Pediatrics: Less than half of undervirilized males receive molecular Dx

July 10, 2017

The finding that the glans is a significant variable in hypospadias complications and an evaluation of external pelvic fixation alone without osteotomy at time of newborn bladder exstrophy closure were among the noteworthy studies in pediatric urology at AUA 2017.

Audrey C. Rhee, MD

The finding that the glans is a significant variable in hypospadias complications and an evaluation of external pelvic fixation alone without osteotomy at time of newborn bladder exstrophy closure were among the noteworthy studies in pediatric urology at the AUA annual meeting in Boston. The take-homes were presented by Audrey C. Rhee, MD, of Cleveland Clinic, Cleveland.

 

Caudal block during penile procedures is more efficacious for the early postoperative period but at increased cost and time compared to the penile block.

 

Defects in the KCTD13 gene can result in hypospadias, cryptorchidism, and other lower urinary tract aberrant development. With loss of expression, mice were more likely to have cryptorchidism, smaller testes, and smaller baculum with decreased sperm count and motility.

 

Less than half of undervirilized males receive a molecular diagnosis. Next-generation sequencing can result in a higher rate of molecular diagnosis than more traditional genetic testing.

 

Upon review of 536 consecutive tubularized incised plate repairs for hypospadias, the combination of glans groove graded as deep moderate or shallow absent and the urethral plate graded as robust or poor spongiosum was the main risk factor for complications.

 

A study demonstrated effective primary proximal hypospadias repair with the use of buccal mucosa as a graft for urethroplasty. The complication rate was similar to that of other studies using prepuce, suggesting this may be a preferable option over prepuce for the most severe cases of proximal hypospadias.

 

 

Continue to the next page for more take-home messages.

 

  • The glans proved to be a significant variable in hypospadias complications, specifically glans dehiscence. Authors suggest a staged approach, with a free graft of buccal mucosa placed in a deeply incised groove of the glans as a first stage followed by tubularization 6 to 12 months later.

  • In a murine model, researchers destabilized a major plaque protein, uroplakin, during urinary tract obstruction and found that this promoted the progression of obstructive injury. This suggests that urothelial remodeling is a protective feature during congenital urinary tract obstruction.

  • Type of urinary diversion is not predictive of end-stage renal disease incidence in neonates with stage 3 chronic kidney disease and posterior urethral valve. Post-intervention GFR revealed no significant difference between additional urinary diversion and valve ablation alone at 10 years.

  • Children with an elevated ureteral diameter ratio are at increased risk of breakthrough urinary tract infections independent of reflux grade.

  • In a resource-deprived center, complete primary repair of bladder exstrophy was successfully performed in an older cohort of children, but rates of incontinence are high. The incontinence may imply that ensuring a patent bladder neck, even at the expense of continence, may preserve upper tracts and be a safe option in this challenging cohort.

  • A video on intraoperative MRI-guided pelvic floor navigation during exstrophy closure showed preoperative MRI 3D imaging and subsequent use of the Brainlab computer in real time provided anatomic information regarding the pelvic floor, specifically the release of the urogenital diaphragm fibers and muscle fibers of the diastatic pubic rami.

  • External pelvic fixation alone without osteotomy at time of newborn bladder exstrophy closure is technically feasible with decreased hospital stay and no need for lower-extremity immobilization.

  • In patients undergoing hypospadias repair with either pudendal or caudal block, there was no difference in intraoperative opioid requirement or postoperative opioid doses, and the postoperative length of stay was shorter in the pudendal block cohort.

  • In 22 patients who received tamsulosin prior to ureteroscopy, 19 had successful navigation of the ureter. Of 19 patients who did not take the alpha-blocker, only 10 had successful ureteral navigation.

  • In the setting of renal trauma, delayed imaging should be included at the time of initial study. At one institution, 32% of all patients undergo a repeat scan, 54% of whom get delayed imaging, which translates to the number needed to treat as three patients to obtain the proper initial CT scan with delayed images to prevent one repeat CT scan.

  • In a study of renal transplantation, there was no correlation between duration of anuria and GFR deterioration.

  • In pediatric renal transplantation, there was a 15% overall rate or ureteral complications, which occurred in 13% of those with normal bladders and 20% of those with pre-existing bladder pathology, which was not found to be statistically significant.

  • Pediatric spina bifida patients who transitioned successfully into the adult clinic were more likely to have bladder management programs and less likely to under urologic procedures as an adult.

  • Myelomeningocele patients who failed to follow up for health care after 18 months were surveyed on where, why, and how they receive care. The lack of follow-up is multi-factorial, including provider communication, patient preference, and self-management and support. Insurance contributed but to a lesser degree.

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