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.

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.

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.

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: 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.

Biliary disease in children.

Biliary diseases in children are infrequent; however, they can be associated with high morbidity and mortality if an accurate diagnosis is not made and adequate treatment provided in a timely fashion. Biliary atresia, choledochal cysts, gallbladder disease, and Alagille syndrome can be associated with similar clinical symptoms, laboratory findings, and radiographic findings, which makes accurate diagnosis difficult. The correct treatment for each of these clinical entities is different and can significantly reduce morbidity and mortality from these diseases. In this article, we discuss the epidemiology, approach to diagnosis, prognosis, and treatment modalities for these four disease processes.

Choledochoceles: are they choledochal cysts?

OBJECTIVE: The aim of this analysis was to report a multidisciplinary series comparing choledochoceles to Todani Types I, II, IV, and V choledochal cysts. SUMMARY BACKGROUND DATA: Choledochoceles have been classified as Todani Type III choledochal cysts. However, most surgical series of choledochal cysts have reported few choledochoceles because they are managed primarily by endoscopists. METHODS: Surgical, endoscopic, and radiologic records were reviewed at the Riley Children's Hospital and the Indiana University Hospitals to identify patients with choledochal cysts. Patient demographics, presenting symptoms, radiologic studies, associated abnormalities, surgical and endoscopic procedures as well as outcomes were reviewed. RESULTS: A total of 146 patients with "choledochal cysts" including 45 children (31%) and 28 with choledochoceles (18%) were identified, which represents the largest Western series. Patients with choledochoceles were older (50.7 vs. 29.0 years, P < 0.05) and more likely to be male (43% vs. 19%, P < 0.05), to present with pancreatitis (48% vs. 24%, P < 0.05) rather than jaundice (11% vs. 30%, P < 0.05) or cholangitis (0% vs. 21%, P < 0.05), to have pancreas divisum (38% vs. 10%, P < 0.01), and to be managed with endoscopic therapy (79% vs. 17%, P < 0.01). Two patients with choledochoceles (7%) had pancreatic neoplasms. CONCLUSIONS: Patients with choledochoceles differ from patients with choledochal cysts with respect to age, gender, presentation, pancreatic ductal anatomy, and their management. The association between choledochoceles and pancreas divisum is a new observation. Therefore, we conclude that classifications of choledochal cysts should not include choledochoceles.

Choledochal cyst in two pediatric heart transplant patients.

Choledochal cyst is a relatively uncommon entity in Western countries. No reports of choledochal cyst in heart transplant patients have been reported to date. We report two cases of choledochal cyst in pediatric heart transplant recipients, one with post-transplant lymphoproliferative disorder (PTLD) within the cyst. The first patient had abdominal pain, increased liver enzymes and was seropositve for Epstein-Barr virus. A choledochal cyst with PTLD was removed 4 years after heart transplantation. The second patient presented 14 years after heart transplantation with a choledochal cyst that was excised for severe abdominal pain. This previously unreported association between choledochal cysts in conjunction with PTLD and heart transplantation is interesting and a possible common pathogenesis is proposed. The management and alternative treatments were briefly noted. We recommend an aggressive treatment for patients with suspected choledochal cyst after heart transplantation because of the increased potential for malignant transformation.

The current management of choledochal cysts.

Choledochal cyst disease is uncommon. The presentation of the disease is being seen more commonly in the adult population than in the pediatric population, particularly in the West, making this a diagnosis a general surgeon should consider when evaluating a patient with biliary disease. The diagnosis of this disease has been greatly facilitated by improving technologies, particularly MRCP and interventional techniques of PTC. The consequences of not treating choledochal cysts can result in malignant transformation. When possible, complete surgical excision of the cyst is recommended and should be performed as early as possible to prevent complications and the progression of liver fibrosis. Long-term follow-up is required for surveillance for late complications and for cancer, particularly in type IV and V choledochal cysts where complete excision is not possible.

Case of a closed choledochocele with biliary and pancreatic drainage via the accessory duct causing acute pancreatitis.

Type III choledochal cysts, also called choledochoceles, are rare cystic dilatations of the terminal biliary tree. We report the case of a 'closed choledochocele', in which the bile and ventral pancreatic ducts emptied into the cyst, but because there was no outlet into the duodenum, drainage was retrograde via the accessory duct. This type of choledochocele variant has never previously been described in the medical literature.

[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.

Preoperative endoscopic retrograde cholangiopancreatographic treatment of complicated choledochal cysts in children: a retrospective case series.

We report our experience with endoscopic retrograde cholangiopancreatography-(ERCP-)based interventions in children with complicated choledochal cysts that are refractory to conservative management. Between 1999 and 2006, 42 children (12 boys, 30 girls; median age 3 years, range 2-14.5 years) were admitted for surgical treatment of choledochal cysts. Seven of these patients (16.7%; one boy, six girls; median age 3 years, range 2-12 years) showed signs of complicated choledochal cysts, and presented with pancreatitis/cholangitis (n = 4) and obstructive jaundice (n = 3). The anatomical classification of the cysts was type Ic (n = 3), type If (n = 3), and type IV (n = 1). ERCP was successfully performed in 6/7 patients, and therapeutic interventions included removal of debris (n = 3), sphincterotomy (n = 3), and stent placement (n = 4). One patient required blood transfusion for post-sphincterotomy bleeding. The patient in whom the ERCP failed underwent ultrasound-guided percutaneous transhepatic biliary drainage. Definitive surgery was performed after a median interval of 10 days (range 7-68 days) after the ERCP intervention.

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.

[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.

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.