Diagnosis and management of choledochal cyst: 20 years of single center experience

We report the first case series from Africa and the Middle East on choledochal cyst,a disease which shows significant geographical distribution with high incidence in the Asian population.In this study,the epidemiological data of the patients are presented and analyzed.Attention was paid to diagnostic imaging and its accuracy in the diagnosis and classification of choledochal cyst.Most cases of choledochal cyst disease have type?ⅠandⅣ-A cysts according to the Todani classification system,which support the etiological theories of choledochal cyst,especially Babbitt’s theory of the anomalous pancreaticobiliary duct junction,which are clearly stated.The difficulties and hazards of surgical management and methods used to avoid operative complications are clarified.Early and late postoperative complications are also included.This study should be followed by multicenter studies throughout Egypt to help assess the incidence of choledochal cysts in one of the largest populations in Africa and the Middle East.     

Spontaneous rupture of a type IVA choledochal cyst in a young adult during radiological imaging

A case of a 24-year-old male with jaundice andepigastric pain is reported.The patient underwent athorough clinical,laboratory,and imaging investigation.Computerized tomography revealed a 9 cm×10 cmcholedochal cyst.Magnetic resonance imaging andmagnetic cholangiopancreatography were performed,during which he developed an“acute abdomen”,withradiological evidence of biliary peritoneal leak.Urgentsurgery revealed rupture of the distended malformedcommon bile duct.A peritoneal drain was instilled anda more definitive surgical procedure was accordinglyscheduled.Hepatobiliary scintigraphy following surgeryverified these findings,as well as confirmed the adequacyof the urgent surgery.A combination of radiological andnuclear medicine techniques substantially contributesto the diagnosis of choledochal cyst rupture and theadequacy of surgical intervention.     

Spontaneous rupture of a type ⅣA choledochal cyst in a young adult during radiological imaging

A case of a 24-year-old male with jaundice and epigastric pain is reported. The patient underwent a thorough clinical, laboratory, and imaging investigation.Computerized tomography revealed a 9 cm×10 cm choledochal cyst. Magnetic resonance imaging and magnetic cholangiopancreatography were performed,during which he developed an "acute abdomen", with radiological evidence of biliary peritoneal leak. Urgent surgery revealed rupture of the distended malformed common bile duct. A peritoneal drain was instilled and a more definitive surgical procedure was accordingly scheduled. Hepatobiliary scintigraphy following surgery verified these findings, as well as confirmed the adequacy of the urgent surgery. A combination of radiological and nuclear medicine techniques substantially contributes to the diagnosis of choledochal cyst rupture and the adequacy of surgical intervention.     

Traumatic rupture of a type Ⅳ a choledochal cyst in an adult male

Choledochal cyst (CC) is a rare, congenital anomaly of the bile ducts. We describe a 26-year-old male patient who was transferred to our hospital with a reported traumatic rupture of cystic liver lesions following a fall. At the time of injury, the patient experienced severe abdominal pain. He was found to have peritonitis and abdominal hemorrhage, which is quite rare. Laparot-omy revealed 3000 mL fluid consisting of a mixture of blood, bile and inflammatory effusion in the peritoneal cavity. The liver, gallbladder, spleen, stomach, duo-denum, small intestine, and colon appeared normal. A large cystic mass was discovered near the porta hepatis. This mass, which connected to the hepatic bifurcation and gallbladder had a 5 cm rupture in the right wall with active arterial bleeding. Abdominal com-puted tomography (CT) and emergency laparotomy revealed rupture of a huge type Ⅳa CC. The patient was successfully managed by primary cyst excision, cholecystectomy, and Roux-en-Y end-to-side hepatico-jejunostomy reconstruction. The postoperative course was uneventful and the patient was discharged on the 12 th day of hospitalization. Four weeks after surgery,abdominal CT scan showed pneumatosis in the intra-hepatic bile duct, and intrahepatic dilatation which decreased following adequate biliary drainage. The patient has remained well in the close follow-up period for 9 mo.     

