Biliary atresia-polysplenia syndrome: Surgical and clinical relevance in liver transplantation

Gustavo Varela-Fascinetto, Paolo Castaldo, Ira J. Fox, Debra Sudan, Thomas G. Heffron, Byers W. Shaw, Alan Norman Langnas

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Abstract

Objective: To review a single center's 10-year experience with liver transplantation (LTx) for the biliary atresia-polysplenia syndrome (BA-PS) and to define surgical and clinical guidelines for its management. Summary Background Data: BA is the most common indication for pediatric liver transplantation (LTx) and is associated with PS in 12% of cases. Only a few studies of LTx for BA-PS have been reported, and the optimal management of BA-PS patients undergoing LTx has yet to be determined. Methods: From July 1985 to September 1995, 166 liver transplants were performed in 130 patients with BA and were included in the study. The malformations most commonly associated with BA-PS, surgical techniques used to overcome these anomalies, and surgical pitfalls that could have contributed to the outcome were characterized. Actuarial 10-year patient and graft survival for patients undergoing LTx for BA-PS were calculated and compared to those with isolated BA. Results: Ten patients (7.8%) with BA had associated PS. An additional patient with PS without BA was included in the study. The diagnosis of PS was unknown before the transplantation in 72% of cases. Thirteen liver transplants were performed in these 11 patients. Modifications of the usual surgical technique were used to overcome the complex anatomy encountered. There was no association between the type of anomaly and the outcome, nor were there any significant differences in patient survival (72% vs. 73.5%, p = 0.79) or graft survival (56.4% vs. 54.6%, p = 0.54). Conclusions: The association of BA with various anomalies should be considered a spectrum that may vary widely from patient to patient. The finding of two or more of these malformations in a patient awaiting transplantation should lead the surgeon to look systematically for other associated anomalies. With some special surgical considerations, the outcome in BA-PS patients should not differ from those with isolated BA.

Original languageEnglish (US)
Pages (from-to)583-589
Number of pages7
JournalAnnals of surgery
Volume227
Issue number4
DOIs
StatePublished - Apr 1 1998

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Heterotaxy Syndrome
Biliary Atresia
Liver Transplantation
Graft Survival
Transplantation
Transplants
Liver

ASJC Scopus subject areas

  • Surgery

Cite this

Biliary atresia-polysplenia syndrome : Surgical and clinical relevance in liver transplantation. / Varela-Fascinetto, Gustavo; Castaldo, Paolo; Fox, Ira J.; Sudan, Debra; Heffron, Thomas G.; Shaw, Byers W.; Langnas, Alan Norman.

In: Annals of surgery, Vol. 227, No. 4, 01.04.1998, p. 583-589.

Research output: Contribution to journalArticle

Varela-Fascinetto, G, Castaldo, P, Fox, IJ, Sudan, D, Heffron, TG, Shaw, BW & Langnas, AN 1998, 'Biliary atresia-polysplenia syndrome: Surgical and clinical relevance in liver transplantation', Annals of surgery, vol. 227, no. 4, pp. 583-589. https://doi.org/10.1097/00000658-199804000-00022
Varela-Fascinetto G, Castaldo P, Fox IJ, Sudan D, Heffron TG, Shaw BW et al. Biliary atresia-polysplenia syndrome: Surgical and clinical relevance in liver transplantation. Annals of surgery. 1998 Apr 1;227(4):583-589. https://doi.org/10.1097/00000658-199804000-00022
Varela-Fascinetto, Gustavo ; Castaldo, Paolo ; Fox, Ira J. ; Sudan, Debra ; Heffron, Thomas G. ; Shaw, Byers W. ; Langnas, Alan Norman. / Biliary atresia-polysplenia syndrome : Surgical and clinical relevance in liver transplantation. In: Annals of surgery. 1998 ; Vol. 227, No. 4. pp. 583-589.
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abstract = "Objective: To review a single center's 10-year experience with liver transplantation (LTx) for the biliary atresia-polysplenia syndrome (BA-PS) and to define surgical and clinical guidelines for its management. Summary Background Data: BA is the most common indication for pediatric liver transplantation (LTx) and is associated with PS in 12{\%} of cases. Only a few studies of LTx for BA-PS have been reported, and the optimal management of BA-PS patients undergoing LTx has yet to be determined. Methods: From July 1985 to September 1995, 166 liver transplants were performed in 130 patients with BA and were included in the study. The malformations most commonly associated with BA-PS, surgical techniques used to overcome these anomalies, and surgical pitfalls that could have contributed to the outcome were characterized. Actuarial 10-year patient and graft survival for patients undergoing LTx for BA-PS were calculated and compared to those with isolated BA. Results: Ten patients (7.8{\%}) with BA had associated PS. An additional patient with PS without BA was included in the study. The diagnosis of PS was unknown before the transplantation in 72{\%} of cases. Thirteen liver transplants were performed in these 11 patients. Modifications of the usual surgical technique were used to overcome the complex anatomy encountered. There was no association between the type of anomaly and the outcome, nor were there any significant differences in patient survival (72{\%} vs. 73.5{\%}, p = 0.79) or graft survival (56.4{\%} vs. 54.6{\%}, p = 0.54). Conclusions: The association of BA with various anomalies should be considered a spectrum that may vary widely from patient to patient. The finding of two or more of these malformations in a patient awaiting transplantation should lead the surgeon to look systematically for other associated anomalies. With some special surgical considerations, the outcome in BA-PS patients should not differ from those with isolated BA.",
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AU - Varela-Fascinetto, Gustavo

