Diagnosis and management of biliary cystic malformations in neonates

Tanaka, Natsumi; Ueno, Takayoshi; Takama, Yuichi; Fukuzawa, Masahiro

doi:10.1016/j.jpedsurg.2010.06.042

Cited by 26 publications

(11 citation statements)

References 13 publications

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“…The γ‐GT level greater than 300 U/L has a specificity of 98.1% in the diagnosis of BA . However, we did not observe a significant difference between CC and CBA in our study, which is consistent with previous study . Serum TBA, TBIL and DBIL levels of the CBA group were much higher than those of the CC group with a statistically significant difference.…”

Section: Discussioncontrasting

confidence: 44%

Differentiation between cystic biliary atresia and choledochal cyst: A retrospective analysis

Tang

Zhang

Liu

et al. 2017

J Paediatrics Child Health

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Section: Discussioncontrasting

confidence: 44%

Differentiation between cystic biliary atresia and choledochal cyst: A retrospective analysis

Tang

Zhang

Liu

et al. 2017

J Paediatrics Child Health

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“…(12, 13) Furthermore, choledochal cysts and biliary atresia are sometimes found simultaneously, a disorder termed biliary cystic malformations. (14) In our study, the association between choledochal cysts or biliary atresia and congenital cardiac anomalies was most robust in infancy, supporting a unifying embryologic or genetic cause. Alagile syndrome, an autosomal dominant, congenital syndromic paucity of the interlobular bile ducts which leads to cholestasis in the first year of life, also includes branch pulmonary artery hypoplasia or stenosis as one if its main features.…”

Section: Discussionsupporting

confidence: 67%

Associations between pediatric choledochal cysts, biliary atresia, and congenital cardiac anomalies

Murphy¹,

Axt²,

Lovvorn³

2012

Journal of Surgical Research

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Background In our institutional experience treating pediatric choledochal cysts over the last 12 years, we noted 7/32 patients (21.9%) had comorbid congenital cardiac anomalies. This association has not been previously described other than in isolated case reports. We aimed to quantify this association on a national level. Materials and Methods We queried the 2009 Healthcare Cost and Utilization Project Kids' Inpatient Database. Patients with a diagnosis of choledochal cyst (ICD-9-CM 75169, 75162, 75160) or biliary atresia (75161) were identified. Cardiac anomalies were defined using the Clinical Classification Software code (CCS 213). Comorbid choledochal cysts or biliary atresia and congenital cardiac anomalies were quantified in both infant (<12 mos) and child (1–18 yrs) subpopulations. Results Of 1,646 estimated discharges for patients with choledochal cysts, 506 (30.7%) were for patients who also had congenital cardiac anomalies, compared to 2.6% in the general hospitalized population (χ2, p<0.0001). The frequency of congenital cardiac anomalies was lower in 1,973 hospitalizations for biliary atresia (13.8%) than in those for patients with choledochal cysts (χ2, p<0.0001). Cardiac anomalies were detected in 44.9% of choledochal cyst hospitalizations for infants <12 months (vs. 3.44% general hospitalized population; χ2, p<0.0001), but in 6.9% of for children ages 1–18 yrs (vs. 1.3% general hospitalized population; χ2, p<0.0001). Conclusions A strong association was observed between pediatric choledochal cysts and congenital cardiac anomalies that more commonly manifests in infancy. When choledochal cysts are diagnosed either prenatally or in infancy, we suggest echocardiographic screening for cardiac anomalies, which may impact timing of surgery and anesthetic planning.

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“…[13] The specific surgical procedure varies based on Todani classification and the extent of disease.…”

Section: Discussionmentioning

confidence: 99%

Spontaneous resolution of choledochal cyst

et al. 2017

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Choledochal cysts (CCs) are rare, congenital cystic dilations of the hepatobiliary tree that require surgical resection to avoid complications such as increased risk of malignancy. A 25-week gestational age male infant developed acholic stools, elevated alkaline phosphatase, and ultrasound findings consistent with CC Todani Type IVA. Surgery was deferred due to the patient's low weight. The patient's symptoms and radiographic findings subsequently resolved spontaneously. CCs have not been previously reported in extremely preterm infants. There are rare reported cases of spontaneously resolving hepatic cysts, all containing key differences from our patient. In patients in whom immediate surgery is not feasible, conservative management with close follow-up and serial ultrasound examinations would appear to be a reasonable course of action.

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Diagnosis and management of biliary cystic malformations in neonates

Cited by 26 publications

References 13 publications

Differentiation between cystic biliary atresia and choledochal cyst: A retrospective analysis

Differentiation between cystic biliary atresia and choledochal cyst: A retrospective analysis

Associations between pediatric choledochal cysts, biliary atresia, and congenital cardiac anomalies

Spontaneous resolution of choledochal cyst

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