Biliary atresia with hyaline cartilage at the porta hepatis

2016 ◽  
Vol 12 (3) ◽  
pp. 122-125
Author(s):  
Rakesh Kumar Gupta ◽  
Vineeta V. Batra ◽  
Mamta Sengar ◽  
Puja Sakhuja ◽  
Sheetal N. Rooge
Keyword(s):  
Biliary Atresia ◽  
Hyaline Cartilage ◽  
Porta Hepatis

scholarly journals Correctable biliary atresia and cholangiocarcinoma: a case report of a 63-year-old patient

2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Masaki Nio ◽  
Motoshi Wada ◽  
Hideyuki Sasaki ◽  
Hiromu Tanaka ◽  
Masatoshi Hashimoto ◽  
...  
Keyword(s):  
Biliary Atresia ◽  
Liver Tumors ◽  
Malignant Tumors ◽  
Porta Hepatis ◽  
Case Presentation ◽  
Cancer Occurrence ◽  
Long Term Survivors ◽  
Near Future

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.

scholarly journals Liver stiffness measurement predicts the difficulty of Kasai procedure in biliary atresia: a single center retrospective analysis of 199 patients

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Qiulong Shen ◽  
Yajun Chen ◽  
Chunhui Peng ◽  
Wenbo Pang ◽  
Zengmeng Wang ◽  
...  
Keyword(s):  
Diagnostic Accuracy ◽  
Biliary Atresia ◽  
Liver Stiffness ◽  
Porta Hepatis ◽  
Liver Stiffness Measurement ◽  
Stiffness Measurement ◽  
Kasai Procedure ◽  
Group A ◽  
Group B ◽  
Sensitivity Specificity

Abstract Background Kasai procedure is the standard initial treatment of infants with biliary atresia. The key to perform a successful surgery is to accurately remove the fibrous portal plate near the liver hilum. Yet how to estimate surgical difficulty pre-operatively remains unclear. This study aims to design an algorithm that predicts the difficulty of Kasai procedure using liver stiffness measurement (LSM). Methods One hundred ninety-nine patients were included from April 2012 to December 2016. The patients were all surgically diagnosed with biliary atresia. Group A comprised of patients with porta hepatis retraction (the angle between the plane of the fibrous porta plate and the plane of the medial liver closest to the plate was equal to or smaller than 90°), group B comprised of patients without porta hepatis retraction (the angle between the plane of the fibrous porta plate and the plane of the medial liver closest to the plate was greater than 90°). Liver function measurements and LSM were measured for all patients within three days before surgery. Results Our study included 19 cases in group A (9 males, 10 females) and 180 cases in group B (87 males, 93 females). LSM had statistical differences between the two groups, 28.10(14.90) kPa VS 10.89(7.10) kPa, P = 0.000. There was a significant relationship between LSM and operative age, TBA, AST, GGT (P = 0.000, 0.003, 0.003, 0.012, correlation coefficient = 0.323, 0.213, 0.207, 0.179). The AUROC of LSM was 0.919. When the cutoff value was 15.15 kPa(OR = 3.989; P = 0.000), the sensitivity, specificity, PPV, NPV and diagnostic accuracy were 0.947, 0.750, 0.285, 0.992 and 0.768, respectively. When the value was 23.75 kPa(OR = 3.483; P = 0.000), the sensitivity, specificity, PPV, NPV and diagnostic accuracy were 0.631, 0.950, 0.571, 0.960 and 0.919, respectively. Conclusions LSM can be used to predict the difficulty in dissecting fibrous portal plate, and in turn, the difficulty of Kasai procedure. LSM > 23.75 kPa suggests a more complicated surgery.

Extensive dissection at the porta hepatis for biliary atresia

1994 ◽  
Vol 29 (7) ◽  
pp. 896-899 ◽  
Author(s):  
Akihiro Toyosaka ◽  
Eizo Okamoto ◽  
Tatsuo Okasora ◽  
Katsuyoshi Nose ◽  
Yoshifumi Tomimoto ◽  
...  
Keyword(s):  
Biliary Atresia ◽  
Porta Hepatis
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