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Choledochal malformation

Jim C H Wilde, Ana M Calinescu, Barbara E Wildhaber
Biliary atresia (BA) and congenital choledochal malformations (CCM) are rare. Both pathologies must (BA) or may (CCM) be operated during the neonatal period or early infancy. We briefly describe the classical operative techniques for both pathologies, followed by the most common and severe perioperative complications encountered during and up to 30 days after surgery in children operated for BA and CCM early in life. For patients with BA, intestinal complications represent the most common and hazardous perioperative surgical complications...
March 6, 2018: European Journal of Pediatric Surgery
A Ten Hove, V E de Meijer, J B F Hulscher, R H J de Kleine
BACKGROUND: Choledochal malformations comprise various congenital cystic dilatations of the extrahepatic and/or intrahepatic biliary tree. Choledochal malformation is generally considered a premalignant condition, but reliable data on the risk of malignancy and optimal surgical treatment are lacking. The objective of this systematic review was to assess the prevalence of malignancy in patients with choledochal malformation and to differentiate between subtypes. In addition, the risk of malignancy following cystic drainage versus complete cyst excision was assessed...
February 26, 2018: British Journal of Surgery
James S Banks, Gaurav Saigal, Joseph M D'Alonzo, Maria D'Almeida Bastos, Nguyen V Nguyen
OBJECTIVE: The purpose of this article is to review the relevant pathophysiologic features, appearances, and surgical implications of choledochal malformations. CONCLUSION: Choledochal malformations, colloquially called choledochal cysts, initially described in 1723, have been recategorized multiple times, the most widely accepted being the Todani classification based on morphologic features and location. Although readily applied to imaging findings, this classification system does not correlate well with clinical and surgical management...
February 15, 2018: AJR. American Journal of Roentgenology
A Grieve, D Harrison, C Westgarth-Taylor, J Loveland
BACKGROUND: Choledochal malformation (CM) is a well-described and relatively rare condition. CMs may present on antenatal ultrasound screening, through childhood and into adulthood. The aetiology is not well understood but the association with a pancreaticobiliary malunion predisposes to the development of CMs. OBJECTIVES: To review the experience of CMs in the Department of Paediatric Surgery, University of the Witwatersrand, Johannesburg to improve our patient care for this population...
November 6, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
Jue Tang, Dan Zhang, Wei Liu, Ji-Xiao Zeng, Jia-Kang Yu, Yi Gao
AIM: Cystic biliary atresia (CBA) can be easily misdiagnosed as choledochal cyst (CC). Some patients have already progressed to severe liver fibrosis and missed the optimal surgical time, when the differential diagnosis is made. We aim to determine the differentiation between CBA and CC, and to validate the value of aspartate aminotransferase-to-platelet ratio index (APRI) in the assessment of liver fibrosis and prediction of post-operative outcome for infants with biliary cystic malformations (BCMs)...
November 3, 2017: Journal of Paediatrics and Child Health
Maria H A van den Eijnden, Ruben H J de Kleine, Ivo de Blaauw, Paul G J M Peeters, Bart P G Koot, Matthijs W N Oomen, Cornelius E J Sloots, W G van Gemert, David C van der Zee, L W E van Heurn, Henkjan J Verkade, Jim C H Wilde, Jan B F Hulscher
INTRODUCTION: A choledochal malformation (CM) is a rare entity, especially in the Western world. We aimed to determine the incidence of CM in the Netherlands and the outcome of surgery for CM in childhood. METHODS: All pediatric patients who underwent a surgical procedure for type I-IV CM between 1989 and 2014 were entered into the Netherlands Study group on choledochal cyst/malformation. Patients with type V CM were excluded from the present analysis. Symptoms, surgical details, short-term (<30 days) and long-term (>30 days) complications were studied retrospectively...
October 2017: World Journal of Surgery
Ashish Gupta, Karikal Chakaravarthi, Lileswar Kaman
With the advent of newer radiological investigations, choledochal cysts are being diagnosed more often in present era. These cysts are commonly diagnosed in early childhood and infancy, although some go undetected to be diagnosed in adulthood. These malformations are associated with multiple complications like cholangitis, jaundice, pancreatitis, rupture or even malignancy. Here we describe a post partum female, who was diagnosed to have choledochal cyst during sixth month of pregnancy. She presented with obstructive jaundice in cholangitis and was subjected to endoscopic retrograde cholangiopancreatography (ERCP) with stenting...
