RESUMEN
PURPOSE: Pancreaticobiliary maljunction (PBM) without biliary dilatation is a condition in which dilatation of the bile duct is not seen in patients with PBM. Recently, the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) published new diagnostic criteria for PBM. In these criteria, biliary dilatation is defined according to the standard diameter at each age. We reviewed cases of pediatric patients with PBM without biliary dilatation. METHODS: From 1992 to 2019, 134 patients with PBM were treated in our institution. Among these, 7 patients were retrospectively diagnosed with PBM without biliary dilatation. The clinical information was retrospectively assessed in these patients. RESULTS: Of the seven patients, six were female. All patients had symptoms similar to those of patients with congenital biliary dilatation. In all seven patients, the diagnosis of PBM was made before definitive surgery. Six patients had type B PBM, and one had type D PBM. All patients underwent extrahepatic bile duct resection and hepaticojejunostomy, and their symptoms resolved. One patient experienced postoperative complications of anastomotic leakage followed by anastomotic stricture. CONCLUSION: The present report revealed important clinical features of this entity. However, there are still some issues that need to be discussed, and further research is needed.
Asunto(s)
Conductos Biliares Extrahepáticos/cirugía , Mala Unión Pancreaticobiliar/cirugía , Fuga Anastomótica , Conductos Biliares/patología , Procedimientos Quirúrgicos del Sistema Biliar/métodos , Niño , Preescolar , Quiste del Colédoco , Dilatación Patológica , Femenino , Humanos , Lactante , Yeyunostomía/métodos , Masculino , Mala Unión Pancreaticobiliar/clasificación , Mala Unión Pancreaticobiliar/diagnóstico , Mala Unión Pancreaticobiliar/patología , Complicaciones Posoperatorias , Estudios RetrospectivosAsunto(s)
Neoplasias del Sistema Biliar/diagnóstico , Mala Unión Pancreaticobiliar/diagnóstico , Adulto , Anciano , Antimetabolitos Antineoplásicos/uso terapéutico , Neoplasias del Sistema Biliar/etiología , Neoplasias del Sistema Biliar/cirugía , Colecistectomía , Desoxicitidina/análogos & derivados , Desoxicitidina/uso terapéutico , Femenino , Hepatectomía , Humanos , Masculino , Persona de Mediana Edad , Mala Unión Pancreaticobiliar/complicaciones , Mala Unión Pancreaticobiliar/cirugía , Pancreaticoduodenectomía , Pronóstico , GemcitabinaAsunto(s)
Neoplasias de los Conductos Biliares/diagnóstico , Carcinoma Ductal/diagnóstico , Neoplasias de la Vesícula Biliar/diagnóstico , Vesícula Biliar/patología , Conductos Pancreáticos/patología , Mala Unión Pancreaticobiliar/diagnóstico , Adenocarcinoma/complicaciones , Adenocarcinoma/diagnóstico , Adenocarcinoma/cirugía , Anciano , Neoplasias de los Conductos Biliares/complicaciones , Neoplasias de los Conductos Biliares/cirugía , Carcinoma Ductal/complicaciones , Carcinoma Ductal/cirugía , Colecistectomía , Femenino , Vesícula Biliar/cirugía , Neoplasias de la Vesícula Biliar/complicaciones , Neoplasias de la Vesícula Biliar/cirugía , Humanos , Conductos Pancreáticos/cirugía , Mala Unión Pancreaticobiliar/complicaciones , Mala Unión Pancreaticobiliar/cirugía , PancreaticoduodenectomíaAsunto(s)
Quiste del Colédoco/diagnóstico por imagen , Conducto Cístico/diagnóstico por imagen , Mala Unión Pancreaticobiliar/diagnóstico por imagen , Síndrome Poscolecistectomía/diagnóstico por imagen , Colangiopancreatografia Retrógrada Endoscópica , Colecistectomía Laparoscópica , Quiste del Colédoco/complicaciones , Quiste del Colédoco/diagnóstico , Femenino , Humanos , Imagenología Tridimensional , Imagen por Resonancia Magnética , Mala Unión Pancreaticobiliar/complicaciones , Mala Unión Pancreaticobiliar/diagnóstico , Síndrome Poscolecistectomía/complicaciones , Síndrome Poscolecistectomía/diagnóstico , Adulto JovenRESUMEN
