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The management of end-stage renal disease in infants with imperforate anus

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Abstract

Genitourinary malformations are frequently associated with imperforate anus, and death from renal failure is reported in up to 6% of children with supralevator imperforate anus. In recent years, advances in renal transplantation and the management of end-stage renal disease (ESRD) have extended these therapies to infants in the first 2 years of life. In infants with imperforate anus and ESRD, it is unclear if the additional burdens of the anorectal malformation and its staged repair contraindicate dialysis and transplantation. This report describes our experience with three such infants and outlines an approach to their care, addressing the following key issues: the initial surgical management of the imperforate anus, the careful search for associated urinary tract and other malformations, the ESRD management, and the appropriate timing of the staged bowel reconstruction and renal transplantation. These cases confirm that such children may be successfully managed by dialysis and renal transplantation co-ordinated with bowel reconstruction; however, there remain the longterm risks of immunosuppression, bladder and bowel dysfunction, and associated congenital anomalies.

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References

  1. Weber TR, Smith W, Grosfeld JL (1980) Surgical experience in infants with the VATER association. J Pediatr Surg 15:849–853

    PubMed  Google Scholar 

  2. McLorie GA, Sheldon CA, Fleisher M, Churchill BM (1987) The genitourinary system in patients with imperforate anus. J Pediatr Surg 22:1100–1104

    PubMed  Google Scholar 

  3. Najarian JS, Frey DJ, Matas AJ, Gillingham KJ, So SK, Cook M, Chavers B, Mauer SM, Nevins TE (1990) Renal transplantation in infants. Ann Surg 212:353–367

    PubMed  Google Scholar 

  4. Najarian JS, Almond PS, Mauer M, Chavers B, Nevins T, Kashtan C, Matas AJ (1992) Renal transplantation in the first year of life: the treatment of choice for infants with end-stage renal disease. J Am Soc Nephrol 2:S228-S233

    PubMed  Google Scholar 

  5. Briscoe DM, Kim MS, Lillehei C, Eraklis AJ, Levey RH, Harmon WE (1992) Outcome of renal transplantation in children less than two years of age. Kidney Int 42:657–662

    PubMed  Google Scholar 

  6. Kohaut EC, Whelchel J, Waldo FB, Diethelm AG (1987) Aggressive therapy of infants with renal failure. Pediatr Nephrol 1:150–153

    PubMed  Google Scholar 

  7. Davis ID, Chang PN, Nevins TE (1990) Successful renal transplantation accelerates development in young uremic children. Pediatrics 86:594–600

    PubMed  Google Scholar 

  8. Grosfeld JL (1991) Pediatric Surgery. In: Sabiston DC (ed) Textbook of surgery: the biological basis of modern medical practice, 14th edn. Saunders, Philadelphia, p 1149

    Google Scholar 

  9. Laberge JM, Bosc O, Yazbec S, Youssef S, Ducharme JC, Guttman FM, N'guyen LT (1983) The anterior perineal approach for pull through operations in high imperforate anus. J Pediatr Surg 18: 774–778

    PubMed  Google Scholar 

  10. Singh MP, Haddadin A, Zachary RB, Piling DW (1974) Renal tract disease in imperforate anus. J Pediatr Surg 9:197–202

    PubMed  Google Scholar 

  11. Muraji T, Mahour GH (1984) Surgical problems in patients with VATER-associated anomalies. J Pediatr Surg 19:550–554

    PubMed  Google Scholar 

  12. Wiener ES, Kiesewetter WB (1973) Urologic abnormalities associated with imperforate anus. J Pediatr Surg 8:151–157

    PubMed  Google Scholar 

  13. Fleming SE, Hall R, Gysler M, McLorie GA (1986) Imperforate anus in females: frequency of genital tract involvement, incidence of associated anomalies, and functional outcomes. J Pediatr Surg 21: 146–150

    PubMed  Google Scholar 

  14. Hall JW, Tank ES, Lapides J (1980) Urogenital anomalies and complications associated with imperforate anus. J Urol 103:810–814

    Google Scholar 

  15. Warady BA, Stall C, Paulsen J, Johnson CB, Sedman A, Lum GM (1986) A unique approach to peritoneal dialysis in infants. Am J Kidney Dis 7:235–240

    PubMed  Google Scholar 

  16. Warady BA, Kriley M, Lovell H, Farrell SE, Hellerstein S (1988) Growth and development of infants with end-stage renal disease receiving long-term peritoneal dialysis. J Pediatr 112:714–719

    PubMed  Google Scholar 

  17. Murugasu B, Conley SB, Lemire JM, Portman RJ (1991) Fungal peritonitis in children treated with peritoneal dialysis and gastrostomy feeding. Pediatr Nephrol 5:620–621

    PubMed  Google Scholar 

  18. Wood EG, Bunchman TE, Khurana R, Fleming SS, Lynch RE (1990) Complications of nasogastric and gastrostomy feeding tube feedings in children with end-stage renal disease. Adv Perit Dial 6: 262–264

    PubMed  Google Scholar 

  19. Miller LC, Lum CT, Bock GH, Simmons RL, Najarian JS, Mauer SM (1983) Transplantation of the adult kidney into the very small child: technical considerations. Am J Surg 145:243–247

    PubMed  Google Scholar 

  20. Mollard P (1984) Les imperforations anales hautes, traitement et résultats. Chir Pediatr 25:305–310

    PubMed  Google Scholar 

  21. Ditesheim JA, Templeton JM (1987) Short-term vs long-term quality of life in children following repair of high imperforate anus. J Pediatr Surg 22:581–587

    Google Scholar 

  22. Sonnino RE, Reinberg O, Bensousson AL, Laberge JM, Blanchard H (1991) Gracilis muscle transposition for anal incontinence in children: long-term follow-up. J Pediatr Surg 26:1219–1233

    PubMed  Google Scholar 

  23. Smith ED (1968) Urinary anomalies and complications in imperforate anus. J Pediatr Surg 3:337–349

    PubMed  Google Scholar 

  24. Belman AB, King LR (1972) Urinary tract abnormalities associated with imperforate anus. J Urol 108:823–825

    PubMed  Google Scholar 

  25. Karrer FM, Flannery AM, Nelson D, McLone DG, Raffensperger JG (1988) Anorectal malformations: evaluation of associated spinal dysraphic syndromes. J Pediatr Surg 23:45–48

    PubMed  Google Scholar 

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Sharma, A.K., Kashtan, C.E. & Nevins, T.E. The management of end-stage renal disease in infants with imperforate anus. Pediatr Nephrol 7, 721–724 (1993). https://doi.org/10.1007/BF01213332

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  • DOI: https://doi.org/10.1007/BF01213332

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