Summary
Reports on long-term survival rates after Kasai's HPE reveal that 23 % of patients are healthy ten and more years after surgery. Therefore we studied again the anatomy and histology of the porta hepatis and the portal tracts in order to improve this surgical procedure. Methods: Autopsied livers of 6 children with and 6 without EHBA were investigated with regard to the topographical and histological anatomy of the major intra- and extrahepatic bile ducts. Results: On the basis of anatomical findings we have modified the conventional Kasai's HPE and developed an extended exploration of the porta hepatis. The procedure and surgical technique is described in this paper.
Résumé
Les rapports sur les taux de survie à long terme après intervention de Kasai révèlent que seulement 23 % sont en bonne santé, 10 ans et plus, après la chirurgie. Nous étudions l'anatomie et l'histologie de la porta hepatis dans le but d'améliorer le geste chirurgical. Méthodes: Les foies de 6 enfants autopsiés avec ou sans atrésie des voies biliaires sont étudiés en considérant l'anatomie des voies biliaires intra et extra hépatiques. Résultats: Sur les bases de cette étude anatomique nous avons modifié l'intervention de Kasai et développé une exploration extensive de la porta hepatis. Ce procédé est décrit dans cette publication.
Resumen
Los estudios de sobrevivencia a largo plazo de pacientes tratados mediante hepato-porto-enterostomía de Kasai muestran que solamente el 23 % siguen vivos y sanos 10 años después de la operación. Por ello hemos estudiado de nuevo la anatomía e histología de la porta hepatis y los restos biliares para mejorar la operación. Métodos: Los hígados de 6 niños autopsiados con atresia biliar con y los de 6 sin ella fueron estudiados para determinar la anatomía histológica y topográfica de los conductos intra y extra-hepáticos mayores. Resultados: Basándonos en los hallazgos anatómicos hemos modificado la hepato-porto-enterostomía clásica de Kasai y desarrollado una exploración más extensa de la porta hepatis. Esta técnica se describe en este artículo.
Zusammenfassung
Große Serien zeigen, dass 23 % der Kinder mit EHGA 10 und mehr Jahre nach Kasai's HPE geheilt sind. Dieses (unbefriedigende) Ergebnis war Anlass, die Topographie der Leberpforte erneut zu studieren, um das Operationsverfahren zu verbessern. Methode: An 6 Lebern von Kindern mit und 6 Kindern ohne EHGA wurde die Anatomie der intrahepatischen und extrahepatischen Gallengänge makroskopisch und mikroskopisch untersucht. Ergebnisse: Auf der Basis dieser Ergebnisse wurde die HPE nach Kasai modifiziert und ein eigenes Operationsverfahren beschrieben.
Key words
Extrahepatic biliary atresia - Extended hepatoporto-enterostomy
Mots-clés
Porta hepatis - Atrésie des voies biliaires
Palabras clave
Atresia biliar - Portoenterostomía de Kasai - Hígado
Schlüsselwörter
Anatomie der Leberpforte - Hepato-Enterostomie - Gallengangsatresie
References
-
1
Altman R P, Chandra R, Lilly J R.
Ongoing cirrhosis after successful portoenterostomy in infants with biliary atresia.
J Pediatr Surg.
1975;
10
685-688
-
2 Caccia G. et al .Clinical results on 90 patients with biliary atresia: Proc. of the 4th Intern. Symposium on Biliary Atresia, Sendai, 1984. Postgrad Serv Tokyo. 1987: 281
-
3
Chiba T, Kasai M, Sasano N.
Histopathological studies on intrahepatic bile ducts in the vicinity of porta hepatis in biliary atresia.
Tohoku J Exp Med.
1976;
12
199-207
-
4
Endo M, Masuyama H, Watanabe K. et al .
Calculation of biliary atresia prognosis index using a multivariantive linear model.
J Pediatr Surg.
1995;
30
1575-1579
-
5
Gauthier M, Jehan P, Odivevre M.
Histologic study of biliary fibrous remnants in 48 cases of extrahepatic biliary atresia: correlation with postoperative bile flow restoration.
J Pediatr.
1976;
89
704-708
-
6
Ito T, Nagaya M. et al .
Modified hepatic portal enterostomy for biliary atresia.
Z Kinderchir.
1984;
39
242-254
-
7
Kasai M.
Treatment of biliary atresia with special reference to hepatic portoenterostomy and its modification.
Prog Pediatr Surg.
1974;
6
50-52
-
8
Kimura S, Tomomatsu Y, Togon J.
Studies on the postoperative changes in the liver tissue of long-term survivors after successful surgery for biliary atresia.
Z Kinderchir.
1980;
31
228-231
-
9
Kitamura T. et al .
Long-term results after operation for congenital biliary atresia in 144 personal cases.
Z Kinderchir.
1980;
31
239-242
-
10
Kobayashi A, Utsunomiya T, Ohbe Y, Shimuzu K.
Ascending cholangitis after successful surgical repair of biliary atresia.
Arch Dis Child.
1973;
48
697-702
-
11
Matsuo S, Suita S. et al .
Long-term results and clinical problems after portoenterostomy in patients with biliary atresia.
Eur J Pediatr Surg.
1998;
8
142-145
-
12
Miyata M. et al .
Long-term results of hepatic portoenterostomy for biliary atresia.
Surgery.
1987;
76
234-237
-
13
Mustard R, Shandling B, Gillam J.
The Kasai operation for biliary atresia. Experience with 20 cases.
J Pediatr Surg.
1979;
14
511
-
14
Ohi R, Nio M, Chiba T. et al .
Long-term follow-up after surgery for patients with biliary atresia.
J Pediatr Surg.
1990;
25
442-445
-
15
Ohi M, Hobeldin M, Chen T. et al .
The Kasai procedure in the treatment of biliary atresia.
J Pediatr Surg.
1995;
30
1077-1081
-
16 Sacki M. et al .Problems in long-term survivors of biliary atresia: Proc. of the 4th Intern. Symposium on Biliary Atresia, Sendai, 1986. Postgrad Serv Tokyo. 1987: 287
-
17
Schweizer P.
Treatment of extrahepatic biliary atresia. Results and long-term prognosis after hepatic-portoenterostomy.
Pediatr Surg Intern.
1986;
1
30-36
-
18
Schweizer P, Lünzmann K.
Hepatic bile duct atresia: How efficient is the hepatoporto-enterostomy.
Eur J Pediatr Surg.
1998;
8
150-154
-
19
Schweizer P, Kirschner H, Schittenhelm C.
Anatomy of the porta hepatis as a rational basis for the hepatoporto-enterostomy.
Eur J Pediatr Surg.
1998;
9
13-18
-
20 Schweizer M. Ätiologie, Pathogenese, Therapie und Prognose der Extrahepatischen Gallengangsatresie. Inauguraldissertation, Tübingen. 1999
-
21
Toyosaka A, Okamoto E, Okasora T. et al .
Outcome of 21 patients with biliary atresia living more than 10 years.
J Pediatr Surg.
1993;
28
1498-1501
Prof. Dr. P. Schweizer
Department of Pediatric Surgery
University of Tübingen
Hoppe-Seyler-Straße 3
72076 Tübingen
Germany