April 2017: Gastroenterology Research
Aditi Arora, Pradyumn Singh, Nidhi Anand, Nuzhat Husain
Choledochal cyst is a rare congenital malformation, particularly when associated with stones in cyst (cystolithiasis), gallstones and heterotopic pancreatic tissue within the cyst wall. The current case represents a 5-year-old boy with abdominal pain, pale colored stools, and jaundice. Magnetic resonance cholangiopancreatography showed a cystic lesion, arising from common bile duct with cystolithiasis and cholelithiasis. He underwent excision of choledochal cyst and gallbladder with Roux-en-Y hepaticojejunostomy...
April 10, 2017: BMJ Case Reports
A Sánchez Galán, A Vilanova Sánchez, L Martínez Martínez, M Dore Reyes, M Romo Muñoz, V Núñez Cerezo, F Hernández Oliveros, M López Santamaría
AIM: In patients with a choledochal cyst, the malformation and subsequent biliodigestive reconstruction lead to a non-physiological state in the digestive tract and biliary excretion that could affect the child's growth. The effect of this malformation and its surgical treatment are reviewed here in a large patient cohort. MATERIAL AND METHODS: Retrospective review of patients undergoing surgery for choledochal cyst in the last 20 years. Nutrition and liver function parameters, weight and height data were collected from medical records...
July 10, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
Maria H A van den Eijnden, Ruben H de Kleine, Ivo de Blaauw, Paul M J G Peeters, Bart G P Koot, Matthijs W N Oomen, Cornelius E J Sloots, Wim G van Gemert, David C van der Zee, L W Ernest van Heurn, Henkjan J Verkade, Jim C H Wilde, Jan B F Hulscher
INTRODUCTION: Choledochal malformations (CMs) are increasingly diagnosed antenatally. There is a dilemma between early surgery to prevent CM-related symptoms and postponing surgery to reduce complications. We aimed to identify the optimal timing of surgery in asymptomatic neonates with antenatally diagnosed CM and to identify predictors for development of symptoms. METHODS: Using the Netherlands Study group on CHoledochal Cyst/malformation (NeSCHoc) we retrospectively collected demographic, biochemical and surgical data from all Dutch patients with an antenatally detected CM...
July 2017: Journal of Pediatric Surgery
Adrián Ransom-Rodríguez, Ruben Blachman-Braun, Emilio Sánchez-García Ramos, Jesús Varela-Prieto, Erick Rosas-Lezama, Miguel Ángel Mercado
Choledochal cysts are rare congenital malformations of the bile duct characterized by dilatations of the intrahepatic and/or extrahepatic portion of the biliary tree, they are associated to an anomalous arrangement of the pancreaticobiliary duct. Pancreas divisum results from a fusion failure of the pancreatic buds. The coexistence of pancreas divisum and choledochal cyst in adults has been reported in less than 10 well documented cases. This article presents a case of a 42-year-old Peruvian man with intermittent episodes of abdominal pain, initially diagnosed with choledocholithiasis, who underwent open cholecystectomy...
February 2017: Annals of Hepato-Biliary-Pancreatic Surgery
Inna N Lobeck, Phylicia Dupree, Richard A Falcone, Tom K Lin, Andrew T Trout, Jaimie D Nathan, Greg M Tiao
BACKGROUND: Choledochoceles may cause biliary obstruction and harbor malignancy. We conducted a 40-year systematic review of the literature for this rare anomaly. METHODS: PubMed and Cochrane databases were accessed 1975-2015 using terms "choledochocele" or "choledochal cyst". Studies reviewed that met the following criteria: English language, published 1975-2015 with human subjects. RESULTS: 325 patients with a choledochocele were identified, including 71 case reports and 254 cases within institutional reviews...
April 2017: Journal of Pediatric Surgery
George Younan, Max Schumm, Fadwa Ali, Kathleen K Christians
Introduction. Gallbladder volvulus is a rare, potentially fatal condition unless diagnosed and treated early. Choledochal cysts are rare congenital malformations of the biliary tree predisposing to different pathologies and posing the risk of degradation into cholangiocarcinoma and gallbladder cancer. Dealing with both diseases at once has not been published yet in the literature. Presentation of Case. We report a case of gallbladder volvulus in an elderly female who happened to have a concomitant type I choledochal cyst...
