Urethral abnormalities in male neonates with VATER association.

Fernbach, Sandra K.

doi:10.2214/ajr.156.1.1898547

Cited by 23 publications

(12 citation statements)

References 8 publications

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“…These included urethral abnormalities, such as stenotic urethra, urethrocutaneous fistula, and chordee, and duplicated urethra. Fernbach [16] reported that eight of 20 VATER patients in their study had urethral anomalies, which included megalourethra, urethral duplication, anterior urethral valve, hypospadias, and congenital stricture. Due to the high incidence of urethral anomalies, the author recommended doing a voiding cystourethrogram on all males with VATER association even when clinical symptoms are not present.…”

Section: Discussionmentioning

confidence: 99%

Chronic kidney disease in the VACTERL association: clinical course and outcome

et al. 2009

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Approximately 60% of VACTERL (vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula with atresia, renal defects/radial limb dysplasia) patients have renal anomalies that can be associated with chronic kidney disease (CKD). With improved medical care, a large proportion of these patients survive into adulthood. Longitudinal follow-up data regarding the management of kidney disease in these children is lacking. Twelve VACTERL patients with CKD stage 2-5 and 12 age-matched controls with similar urologic anomalies and CKD [mean follow-up period 15.0 +/- 1.4 (SE) and 11.9 +/- 2.1 years, respectively] were identified in a single center. Eight VACTERL patients progressed to end-stage renal disease (ESRD) compared to four controls (66.7 vs. 33.3%, respectively). Six VACTERL patients were dialyzed pre-transplant. Of the four patients on peritoneal dialysis (PD), three had to be switched to hemodialysis due to complications, whereas two of the three controls on PD did not experience significant problems. Seven VACTERL patients underwent renal transplantation compared to four controls. Mean creatinine clearance 2 years post-transplant was 65.8 +/- 6.3 in VACTERL patients vs. 87.8 +/- 7.1 ml/min per 1.73 m(2) in controls (p = 0.03). VACTERL patients had a significantly lower mean height standard deviation score than the controls (-2.34 +/- 0.41 vs. -1.27 +/- 0.24, respectively; p < 0.05). Based on these results, VACTERL patients with CKD develop ESRD more frequently, experience more complications with dialysis, may have a poorer transplant outcome, and have more severe growth failure than controls.

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Section: Discussionmentioning

confidence: 99%

Chronic kidney disease in the VACTERL association: clinical course and outcome

et al. 2009

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“…Preexisting fusion of mesoderm at the dorsal aspect of the cloacal folds might be present, which prevents normal caudal descent of the urogenital septum and results in a urorectal septal defect. Recent reports describe a possible genetic link between abnormalities of the Sonic Hedgehog gene (Shh) and midline developmental abnormalities such as those seen with the VATERL association [40, 47, 51]. …”

Section: Congenital Anomalies Of the Urethramentioning

confidence: 99%

Congenital anomalies of the male urethra

2007

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“…The combination of congenital megalourethra with more complex anomalies, for example, VACTERL or VATER association has also been described (Fernbach, 1991;Krapp et al, 2002;Ardiet et al, 2003;Vaux et al, 2005). The frequent association with these anomalies suggests that megalourethra is most likely part of a larger mesodermal syndrome comprising gastrointestinal anomalies with a functional obstruction of the urinary tract as the other anomalies are not readily explained by the previously Tissue bridge connecting the twins, indicating possible omphalopagus twins.…”

Section: Discussionmentioning

confidence: 99%