Bile-duct diameter at the porta hepatis and liver biopsies in Indian children with biliary atresia

Abstract Background Although cancer occurrence following surgery for biliary atresia has gradually increased, the development of cholangiocarcinoma in a native liver survivor of biliary atresia is extremely rare. Case presentation A 3-month-old female patient with the correctable type of biliary atresia underwent a cystoduodenostomy. At 16 years of age, she underwent multiple surgeries including lysis of intestinal adhesions, ileostomy, and gastrojejunostomy at another hospital. At 54 years of age, she underwent lithotomy at the porta hepatis, resection of the residual cystic bile duct with gallbladder, and hepaticojejunostomy in Roux-en-Y fashion. As she approached the age of 63, her computed tomography scan showed no liver tumors. In the following year, she developed cholangiocarcinoma at the porta hepatis and underwent chemotherapy. However, the cancer progressed, and she died before she reached the age of 64 years. Conclusions Cholangiocarcinoma is extremely rare in patients with biliary atresia. However, physicians should follow up patients with biliary atresia as closely as possible, as malignant tumors secondary to biliary atresia may increase in number in the near future because of the growing number of long-term survivors with biliary atresia.

Download Full-text

Correlation of common bile duct diameter by ultrasound and cholangiography

Gastrointestinal Endoscopy ◽

10.1016/s0016-5107(96)80338-5 ◽

1996 ◽

Vol 43 (4) ◽

pp. 375

Author(s):

EP Bouras ◽

KM McGrath ◽

D Ciaccia ◽

JE Onken

Keyword(s):

Bile Duct ◽

Common Bile Duct ◽

Duct Diameter ◽

Common Bile Duct Diameter

Download Full-text

Key Histopathologic Features of Liver Biopsies That Distinguish Biliary Atresia From Other Causes of Infantile Cholestasis and Their Correlation With Outcome

The American Journal of Surgical Pathology ◽

10.1097/pas.0000000000000755 ◽

2016 ◽

Vol 40 (12) ◽

pp. 1601-1615 ◽

Cited By ~ 37

Author(s):

Pierre Russo ◽

John C. Magee ◽

Robert A. Anders ◽

Kevin E. Bove ◽

Catherine Chung ◽

...

Keyword(s):

Biliary Atresia ◽

Liver Biopsies ◽

Infantile Cholestasis ◽

Histopathologic Features

Download Full-text

Armed CD4+ Th1 effector cells and activated macrophages participate in bile duct injury in murine biliary atresia

Clinical Immunology ◽

10.1016/j.clim.2005.01.012 ◽

2005 ◽

Vol 115 (2) ◽

pp. 200-209 ◽

Cited By ~ 69

Author(s):

Cara L. Mack ◽

Rebecca M. Tucker ◽

Ronald J. Sokol ◽

Brian L. Kotzin

Keyword(s):

Bile Duct ◽

Biliary Atresia ◽

Bile Duct Injury ◽

Activated Macrophages ◽

Effector Cells

Download Full-text

Bile duct varices presenting as a porta hepatis mass

Australasian Radiology ◽

10.1111/j.1440-1673.2005.01474.x ◽

2005 ◽

Vol 49 (5) ◽

pp. 415-417

Author(s):

MK House ◽

RN Gibson

Keyword(s):

Bile Duct ◽

Porta Hepatis

Download Full-text

YAP Activation and Implications in Patients and a Mouse Model of Biliary Atresia

Frontiers in Pediatrics ◽

10.3389/fped.2020.618226 ◽

2021 ◽

Vol 8 ◽

Author(s):

Chao Zheng ◽

Jiaqian Luo ◽

Yifan Yang ◽

Rui Dong ◽

Fa-Xing Yu ◽

...

