Familial adenomatous polyposis (Gardner's syndrome) and thyroid carcinoma

Summary The case history of a 24-year-old woman with Gardner's syndrome [familial adenomatous polyposis (FAP)] and papillary thyroid carcinoma is presented, representing the 37th report of this association. Although FAP is transmitted as an autosomal dominant trait with similar penetrance in both sexes, thyroid carcinoma has been found almost exclusively in women (94.3%). The majority have been papillary carcinomas (88.5%), which have become apparent during the third decade (average 23.6, range 16–40 years). Most (55.5%) thyroid carcinomas have been discovered 1–17 years after FAP was identified, although some have been found before (29.6%), or at the same time (14.8%) FAP was diagnosed. Multicentric papillary carcinomas have been reported in 64% (14 of 22) of FAP patients, a frequency at least twofold greater than usual. Although papillary carcinoma found before age 30 (as it was in most patients with FAP) typically has an excellent prognosis, one patient with FAP developed distant metastases from thyroid carcinoma and a 28-year-old woman's death was attributed to papillary carcinoma. The high frequency of multicentric papillary thyroid carcinoma in young patients with FAP and the potential for metastases and death due to thyroid carcinoma warrant aggressive diagnostic screening at regular intervals with neck palpation, ultrasonography, and if necessary, fineneedle aspiration biopsy. When thyroid carcinoma is found, total or near-total thyroidectomy should be considered because of the tumor's high likelihood of being multifocal. Since almost 30% of the thyroid carcinomas associated with FAP have been diagnosed 4–12 years before polyposis was identified, young patients presenting with thyroid carcinoma should be questioned regarding bowel function and a family history of gastrointestinal disease, and consideration should be given to periodic testing for fecal occult blood.The views expressed herein are those of the authors and do not necessarily reflect those of the Army or the Department of Defense.     

Adenomas of the common bile duct in familial adenomatous polyposis

Familial adenomatous polyposis(FAP)or Gardner’s syndrome is often accompanied by adenomas of the stomach and duodenum.We experienced a case of adenomas of the common bile duct in a 40-year-old woman with FAP presenting with acute cholangitis.Only 8 cases of adenomas or adenocarcinoma ofthe common bile duct have been reported in the literature in patients with FAP or Gardner’s syndrome.Those patients presented with acute cholangitis or pancreatitis.Local excision or Whipple procedure may be the reasonable surgical option.     

胆总管结石与胆源性胰腺炎的相关性研究

目的 探讨胆总管结石的大小、部位等相关因素与胆源件胰腺炎的相关性.方法 回顾性分析2002年1月至2008年12月收治的3497例胆总管结石患者的临床资料,并根据胆总管结石的大小,结石部位分组,比较各组并发胰腺炎的发病率.结果 胆总管结石患者并发胆源性胰腺炎时,以发热、腹痛、黄疸等症状更为明显,血清淀粉酶显著增高,其中胆总管结石的大小与胆源性胰腺炎严重程度呈负相关,位于胆总管下段与壶腹部的结石者易并发胰腺炎.结论 对位于胆总管下段或壶腹部的小结石应早期行内镜下取石治疗,以预防急性胆源性胰腺炎的发生.     

Role of prophylactic endoscopic sphincterotomy in patients with acute biliary pancreatitis due to transient common bile duct obstruction

BACKGROUND AND AIM: The role of prophylactic endoscopic sphincterotomy in patients with transient common bile duct obstruction is controversial. The aim of this study was to assess the value of performing prophylactic endoscopic sphincterotomy in patients suffering from acute biliary pancreatitis and absent common bile duct stones on endoscopic retrograde cholangiopancreatography (ERCP). METHODS: Hospital notes of patients admitted to our unit with a diagnosis of acute pancreatitis from January 2000 to January 2005 were reviewed. Endoscopic sphincterotomy was performed when patients were deemed unfit for cholecystectomy, suffering from a severe attack of acute pancreatitis and/or showing evidence of transient common bile duct obstruction. The outcomes of patients with and without endoscopic sphincterotomy were compared. RESULTS: A total of 427 patients were admitted with a diagnosis of acute pancreatitis during the study period. Eighty-eight patients with absent common bile duct stones on ERCP were identified. Endoscopic sphincterotomy was performed in 71 patients and not performed in 17 patients. There was no significant difference in recurrent pancreatitis rates (1.4% vs 5.8%, P = 0.35), recurrent biliary complication rates (5.6% vs 5.9%, P = 1) or mortality rates (5.8% vs 1.5%, P = 0.35). The time to recurrent complications (38.4 days vs 41.0 days, P = 0.38) was not significantly different between the two groups. There was no ERCP-related morbidity or mortality. CONCLUSION: Prophylactic endoscopic sphincterotomy is not recommended in patients with transient common bile duct obstruction or as an option to cholecystectomy in elderly patients. Early cholecystectomy should be performed.     