A 10-year experience in the management of gallbladder cancer

BackgroundThe aim of this retrospective study was to review all patients diagnosed with gallbladder cancer over a 10-year period to assess variables affecting survival.MethodsPatients diagnosed with gallbladder cancer from January 1990 to December 1999 were identified from the Lothian Surgical Audit database and a case-note review was performed.ResultsThe 44 patients who were studied (33 women, 11 men) had a mean age of 66 years (range 42–90 years). The diagnosis was established preoperatively in 25 patients (57%), intraoperatively in 5 patients (11%) and incidentally following pathological examination of cholecystectomy specimens in 14 patients (32%). None of the 25 patients diagnosed preoperatively underwent curative operations (median survival 4 months). All five patients diagnosed at the time of attempted cholecystectomy had advanced irresectable disease (median survival 1 month). The overall median survival in 14 patients with an incidental diagnosis of gallbladder cancer was 16 months; however, in eight of these patients who were considered to have had a potentially curative resection, the median survival was 38 months.DiscussionThe prognosis for patients diagnosed preoperatively or at the time of cholecystectomy is very poor. Patients with an incidental finding of gallbladder cancer have a significantly better prognosis and should be considered for further radical re-resection.     

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.     

Mutagenic activity in stones from a patient with a congenital choledochal cyst

Summary A 12-year-old boy presenting a congenital choledochal cyst complicated with stones and chronic recurrent colangitis was subjected to surgery for cyst resection with a Roux-Y hepatojejunostomy. Potential carcinogenic factors were looked for in the cyststones using the Salmonella typhimurium plate test of Ames. High mutagenic activity was found in the stone extract incubated with the TA 98 tester strain, but not with the TA 100 strain. The test was negative with stone extracts obtained from seven patients operated on for chronic gallbladder disease. This study demonstrates the presence of a mutagenic chemical in the biliary tree of a patient with a clinical condition commonly associated with biliary tract cancer.Supported by Grants (N^o 10/80, 71/82, and 94/82) from the Direción de Investigación de la Pontificia Universidad Católica de Chile     

Course of choledochal cysts according to the type of treatment

Abstract

Objective. To compare natural course and treatment results of various types of choledochal cysts. Material and methods. Complication rate in treated (T) and natural course in not treated (NT) patients with choledochal cysts were compared. The level of bilioenteric anastomosis after cyst resection was categorized and their post-procedure courses were analyzed. Results. A total of 204 patients (T, 174; NT, 30) were included. Patients with initial malignancies were excluded from the outcome analysis (n = 13). Of the remaining 191 patients, the mean follow-up time was 35.9 months (range 6?310.7 months). Biliary stricture was the most troublesome post-operative complication, and occurred in 10 patients (6.1%). It occurred significantly more frequently in the above hilar anastomosis group than in at or below (p-trend = 0.017), and also with older age (p-trend = 0.019). Common bile duct stone was the most frequent adverse event among NT (42.9%). Overall, there were fewer complications among treated patients (T, 17.2%; NT, 67.9%; p < 0.001). Anomalous pancreaticobiliary union (APBU) was present in 69 patients (37.5%), which was associated with higher prevalence of carcinoma (APBU-present, 13%; APBU-absent, 3.5%; p = 0.019). No malignancy developed in both T and NT groups during follow-up. Conclusions. This study suggests, that even asymptomatic choledochal cysts should be treated, not only for prevention of malignancies, but also to lower the future adverse events. Bilioenteric anastomosis at larger caliber duct is recommended for prevention of post-operative biliary strictures.     

Prenatal diagnosis of choledochal cyst using magnetic resonance imaging： A case report

Choledochal cysts are congenital anomalies of the biliary ducts, characterized by cystic dilatation of the ducts. Prenatal diagnosis of this anomaly using ultrasonography (US) has been well documented. Magnetic resonance imaging (MRI) has recently become an important complement to US in prenatal diagnosis of fetal anomalies. We herein report a patient in whom at 24 wk' gestation US suggested a right upper quadrant abdominal cyst and in whom at 26 wk' gestation MRI more clearly delineated the cyst and its surrounding structures and suggested a choledochal cyst, which was confirmed at postnatal surgery and histopathology.     

