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

Tang, Jue; Zhang, Dan; Liu, Wei; Zeng, Jixiao; Yu, Jian; Gao, Yi

doi:10.1111/jpc.13779

Cited by 13 publications

(9 citation statements)

References 37 publications

(74 reference statements)

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“…During the same period, the lesions affecting liver function in children with CCs were milder than those in children with CBA, which was likely the cause of the less critical cholestasis. The ALT, AST, TBIL, and DBIL levels in the CBA group were all significantly higher than those in the CC group in this study, which was consistent with the results reported in several existing studies ( 17 ). This indicated that these indexes could be used as sensitive indicators to distinguish CBA from CCs.…”

Section: Discussionsupporting

confidence: 93%

Comparative analysis of cystic biliary atresia and choledochal cysts

et al. 2022

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ObjectiveCystic biliary atresia (CBA) is a rare and peculiar type of biliary atresia (BA) that is easily confused with infantile choledochal cysts (CCs). This study explored information for early CBA diagnosis and treatment.MethodThe authors retrospectively analyzed the clinical data of 32 children with hilar cysts from January 2013 to May 2021. According to the diagnosis, they were divided into the CBA (n = 12) and CC (n = 20) groups. Patient features, biochemical indexes, preoperative ultrasound characteristics, cholangiography features, and intraoperative findings were analyzed and compared between the two groups.ResultsThe alanine aminotransferase, aspartate aminotransferase, total bilirubin, and direct bilirubin levels in the CBA group were higher than in the CCs group (P < 0.05). Additionally, B-mode ultrasound showed a cystic mass in front of the hepatic hilum, and the cyst size was much smaller in the CBA group compared with the CC group (2.2 ± 1.3 cm vs. 6.0 ± 2.2 cm, P < 0.001). Among all of the parameters, cyst width was the most accurate for identifying CBA and CCs. A cutoff value of 2.5 cm (area under the curve, 0.98, P < 0.001) showed 90.9% sensitivity and 95% specificity for cyst size.ConclusionFor children with early-onset severe jaundice, and if the width of the cystic mass was ≤2.5 cm, a diagnosis of CBA was highly likely. Early cholangiography and surgical treatment are necessary for the effective treatment of these infants.

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Section: Discussionsupporting

confidence: 93%

Comparative analysis of cystic biliary atresia and choledochal cysts

et al. 2022

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“…Diagnostic confusion of cystic biliary atresia with congenital choledochal cyst might have been the cause of the delayed diagnosis in some cases in this study, as previously reported (12,13). At the time of diagnosis, obstructed congenital choledochal cyst needs to be differentiated from cystic biliary atresia (14–16).…”

Section: Discussionsupporting

confidence: 68%

The Outcome of Patients With Cystic Biliary Atresia With Intact Proximal Hepatic Ducts Following Hepatic-Cyst-Jejunostomy

Asai

Wang

et al. 2022

Journal of Pediatric Gastroenterology &Amp; Nutrition

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Objectives: To determine the outcomes of patients with cystic biliary atresia by correlating the anatomy of the hepatic ducts with the choice of biliary reconstruction surgery. Background: The Kasai hepatoportoenterostomy (Kasai) is the initial surgical procedure offered to most patients with biliary atresia. In contrast, a hepatic-cyst-jejunostomy has been reported to be effective in patients with the cystic form of biliary atresia. Methods and Results:We performed an international multicenter retrospective review. Two hundred eighty-seven patients were included, and 33 cases of cystic biliary atresia were identified. Outcomes were the serum total bilirubin level 3 months post-surgery and native liver survival at 2 years of age and were compared between cases who received the Kasai versus hepatic-cyst-jejunostomy in correlation to the anatomy of proximal hepatic ducts. The patients were categorized into 3 anatomical groups: patent intact hepatic ducts (n = 10), patent hypoplastic hepatic ducts (n = 13), and obliterated hepatic ducts (n = 10). All 10 patients with patent intact hepatic duct group underwent hepatic-cyst-jejunostomy, and 9 experienced bile drainage and native liver survival. Among the 13 patients with hypoplastic hepatic ducts, 11 underwent the Kasai procedure, and 9 had bile drainage, whereas 2 underwent hepatic-cyst-jejunostomy, and one survived with the native liver. All of the patients with obliterated hepatic ducts underwent the Kasai procedure; 5 established biliary drainage and survived with the native liver. Of 5 who did not drain, 3 underwent liver transplantation.Conclusions: In patients with cystic biliary atresia, the subset with a connection between cyst and intrahepatic bile ducts via intact proximal hepatic ducts had favorable clinical outcomes following hepatic-cyst-jejunostomy.

