Skip to main content
Log in

Combined heart-lung-liver, double lung-liver, and isolated liver transplantation for cystic fibrosis in children

  • Original Article
  • Published:
Transplant International

Abstract

Between June 1990 and September 1995, 8 of 24 children with cystic fibrosis (CF) who were accepted either for combined transplantation or isolated liver transplantation died while waiting for a graft; 11 underwent transplantation and 5 are currently on the waiting list. Of the 11 children who had surgery, 7 (group 1) underwent one of the following procedures: heartlung-liver (n = 4), sequential double lung-liver (n = 2), or bilateral lobar lung from a split left lung and reduced liver (n = 1). During the same period, the four other children (group 2) underwent isolated liver transplantation (three full-size livers, one partial liver). There was one perioperative death in each group. Pulmonary infection was the most common cause of morbidity in group 1. Other complications in group 1 included tracheobronchial stenosis (n = 2), biliary stricture (n = 2), and severe ascites (n = 2). All were successfully treated. Obliterative bronchiolitis developed in three patients. This was treated with FK 506. In group 2, pulmonary function tests improved or remained stable after liver transplantation. Surgical complications in group 2 included severe ascites (n =1), biliary stricture (n = 1), and abscess of the liver (n = 1). Actuarial survival was 85.7 % ± 2 % in group 1 at 1 year; it remained unchanged at 3 years and was 64.2 % at 5 years.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Bismuth H, Houssin D (1984) Reducedsized orthotopic liver graft in hepatic transplantation in children. Surgery 95: 367–370

    PubMed  Google Scholar 

  2. Broelsch CE, Emond JC, Whitington PF, Thistlethwaite JR, Baker AL, Lichtor JL (1990) Application of reduced-size liver transplants as split grafts, auxiliary orthotopic grafts, and living related segmental transplants. Ann Surg 212: 368–377

    PubMed  Google Scholar 

  3. Calne RY, Sells RA, Pena JR, Davis DR, Millard PR, Herbertson BM, Binns RM, Davies DAL (1969) Induction of immunological tolerance by porcine liver allografts. Nature 223: 472–476

    PubMed  Google Scholar 

  4. Colonna JO II, Shaked A, Gomes AS, Colquhoun SD, Jurim O, Mc Diarmid SV, Millis JM, Goldstein LI, Busuittil RW (1992) Biliary strictures complicat ing liver transplantation. Ann Surg 216: 344–352

    PubMed  Google Scholar 

  5. Cooper JD, Bilingham M, Egan T, et al. (1993) A working formulation for the standardization of nomenclature and for clinical staging of chronic dysfunction in lung allografts. J Heart Lung Transplant 12: 713–716

    PubMed  Google Scholar 

  6. Couetil JP, Grousset A, Benaim D, Sapoval M, Hernigou A, Coppens P, Fayolle P, Carpentier A (1994-95) Misc au point experimentale de la bipartition pulmonaire avec transplantation lobaire bilaterale chez le chien et application en clinique humaine. Chirurgie 120: 512–517

    PubMed  Google Scholar 

  7. Couetil JP, Houssin D, Soubrane O, Chevalier P, Dousset B, Loulmet D, Achkar A, Tolan M, Amrein C, Guinvarch A, Guillemain R, Birnbaum P, Carpentier A (1995) Combined lung and liver transplantation in cystic fibrosis. A 4.5 year experience. J Thorac Cardiovasc Surg 110: 1415–1423

    PubMed  Google Scholar 

  8. Cox KL (1990) The role of liver transplantation in cystic fibrosis patients. Cystic Fibrosis Conference S1.4: 78–79

    Google Scholar 

  9. de Leval MR, Smyth R, Whitehead B, et al. (1991) Heart and lung transplantation for terminal cystic fibrosis: a 4 1/2-year experience. J Thorac Cardiovasc Surg 101: 633–642

    PubMed  Google Scholar 

  10. Dennis C, Caine N, Sharples L, et al. (1993) Heart lung transplantation for end stage respiratory disease in a cystic fibrosis patient at Papworth Hospital. J Heart Lung Transplant 12: 893–902

    PubMed  Google Scholar 

  11. Egan TM, Detterbeck FC, Mill RM, Paradowski LJ, Lackner RP, Ogden WD, Yankaskas JR, Westerman JH, Thompson JT, Weiner MA, Cairns EL, Wilcox BR (1995) Improved results of lung transplantation for patients with cystic fibrosis. J Thorac Cardiovasc Surg 109:224–235

    PubMed  Google Scholar 

  12. Feigelson J, Anagnostopoulos C, Poquet M, Pecan Y, Munck A, Navarro J (1993) Liver cirrhosis in cystic fibrosis — therapeutic implications and long term follow up. Arch Dis Child 68: 653–657

    PubMed  Google Scholar 

  13. Geddes DM (1988) Cystic fibrosis Future trends in care. Thorax 43: 115–130

    Google Scholar 

  14. Gonwa TA, Nery JR, Husberg BO, Klintmalm GB (1988) Simultaneous liver and renal transplantation in man. Transplantation 46: 690–693

