A Single-Center Experience with Kidney Transplantation in the Verteberal, Anal, Cardiac, Tracheoesophageal, Renal, and Limb Birth Detects (VACTERL) Association

• Published in: Transplantation proceedings Vol. 43; no. 4; pp. 1250 - 1251
• Main Authors: Telkes, G, Reusz, G, Szabó, A.J, Langer, R.M
• Format: Journal Article Conference Proceeding
• Language: English
• Published: Amsterdam Elsevier Inc 01-05-2011; Elsevier
• Subjects: Adult; Anal Canal - abnormalities; Biological and medical sciences; Child; Complex syndromes; Esophagus - abnormalities; Female; Fundamental and applied biological sciences. Psychology; Fundamental immunology; 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; Medical genetics; Medical sciences; Recurrence; Renal Insufficiency - etiology; Renal Insufficiency - surgery; Reoperation; Spine - abnormalities; Surgery; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the urinary system; Time Factors; Tissue, organ and graft immunology; Trachea - abnormalities; Treatment Outcome; Ureterostomy; Urologic Diseases - etiology; Urologic Diseases - surgery; Young Adult; Heart; Center; Tracheoesophageal; Digestive system; Respiratory disease; Diseases of the osteoarticular system; Homotransplantation; Birth; Kidney; Medicine; Experience; Treatment; Urinary system; Anus; Surgery; Limb; Digestive diseases; Graft; Circulatory system; Complex syndrome; Vater syndrome; Kidney transplantation
• ISSN: 0041-1345; 1873-2623
• 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.