Complete obstruction of the lower common bile duct caused by autoimmune pancreatitis: is biliary reconstruction really necessary?

Recent observations suggest that an immune response is involved in the development of chronic pancreatitis. We report a case of autoimmune pancreatitis in a patient who showed complete obstruction of the lower common bile duct. A 63‐year‐old man was admitted to a local hospital, complaining of appetite loss and back pain. The patient had obstructive jaundice, and percutaneous transhepatic gallbladder drainage was performed. Fluorography through the biliary drainage catheter showed complete obstruction of the lower common bile duct. The patient had no history of alcohol consumption and no family history of pancreatic disease. Physical examination revealed an elastic hard mass palpable in the upper abdomen. Abdominal ultrasound and abdominal computed tomography (CT) scans showed enlargement of the pancreas head. While autoimmune pancreatitis was highly likely, due to the patient's high serum immunoglobulin level, the possibility of carcinoma of the pancreas and/or lower common bile duct could not be ruled out. Laparotomy was performed, and wedge biopsy samples from the pancreas head and body revealed severe chronic pancreatitis with infiltration of reactive lymphocytes, a finding which was compatible with autoimmune pancreatitis. Cholecystectomy and biliary reconstruction, using choledochojejunostomy, were performed, because the complete bile duct obstruction was considered to be irreversible, due to severe fibrosis. After the operation, prednisolone (30 mg/day) was given orally for 1 month, and the entire pancreas regressed to a normal size. Complete obstruction of the common bile duct caused by autoimmune pancreatitis has not been reported previously; this phenomenon provides an insight into autoimmune pancreatitis and provokes a controversy regarding whether biliary reconstruction is needed for the treatment of complete biliary obstruction caused by autoimmune pancreatitis.     

Delayed common bile duct obstruction in acute pancreatitis

Summary Common bile duct obstruction during acute pancreatitis usually occurs in the early symptomatic phase of the illness, involves only the distal portion of the common bile duct, and subsides with clinical improvement. We present two cases of persistent common bile duct obstruction that developed 2–3 months after complete clinical subsidence of the initial episode of severe acute pancreatitis and involved a long segment of the common bile duct. After surgical decompression, there was no recurrence of common bile duct obstruction or pancreatitis.     

Double common bile duct： A case report

Double common bile duct （DCBD） is a rare congenital anomaly in which two common bile ducts exist. One usually has normal drainage into the papilla duodeni major and the other usually named accessory common bile duct （ACBD） opens in different parts of upper gastrointestinal tract （stomach, duodenum, ductus pancreaticus or septum）. This anomaly is of great importance since it is often associated with biliary lithiasis, choledochal cyst, anomalous pancreaticobiliary junction （APBJ） and upper gastrointestinal tract malignancies. We recently recognized a rare case of DCBD associated with APB3 with lithiasis in better developed common bile duct. The opening site of ACBD was in the pancreatic duct. The anomaly was suspected by transabdominal ultrasonography and finally confirmed by endoscopic retrograde cholangiopancreatography （ERCP） followed by endoscopic sphincterotomy and stone extraction. According to the literature, the existence of DCBD with the opening of ACBD in the pancreatic duct is most frequently associated with APB3 and gallbladder carcinoma. In case of DCBD, the opening site of ACBD is of greatest clinical importance because of its close implications with concomitant pathology. The adequate diagnosis of this rare anomaly is significant since the operative complications may occur in cases with DCBD which is not recognized prior to surgical treatment.     

Relapsing acute pancreatitis due to ampullary adenoma in a patient with familial adenomatous polyposis

A patient with familial adenomatous polyposis (FAP) presented with a relapsing attack of acute pancreatitis. Evaluation using computed tomography, ultrasonography, and duodenoscopy revealed an ampullary adenoma, which was classified as Spigelman's stage III according to Spigelman's criteria. The patient underwent a pylorus-resected pancreatoduodenectomy, and has had no abdominal pain suggesting acute pancreatitis for 1 year after surgery. Only a few reports of acute pancreatitis due to ampullary neoplasms in patients with FAP are available. Relapsing acute pancreatitis is another surgical indication for premalignant periampullary neoplasms in FAP.     