A 15-year experience of two hundred and twenty five consecutive right hepatectomies

BackgroundIn case of liver tumor, surgical resection is the therapeutic gold standard to increase patient survival. Among liver resections, right hepatectomy (RH) is defined as a major hepatectomy. The first aim of this study was to analyze the overall morbidity and mortality of patients undergoing RH, the second aim was to assess changes in both patients characteristic and surgical parameters and mortality rates in a single center institution.MaterialsFrom 2001 to December 2015, 225 RH were performed in our center. We analyzed two time period: 2001–2007 and 2008–2015.ResultsNinety days post operative mortality was observed in 9 cases (4%) for the overall cohort. We observed a difference between the two groups in the use of Pringle Maneuver (p < 0,001). This result is consistent in each major surgical indication: HCC (p = 0,001), CLM (p = 0,015) and BT (p = 0,015). The estimated blood losses improved (p = 0,028), particularly for the HCC cases (p = 0,024). No difference was observed in terms of number of transfusions received between the two groups. Reduced length of stay was observed in the second group (p < 0,001), more markedly for CLM cases (p = 0,001).ConclusionTo further improve the outcomes of RH, it is important to performed this major hepatectomy in hepatobiliary centers with an overall liver resection experience of at least few hundred cases.     

Surgical management of intrahepatic cholangiocarcinoma: A 31-year experience

Intrahepatic cholangiocarcinoma (ICC) is the second most common malignant primary tumor of the liver. It is, though, a rare tumor and little is known regarding its natural history, clinicopathologic characteristics, or the outcomes of surgical therapy. We reviewed the experience of 61 patients with ICC seen by the surgical service at the Mayo Clinic over a 31-year period. Patient demographic and clinical data were recorded, as were survival statistics. Pathologic data were also obtained and patients stratified according to the TNM classification. Twenty-eight patients were resected for cure. Overall, 45 patients died of ICC. Of the patients resected for cure, survival at 3 years was 60%. No pathologic condition was found to be associated with the development of ICC. Overall survival correlated with stage of the tumor. Among patients resected for cure, stage did not correlate with survival. Prognosis for patients with ICC remains poor; resection, though, appears to prolong survival.     

婴儿肝炎综合征、胆道闭锁、胆总管囊肿与巨细胞病毒感染的关系

目的:探讨人巨细胞病毒(HCMV)感染与婴儿肝炎综合征(IHS)、胆道闭锁、胆总管囊肿的关系．方法:采用ELISA法和免疫组化法对IHS患儿 98例、胆道闭锁患儿50例、胆总管囊肿患儿 50例血尿及其母亲血和乳汁进行检测,同时检测62例非肝胆疾病患儿及其母亲．结果:血CMV-IgM阳性率IHS组61．2％,其母亲33．7％;胆道闭锁组56．0％,其母亲36．0％; 胆总管囊肿组22．0%,其母亲6．0％．对照组阳性率22．6％,其母亲8．1％．患儿尿HCMV抗原阳性率HIS组71．4％,其母亲乳汁HCMV抗原 91．8％;胆道闭锁组62．0％,其母亲82．0％;胆总管囊肿组20．0％,其母亲54．0％．对照组阳性率24．2％,其母亲乳汁56．5％．IHS组及胆道闭锁组患儿及其母亲阳性率分别高于对照组患儿及其母亲(P<0．01),胆总管囊肿组患儿及其母亲阳性率均不高于对照组患儿及其母亲 (P>0．05)．结论:IHS及胆道闭锁的发病与HCMV感染有关,胆总管囊肿的发病与HCMV感染无明显相关性．     

