Presentation and Clinical Outcomes of Choledochal Cysts in Children and Adults

Soares, Kevin C.; Kim, Yuhree; Spolverato, Gaya; Maithel, Shishir K.; Bauer, Todd W.; Marques, Hugo P.; Sobral, Mafalda; Knoblich, Maria; Tran, Thuy B.; Aldrighetti, Luca; Jabbour, Nicolas; Poultsides, George A.; Gamblin, T. Clark; Pawlik, Timothy M.

doi:10.1001/jamasurg.2015.0226

Cited by 102 publications

(128 citation statements)

References 21 publications

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“…[10] In addition, majority of the previous studies reported that adults, compared with children, were more likely to present with abdominal pain. [7,8,10–13] By contrast, a recent multicenter study by Ouaissi et al [14] observed similar proportions of abdominal pain presentation in children and adults. Furthermore, our study demonstrated that the proportion of patients presented with right upper quadrant mass was greater in infants compared with the older children.…”

Section: Discussionmentioning

confidence: 94%

Different clinical presentations of choledochal cyst among infants and older children

et al. 2017

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Choledochal cyst is a rare and often benign congenital cystic dilation throughout the biliary tree. Due to the benign nature of choledochal cyst among early-diagnosed patients, the clinical assumption and diagnosis seem to be of utmost significance. Therefore, we sought to assess different clinical manifestations of choledochal cyst and relevant laboratory findings in infants and older children.Retrospectively, medical records of all patients with the diagnosis of choledochal cyst between 2005 and 2015 were reviewed. Demographic data, initial clinical presentation, positive findings on physical examination, history of any remarkable behavior such as persistent and unexplained crying and poor feeding, and diagnostic imaging modalities were listed. In addition, laboratory values for total and direct bilirubin, alkaline phosphatase, alanine transaminase, aspartate transaminase, prothrombin time, and partial thromboplastin time (PTT) were recorded for each patient. Patients were divided into 2 groups; younger than 1-year-old (infants), and 1 year to 18 years old (older children). Demographic data, clinical data, and laboratory values were compared between the infants and older children.Thirty-two patients with a diagnosis of choledochal cyst were included in the study: 9 patients (28.12%) were infants and 23 patients (71.87%) were older children. Abdominal pain was the most common presenting symptom (62.5%), followed by nausea/vomiting (59.4%) and jaundice (28.1%). None of the patients presented with the classic triad of abdominal pain, jaundice, and right upper quadrant mass. Seventeen older children (73.91%) presented with nausea and vomiting, while 2 subjects (22.22%) in the infantile group presented with this feature (P = .01). Similarly, abdominal pain was found in 20 older children (86.95%); however, none of the infants presented with abdominal pain at diagnosis (P < .001). By contrast, the abdominal mass was more detected in infants than the older children (33.33% vs. 0%, P = .01). In terms of laboratory values, the median PTT was 44 and 36 s in infants and older children, respectively (P = .04).Infants were more likely to present with abdominal mass and older children were more likely to have nausea, vomiting, and abdominal pain. Furthermore, infants had more prolonged PTT than older children, implying a potential bleeding tendency.

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Section: Discussionmentioning

confidence: 94%

Different clinical presentations of choledochal cyst among infants and older children

et al. 2017

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“…The overall incidence of cholangiocarcinoma in patients with untreated cysts is as high as 28% [24] , while after proper treatment, the incidence could be as low as around 3% [25] .…”

Section: Congenital Abnormalitiesmentioning

confidence: 99%

Intrahepatic cholangiocarcinoma: Epidemiology, risk factors, diagnosis and surgical management

2016

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“…[2,3] Nearly 20% of choledochal cysts are present in adults, and malignant transformation is the most feared complication. [4] A 6% to 30% risk of developing malignancy is estimated in patients with choledochal cysts; the risk is low in childhood (<1%) but increases to about 30% to 40% with age greater than 50 years. [5,6] …”

Section: Introductionmentioning

confidence: 99%

The distal classification and management of choledochal cyst in adults

Liu

Sun²,

Guo³

et al. 2017

Medicine

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Todani classification is extensively used to guide the surgical strategy of choledochal cysts, but no systematic investigations on the distal management of intrapancreatic choledochal cysts have been conducted. This study reports the distal classification and management of choledochal cysts in adults based on the relation between the cyst and pancreatic duct. Patients with choledochal cyst who underwent operation, including distal management, in our department from January 2009 to December 2014 were retrospectively reviewed. Patients presenting symptoms, coexisting diseases, surgical treatment, perioperative complications, and long-term follow-up according to the distal classification of choledochal cyst were analyzed. A total of 54 patients with choledochal cyst were included in the present retrospective study. Based on the distal classification of choledochal cyst, 39 patients (72.22%) were type 1, 13 patients (24.07%) were type 2, and 2 patients (3.70%) were type 3. Thirty-nine type 1 patients and 10 type 2 patients underwent excision of intrapancreatic choledochal cyst or bile duct. Three type 2 patients received excision of distal cylindrical cyst and papilla, followed by pancreatic duct plasty with duodenum mucosa. One type 3 patient underwent endoscopic sphincteroplasty, and another type 3 patient underwent transduodenal sphincteroplasty. After the operation, 11 patients (20.37%, 11/54) had short-term perioperative complications. The long-term follow-up results showed that the satisfactory rate (excellent and good outcomes) was 95.83%. Current distal classification of choledochal cysts could provide a more targeted strategy for complete excision to eliminate potential dead space within the pancreas, protect the pancreatic duct, and prevent reoperation.

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Presentation and Clinical Outcomes of Choledochal Cysts in Children and Adults

Cited by 102 publications

References 21 publications

Different clinical presentations of choledochal cyst among infants and older children

Different clinical presentations of choledochal cyst among infants and older children

Intrahepatic cholangiocarcinoma: Epidemiology, risk factors, diagnosis and surgical management

The distal classification and management of choledochal cyst in adults

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