Technical aspects in the laparoscopic management of complicated common bile duct stones

The management of common bile duct (CBD) stones traditionally required open laparotomy and bile duct exploration. With the advent of endoscopic and laparoscopic technology in the latter half of last century, endoscopic retrograde cholangiopancreatography (ERCP) and laparoscopic cholecystectomy (LC) has become the mainstream treatment for CBD stones and gallstones in most medical centers around the world. However, in certain situations, ERCP cannot be feasible because of difficult cannulation and extraction. ERCP can also be associated with potential serious complications, in particular for complicated stones requiring repeated sessions and additional maneuvers. Since our first laparoscopic exploration of the CBD (LECBD) in 1995, we now adopt the routine practice of the laparoscopic approach in dealing with endoscopically irretrievable CBD stones. The aim of this article is to describe the technical details of this approach and to review the results from our series.     

Primary closure of the common bile duct in open laparotomy for common bile duct stones

It is common these days to treat common bile duct (CBD) stones using endoscopic techniques. However, severe complications sometimes lead to death despite the great benefit of these techniques. If the patient has many and/or large stones, it can take considerable time for duct clearance, and this is associated with high costs. Therefore, we do not hesitate to choose surgical procedures when necessary. In this study, our aim was to evaluate the usefulness of primary closure of the CBD in open laparotomy for CBD stones. Thirty-four patients with CBD stones were operated on by open laparotomy; primary closure was done in 17 patients (group PC), and T-tube insertion was done in 17 (group TT). We compared the patients' medical records, clinical features, laboratory data, complications, and postoperative hospital admission days. There were no significant intergroup differences in patients' medical records, clinical features, or laboratory data, except for the number of CBD stones. There were no differences in complications. All complications were minor and needed no extra care. The number of postoperative hospital admission days showed a significant difference: 18.3 days in group PC and 31.5 in group TT. There are so many methods to treat CBD stones now that the selection of the procedure can be important for the patient's benefit. We prefer primary closure, to get better quality of life postoperatively and to avoid further operations and any severe complications.     

The case for laparoscopic common bile duct exploration

The modern surgeon's approach to choledocholithiasis depends his or her view of cholangiography. During the early 1990 there was a swing away from cholangiography, which had previously been common practice. This was because of perceptions of difficulty with the technique, the time it took, and perhaps an implied increase in costs because of the time factor. There was no evidence on which to base this decision. This led to a marked upswing in the use of endoscopic retrograde cholangiopancreatography (ERCP). There were a large number of ERCPs with normal results performed prior to laparoscopic cholecystectomy. This paper states the case for intraoperative cholangiography and common bile duct clearance at the time of cholecystectomy. It is hoped that this technique will be adopted so patients can undergo a single procedure to remove their gallstones and common bile duct stones if they exist and to decrease the incidence of normal preoperative ERCPs and the need for a second procedure postoperatively to clear stones if they are found.     

胆总管神经纤维瘤1例报告

1病例资料 患者女性,47岁,因发现＂皮肤、巩膜黄染8 d＂于2015年10月8日入本院。病程中无明显腹痛、腹胀,寒战高热等症状。既往：2015年4月30日于吉林市华侨医院诊断为胆囊结石,胆管结石行腹腔镜胆囊切除术、胆道探查取石、T管引流术。5年前因子宫肌瘤行子宫次全切术。20年前行阑尾切除术。体格检查：皮肤、巩膜黄染。     

Risk factors for the development of post-endoscopic retrograde cholangiopancreatography pancreatitis in patients with asymptomatic common bile duct stones

BACKGROUND Previous studies have revealed that patients with asymptomatic common bile duct(CBD) stones are at a high risk of developing post-endoscopic retrograde cholangiopancreatography(ERCP) pancreatitis(PEP). However, no studies to date have addressed the risk factors for PEP in patients with asymptomatic CBD stones.AIM To examine the risk factors for PEP in patients with asymptomatic CBD stones.METHODS Using medical records of three institutions in Japan for 6 years, we identified a total of 1135 patients with choledocholithiasis including 967 symptomatic patients and 168 asymptomatic patients with native papilla who underwent therapeutic ERCP. We performed univariate and multivariate analyses to examine the risk factors for PEP in the 168 patients with asymptomatic CBD stones.RESULTS The overall incidence rate of PEP in all the patients with during study period was4.7%(53/1135). Of the 168 patients with asymptomatic CBD stones, 24(14.3%)developed PEP. In univariate analysis, precut sphincterotomy(P = 0.009) and biliary balloon sphincter dilation(P = 0.043) were significant risk factors for PEP.In multivariate analysis, precut sphincterotomy(P = 0.002, 95%CI: 2.2-27.8, odds ratio = 7.7), biliary balloon sphincter dilation(P = 0.015, 95%CI: 1.4-17.3, odds ratio = 4.9), and trainee endoscopists(P = 0.048, 95%CI: 1.01-8.1, odds ratio = 2.9)were significant risk factors for PEP.CONCLUSION ERCP for asymptomatic CBD stones should be performed by experienced endoscopists. When performing precut sphincterotomy or biliary balloon sphincter dilation in patients with asymptomatic CBD stones, the placement of a prophylactic pancreatic stent is strongly recommended to prevent PEP.     

