Congenital Bile Duct Dilatation Research Articles

CHOLEDOCAL CYST Pada DEWASA: PERAN ULTRASONOGRAFI

Choledocal cyst (CC) is a rare congenital cystic dilatation of bile duct. The etiology is unknown, and likely multifactorial and it is uncertain whether they are congenital or acquired. Ultrasonography is initial imaging which can be used to evaluate biliary system. We report 43 year old female presented with icteric for 3 months. She had dark-color urine and white stool. She didn’t complain about abdominal pain or palpable mass in her abdomen.We performed ultrasonography and found dilatation of common bile duct for ± 7.4 cm. Gall bladder and another intrahepatic structures were normal. The patient was referred to digestive surgeon in adequate facility. Conclusion: Ultrasonography is initial and non-invasive imaging for viewing biliary system. Early detection and management can prevent morbidity and improve patient’s quality of life.

Neuroendocrine carcinoma of the common bile duct associated with congenital bile duct dilatation: a case report

BackgroundCholangiocarcinoma is frequently observed in patients with congenital bile duct dilatation (CBDD). Most cholangiocarcinomas are adenocarcinomas. Other types, especially neuroendocrine carcinomas (NECs), are rare. To the best of our knowledge, this is the third reported case of an NEC of the common bile duct associated with CBDD and the first to receive adjuvant chemotherapy for advanced disease.Case presentation A 29-year-old woman presented with upper abdominal pain. Preoperative imaging indicated marked dilatation of the common bile duct and a tumor in the middle portion of the common bile duct. She was suspected of having distal cholangiocarcinoma associated with CBDD and underwent pylorus-preserving pancreaticoduodenectomy. Pathological and immunohistological findings led to a final diagnosis of large-cell NEC (pT3aN1M0 pStageIIB). The postoperative course was uneventful, and she was administered cisplatin and irinotecan every 4 weeks (four cycles) as adjuvant chemotherapy. She has remained recurrence-free for 16 months.ConclusionsNEC might be a differential diagnosis in cases of cholangial tumor associated with congenital bile duct dilatation. This presentation is rare and valuable, and to establish better treatment for NEC, further reports are necessary.

Management of Congenital Cystic Dilation of Common Bile Duct in Dakar (Senegal)

Congenital cystic dilatation of common bile duct is a rare condition in Africa and the West. Its discovery is often fortuitous. The diagnosis is based on imagery. Our goal was to report 3 cases followed by a literature review. Three patients were received, most often for pain of the right hypochondrium. There were 2 women and 1 man aged between 16 and 27 years old. The physical examination was normal. The abdominal CT scan allowed us to diagnose a IA Todani cyst in all patients, including one degenerate. Cholecystectomy with resection of the bile duct, followed by hepatico-jejunal anastomosis on anse-en-Y of Roux was performed in 2 patients. The immediate suites were simple. Histology showed inflammation on 2 operative specimen and cholangiocarcinoma on the 3rd. Choledochal DKC is a rare condition, often revealed in adults by complications. Bilio-pancreatic CT is an alternative to cholangio-MRI for its diagnosis with a type I Todani easily recognized. The treatment is surgical with a bad prognosis in case of degeneration.

Planned hepatectomy for the"central type"intrahepatic and extrahepatic choledochal cysts