    PubMed  Google Scholar 

  15. Hobeika J, Houssin D, Bernard O, Devictor D, Grimon G, Chapuis Y (1994) Orthotopic liver transplantation in children with chronic liver disease and severe hypoxemia. Transplantation 57:224–228

    PubMed  Google Scholar 

  16. Hodson ME, Madden BP, Steven MH, Tsang VT, Yacoub MH (1991) Non-invasive mechanical ventilation for cystic fibrosis patients — a potential bridge to transplantation. Eur Respir J 4: 524–527

    PubMed  Google Scholar 

  17. Kalayoglu M, Sollinger HW, Stratta RJ, et al (1988) Extended preservation of the liver for clinical transplantation. Lancet 1:617–619

    PubMed  Google Scholar 

  18. Kamada N, Davies HS, Roser B (1981) Reversal of transplantation immunity by liver grafting. Nature 292: 840–842

    PubMed  Google Scholar 

  19. Lange PA, Stoller JK (1995) The hepatopulmonary syndrome. Ann Intern Med 122: 521–529

    PubMed  Google Scholar 

  20. Madden BP, Hodson ME, Tsang V, Radley-Smith R, Khaghani A, Yacoub MH (1992) Intermediate-term results of heart-lung transplantation for cystic fibrosis. Lancet I: 1583–1587

    Google Scholar 

  21. Mieles LA, Orenstein D, Teperman L, Podesta L, Koneru B, Starzl TE (1989) Liver transplantation in cystic fibrosis. Lancet 1: 1073

    PubMed  Google Scholar 

  22. Noble-Jamieson G, Valente J, Barnes ND, Friend PJ, Jamieson NV, Rasmussen A, Calne RY (1994) Liver transplantation for hepatic cirrhosis in cystic fibrosis. Arch Dis Child 71: 349–352

    PubMed  Google Scholar 

  23. Park RM, Grand RJ (1981) Gastrointestinal manifestations of cystic fibrosis: a review. Gastroenterology 81: 1143–1161

    Google Scholar 

  24. Pasque MK, Cooper JD, Kaiser LR, Haydock DA, Triantafillou A, Trulock EP (1990) Improved technique for bilateral lung transplantation: rationale and initial clinical experience. Ann Thorac Surg 49: 785–791

    PubMed  Google Scholar 

  25. Penketh ARL, Wise A, Mearns MB, Hodson ME, Batten JC (1987) Cystic fibrosis in adolescents and adults. Thorax 42: 526–532

    PubMed  Google Scholar 

  26. Ramirez JC, Patterson GA, Winton TL, de Hoyos AL, Miller JD, Maurer JR (1992) Bilateral lung transplantation for cystic fibrosis. J Thorac Cardiovasc Surg 103:287–294

    PubMed  Google Scholar 

  27. Scott-Jupp R, Lama M, Tanner MS (1991) Prevalence of liver disease in cystic fibrosis. Arch Dis Child 66: 698–701

    PubMed  Google Scholar 

  28. Smith J, Wallwork J (1995) Retransplantation in heart-lung recipient with obliterative bronchiolitis (letter). J Thorac Cardiovasc Surg 109: 818

    Google Scholar 

  29. Starnes VA, Lewiston N, Theodore J, Stoehr C, Stinson E, Shumway NE, Oyer PE (1992) Cystic fibrosis. Target population for lung transplantation in North America in the 1990 s. J Thorac Cardiovasc Surg 103: 1008–1014

    PubMed  Google Scholar 

  30. Starnes VA, Barr ML, Cohen RG (1994) Lobar transplantation. Indications, techniques and outcome. J Thorac Cardiovasc Surg 108: 403–411

    PubMed  Google Scholar 

  31. Stern RC, Stevens DP, Boat TF, Doershuk CF, Izant RJ Jr, Matthews LW (1976) Symptomatic hepatic disease in cystic fibrosis: incidence, course, and outcome of portal systemic shunting. Gastroenterology 70: 645–649

    Google Scholar 

  32. Van Obbergh L, Carlier M, de Clety SE, Sokal E, Rennotte MT, Veyckemans F, Dekock M, Frans A, Otte JB (1993) Liver transplantation and pulmonary gas exchanges in hypoxemic children. Am Rev Respir Dis 148:1408–1410

    PubMed  Google Scholar 

  33. Wood A, Higgenbottam T, Jackson M, Scott J, Stewart S, Wallwork J (1989) Airway mucosal bielectric potential difference in cystic fibrosis after lung transplantation. Am Rev Respir Dis 140:1645–1649

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

About this article

Cite this article

Couetil, J.P.A., Soubrane, O., Houssin, D.P. et al. Combined heart-lung-liver, double lung-liver, and isolated liver transplantation for cystic fibrosis in children. Transplant Int 10, 33–39 (1996). https://doi.org/10.1007/BF02044339

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02044339

Key words

Navigation