title: A single-center experience with kidney transplantation in the verteberal, anal, cardiac, tracheoesophageal, renal, and limb birth detects (VACTERL) association.
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.
authors.affiliation: Department of Transplantation and Surgery, Semmelweis University, Budapest, Hungary. telkesdr@gmail.com
authors.forename: G
authors.initials: G
authors.lastname: Telkes
authors.forename: G
authors.initials: G
authors.lastname: Reusz
authors.forename: A J
authors.initials: AJ
authors.lastname: Szabó
authors.forename: R M
authors.initials: RM
authors.lastname: Langer
mesh_terms: D000328:Adult; D001003:Anal Canal / Q000002:abnormalities; D002648:Child; D004947:Esophagus / Q000002:abnormalities; D005260:Female; D006084:Graft Rejection / Q000209:etiology / Q000601:surgery; D006085:Graft Survival; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D007668:Kidney / Q000002:abnormalities; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000601:surgery*; D016030:Kidney Transplantation / Q000009:adverse effects; D017880:Limb Deformities, Congenital / Q000150:complications*; D008297:Male; D012008:Recurrence; D051437:Renal Insufficiency / Q000209:etiology / Q000601:surgery*; D012086:Reoperation; D013131:Spine / Q000002:abnormalities; D013997:Time Factors; D014132:Trachea / Q000002:abnormalities; D016896:Treatment Outcome; D014519:Ureterostomy; D014570:Urologic Diseases / Q000209:etiology / Q000601:surgery; D055815:Young Adult