John F. Schillinger

Prune Belly Syndrome in the Female Subject

The prune belly syndrome of marked abdominal muscle deficiency and associated genitourinary anomalies has been found almost exclusively in male subjects. Of the few reported cases of female patients with this entity most are questionable or incomplete forms. The light microscopic histology and electron microscopic ureteral abnormalities in male subjects have been reported previously. A female newborn with marked abdominal muscle deficiency and severe bilateral ureteropyelocaliectasis is described. Chromosomal studies showed 46 XX karyotype. Histologic examination of the ureter by light microscopy was compatible with the diagnosis.

Surgical Correction of Vesicoureteral Reflux in Children with Neurogenic Bladder

The reported success rate for correction of vesicoureteral reflux in the neurogenic bladder has been low. Reflux was corrected in 33 of 37 ureters in 23 selected children with neurogenic bladder-treated by reimplantation. Selection was based on good bladder capacity, little or no trabeculation and the expectation that the bladder would be useful in future management of the patient. Preoperative preparation and surgical technique are emphasized.

Posttraumatic Arterial Priapism In A 7-Year-Old Boy: Successful Management by Percutaneous Transcatheter Embolization

Priapism may be classified as the classical veno-occlusive form or the more recently described high flow, arterial type. We report a case of posttraumatic arterial priapism in a 7-year-old boy. The successful management with percutaneous transcatheter embolization of the internal pudendal artery is described.

Continence in Cases of Bladder Exstrophy

The relationship of the onset of continence, staged surgical procedures and continence length was investigated in 21 patients with closed continent exstrophy. Continence occurred within a year of operation in 13 patients and 10 of these 13 patients had a Young-Dees urethroplasty and Marshall-Marchetti-Krantz suspension. In 11 of these 13 patients the continence length was greater than 1 cm. In the remaining 8 patients continence was delayed beyong a year. Only 1 of these 8 patients had undergone both bladder neck procedures and in 5 patients the continence length was greater than 1 cm.

Pediatric Articles: Primary Carcinoid of the Prostate in Conjunction With Multiple Endocrine Neoplasia IIb in a Child

Prostatic neoplasms are rare in childhood. We report a case of primary prostatic carcinoid in a 7-year-old boy who was subsequently diagnosed with multiple endocrine neoplasia IIb. To our knowledge this is the first report of either pediatric carcinoid of the prostate or of prostatic carcinoid in conjunction with neuronal intestinal dysplasia and medullary thyroid carcinoma suggestive of multiple endocrine neoplasia IIb. Management and histogenesis regarding this prostatic tumor are discussed as is a possible association with other neuroendocrine tumors.

Surgical Treatment of the Massively Dilated Ureter in Children. Part I. Management by Cutaneous Ureterostomy

One of the major therapeutic challenges facing the pediatric urologist is the massively dilated ureter. When conservative measures, such as relief of obstruction or treatment of infection, have failed or are likely to fail, surgical treatment must be directed at the massive dilatation itself. These circumstances were encountered in 366 massively dilated ureters in 244 children during a 10-year period. Loop and/or terminal cutaneous ureterostomy was used to manage 125 of these ureters in 74 children. Indications for temporary non-intubated proximal urinary diversion included uncontrolled infection, sepsis, azotemia, significant ureteral redundancy and tortuosity, and questionable over-all renal function. Advantages of loop ureterostomy included more proximal drainage and less stomal problems but terminal ureterostomy required a significantly lesser number of surgical procedures in reconstructing the urinary tract. Temporary splinting of the ureter in dry ureteral reimplantations has diminished markedly the incidence of ureterovesical complications. No instances of permanent vesical contracture were noted after long periods of defunctionalization. After initial reconstruction of 47 cutaneous ureterostomies good results by all parameters were obtained in 68 per cent. Additional surgical procedures in selected initial failures have resulted in a final success rate of 85 per cent.

Bladder exstrophy: Penile lengthening procedure

A review of bladder exstrophy experience at our institution was undertaken with reference to penile adequacy and intersymphyseal distance. Drift of the symphysis apart even with iliac osteotomy was noted to occur within two years of initial closure but remained stable after three years. This drift can be prevented if the sacral tuberous and sacral spinous ligaments are sectioned. Further, the adequacy of the phallus seems to depend directly on the intersymphyseal distance. Thus, we suggest a variation in the staged approach to bladder exstrophy repair with these facts in mind.

Urologic abnormalities found with imperforate anus

Abstract This is a retrospective review of 28 cases of imperforate anus with respect to congenital anomalies of the urinary tract and hydronephrosis. A large number of solitary kidneys have been found. It is further noted that hydroureters often found on initial intravenous pyelography will regress after colostomy alone.

Bladder diverticula causing posterior urethral obstruction in children.

Two cases of bladder diverticula causing posterior urethral obstruction, which was corrected surgically, are presented. Five similar cases have been found in the literature.

Gas cystometry in cases of continent bladder exstrophy.

Cystometry in 21 cases of closed continent bladder exstrophy revealed that 14 patients had a normal reflexic bladder. A simultaneous anal plug electromyogram showed an appropriate response in all patients when correlated with a cystometrogram.