Smallest Todani’s type II choledochal cyst

A choledochal cyst is defined as an isolated or combined congenital dilation of the extra hepatic or intrahepatic biliary tree. Todani and colleagues proposed the five types of congenital choledochal cysts which have gained widespread acceptance. Type II choledochal cyst, a diverticulum of common bile duct, is rarest, and most reported cases of Type II were as large as several centimeters in size. We herein report the case of a small Type II choledochal cyst which was resected at pancreatoduodenectomy for carcinoma of the papilla of Vater. A 58-year-old Japanese male was referred to our hospital for the evaluation of jaundice. Preoperative cholangiogram via the percutaneous transhepatic biliary drainage tube revealed a complete obstruction at the narrow terminal segment. Furthermore, a small diverticular protrusion was demonstrated on the lower part of the common bile duct. The resected specimen showed a 2.2 x 1.7 x 1.2 cm carcinoma of the major papilla, and a deep, 2 mm in diameter and 5 mm in depth, depression on the posterior wall of the common bile duct. The anomalous pancreatobiliary duct was not seen. The deep depression was confirmed microscopically to penetrate the fibromuscular layer of the common bile duct and diagnosed as a Todani's Type II choledochal cyst. To our knowledge, the current case is the smallest Type II choledochal cyst which was completely resected.     

Computed tomography virtual endoscopy with angiographic imaging for the treatment of type IV-A choledochal cyst

Type IV-A choledochal cysts (CCs) are a congenital biliary anomaly which involve dilatation of the extrahepatic and intrahepatic bile ducts. We present the case of a 30-year-old woman with type IV-A CC, on whom three-dimensional computed tomography (3D CT) and virtual endoscopy were performed. 3D CT revealed partial dilatation in the posterior branch of the intrahepatic bile duct and a relative stricture between it and the extrahepatic bile duct. Virtual endoscopy showed that this stricture was membrane-like and separated from the surrounding blood vessels. Based on these image findings, complete cyst resection, bile duct plasty for the stricture, and hepaticojejunostomy were safely performed. To the best of our knowledge, there are no reports of imaging by virtual endoscopy of the biliary tract which show the surrounding blood vessels running along the bile duct.     

Diagnosis and treatment of congenital choledochal cyst: 20 years' experience in China

AIM To summarize the experience of diagnosis andtreatment of congenital choledochal cyst in the past 20years(1980-2000).METHODS The clinical data of 108 patients admitted from1980 to 2000 were analyzed retrospectively.RESULTS Abdominal pain,jaundice and abdominal masswere presented in most child cases.Clinical symptoms inadult cases were non-specific,resulting in delayeddiagnosis frequently.Fifty-seven patients(52.7%)hadcoexistent pancreatiobiliary disease.Carcinoma of thebillary duct occurred in 18 patients(16.6%).Ultrasonicexamination was undertaken in 94 cases,ERCP performedin 46 cases and CT in 71 cases.All of the cases werecorrectly diagnosed before operation.Abnormalpancreatobiliary duct junction was found in 39 patients.Before 1985 the diagnosis and classification of congenitalcholedochal cyst were established by ultrasonographypreoperatively and confirmed during operation,the mainprocedures were internal drainage by cyst enterostomy.After 1985,the diagnosis was established by ERCP andCT,and cystectomy with Roux-en-Y hepaticojejunostomywas the conventional procedures.In 1994,we reported anew and simplified operative procedure in order to reducethe risk of choledochal cyst malignancy.Postoperativecomplication was mainly retrograde infection of biliarytract,which could be controlled by the administration ofantibiotics,there was no perioperative mortality.CONCLUSION The concept in diagnosis and treatment ofcongenital choledochal cyst has obviously been changedgreatly.CT and ERCP were of great help in theclassification of the disease.Currently,cystectomy withRoux-en-Y hepaticojejunostomy is strongly recommendedas the choice for patients with type Ⅰ and type Ⅳ cysts.Piggyback orthotopic liver transplantation is indicated intype Ⅴ cysts(Caroli's disease)with frequently recurrentcholangitis.