[Cyst of the broken common bile duct: literature review]. / Kyste du cholédoque rompu: revue de la littérature.

Cystic malformations of the bile ducts are rare congenital disorders, with an incidence of 1/2000000 live births. Complication including the angiocholitis, chronic pancreatitis, progressive biliary cirrhosis, portal hypertension or gallbladder lithiases can reveal severe disorder. Spontaneous perforation is one of the rare complications described for the first time in 1934 by Weber. We report the case of an 18-month old baby admitted with subocclusive syndrome with biliary peritonitis. Ultrasound was performed showing abdominal effusion with cystic formation communicating with the bile ducts associated with subcapsular effusion of the liver confirmed by a scanner. Treatment was based on peritoneal toilet with redon drain at the level of the perforation and subhepatic drain without cyst excision. The patient was re-admitted 6 months after this incident to be definitively treated.

Transitional care for patients with surgical pediatric hepatobiliary disease: Choledochal cysts and biliary atresia.

Choledochal cysts (CDCs) and biliary atresia (BA) are rare pediatric hepatobiliary anomalies that require surgical intervention due to increased risk of malignancy and liver failure, respectively. The underlying disease and operative procedures place patients at risk for long-term complications, which may continue to affect them into adulthood. Lack of a transitional care model in the health-care system potentiates the challenges they will face following aging out of their pediatric providers' care. We sought to elucidate the long-term complications and challenges patients with CDCs and BA face, review the current literature regarding transitioning care, and propose guidelines aiding adult providers in continued care and surveillance of these patients. A literature review was performed to assess short-term and long-term complications after surgery and the current standards for transitioning care in patients with a history of CDCs and BA. While transitional programs exist for patients with other gastrointestinal diseases, there are few that focus on CDCs or BA. Generally, authors encourage medical record transmission from pediatric to adult providers, ensuring accuracy of information and compliance with treatment plans. Patients with CDCs are at risk for developing biliary malignancies, cholangitis, and anastomotic strictures after resection. Patients with BA develop progressive liver failure, necessitating transplantation. There are no consensus guidelines regarding timing of follow up for these patients. Based on the best available evidence, we propose a schema for long-term surveillance.

Choledochocele as an atypical cause of relapsing acute pancreatitis: diagnosis and endoscopic treatment.

The choledochocele is a cystic dilatation of the intraduodenal portion of the bile duct and corresponds to the type III biliary cysts in the Todani ´s classification. Eventhough the majority of patients remain asymptomatic they can be an atypical cause of abdominal pain or relapsing acute pancreatitis events. The risk of malignancy is lower than other choledochal cyst (<2,5%). The treatment is based on surgical or new endoscopic techniques of resection. In some of the cases an endoscopic sphincterotomy is the first approach.

Japanese clinical practice guidelines for congenital biliary dilatation.

Until now, there have been no practical clinical guidelines for congenital biliary dilatation (CBD). In this review article, the Japanese Study Group on Congenital Biliary Dilatation (JSCBD) propose to establish clinical practice guidelines for CBD. Because the evidence-based literature is relatively small, we decided to create guidelines based on the consensus of experts, using the medical literature for reference. A total of 20 clinical questions (CQs) were considered by the members of the editorial committee responsible for the guidelines. The CQs included the distinct aspects of CBD: (1) Concepts and Pathology (three CQs); (2) Diagnosis (six CQs); (3) Pancreaticobiliary Complications (three CQs); Treatments and Prognosis (eight CQs). Each statements and comments for CQs were made by the guidelines committee members. CQs were finally approved after review by members of the editorial committee and the guidelines evaluation board of CBD. These guidelines were created to provide assistance in the clinical practice of CBD management; their contents focus on clinical utility, and they include general information on CBD to make this disease more widely recognized.

Ectopic Opening of the Common Bile Duct Accompanied by Choledochocele and Pancreas Divisum.

A 32-year-old woman was referred due to abdominal pain and elevated liver enzymes. Computed tomography and magnetic resonance imaging showed ectopic opening of the common bile duct (CBD) into the duodenal bulb. Esophagogastroduodenoscopy showed a hemispheric bulge in the duodenal bulb. Endoscopic retrograde cholangiopancreatography (ERCP) revealed the bulge to be cystic dilatation of the CBD. ERCP also showed no communication between the ventral and dorsal pancreatic ducts. We diagnosed the patient with ectopic opening of the CBD accompanied by choledochocele and pancreas divisum. Endoscopic incision was performed for the treatment of the choledochocele. The patient's symptoms and elevated liver enzymes improved after treatment.

[Choledochal cyst during pregnancy. Report of 3 cases and a literature review]. / Quiste de colédoco y embarazo. Reporte de 3 casos y revisión de la bibliografía.

