Cystic biliary atresia or atretic choledochal cyst: A continuum in infantile obstructive cholangiopathy

Mahalik, Santosh Kumar; Mitra, Suvradeep; Patra, Susama; Das, Kanishka

doi:10.1080/15513815.2019.1627621

Cited by 12 publications

(17 citation statements)

References 8 publications

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“…A few cases of CC and BA were included in our previous publications. 2,8 Follow-up data of 12 cases of pediatric CC were obtained. Recurrence of any clinical symptoms was labeled as the events.…”

Section: Methodsmentioning

confidence: 99%

Diminution of the Primary Cilia From the Intrahepatic Cholangiocytes in a Pediatric Choledochal Cyst

Mitra¹,

Ayyanar²,

Mahalik

et al. 2021

Applied Immunohistochemistry &Amp; Molecular Morphology

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Choledochal cyst (CC) is a cystic disease predominantly involving the extrahepatic biliary tree. Biliary atresia (BA), another disorder of the extrahepatic biliary tree, is sometimes considered to be in the same spectrum as pediatric CC. Recently, the absence and/or the structural abnormality of the primary cilia in the intrahepatic cholangiocytes have been implicated in the pathogenesis of BA. We aimed to evaluate the expression of primary ciliary proteins in the intrahepatic cholangiocytes in cases of pediatric CC and compare it with normal control and BA. We performed immunohistochemistry for primary ciliary proteins (acetylated-α-tubulin and double-cortin domain containing 2) on the liver biopsies of control liver (n = 5), pediatric CC (n = 13), and BA (n = 14). We also compared the expression with various clinical, biochemical, histopathologic (portal fibroinflammation and ductal plate malformation), and immunohistochemical (proliferative index) data. There was significant loss of primary cilia from the intrahepatic cholangiocytes in cases of CC and BA as compared with the normal control by both immunostains (CC: P = 0.003 and 0.001, respectively; BA: P = 0.001 and 0.001, respectively). There was no significant difference between the CC and BA in terms of ciliary protein loss. The loss of the ciliary proteins occurred irrespective of the proliferative (MIB-1 labeling) index, portal fibroinflammation, or ductal plate malformation. The loss of cilia did not correlate with the clinical follow-up in cases of pediatric CC. The loss of primary cilia from the intrahepatic cholangiocytes may be crucial in the etiopathogenesis of pediatric CC.

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Section: Methodsmentioning

confidence: 99%

Diminution of the Primary Cilia From the Intrahepatic Cholangiocytes in a Pediatric Choledochal Cyst

Mitra¹,

Ayyanar²,

Mahalik

et al. 2021

Applied Immunohistochemistry &Amp; Molecular Morphology

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“…The etiology and pathogenesis of biliary atresia remain unclear, although viral, genetic, toxic, and autoimmune origins have been proposed as possibilities [ 39 ]. Most cases of biliary atresia are isolated; however, about 10%-35% are associated with congenital anomalies such as situs inversus or polysplenia [ 38 , 40 , 41 ].…”

Section: Porta Hepatis and Hepatic Cystic Massesmentioning

confidence: 99%

“…Absence of the common bile duct, non-dilated bile ducts, enlargement of the hepatic artery (>1.5 mm) and the presence of hepatic subcapsular flow are other important findings. Diagnostic accuracy for biliary atresia can be improved to 98% when the findings are combined [ 38 , 41 - 45 ]. The diagnosis of biliary atresia can be confirmed with an intraoperative cholangiogram and liver biopsy [ 40 , 41 , 46 ].…”

Section: Porta Hepatis and Hepatic Cystic Massesmentioning

confidence: 99%

“…Diagnostic accuracy for biliary atresia can be improved to 98% when the findings are combined [ 38 , 41 - 45 ]. The diagnosis of biliary atresia can be confirmed with an intraoperative cholangiogram and liver biopsy [ 40 , 41 , 46 ]. Cystic biliary atresia must be differentiated from choledochal cyst, as these two entities have dramatically different management approaches and prognoses.…”

Section: Porta Hepatis and Hepatic Cystic Massesmentioning

confidence: 99%

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Approach to the postnatal sonographic evaluation of prenatally detected abdominopelvic cysts

Muthee

Bray

2022

Ultrasonography

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Prenatally detected abdominal and pelvic masses are commonly cystic in morphology and usually seen at mid-trimester sonography. Sonography is the favored imaging modality for postnatal evaluation of these lesions in newborns, given the availability, low cost, lack of ionizing radiation, lack of sedation, and high spatial resolution in small patients. The differential diagnosis of abdominopelvic cystic masses in the newborn is broad given that they can arise from many organs and may have overlapping features on imaging. This article illustrates an approach to the postnatal sonographic evaluation of prenatally detected cystic abdominal and pelvic masses based on their anatomic location as well as distinctive sonographic characteristics, which can aid in accurate diagnosis and guide appropriate management.

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“…Differentiation is also difficult in prenatal assessments [19,20]. Some authors suggested that cBA and CC could be interim entities belonging to the same pathologic continuum [21]. However, limited studies have focused on the differentiation of cBA with a large extrahepatic cyst from CC type Ia/b using various imaging modalities and prenatal images.…”

Section: Introductionmentioning

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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Cystic biliary atresia or atretic choledochal cyst: A continuum in infantile obstructive cholangiopathy

Cited by 12 publications

References 8 publications

Diminution of the Primary Cilia From the Intrahepatic Cholangiocytes in a Pediatric Choledochal Cyst

Diminution of the Primary Cilia From the Intrahepatic Cholangiocytes in a Pediatric Choledochal Cyst

Approach to the postnatal sonographic evaluation of prenatally detected abdominopelvic cysts

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

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