BACKGROUND: Pancreatic juice reflux to the common bile duct and gallbladder is observed in the pancreaticobiliary maljunction (PBM), and various pathological conditions occur in the biliary tract. However, the mechanism of pancreatic juice reflux has not been discussed yet. This study aimed to investigate the mechanism of this phenomenon from the perspective of the fluid dynamics theory. METHODS: A fluid dynamics model of PBM without biliary dilatation having gallbladder function and of the pressure of sphincter of Oddi was developed. Water (as bile juice and pancreatic juice) was flowed to these models with a flow rate similar to that in humans. Pancreatic and bile juice flow and bile duct pressure were observed in three phases of gallbladder function. Moreover, the same experiment was performed in the PBM without biliary dilatation model without gallbladder. RESULTS: Pancreatic juice reflux could be observed when the gallbladder was passively expanded with the pressure in the bile duct lower than that in the sphincter of Oddi. However, pancreatic juice reflux was not observed in the model without gallbladder. CONCLUSIONS: Gallbladder function may be strongly involved in pancreatic juice reflux in PBM without biliary dilatation. Cholecystectomy may be able to stop the reflux of pancreatic juice.
Asunto(s)
Conducto Colédoco/fisiopatología , Conductos Pancreáticos/fisiopatología , Jugo Pancreático , Mala Unión Pancreaticobiliar/fisiopatología , Colangiopancreatografia Retrógrada Endoscópica , Conducto Colédoco/diagnóstico por imagen , Humanos , Hidrodinámica , Conductos Pancreáticos/diagnóstico por imagen , Mala Unión Pancreaticobiliar/diagnósticoRESUMEN
BACKGROUND: In pancreaticobiliary maljunction (PBM), reflux of pancreatic juice and bile produces various pathological conditions in the biliary tract and pancreas. Clinical features according to the classification of PBM by confluence between the distal bile duct and the main pancreatic duct proposed in 2015 were evaluated in children. METHODS: Clinical features and complicating diseases according to the PBM classification were evaluated in 168 adult PBM patients. Patency of Santorini duct and associated biliary carcinomas were evaluated in 123 patients. RESULTS: Similar to children, there were significant differences in age (P < 0.01) and type of common bile duct (P < 0.01) between the groups of the classification. Unlike in children, there was no significant difference in the incidence of abdominal pain and hyperamylasemia. There were 87 associated biliary carcinomas (79 gallbladder carcinomas and eight cholangiocarcinomas). PBM patients with a cudgel-type Santorini duct, which is greater than 2 mm in diameter, did not develop biliary carcinomas, compared to 61.1% of those with other types of Santorini duct (P < 0.01). CONCLUSIONS: Clinical features according to the PBM classification in adults were different from those in children. Although biliary carcinomas were frequently seen in adult PBM patients, none of those with a cudgel-type Santorini duct developed biliary carcinoma.
Asunto(s)
Mala Unión Pancreaticobiliar/clasificación , Mala Unión Pancreaticobiliar/diagnóstico , Adulto , Anciano , Colangiopancreatografia Retrógrada Endoscópica , Femenino , Humanos , Masculino , Persona de Mediana Edad , Mala Unión Pancreaticobiliar/complicacionesRESUMEN
Pancreatobiliary maljunction (PBM) is a rare congenital malformation, often associated with adenocarcinoma. However, PBM accompanying gallbladder carcinosarcoma has rarely been reported. A 72-year-old woman was referred to our hospital, complaining of abdominal pain. Computed tomography showed a polypoid mass in the gallbladder. Endoscopic retrograde cholangiopancreatography showed PBM, and aspirated bile demonstrated elevated levels of pancreatic-type amylase (26,780 U/L) and cancer cells. Extended cholecystectomy was performed. Histologically, the tumor had adenocarcinoma, squamous cell carcinoma and sarcoma components. Despite the large tumor size (84 mm) and intra-vessel cancer permeations, this patient has been healthy for 73 months since the surgery.