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Published on: December 20, 2014
The management of end-stage renal disease in infants with imperforate anus
A K Sharma1, C E Kashtan, T E Nevins
1Department of Pediatrics, University of Minnesota Hospital and Clinic, Minneapolis 55455.
Insights
Infants with imperforate anus and end-stage renal disease (ESRD) can undergo successful dialysis and renal transplantation alongside staged bowel reconstruction. Careful management is crucial due to associated anomalies and long-term risks.
Area of Science:
- Pediatric Surgery
- Nephrology
- Congenital Malformations
Background:
- Genitourinary malformations often accompany imperforate anus, with renal failure causing mortality in up to 6% of supralevator cases.
- Advances in renal transplantation and end-stage renal disease (ESRD) management now permit treatment for infants within their first two years of life.
Observation:
- The feasibility of dialysis and renal transplantation in infants with imperforate anus and ESRD, considering the complexities of anorectal malformation repair, remains unclear.
- This report details the care of three infants with imperforate anus and ESRD, focusing on initial surgical management, comprehensive malformation screening, ESRD treatment, and coordinated bowel reconstruction with renal transplantation.
Findings:
- Successful management through coordinated dialysis, renal transplantation, and staged bowel reconstruction is achievable for infants with imperforate anus and ESRD.
- These cases demonstrate the viability of intensive, multidisciplinary care for this complex patient population.
Implications:
- Children with imperforate anus and ESRD can benefit from timely renal replacement therapy and surgical reconstruction.
- Long-term considerations include risks associated with immunosuppression, potential bladder and bowel dysfunction, and management of co-existing congenital anomalies.
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 long-term risks of immunosuppression, bladder and bowel dysfunction, and associated congenital anomalies.
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