Keyword(s):

Bile Duct ◽

Liver Fibrosis ◽

Mouse Model ◽

Biliary Atresia ◽

Signaling Pathway ◽

Target Genes ◽

Hippo Signaling ◽

Mice Model ◽

Hippo Signaling Pathway ◽

Ductal Cells

Background and Aim: Biliary atresia (BA), an inflammatory destruction of the bile ducts, leads to liver fibrosis in infants and accounts for half of cases undergoing pediatric liver transplantation. Yes-associated protein (YAP), an effector of the Hippo signaling pathway, is critical in maintaining identities of bile ductal cells. Here, we evaluated the expression of YAP and YAP target genes in BA livers and a rhesus rotavirus (RRV)-induced BA mice model.Methods: Liver specimens collected from 200 BA patients were compared with those of 30 non-BA patients. Model mice liver tissues were also collected. The expression of YAP and YAP target genes were measured by transfection, RNA-seq, immunohistochemistry, immunoblot, and quantitative PCR. Masson's trichrome staining and the Biliary Atresia Research Consortium (BARC) system were utilized to score liver fibrosis status.Results: The expression of YAP is elevated and positively correlated with fibrosis in BA livers. Moreover, ANKRD1, which is identified as the target gene of YAP, is also highly expressed in BA livers. Consistent with clinical data, YAP and ANKRD1 are significantly upregulated in RRV-induced BA mouse model.Conclusions: YAP expression is closely correlated with the bile duct hyperplasia and liver fibrosis, and may serve as an indicator for liver fibrosis and BA progression. This study indicates an involvement of the Hippo signaling pathway in the development of BA, and the YAP induced ANKRD1 expression may also be related to bile duct hyperplasia in BA. This provides a new direction for more in-depth exploration of the etiology and pathogenesis of biliary atresia.

Download Full-text

Cystic form of biliary atresia. Treatment experience

Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care ◽

10.17816/psaic603 ◽

2020 ◽

Vol 10 (1) ◽

pp. 17-24

Author(s):

Irina I. Borisova ◽

Anatoliy V. Kagan ◽

Svetlana A. Karavaeva ◽

Aleksey N. Kotin

Keyword(s):

Surgical Treatment ◽

Bile Duct ◽

Common Bile Duct ◽

Biliary Atresia ◽

Bile Ducts ◽

Surgical Intervention ◽

Ultrasound Examination ◽

Duct Cyst ◽

Cystic Form ◽

The Common

Background. The cystic form of biliary atresia is a rare form of atresia of the biliary tract, which is a relatively favorable variant of the defect and can be diagnosed antenatally. In practice, it is important not only to suspect this diagnosis, but also to differentiate this variant of impaired development of the external bile duct from the cyst of the common bile duct. This is due to the difference in approaches and methods of surgical treatment of choledochal cysts and biliary atresia. Obliteration (atresia) of the bile ducts in the absence of timely surgical intervention quickly leads to the progression of cirrhosis and the development of liver failure. The method of choice in the treatment of AD is Kasai surgery, often palliative in nature, but allowing to delay the time until liver transplantation. The cyst of the common bile duct rarely requires early surgical treatment, and the risk of cirrhosis is significantly lower. Surgical intervention is aimed at removing the cyst and restoring the flow of bile by anastomosing the external bile ducts with the intestines, which is a radical method of treatment and leads to the recovery of the child. External similarity in ultrasound examination of the fetus and newborn baby of the cystic form of biliary atresia of the bile ducts with a cyst of the common bile duct does not always allow differentiation of one defect from another, which can lead to untimely correction of the defect and an unfavorable outcome. Aim. Demonstrate a rare type of biliary atresia. Materials and methods. Between 2001 and 2019, 33 patients with biliary atresia were treated in the Childrens City Multidisciplinary Clinical Specialized Center for High Medical Technologies in St. Petersburg, only two patients had a cystic form. Both children were initially treated as patients with bile duct cyst. Children were operated on at the age of 2 and 3.5 months. The first patient underwent surgery Kasai, the second hepaticoyunoanastomosis. Results. During the observation period (9 years and 4 years), the synthetic function of the liver is normal, and there are currently no indications for transplantation. Conclusion. If a fetus or a newborn with neonatal jaundice is detected during ultrasound examination of a cystic formation in the gates of the liver, it is very important to correctly and quickly make a differential diagnosis between the cystic form of biliary atresia of the biliary tract and the common bile duct cyst.

Download Full-text