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“…In a previous study, the most frequently described difference between cBA and CC was the cyst size. Several studies mentioned that cysts were significantly smaller on postnatal US in cBA than in CC, with cutoff values ranging from 1.5 to 3.5 cm [5,[24][25][26]. Even though the cyst size in cBA can be variable, and cysts can extend below the hepatic hilum, the cysts in infants with cBA were also significantly smaller than those in infants with CC in our study.…”

Section: Discussionmentioning

confidence: 45%

“…cBA can be misdiagnosed as CC, especially when the cyst is large and not limited to the periportal tract. However, it is important to differentiate these two disease entities early in infancy because they have different clinical outcomes without an early intervention [24]. Our study showed that an increased triangular cord thickness (cutoff value of 4 mm on US, the same cutoff as in the original research on BA [10]), smaller cyst size, GB mucosal irregularity, and non-visibility of the distal bile duct on US or MRI were the key diagnostic features for differentiating cBA from CC type Ia/b, with excellent diagnostic performance, even though T.bil and γGT levels were not significantly different between the two groups.…”

Section: Discussionmentioning

confidence: 99%

Key imaging features for differentiating cystic biliary atresia from choledochal cyst: prenatal ultrasonography and postnatal ultrasonography and MRI

et al. 2021

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Purpose: This study compared clinical and radiologic differences between cystic biliary atresia (cBA) and choledochal cyst (CC) type Ia/b. Methods: Infants (≤12 months old) who were diagnosed with cBA or CC type Ia/b from 2005 to 2019 were retrospectively reviewed. Imaging features on preoperative ultrasonography (US) and magnetic resonance imaging (MRI) were compared between the cBA and CC groups. Logistic regression and area under the receiver operating characteristic curve (AUC) analyses were performed for the diagnosis of cBA. Changes in cyst size were also evaluated when prenatal US exams were available. Results: Ten patients (5.5% of biliary atresia cases) with cBA (median age, 48 days) and 11 infants with CC type Ia/b (Ia:Ib=10:1; median age, 20 days) were included. Triangular cord thickness on US (cutoff, 4 mm) showed 100% sensitivity and 90.9% specificity (AUC, 0.964; 95% confidence interval [CI], 0.779 to 1.000) and cyst size on MRI (cutoff, 2.2 cm) had 70% sensitivity and 100% specificity (AUC, 0.900; 95% CI, 0.690 to 0.987) for diagnosing cBA. Gallbladder mucosal irregularity on US and an invisible distal common bile duct on MRI were only seen in the cBA group (10 of 10). Only the CC group showed prenatal cysts exceeding 1 cm with postnatal enlargement. Conclusion: Small cyst size (<1 cm) on prenatal US, triangular cord thickening (≥4 mm) and gallbladder mucosal irregularity on postnatal US, and small cyst size (≤2.2 cm) and an invisible distal common bile duct on MRI can discriminate cBA from CC type Ia/b in infancy.

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

Abstract: There is still certain difficulty in the early identification of CBA and CC clinically. Liver fibrosis could occur as early as infantile period in both CBA and CC. In infants with BCMs, APRI can be used as a non-invasive method to detect liver fibrosis in early stages.

Cited by 13 publications

References 37 publications

Comparative analysis of cystic biliary atresia and choledochal cysts

Comparative analysis of cystic biliary atresia and choledochal cysts

The Outcome of Patients With Cystic Biliary Atresia With Intact Proximal Hepatic Ducts Following Hepatic-Cyst-Jejunostomy

Key imaging features for differentiating cystic biliary atresia from choledochal cyst: prenatal ultrasonography and postnatal ultrasonography and MRI

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