary tract in a 39 year-old woman, 17 years after the initial excision of a choledochal cyst with Roux-en-Y hepaticojejunostomy. Long-term follow-up may be recommended even in patients who have undergone excisional surgery for choledochal cyst, because it is possible that cancer associated with the intrapancreatic biliary tract may develop.