Objective To retrospectively study the clinical value and the advantages inplanned hepatectomyfor thecentral typeintrahepatic and extrahepatic choledochal cysts. Methods The clinical data of 7 patients with thecentral typeof intrahepatic and extrahepatic choledochal cysts which were treated withplanned hepatectomyfrom January 2014 through April 2017 at the Department of Biliary Tract Surgery of the Eastern Hepatobiliary Surgery Hospital, Second Military Medical University were retrospectively analyzed. Results All the patients completed radical resection of the intrahepatic and extrahepatic choledochal cysts in accordance with theplanned hepatectomy. The operations included 6 patients who were treated with percutaneous transhepatic cholangial drainage (PTCD) and 5 patients with portal vein embolization (PVE) prior to the surgical excision. Combined right liver resection was performed in 6 patients, and combined left liver resection in one patient. All the 7 patients had a history of chronic cholangitis. Liver volume tests demonstrated that the hemiliver volume to be removed (the embolized hemiliver) significantly decreased after PVE, whereas the hemilivers to be persevered were remarkably enlarged. No complication associated with PTCD and PVE occurred. The mean postoperative hospitalization was 12 days. Liver function tests suggested all the patients recovered well. No postoperative complication of bleeding, infection or liver function failure was observed, except in one patient who experienced pleural and abdominal effusion. Conclusions Combined subtotal hepatectomy may increase the risk of complications associated with thecentral typeintrahepatic and extrahepatic choledochal cysts. The surgical strategy in planned hepatectomy can be used effectively to treat thecentral typeof intrahepatic and extrahepatic choledochal cysts, with improved surgical safety, decrease in incidences of postoperative liver function failure and residual choledochal cysts. Key words: Intrahepatic and extrahepatic bile duct cysts; Congenital cystic dilatation of bile duct; Planned hepatectomy; Percutaneous transhepatic cholangial drainage (PTCD); Portal vein embolization (PVE)

Genetic study of congenital bile-duct dilatation identifies de novo and inherited variants in functionally related genes.

BackgroundCongenital dilatation of the bile-duct (CDD) is a rare, mostly sporadic, disorder that results in bile retention with severe associated complications. CDD affects mainly Asians. To our knowledge, no genetic study has ever been conducted.MethodsWe aim to identify genetic risk factors by a “trio-based” exome-sequencing approach, whereby 31 CDD probands and their unaffected parents were exome-sequenced. Seven-hundred controls from the local population were used to detect gene-sets significantly enriched with rare variants in CDD patients.Results Twenty-one predicted damaging de novo variants (DNVs; 4 protein truncating and 17 missense) were identified in several evolutionarily constrained genes (p < 0.01). Six genes carrying DNVs were associated with human developmental disorders involving epithelial, connective or bone morphologies (PXDN, RTEL1, ANKRD11, MAP2K1, CYLD, ACAN) and four linked with cholangio- and hepatocellular carcinomas (PIK3CA, TLN1 CYLD, MAP2K1). Importantly, CDD patients have an excess of DNVs in cancer-related genes (p < 0.025). Thirteen genes were recurrently mutated at different sites, forming compound heterozygotes or functionally related complexes within patients.ConclusionsOur data supports a strong genetic basis for CDD and show that CDD is not only genetically heterogeneous but also non-monogenic, requiring mutations in more than one genes for the disease to develop. The data is consistent with the rarity and sporadic presentation of CDD.Electronic supplementary materialThe online version of this article (doi:10.1186/s12920-016-0236-z) contains supplementary material, which is available to authorized users.

Effects of conventional versus laparoscopic surgery in treatment of congenital bile duct dilatation: a comparative analysis

Effects of conventional versus laparoscopic surgery in treatment of congenital bile duct dilatation: a comparative analysis

共通管から分枝の派生を認めた先天性胆道拡張症の1例

共通管から分枝の派生を認めた先天性胆道拡張症の1例

先天性胆道拡張症に対する分流手術後の両葉多発肝内結石に対し肝左葉切除術と切石用空腸瘻造設術を施行した1例

先天性胆道拡張症に対する分流手術後の両葉多発肝内結石に対し肝左葉切除術と切石用空腸瘻造設術を施行した1例

Surgical treatment of congenital bile duct dilatation in adults

先天性胆管扩张症(bile duct dilatation,BDD)是一种以胆管扩张和胰管-胆管合流异常为特征的胆道疾病,通常在儿童期发病,但也有少部分患者病情迁延至成年.而成人型BDD的病程长,合并症多,部分患者往往接受多次不恰当手术[1].本研究回顾性分析2000年1月至2010年12月昆明医科大学第一附属医院收治的76例行手术治疗的成人BDD患者的临床资料,旨在探讨其外科治疗方法及疗效. 关键词：胆道疾病;胆管扩张症;成人

