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... original classification identified four types of biliary cysts (types I-IV). In 1977, Todani and colleagues [8] modified this classification and added a fifth category of CC, type V biliary cysts or Carol disease (Figure 2) [10]. Apart from types I-V included in the revised Todani classification, isolated cystic dilatation of the cystic duct has been described and suggested as type VI [59]. ...
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Citations
... It was first described by Vater. However, the first authenticated case was reported by Doughlas in 1852 [12]. ...
Background
Choledochal cyst is an uncommon congenital biliary tract abnormality of unknown etiology. Its classical symptoms are jaundice, abdominal pain, and right upper quadrant mass. However, the disease may present with a vague and non-specific chronic abdominal discomfort. Delay in diagnosis and management may increase the risk of complications particularly the malignancy, which can directly affect the prognosis and outcome. Complete excision of the extra-hepatic duct, cholecystectomy, and Roux-en-Y hepaticojejeunostomy through the open surgical intervention or laparoscopic procedure is the mainstay of treatment.
Case Presentation
A 14-year-old male was presented to our hospital complaining of vague abdominal pain for 5 years. The radiologic imaging showed the features of a type IVa choledochal cyst. He underwent complete excision of the extra-hepatic duct, cholecystectomy, and Roux-en-Y hepaticojejeunostomy. Although the bile leakage occurred as a complication of the procedure, our team best managed the patient in the post-operative period until full recovery.
Discussion
The Choledochal cyst was first described by Vater, which is a congenital anomaly that sometimes may remain asymptomatic till adulthood. Surgical intervention is the mainstay of treatment. However, postoperative complications including bile leakage need a close follow-up of the patient.
Conclusion
Diagnostic delay (antenatal and postnatal) and non-specific symptoms will mask the real feature of the disease, especially in low resources settings. Technical facilities and professional care of the patient may preclude complications.
... The original classification identified four types of biliary cysts (types I-IV). In 1977, Todani and colleagues revised the classification and added a fifth category of CC, type V biliary cysts or Carol disease [5,6]. Prenatal ultrasonography is now being routinely performed worldwide and the detection of choledochal cysts have been reported in fetus as early as 15-16 weeks of gestation. ...
... In infant form, symptoms such as obstructive jaundice, clay colored stools, and hepatomegaly proved to be difficult to be distinguished from biliary atresia. There are three main symptoms in the classical clinical triad: recurrent jaundice found in 69-75% of cases, right upper quadrant pain found in 47-60% of cases, and right upper quadrant mass found in 47-80% of cases, but the classic triad only presents in 10% of cases (6-25%) [6] Obstructive jaundice is the main presentation symptom in children [4,8,14] Patient came with symptoms of jaundice and acholic stool. There was no abdominal pain nor palpable mass found. ...
... The exact etiology remains incompletely understood. There are two main theories widely accepted in present days;; (1) reflux of trypsin and other pancreatic enzymes to the bile ducts due to an Anomalous pancreaticobiliary ductal union (APBDU); (2) obstruction of distal common bile duct (CBD) [6]. The etiology in this patient still unknown, there was no reported history of illness during pregnancy period. ...
... Има няколко теории за възникването им -аномално формиране на ампула Vateri, при което ductus pancreaticus и ductus choledochus се вливат под прав ъгъл и образуват общ канал в стената на дванадесетопръстника. Обикновено ъгълът на вливане е остър и дължината на общия канал е до 4 мм, но в случаите на аномално формиране той е по-дълъг, като при пациентите с кисти на холедоха се увеличава до 5-20 мм. Това води до рефлукс на гпанкреатични ензими в жлъчното дърво, повишаване на налягането в него, възпалителна реакция в стената му с последваща кистична дилатация (2,5,7). ...
... Други теории включват вродена слабост на стената на жлъчните пътища, дистална обструкция, намалено количество ганглийни клетки, първична структура на ductus choledochus, нарушения в процеса на реканализация, дисфункция на сфинктера на Odi (5,7) Първата класификация е предложена от Alonso-Lejetal. през 1959 г., като първоначално включва 4 типа жлъчни кисти. ...