Congenital web of the common bile duct in association with cholelithiasis

Congenital web formations are extremely rare anomalies of the extrahepatic biliary tree. The age at presentation and the clinical symptomatology of these anomalies depend on the grade of the biliary obstruction. We report a case of a common bile duct septum in association with cholelithiasis in a 30‐year‐old woman. The diagnosis was made on preoperative magnetic resonance cholangiopancreatography (MRCP) and confirmed with intraoperative cholangiography. Because all known causes of acquired web formation were excluded, a congenital origin of the web was assumed. The patient was treated with a hepaticoduodenostomy above the level of the septum. The embryological aspects of this rare anomaly are described.     

胆总管结扎诱导大鼠肝肺综合征模型的建立初步研究

目的 探讨胆总管结扎术诱导大鼠肝肺综合征(HPS)模型的建立.方法 40只SD大鼠被随机分为实验组30只和对照组(假手术组)10只,采用胆总管结扎术建立肝硬化和HPS模型.使用血气分析仪测定PaO2、PaCO2和pH.结果 在术后1 w,腹部超声检查显示实验组肝脏回声增粗,胆总管轻度扩张.在术后2 w,肝脏被膜不平整,...     

Embryonal rhabdomyosarcoma of the common bile duct mimicking choledochal cyst

Embryonal rhabdomyosarcoma of the common bile duct is a very rare malignancy of childhood. The radiological appearance of the lesion is similar to that of congenital choledochal cyst if there is no local invasion to the adjacent tissues. The authors present a case of embryonal rhabdomyosarcoma of the common bile duct which was considered to be a congenital choledochal cyst preoperatively, and they discuss this very rare childhood tumor, with a brief survey of the literature. In conclusion, it is important to know that this rare tumor can simulate congenital choledochal cyst; it should be considered in the differential diagnosis of obstructive jaundice in children.     

Resected cystadenoma of the common bile duct

Biliary cystadenoma in the extrahepatic bile ducts is a very rare tumor. A 62-year-old woman with jaundice was admitted to our hospital. Imaging studies revealed a 4-cm cystic lesion around the hepatic hilum, compressing the common bile duct (CBD). When laparotomy was performed, a cystic tumor was detected in the hepatic hilum, filling the lumen of the CBD. Bile duct resection that included the tumor was performed, followed by biliary reconstruction. Microscopically, the cyst wall was lined by a single layer of cuboidal epithelial cells, covering an ovarian-like stroma. The degree of atypia was low and warranted the diagnosis of cystadenoma.     

Laparoscopic management of common bile duct stones

We reviewed our experience with the management of common bile duct (CBD) stones in 100 consecutive patients treated laparoscopicaly during the past 9 years (1990—1998) and evaluated the advantages, disadvantages, and feasibility of the treatment, to elucidate reasonable therapeutic strategies for patients harboring CBD stones. We conclude that the most rational management of CBD stones is that which is decided according to the size of the CBD, which, in turn, depends on the size, number, and location of stones. The cystic duct in patients with a non‐dilated CBD is narrow, because the size of the CBD depends on the size and number of stones that have migrated through the narrow cystic duct, and the stones in the non‐dilated CBD are therefore usually small in size and number. Patients with a dilated CBD, however, are good candidates to undergo single‐stage laparoscopic treatment. In our Department, therefore, even if complete removal of stones has failed in patients with non‐dilated CBD, further choledochotomy is not carried out, and a C‐tube is placed through the cystic duct for a subsequent postoperative transduodenal approach, because laparoscopic transcystic CBD exploration and choledochotomy may not be always feasible in those patients with non‐dilated CBD, and spontaneous migration of small stones into the duodenum is frequently noted. In fact, some stones demonstrated on intraoperative cholangiograms were not revealed by postoperative cholangiography. In contrast, retained stones detected postoperatively were successfully removed by postoperative endoscopic sphincterotomy (EST), the endoscopic papillary balloon dilatation technique (EPBDT), or postoperative cholangioscopy (POCS) without any injury to the sphinter of Oddi. With this approach, we believe that the causes of stone recurrence can be avoided in the majority of cases.