Hypospadias with dorsal chordee.
Herein we report on 7 children with hypospadias and dorsal chordee. A theory is proposed to explain this rare combination of anomalies. A method for surgical correction is presented.
Abnormalities, Multiple↗
Biomedical subjects
M K Hanna
Publications and source records attributed to M K Hanna.
Endoscopic treatment of vesicoureteral reflux in 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.
Adolescent↗
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.
Adolescent↗
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.
Disorders of Sex Development↗
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.
Female↗
Vaginal construction.
Construction of the vagina was performed in 5 46 XY, phenotypic female children, by utilizing a vascularized segment of ileum, which is folded and converted into a pouch, then placed between the rectum and bladder, and transplanted to the perineum. The neovaginal opening remained patent in all, mucus discharge decreased with time, and one teenager experienced a satisfactory sexual relationship.
Adolescent↗
Removal of ureteral stone from patient with cross-trigonal ureteral reimplantation.
A ureteral calculus was extracted ureteroscopically from a pelvic kidney in a child who had undergone two open pyelolithotomies, bladder reconstruction of cloacal exstrophy, and bilateral cross-trigonal ureteral reimplantations (Cohen procedure).
Bladder Exstrophy↗
MAGPI hypospadias repair: ambulatory versus inpatient surgery.
Over a one-year period, 96 consecutive children with distal hypospadias underwent mental advancement and glanuloplasty (MAGPI) for hypospadias repair. In a prospective study, 56 were operated on as inpatients, and 40 underwent repair as outpatients. It was determined that MAGPI can be performed safely and cost-effectively on an ambulatory basis.
Ambulatory Surgical Procedures↗
A new method for conversion of ileal conduit to a continent nonrefluxing reservoir: the sandwich technique.
A preliminary report on a method for conversion of the ileal conduit to a nonrefluxing continent reservoir is presented. The original conduit is opened to become a patch of ileum and the ureters are reimplanted in an antirefluxing manner into the ileal conduit patch. A new segment of ileum is isolated and its distal third is constructed into a continent nipple valve by ileoileal intussusception. The proximal two-thirds are opened at the antimesenteric border to form another patch, which then is sutured onto the original ileal patch, thus, forming a sandwich. Reflux was prevented and continence was achieved between clean intermittent catheterizations of the reservoir in 4 patients.
Adolescent↗
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.
Adolescent↗
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.
Appendix↗
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.
Abdominal Muscles↗
Technique and trial of continent ileocystostomy.
To overcome the problem of incontinence which failed to respond to standard measures, an animal model was designed for continent diversion without cystectomy. A tapered terminal ileal segment was implanted in a submucosal tunnel on the anterior aspect of the bladder, and the proximal end of the segment was brought out to the abdominal wall as a stoma that is catheterized intermittently. Ileocystostomy provided continent diversion in 10 dogs at intravesical pressures as high as 45 cm H2O, and there were no changes in serum electrolytes, creatinine, or urea nitrogen concentration.
Animals↗
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.
Animals↗
Alterations of myocardial alpha 1-adrenergic receptors in hypertensive cardiac hypertrophy in the rat.
The hypertrophied myocardium of 2 kidney-1 clip renal hypertensive rats (2K-1C RHR) displays reduced responsiveness to phenylephrine stimulation. We have studied the effects of hypertrophy on alpha 1-receptor binding properties in three models of hypertension. Specific binding density of an alpha 1-selective antagonist [3H]-prazosin to ventricular membrane of 6 and 10 weeks 2K-1C RHR was significantly decreased (45 +/- 9 fmol/mg protein, n = 14, vs 64 +/- 7.8 fmol/mg protein, n = 20, p less than .001) compared to age matched sham operated control group, but with no change in the total alpha 1-receptor content in the hypertrophied ventricles. The 4 week 1K-1C RHR showed a 29% decrease in alpha 1-adrenergic receptor density with no change in affinity. In contrast, the spontaneously hypertensive rats (SHR) showed an increase in alpha 1-receptors in the prehypertensive stage (4 weeks old) (115.2 +/- 4.9 fmol/mg protein, n = 10, vs 88.7 +/- 12 fmol/mg protein, n = 10, p less than .001) and in the adult stage with established hypertension (15 weeks old) (87.1 +/- 10.5 fmol/mg protein, n = 10, vs 67.3 +/- 9 fmol/mg protein, n = 10, p less than .001) compared to age matched or heart weight matched Wistar Kyoto rats (WKY). Reduced responsiveness to phenylephrine in RHR may be due to reduced density of alpha 1-receptors, but the reason for the increase in alpha 1-receptors in SHR's myocardium remains unclear.
Aging↗
Complete penoscrotal transposition.
A patient with complete penoscrotal transposition is described. We believe this represents the first such case associated with a chromosomal anomaly. A review of the literature is given.
Abnormalities, Multiple↗
Intraurethral condylomas acuminata in previous hypospadias repair.
In recent years, the number of reported cases of venereal warts has increased more than fivefold, and various methods of treating these lesions have been described. We herein report a rare case of condylomas acuminata in a hypospadias repair.
Adult↗
Dominantly inherited ureteropelvic junction obstruction.
We report on siblings from two families with unilateral ureteropelvic junction obstruction. HLA studies were undertaken and found to be a useful marker between affected members. We believe that incomplete penetrance with variable expression is the most probable mode of transmission of this disorder.
Adolescent↗