... Може да се осъществи КТ-холангиография с цел по-точно изобразяване на анатомията на жлъчните пътища, но е установена по-малка чувствителност при изобразяването на ductuspancreaticus, който е отговорен за рефлукса на контрастна материя в жлъчните пътища. Нефро-и хепатотосичността на контрастната материя и йонизиращото облъчване са фактори, които поставят КТ на по-заден план в диагностиката в детската възраст (3,5,7). ...
Резюме
Кистите на d. choledochus са рядко вродено заболяване, протичащо с дилатация на жлъчното дърво. Срещат се по-често у жители на Източна Азия, като преобладава женския пол. Проявите са разнообразни, като съществува сериозен риск от сериозни усложнения – злокачествена трансформация, холангит, панкреатит, холелитиаза и руптура. Съществуват различни образни методики за диагностика, като златен стандарт остава магнитно-резонансната холангиопанкреатография. Дефинитивното лечение на заболяването е оперативно.
Abstract
Choledochal cysts are rare congenital cystic dilation of the biliary tract. They present primarily in East Asian populations, and are more prevalent in females. Clinical presentations are various, but choledochal cysts can be associated with serious complications - malignant transformation, cholangitis, pancreatitis, cholelithiasis and rupturing of the cyst. There are different modalities of imaging studies to diagnose the disease, but still the gold standard is MRI-cholangiography. The causal treatment is surgical operation.
Paediatric Surgical Special Edition
Introducción:
Se define como quiste de colédoco gigante aquel con un diámetro ≥ 10 cm. A pesar de que el abordaje laparoscópico ha sido contraindicado, se presenta el caso de un adolescente con un quiste de colédoco gigante resuelto por laparoscopía.
Caso clínico:
Paciente de sexo masculino de 14 años con un quiste de colédoco gigante tratado con anastomosis hepático-duodenal laparoscópica.
Conclusiones:
El tamaño promedio de los quistes de colédoco tratados por laparoscopía es de 40 mm. No se recomienda la resección de quistes gigantes por mínima invasión debido a adherencias y restricción del campo visual. En este caso se realizó un tratamiento laparoscópico de manera exitosa.
Introduction. Cysts of the common bile duct or choledoch cysts (CC) are congenital cystic dilatation of the extrahepatic and / or intrahepatic bile ducts. The etiology is not fully clear, but it has been proven that abnormal pancreatobiliary anastomosis plays a major role in CC formation. Clinical manifestations range from prolonged jaundice in newborns to non-specific abdominal pains in older patients. Purpose. To study clinical manifestations of common bile duct cysts in children of different age, to define diagnostic criteria as well as to analyze outcomes after surgical treatment. Material and methods. In 2001-2019, 27 patients with chronic obstructive CC, aged from several days to 12 y.o., were treated in the Children’s City Multidisciplinary Clinical Specialized Center for High Medical Technologies of St. Petersburg. Most of them (16-59.2%) had CC of type IA by the Todani classification. Ultrasound examination and MRI cholangiography were used for diagnostics. Children with CC of type V (Caroli disease) required a specific approach to their treatment because of the combined malformations in kidneys (polycystic kidney disease); in 19 patients with CC of types I and IV the cystically altered choledoch was resected and hepaticojejunostomy was put on the loop by the P technique. Results. 19 patients were operated on. Good outcomes were in 15 cases. Underlying disease complications in the preoperative period were noted in 8 patients: pancreatitis in 5 patients, perforation of cysts with biliary peritonitis in 3 patients. In the postoperative period, 4 patients had complications: stenosis of the anastomosis in 1; portal hypertension in 1; 3 children developed cholangitis, 1 of them required surgical correction. Out of 5 patients with Caroli disease, 2 children died in the neonatal period, one because his parents refused of treatment. Two children aged 12 and 17 had liver and kidney transplantation. Conclusion. CC clinical manifestations are diverse; asymptomatic course can develop up to some age. Ultrasound and MRI cholangiography are main diagnostic tools. Resection of the cyst with hepaticojejunostomy on the loop by the P technique is a radical approach to the treatment and has good outcomes. Patients with Caroli disease require an individual approach.
