A single-center experience with kidney transplantation in the verteberal, anal, cardiac, tracheoesophageal, renal, and limb birth detects (VACTERL) association

Transplant Proc. 2011 May;43(4):1250-1. doi: 10.1016/j.transproceed.2011.03.079.

Abstract

VACTERL association is a nonrandom association of birth defects in vertebral, anal, cardiac, tracheoesophageal, renal, and limb structures. Renal anomalies are observed in ∼60%-90% of VACTERL patients. We present 3 cases to demonstrate the clinical and surgical challenges that these patients present for renal transplantation. One pediatric and 2 adult patients with the VACTERL association were transplanted at a single center; their follow-up times were 6 years, 4 years, and 3 months. Only 1 of them had a suitable native bladder to receive the kidney graft; the other 2 required bladder augmentation, 1 of which was performed after the loss of the first graft. None of these patients had an uneventful posttransplantation course. Two patients had acute rejection episodes, and 2 had reoperations for urologic complications. One patient needed a surgical intervention owing to a sigmoid prolapse. All 3 grafts worked at last examination. The 2 patients with bladder reconstructions and longer follow-ups suffered recurrent pulmonary and urinary infections and had been hospitalized several times during each posttransplantation year. In conclusion, multiorgan involvement in VACTERL patients greatly complicates medical care after transplantation; urinary tract reconstruction seems to be essential before transplantation.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anal Canal / abnormalities
  • Child
  • Esophagus / abnormalities
  • Female
  • Graft Rejection / etiology
  • Graft Rejection / surgery
  • Graft Survival
  • Heart Defects, Congenital / complications*
  • Humans
  • Kidney / abnormalities
  • Kidney Failure, Chronic / etiology
  • Kidney Failure, Chronic / surgery*
  • Kidney Transplantation* / adverse effects
  • Limb Deformities, Congenital / complications*
  • Male
  • Recurrence
  • Renal Insufficiency / etiology
  • Renal Insufficiency / surgery*
  • Reoperation
  • Spine / abnormalities
  • Time Factors
  • Trachea / abnormalities
  • Treatment Outcome
  • Ureterostomy
  • Urologic Diseases / etiology
  • Urologic Diseases / surgery
  • Young Adult

Supplementary concepts

  • VACTERL association