BACKGROUND: Choledochal cysts are rare. They usually present during childhood in women, but it can also be seen during pregnancy. Clinical signs and symptoms are obscured during this time, thus it can complicate the diagnosis and represent a life threatening complication for both the mother and the child. OBJECTIVE: To communicate the case of 3 pregnant patients with choledochal cyst. CLINICAL CASES: Three pregnant women in which choledochal cyst were diagnosed. Two developed signs of cholangitis. The first one underwent a hepatic-jejunostomy, but had an abortion and died on postoperative day 10. The second one had a preterm caesarean operation due to foetal distress and underwent a hepatic-jejunostomy 4 weeks later; during her recovery she had a gastric perforation and died of septic complications. The third one did not develop cholangitis or jaundice. She had an uneventful pregnancy and had a hepatic-jejunostomy 4 weeks later with good results. CONCLUSIONS: Management of choledochal cysts during pregnancy is related to the presence of cholangitis. When they do not respond to medical treatment, decompression of the biliary tree is indicated. Definitive treatment should be performed after resolution of the pregnancy.

A case series of congenital hepatic hilar cyst: recommendations for diagnosis and management.

BACKGROUND: Congenital hepatic hilar cysts are rare. Some are simple and do not require intervention, but some biliary cystic malformations impose the risk of morbidity and mortality. OBJECTIVES: To assess a series of five patients presenting with congenital hepatic hilar cysts. METHODS: We retrospectively reviewed all cases presenting to our pediatric surgical service between January 2010 and December 2012 and found to have a congenital hepatic hilar cyst. Data regarding clinical, radiological, operative and pathological features were analyzed. RESULTS: Five children with congenital cyst of the hepatic hilum were identified; four of them were diagnosed prenatally. Four children had undergone surgical intervention: one with intrahepatic choledochal cyst, one with epidermoid cyst, and two with biliary atresia and an associated cyst of the common bile duct. In another case of choledochal cyst the treatment was conservative. All children except one had a good prognosis; one child with biliary atresia required liver transplantation. CONCLUSIONS: The differential diagnosis of congenital hepatic hilar cyst includes a broad spectrum of pathologies. It is essential to diagnose biliary atresia as early as possible. Signs such as smaller cysts in association with a hypoplastic gallbladder and direct hyperbilirubinemia may be suggestive of biliary atresia.

Colestasis y pancreatitis como forma de presentación de quiste de colédoco forma frustra / A forme fruste choledochal cyst presenting as cholestasis and pancreatitis

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Endoscopic management of choledochocele complicated with choledocholithiasis and pancreatitis in an old patient.

Choledochocele, or type III choledochal cyst, is a rare congenital disease and is even less common among adults compared with children. In this case, a 75-year-old female was admitted to our hospital presented with epigastric pain and vomiting for one day. Abdominal computed tomography revealed dilated common bile duct, pancreatitis and peripancreatic effusion. The patient was treated with fasting, fluid resuscitation, anti-acid agents, somatostatin and antibiotics. Endoscopic retrograde cholangiopancreatography was employed for the further diagnosis of choledochocele, choledocholithiasis and biliary stenosis. Endoscopic sphincterotomy, stone extraction and plastic stent placement were performed for treatment. The patient recovered quickly after the treatment and no signs of recurrence and complications were observed during the first follow-up. Endoscopic management may be a promising and alternative therapy for choledochocele although long-term follow-up is necessary to confirm the efficacy and safety of this procedure in the future.

Coagulopathy in a subtype of choledochal cyst and management strategy.

AIM: To evaluated our management algorithm of the coagulopathy. We evaluated our management algorithm of the coagulopathy. METHODS: Between October 2001 and January 2013, 160 CDC children with coagulopathy (fibrinogen, FIB < 2 g/L) were recruited. FIB ≥ 1 g/L is generally required for safe elective surgery. We used FIB level as an indicator when: (1) patients with FIB levels between 1-2 g/L underwent one-stage definitive operation; and (2) patients with FIB < 1 g/L underwent 3 d of medical treatment. Thereafter, those with FIB ≥ 1 g/L underwent one-stage definitive operation whereas those with FIB < 1 g/L underwent external biliary drainage to allow liver function improvement. Those patients with liver function improvements underwent definitive operation after 7 d of drainage. RESULTS: After preoperative optimization, 92.5% of CDC children with coagulopathy underwent successful one-stage definitive operation. The remaining 7.5% of CDC children required initial external bile drainage, and underwent definitive operation 11 d after the admission. The mean operative time and postoperative recovery duration were comparable to those with normal coagulations. The median follow-up period was 57 mo. No blood transfusion or other postoperative complications were encountered. CONCLUSION: Following our management protocol, the majority of CDC children with coagulopathy can be managed with one-stage definitive operation.