先天性胆道拡張症分流手術後の肝内結石症に対する肝切除の経験

先天性胆道拡張症分流手術後の肝内結石症に対する肝切除の経験

Relationship between pancreaticobiliary maljunction and gallbladder carcinoma: a meta-analysis

Reports on the relationship between pancreaticobiliary maljunction (PBM) and gallbladder carcinoma (GBC) are conflicting. The frequency of PBM in GBC patients and the clinical features of GBC patients with PBM vary in different studies. English-language articles describing the association between PBM and GBC were searched in the PubMed and Web of Science databases. Nine case-control studies fulfilled the inclusion criteria and addressed the relevant clinical questions of this analysis. Data were extracted independently by two reviewers using a predefined spreadsheet. The incidence of PBM was higher in GBC patients than in controls (10.60% vs 1.76%, OR: 7.41, 95% CI: 5.03 to 10.87, P<0.00001). The proportion of female patients with PBM was 1.96-fold higher than in GBC patients without PBM (80.5% vs 62.9%, OR: 1.96, 95% CI: 1.09 to 3.52, P=0.12). GBC patients with PBM were 10 years younger than those without PBM (SMD: -9.90, 95% CI: -11.70 to -8.10, P<0.00001). And a difference in the incidence of associated gallstone was found between GBC patients with and without PBM (10.8% vs 54.3%, OR: 0.09, 95% CI: 0.05 to 0.17, P<0.00001). Among the GBC patients with PBM, associated congenital dilatation of the common bile duct was present with a higher incidence ranging from 52.2% to 85.7%, and 70.0%-85.7% of them belonged to the P-C type of PBM (the main pancreatic duct enters the common bile duct). No substantial heterogeneity was found and no evidence of publication bias was observed. PBM is a high-risk factor for developing GBC, especially the P-C type of PBM without congenital dilatation of the common bile duct. To prevent GBC, laparoscopic cholecystectomy is highly recommended for PBM patients without congenital dilatation of the common bile duct, especially relatively young female patients without gallstones.

Surgical treatment of giant congenital bile duct dilatation: an analysis of 20 cases

Surgical treatment of giant congenital bile duct dilatation: an analysis of 20 cases

Liver resection for intrahepatic stones in congenital bile duct dilatation

This study reports our clinical experience with liver resection for congenital dilatation of the intrahepatic bile duct and intrahepatic gallstones to evaluate results and define indications for treatment. We studied the clinical data of patients who underwent hepatic resection for intrahepatic lithiasis from January 1992 to December 2008 and assessed the immediate and long-term results of these interventions. Of 49 treated patients, 47 underwent liver resection. In the majority of cases, the disease was limited to the left lobe and left hepatectomy was the most commonly performed surgical procedure. The operative mortality was zero with morbidity in 24.5% of patients. Cholangiocarcinoma was diagnosed in six cases (12.2%). In 91.6% of cases the long-term results were good or satisfactory. Treatment goals in all cases should be the elimination of intrahepatic stones, the prevention of recurrent lithiasis, and prevention or cure of cholangiocarcinoma. Surgical excision is the best possible treatment for symptomatic patients with localized disease and atrophy of the affected liver.

