Moneer K. Hanna

Malignant renal tumors intuberous sclerosis

Although the frequency of renal carcinoma in tuberous sclerosis is lower than that in von Hippel-Lindau syndrome, it occurs bilaterally in 43 percent of patients at a median age of twenty-eight years. Concomitant hamartomas and hyperplastic cystic components may confound the histologic diagnosis of renal carcinoma in tuberous sclerosis.

Reconstruction of umbilicus in bladder exstrophy

A surgical method for reconstruction of the umbilicus in patients with bladder exstrophy is described. The technique is based on the surgical principle of eventual tubularization of a skin strip buried in the subcutaneous space.

Reconstruction of umbilicus during functional closure of bladder exstrophy

In babies born with bladder exstrophy, the umbilicus is caudally displaced, and attached to the upper margin of the exstrophied bladder. Reconstructive surgery often removes the navel. A surgical technique for preservation and repositioning of the umbilicus is presented.

Ureteropelvic junction obstruction during the first year of life

Over the past three years, 28 children less than one year of age, were operated on for ureteropelvic junction obstruction. Our results demonstrate that congenital hydronephrosis most commonly presents antenatally, and that early postnatal correction can be performed safely. The high surgical success rate, with a relatively short hospital stay (3-4 days), is attributed to early diagnosis, when the pelviureteral tissues are relatively healthy and nephron maturation is at maximum. The practice of delaying surgical correction of an obstructed hydronephrotic kidney on the basis of stable renal function is unwarranted, since it denies such kidneys their full potential for recoverability.

Continent Diversion in Children: Modification of Kock Pouch

Continent urinary diversion with the ileum was performed in 10 children. The techniques described by Kock and modified by Skinner were modified further to avoid use of staples and a polypropylene (Marlex) collar with their potential hazards. The afferent and efferent limbs were fixed at 2 points internally to the wall of the pouch and externally by apposed multiple incisions and sutures to prevent nipple dessusception. A continent, nonrefluxing reservoir was achieved in all children. The stoma is placed at the umbilicus, which is more pleasing aesthetically, is less compromising to the abdominal wall musculature and is easier to catheterize by chair-bound, obese girls and by children with limited fine finger movements or mild spasticity.

Simultaneous repair of bilateral ureteropelvic junction obstruction.

Fifteen children with bilateral ureteropelvic junction obstruction underwent simultaneous repair as a single procedure. Dismembered pyeloplasty and ureterocalycostomy were the two procedures used. Ten were performed through anterior subcostal incisions. Posterior lumbotomy provided adequate exposure in 5 children and further contributed to shortened hospitalizations. The functional and anatomic results were excellent. Simultaneous bilateral pyeloplasty through posterior lumbotomy is a safe and cost-effective alternative to staged procedures for bilateral ureteropelvic junction obstruction.

Effect of nitroglycerin ointment on penile skin flap survival in hypospadias repair Experimental and clinical studies

The mobilization of penile neurovascular skin flaps is associated with vasospasm, which may result in ischemic necrosis. A prospective clinical trial of nitroglycerin was performed in 40 children undergoing hypospadias repair. Quantitative fluorescein studies were performed in another group of patients who underwent circumcision. These studies represent the first clinical application of nitroglycerin ointment in human subjects. Our preliminary data suggest that nitroglycerin ointment can enhance survival of skin flaps.

Endoscopic treatment of vesicoureteral refluxin children with neurogenic bladders

Endoscopic subureteral injection of Teflon was performed in 12 children (20 ureters) with neurogenic bladders and vesicoureteral reflux (grades III-IV). Follow-up evaluation by ultrasonography and voiding cystourethrogram at three-month intervals up to two years revealed successful correction of the reflux in 70 percent of the ureters.

A Report of 4 Patients with the Drash Syndrome and a Review of the Literature

Four patients with the Drash syndrome, represented by the triad of male pseudohermaphroditism, progressive renal insufficiency and Wilms tumor are discussed. This syndrome, which some have suggested should be expanded to include patients with nephropathy, Wilms tumor and any abnormality of gonadal differentiation, appears early in life. The first sign usually is genital ambiguity. However, proteinuria, hematuria and hypertension eventually require renal biopsy, which may reveal a variety of glomerular and interstitial changes. The nephropathy progresses rapidly, is unresponsive to steroids and eventually requires dialysis. Wilms tumor may appear as a mass on ultrasound or it may not be recognized until nephrectomy or even autopsy. Young children with gonadal dysgenesis and nephropathy are at high risk for nephroblastoma and gonadal tumors and, therefore, prophylactic nephrectomy and gonadectomy should be considered early in the course of this disease.

TOTAL REPLACEMENT OF BLADDER AND URETHRA BY CECUM AND APPENDIX IN BLADDER EXSTROPHY

The first case of total bladder and urethral replacement by cecoappendiceal unit was achieved in a five-year-old boy born with bladder exstrophy. Residual vesical and prostatic tissue was utilized to provide continence successfully.