Outcomes of choledochal cysts with or without intrahepatic involvement in children after extrahepatic cyst excision and Roux-en-Y hepaticojejunostomy

Congo, Kisa; Lopes, Maria Francelina; Oliveira, Patrícia Horta; Matos, Hugo; Basso, Susana; Reis, Aldina

doi:10.1016/s1665-2681(19)31468-1

Cited by 30 publications

(19 citation statements)

References 19 publications

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“…They affect about 1 in 100 000–150 000 live births in Western populations and up to 1 in 1000 live birth in Japan with a 3–4:1 female to male distribution . The majority (75–85%) of CDCs are identified within the first decade of life, especially as the number diagnosed prenatally during ultrasound exams increases . The classic presentation is the triad of jaundice, a palpable right upper quadrant mass, and abdominal pain.…”

Section: Choledochal Cystsmentioning

confidence: 99%

“…They tend to present more often in adults. Type V CDCs (20%) (Caroli's disease) involve multiple intra‐hepatic cysts with associated hepatic fibrosis …”

Section: Choledochal Cystsmentioning

confidence: 99%

“…8,9 The majority (75-85%) of CDCs are identified within the first decade of life, especially as the number diagnosed prenatally during ultrasound exams increases. 10,11 The classic presentation is the triad of jaundice, a palpable right upper quadrant mass, and abdominal pain. However, these symptoms are seen together in only about 20% of patients.…”

Section: Choledochal Cystsmentioning

confidence: 99%

“…Type V CDCs (20%) (Caroli's disease) involve multiple intra-hepatic cysts with associated hepatic fibrosis. 9,10 Choledochal cysts are thought to arise due to an anomalous pancreaticobiliary junction with a long common channel, leading to reflux of pancreatic secretions into the biliary tract with resultant injury and cyst formation. 8,9 If left untreated, CDCs have a high risk of malignant transformation.…”

Section: Choledochal Cystsmentioning

confidence: 99%

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Transitional care for patients with surgical pediatric hepatobiliary disease: Choledochal cysts and biliary atresia

Aspelund

Mahdi

Rothstein

et al. 2019

J of Gastro and Hepatol

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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.

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Section: Choledochal Cystsmentioning

confidence: 99%

“…They tend to present more often in adults. Type V CDCs (20%) (Caroli's disease) involve multiple intra‐hepatic cysts with associated hepatic fibrosis …”

Section: Choledochal Cystsmentioning

confidence: 99%

Section: Choledochal Cystsmentioning

confidence: 99%

Section: Choledochal Cystsmentioning

confidence: 99%

See 2 more Smart Citations

Transitional care for patients with surgical pediatric hepatobiliary disease: Choledochal cysts and biliary atresia

Aspelund

Mahdi

Rothstein

et al. 2019

J of Gastro and Hepatol

View full text Add to dashboard Cite

show abstract

“…L'étiopathogénie reste imprécise, l'hypothèse la plus communément admise étant que le KCC est le résultat d'une anomalie de la jonction pancréatico-biliaire. Sa prévalence est variable, estimée de 1/13 000-15 000 à 1/100 000-200 000 naissances vivantes [2,3], plus élevée dans les pays asiatiques, en particulier au Japon où elle est estimée à 1/1000 naissances vivantes [4]. Bien que notre observation ait concerné un jeune garçon d'origine asiatique, la fréquence du KCC est plus grande chez les filles (sexratio = 0,25) [3].…”

Section: Discussionunclassified