Biliary cyst in pregnancy managed by percutaneous drainage: a case report.

BACKGROUND: A maternal biliary cyst diagnosed in pregnancy is extremely rare. The condition can be asymptomatic or can present with chronic and intermittent abdominal pain, rupture of the cyst, and liver function test abnormalities. Management depends on the gestational age and the patient's condition when it is diagnosed. CASE: We present a case of a symptomatic type IV biliary cyst with abnormal liver function tests diagnosed in the second trimester of pregnancy and treated with a percutaneous drainage. The patient delivered vaginally at term and developed obstruction of the percutaneous drain with superimposed bacterial infection postpartum. She was lost to follow-up intermittently throughout her treatment. CONCLUSION: Symptomatic biliary cyst in pregnancy may require palliative invasive intervention until delivery.

Choledochal cysts: diagnosis and treatment.

The aim of this study is to show the different diagnostic procedures and treatment in patients diagnosed with congenital choledochal cysts. Choledochal cysts are congenital anomalies of the bile ducts and include cystic dilatation of the extrahepatic and intrahepatic biliary ducts or both. The study shows ten patients diagnosed as having choledochal cysts. Diagnosis was established by clinical and radiographic findings including: ultrasound (US), magnetic resonance cholangiopancreatograpy (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic cholangiography (PTC) and cytological examination of the bile juice. In the study choledochal cysts were classified according to the Todani classification. Most common cysts were type I (six cases); type III (one case), type IVa (one case) and two patients were type V cysts (Caroli disease). The most frequent symptoms were abdominal pain, jaundice and cholangitis. US findings were sensitive for the preliminary diagnosis of choledochal cysts in all the patients. MRCP accurately defined the cyst anatomy and the site of the biliary origin in all the cases with extrahepatic cysts. In three cases ERCP clearly demonstrated the cyst and by PTC smaller cysts were well defined. Cytological examination of the bile juice obtained during the PTC procedure showed malignant cells in one case. Therefore pancreaticoduodenectomy was performed and pathological examination showed associated cholangiocarcinoma. Five years after the operation the patient was well and free of the disease. Five patients underwent surgical treatment with a total cyst excision and Roux-en-Y hepaticojejunostomy while the surgical approach in two patients was partial cyst excision and cystojejunostomy. Patients with Caroli disease were conservatively treated and 3 with interventional endoscopic procedures. Despite US evidence suggesting choledochal cyst diagnosis, other supportive radiographic imaging modalities such as MRCP, ERCP and PTC are required to define the precise cyst anatomy and are essential for the preoperative assessment. Total cyst excision is recommended for reducing cyst-related complications and risk of cholangiocarcinoma.

Choledochal cysts: our ten year experience.

We present our experience in the management of choledochal cysts from 1999 to 2009. A retrospective review of all charts with a diagnosis of choledochal cysts in our institution in this ten-year period. Data was collated using Excel. A total of 17 patients were diagnosed with choledochal cyst: 9 females and 8 males. The average age at diagnosis was 28 months (range from 0 to 9 years). The most common presenting symptoms were obstructive jaundice 6 (35%) and abdominal pain and vomiting 4 (23%). Ultrasound (US) was the initial diagnostic test in all cases with 4 patients requiring further investigations. All patients underwent Roux-en-Y Hepaticojejunostomy. The average length of stay was 11 days. Patients were followed up with Liver Function Tests (LFTS) and US 4-6 weeks post-operatively. Three patients developed complications including post-op collection, high drain output requiring blood transfusion and adhesive bowel obstruction. Our overall experience with choledochal cyst patients has been a positive one with effective management and low complication rates.

Management of choledochal cyst with portal hypertension.

Portal hypertension (PHT) is a rare complication associated with choledochal cysts. Management issues of PHT patients are inadequately addressed, as its incidence is low and underlying causes variable. We report three cases of choledochal cyst with PHT. All patients had type IVa choledochal cysts, and the causes of PHT were secondary biliary cirrhosis (SBC) (two cases) and alcoholic liver disease (one case). Clinical presentation included jaundice, gastrointestinal bleeding and ascites. One patient with SBC successfully underwent excision with Rouxen-Y hepaticojejunostomy, while the patient with cholangitis was managed with endoscopic retrograde cholangiopancreatography stenting. The last patient with alcoholic liver disease was managed conservatively for seven years and died of liver failure. Management of choledochal cysts depends on the severity of liver disease in cases of cirrhosis of unrelated cause, while those with SBC should be considered for surgical management. Endoscopic stenting may be considered as a temporary measure in high-risk cases.