AU - Castaldo, Paolo

AU - Fox, Ira J.

AU - Sudan, Debra

AU - Heffron, Thomas G.

AU - Shaw, Byers W.

AU - Langnas, Alan Norman

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N2 - Objective: To review a single center's 10-year experience with liver transplantation (LTx) for the biliary atresia-polysplenia syndrome (BA-PS) and to define surgical and clinical guidelines for its management. Summary Background Data: BA is the most common indication for pediatric liver transplantation (LTx) and is associated with PS in 12% of cases. Only a few studies of LTx for BA-PS have been reported, and the optimal management of BA-PS patients undergoing LTx has yet to be determined. Methods: From July 1985 to September 1995, 166 liver transplants were performed in 130 patients with BA and were included in the study. The malformations most commonly associated with BA-PS, surgical techniques used to overcome these anomalies, and surgical pitfalls that could have contributed to the outcome were characterized. Actuarial 10-year patient and graft survival for patients undergoing LTx for BA-PS were calculated and compared to those with isolated BA. Results: Ten patients (7.8%) with BA had associated PS. An additional patient with PS without BA was included in the study. The diagnosis of PS was unknown before the transplantation in 72% of cases. Thirteen liver transplants were performed in these 11 patients. Modifications of the usual surgical technique were used to overcome the complex anatomy encountered. There was no association between the type of anomaly and the outcome, nor were there any significant differences in patient survival (72% vs. 73.5%, p = 0.79) or graft survival (56.4% vs. 54.6%, p = 0.54). Conclusions: The association of BA with various anomalies should be considered a spectrum that may vary widely from patient to patient. The finding of two or more of these malformations in a patient awaiting transplantation should lead the surgeon to look systematically for other associated anomalies. With some special surgical considerations, the outcome in BA-PS patients should not differ from those with isolated BA.

AB - Objective: To review a single center's 10-year experience with liver transplantation (LTx) for the biliary atresia-polysplenia syndrome (BA-PS) and to define surgical and clinical guidelines for its management. Summary Background Data: BA is the most common indication for pediatric liver transplantation (LTx) and is associated with PS in 12% of cases. Only a few studies of LTx for BA-PS have been reported, and the optimal management of BA-PS patients undergoing LTx has yet to be determined. Methods: From July 1985 to September 1995, 166 liver transplants were performed in 130 patients with BA and were included in the study. The malformations most commonly associated with BA-PS, surgical techniques used to overcome these anomalies, and surgical pitfalls that could have contributed to the outcome were characterized. Actuarial 10-year patient and graft survival for patients undergoing LTx for BA-PS were calculated and compared to those with isolated BA. Results: Ten patients (7.8%) with BA had associated PS. An additional patient with PS without BA was included in the study. The diagnosis of PS was unknown before the transplantation in 72% of cases. Thirteen liver transplants were performed in these 11 patients. Modifications of the usual surgical technique were used to overcome the complex anatomy encountered. There was no association between the type of anomaly and the outcome, nor were there any significant differences in patient survival (72% vs. 73.5%, p = 0.79) or graft survival (56.4% vs. 54.6%, p = 0.54). Conclusions: The association of BA with various anomalies should be considered a spectrum that may vary widely from patient to patient. The finding of two or more of these malformations in a patient awaiting transplantation should lead the surgeon to look systematically for other associated anomalies. With some special surgical considerations, the outcome in BA-PS patients should not differ from those with isolated BA.

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