2016: Case Reports in Surgery
Christos Kaselas, Dimitrios Patoulias, Ioannis Patoulias, Ioannis Spyridakis
Choledochal cysts are rare congenital malformations, comprising of dilatation of the biliary tree of different localization. Classically, classification of choledochal cysts describes five variants of the disease. Type VI choledochal cyst is considered by many authors as the sixth variant of this classification and is described either as a dilatation of the cystic duct or as a cystic duct cyst. We present a rare case of cystic duct dilatation that presented with acute cholangiopancreatitis and a clinical picture consistent of a choledochal cyst in a 10-year-old female patient...
June 2016: Journal of Clinical and Diagnostic Research: JCDR
Nursel Yurttutan, Suleyman Cuneyt Karakus, Naim Koku, Mustafa Demirci, Ramazan Ucak
Choledochal cyst is a dilation that encloses the intrahepatic or both extra- and intrahepatic portions of the biliary ducts. Postnatally, ultrasonography is the initial diagnostic modality of choice, allowing for precise measurements of intra- or extrahepatic duct dilatation and identification of stones and sludge. Symptoms depend on the age at presentation. Common bile duct malformations should be considered as a differential diagnosis of a cystic mass regardless of the cyst's size or patient's age, especially in children presenting with abdominal pain, jaundice, and palpable mass...
May 2016: Korean Journal of Pediatrics
Enrico La Pergola, Yoh Zen, Mark Davenport
PURPOSE: There is a predisposition to the development of malignancy in congenital choledochal malformation (CCM) although the degree of risk is unknown. We investigated the role of CA19-9 in bile and the MIB-1 (Ki-67) epithelial proliferation index as markers of an at risk choledochal epithelium at the time of definitive surgery. METHODS: Bile collected at surgery was analyzed for levels of amylase (as a surrogate of pancreatic reflux) and CA19-9. Immunohistochemical staining for CA19-9 and MIB-1 index (expressed as %) was performed on resected specimens...
September 2016: Journal of Pediatric Surgery
John A Sandoval, Israel Fernandez-Pineda, Alpin D Malkan
OBJECTIVE: A small percentage of pediatric solid cancers arise as a result of clearly identified inherited predisposition syndromes and nongenetic lesions. Evidence supports preemptive surgery for children with genetic [multiple endocrine neoplasia type 2 (MEN2), familial adenomatous polyposis syndrome (FAP), hereditary nonpolyposis colorectal cancer (HNPCC), and hereditary diffuse gastric cancer (HDGC) and nongenetic [thyroglossal duct cysts (TGDC), congenital pulmonary airway malformations (CPAM), alimentary tract duplication cysts (ATDC), and congenital choledochal cysts (CCC)] developmental anomalies...
April 2016: Journal of Pediatric Surgery
Silvia Zoetsch, Georg Singer, Erich Sorantin, Alan W Flake, Holger Till
INTRODUCTION: The congenital combination of duodenal atresia and choledochal cyst has only been reported in a few children. None of these patients had an intrapancreatic choledochocele causing persistent hyperbilirubinemia in the newborn period. PRESENTATION OF CASE: A female newborn presented with duodenal atresia and received a duodeno-duodenostomy on day two of life. The postoperative course was uneventful except for progressive hyperbilirubinemia and elevation of liver enzymes...
2016: International Journal of Surgery Case Reports
R Weng, W Hu, S Cai, X Guo, Q Luo
To investigate the clinical significance of prenatal diagnosis and prognosis evaluation of congenital choledochal cyst (CCC), we reviewed CCC cases of diagnosed antenatally in our hospital from 2007 to 2013, summarised and analysed prenatal sonographic features and clinical outcomes, and followed these cases up to six months after birth. We found that induced labour was conducted in 7 cases, and term labour progressed smoothly in 14 cases among the 21 cases. Operations were completed within 3 months after birth and all the operation cases received a good prognosis...
2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
Kathryn E Ford, Lilli R L Cooper, Mark Davenport
PURPOSE: Excision and biliary reconstruction using a Roux loop is the current standard for choledochal malformation (CM). This is un-physiological, delivering bile beyond the duodenum and excluding a significant length of the jejunum from intestinal absorption. We investigated whether this had an effect on post-operative growth. METHODS: Retrospective case-note analysis of children surgically treated for CM. Growth variables were converted to standard deviation scores (SDS) and compared against population norms...
November 2015: Pediatric Surgery International
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