Congenital dilatation of the common bile duct and pancreaticobiliary maljunction—clinical implications

CLINICAL CONDITIONS AND DIAGNOSIS: Congenital dilatation of the common bile duct is a disease in which the extrahepatic bile duct, or both the extra and intrahepatic bile ducts, is dilated in various ways. Pancreaticobiliary maljunction is a disease in which the pancreatic duct meets the bile duct outside of the duodenal wall beyond the sphincter Oddi. Recently, these diseases have been thought to be closely related to each other but to be different malformations. Biliary tract carcinoma, especially bile duct carcinoma, is found in about 30% of patients with congenital dilatation of the bile duct. The concomitance of bile and pancreatic juice and their stasis in the biliary tract induce cellular proliferation and reproduction and stimulate genetic alterations in biliary epithelium, which may play an important role in carcinogenesis of the bile duct. Endoscopic retrograde cholangiopancreatography is useful for examining pancreaticobiliary maljunction. The operation is dilated bile duct resection and hepaticojejunostomy, which ensure that pancreatic juice and bile do not mix in the bile duct. Gallbladder carcinoma develops in more than 90% of pancreaticobiliary maljunction without bile duct dilatation.

B-036 PAR2(protease activated receptor-2) in proliferation of biliary epithelium in congenital bile duct dilatation(Oral Presentation Hepatobiliary, pancreas,Better Life for Sick Children, Better Future for Pediatric Surgery,the 45th Annual Meeting of Japanese Society of Pediatric Surgeons)

B-036 PAR2(protease activated receptor-2) in proliferation of biliary epithelium in congenital bile duct dilatation(Oral Presentation Hepatobiliary, pancreas,Better Life for Sick Children, Better Future for Pediatric Surgery,the 45th Annual Meeting of Japanese Society of Pediatric Surgeons)

Familial occurrence of congenital bile duct dilatation

Congenital bile duct dilatation (CBD) that developed in a parent and son is presented. Familial occurrence of CBD is rare, with only a few male cases having been reported. Since the initial report of CBD occurring in siblings in 1981, a total of 20 cases (10 pairs) have been published as of 2007. Clinical and genetic features of CBD are discussed.

A case of Todani's type Ib congenital cystic dilatation of the common bile duct

A case of Todani's type Ib congenital cystic dilatation of the common bile duct

Pseudopancreatitis and choledochal cyst.

choledocal cysts (1). Although it is considered a congenital illness, 30% of cases are diagnosed in the adult age. Clinical symptoms are quite variable: the classical triad of abdominal pain, jaundice and abdominal mass is exceptional in adult subjects. This disease occasionally presents with epigastric pain, nausea, vomiting, fever, increased blood bilirubin, and increased blood amylase, suggesting the diagnosis of acute pancreatitis. However, this diagnosis that is not confirmed by imaging techniques or laparotomy studies. This form of clinical presentation of choledochal cysts has been designated pseudopancreatitis (4) or fictitious pancreatitis (3), which usually has a favorable outcome. In this report, we present two cases that clinically started with the aforementioned symptoms, and in which a diagnosis of congenital cystic dilatation of the common bile duct was finally established. No acute pancreatitis was confirmed. In both cases a surgical operation was performed with resection of the dilated bile duct and restoring biliary-enteric continuity using a Roux-en-Y hepaticojejunostomy (5). Results of this treatment was satisfactory in both patients. This type of pathology is controversial as to whether increased blood amylase is due to a mild form pancreatitis or it is not caused by a acute pancreatitis (3,4). The clinical, anatomical and histological features of these cases support the role of pancreatic reflux and the obstructive component associated with the pathophysiology of choledochal cysts (5). Both the observation of normal pancreas during surgical laparotomy and the absence of morphological pancreatic alterations on radiologic imaging in our two cases support the latter option. Considering all this, the term “pseudopancreatitis” seems to be appropriate to define this characteristic manifestation of choledochal cysts. Pseudopancreatitis and choledochal cyst

人間ドッグにて偶然発見された早期胆嚢癌合併先天性胆道拡張症の1例

人間ドッグにて偶然発見された早期胆嚢癌合併先天性胆道拡張症の1例

8. Lack of evidence for reovirus infection in tissues from patients with biliary atresia and congenital dilatation of bile duct

8. Lack of evidence for reovirus infection in tissues from patients with biliary atresia and congenital dilatation of bile duct