Kevin A. Burbige

Pregnancy and sexual function in women with bladder exstrophy

As a result of improved surgical and medical treatment, an increasing number of women with bladder exstrophy are reaching childbearing age. Unfortunately, little data exist regarding their sexual capacity, fertility, and potential complications of pregnancy. To assess these parameters, the medical records of 40 women ranging in age from nineteen to thirty-six years who had been treated in infancy for bladder exstrophy were reviewed. The 14 pregnancies in 11 women (25%) resulted in 9 successful deliveries, 3 spontaneous abortions, and 2 elective abortions. Nine women in this group had previous urinary diversion. Twelve of the 40 women agreed to participate in a survey of sexual capacity. Eight of this group reported that they engaged in regular sexual activity. Six reported regular orgasms, 4 dyspareunia, and 5 dysmenorrhea. Five of these women had achieved 7 pregnancies, and only 1 woman in this group who desired pregnancy had been unable to conceive. Complications during pregnancy included: uterine prolapse in 7, acute pyelonephritis in 1, prolapsed ileal conduit in 1, and transient urinary incontinence in 1.

Long-term followup of 207 patients with bladder exstrophy: an evolution in treatment.

Between 1945 and 1985 a total of 207 patients (male-to-female ratio 2:1) was treated at our institution for exstrophy of the bladder. Primary anatomical bladder closure was performed in 137 patients, of whom 42 (31 per cent) eventually required urinary diversion. Primary urinary diversion was the initial treatment in 70 patients, including ureterosigmoidostomy in 40, ileal conduit in 17, colon conduit in 11 and cutaneous ureterostomy in 2. Secondary urinary diversion was necessary in 35 patients and included continent diversion in 7. Primary anatomical bladder closure, done within 72 hours of birth and followed by staged reconstruction of the bladder neck, was the most successful surgical regimen for the treatment of bladder exstrophy. Acceptable urinary continence was achieved in 82 per cent of our patients with this approach. However, multiple procedures often were required to achieve continence.

Prune Belly Syndrome: 35 Years of Experience

Between 1949 and 1984, 50 children with the prune belly syndrome were treated at our institution. The modes of evaluation and treatment, and the long-term results are discussed.

Diagnosis and Surgical Correction of the Pediatric Varicocele

We reviewed our experience with the diagnosis and management of varicoceles in 40 patients 9 to 18 years old. Of the patients 29 underwent varicocelectomy and 11 are being managed conservatively with periodic followup. The results of therapy and criteria for surgical intervention are discussed.

Meconium hydrocele: an unusual etiology of a scrotal mass in the newborn

The in utero leakage of meconium from the gastrointestinal tract into the peritoneal cavity results in the clinical entity known as meconium peritonitis. A patent processus vaginalis in the male fetus may lead to the migration of meconium into the scrotum, and these infants can present with soft meconium-filled hydroceles at birth. Local tissue reaction during the first several weeks of life results in calcification of these hydroceles into hard tumor-like lesions that may mimic testicular neoplasia. We report an atypical case of meconium hydrocele in a newborn.

Salt losing nephropathy simulating congenital adrenal hyperplasia in infants with obstructive uropathy and/or vesicoureteral reflux--value of ultrasonography in diagnosis.

Salt losing nephropathy, occurring predominantly in male infants, has been reported in association with a spectrum of urologic diseases including obstructive uropathy and massive, infected vesicoureteral reflux (VUR). This has been called pseudo-hypoaldosteronism (PHA) or alternatively, pseudo salt-losing congenital adrenal hyperplasia (CAH), and is thought to reflect a tubular unresponsiveness to aldosterone. We report our experience with six cases, discuss one case in detail and review the 39 cases previously reported. A one month old male infant presented with a left upper quadrant mass. Signs and symptoms included vomiting, dehydration, hyponatremia and hyperkalemia. This suggested the diagnosis of CAH for which therapy was instituted. Ultrasonographic examination subsequently revealed the mass to be a urinoma in an infant with posterior urethral valve (PUV) and obstructive hydronephrosis.

Extravesical Ureteral Reimplantation: Results in 128 Patients

PURPOSEnWe evaluated the results of extravesical ureteral reimplantation in children.nnnMATERIALS AND METHODSnWe reviewed the records of 128 children (174 ureters) who underwent ureteral reimplantation via extravesical techniques. Primary vesicoureteral reflux was the most common diagnosis (73 patients).nnnRESULTSnThe extravesical technique produced a successful result in all patients (no reflux or obstruction). The 2 complications included postoperative urosepsis and transient urinary retention.nnnCONCLUSIONSnExtravesical ureteral reimplantation is a reliable procedure with predictable results comparable to those of more traditional intravesical techniques.

Contained Neonatal Urinoma: Management and Functional Results

Between 1970 and 1985, 10 male newborns with a contained urinoma were treated at our institution. An underlying congenital obstructive uropathic condition was discovered in every patient, the most common of which was posterior urethral valves. All 10 patients underwent surgical correction of the primary obstructive process. Direct drainage of the urinoma was required in 4 patients because of progressive clinical symptoms. Radiographic and renal functional parameters returned to normal at followup. Clinical presentation, evaluation, treatment and pathophysiology of this rare entity are discussed.

Uterus Didelphys and Vaginal Duplication with Unilateral Obstruction Presenting as a Newborn Abdominal Mass

Vaginal duplication with uterus didelphys is a rare anomaly in which unilateral obstruction of either vaginal orifice may result in hydrocolpos but with normal-appearing external genitalia in the female newborn. We recently treated 2 female infants with this anomaly who presented with a lower abdominal mass that persisted after bladder drainage. Both infants had evidence of urological abnormalities on the side ipsilateral to the obstructed vagina. One infant had renal agenesis and 1 had severe renal dysplasia. Owing to the high incidence of associated renal abnormalities excretory urography, voiding cystourethrography and abdominal ultrasound are essential to the evaluation. Precise diagnosis and careful evaluation are mandatory to avoid unnecessary loss of essential normal müllerian structures.

The complications of urinary tract reconstruction

Urinary tract reconstruction has benefited a vast number of patients with dysfunctional lower urinary tracts caused by congenital abnormalities, previous surgery, or both. Reconstructive efforts have been innovative and continue to evolve. With this evolution, new complications continue to appear, and in order to minimize the risk to the patient, we must recognize our previous lessons. Appropriate patient selection is essential in achieving a successful outcome in this group. The patients neurologic status, urologic anatomy, renal function, and motivation are also important factors in choosing the appropriate patient and correct surgical approach. Close follow-up remains the single most important element in assuring long-term well-being for most of these patients.