George W. Kaplan

Prostatic Utricle Cysts (Müllerian Duct Cysts)

A series of 88 male patients with prostatic utricle cysts (müllerian duct) has been compiled by adding our 3 patients to 85 cases reported. Irritative lower urinary tract symptoms were the most common presenting complaint (42 per cent), while lower obstructive symptoms were noted in 29 per cent. Of the 20 clinical case reports in boys 25 per cent presented with epididymitis often in an undescended gonad. A cystic rectal mass found in half of the patients represents the most common presenting sign, while hypospadias occurred in a fourth. Unilateral renal dysgenesis or agenesis was associated in 10 per cent of the reports. While the suprapubic surgical approach to excision has been used most commonly it has proved unsatisfactory in two-thirds of the cases. The posterior approach was used successfully in 2 boys with preservation of erectile potency in both. The wide spectrum of histopathology of these cysts prevents clear characterization. A 3% incidence of malignancy in prostatic utricle cysts is noted.

RENAL FUNCTION BEFORE AND AFTER PYELOPLASTY: DOES IT IMPROVE?

PURPOSE Controversy exists concerning the timing of surgical correction of presumed ureteropelvic junction obstruction. Different opinions on the timing of intervention focus on renal function deterioration with time, poor initial relative function, or obstructive drainage curves and/or drainage time on diuretic renography. We retrospectively determined whether there is any improvement in renal function after pyeloplasty for presumed renal obstruction. MATERIALS AND METHODS We retrospectively reviewed the charts of patients who underwent pyeloplasty between 1990 and 1997 in whom preoperative and postoperative diuretic renography data were available. Patients were excluded from review when they had bilateral ureteropelvic junction obstruction, a solitary kidney, associated vesicoureteral reflux, or other bladder or ureteral abnormalities, and when preoperative and postoperative renography studies were not available. RESULTS Complete data were available for review in 79 patients 2 weeks to 18 years old (median age 6 months). Of the patients 73% were male and 73% of the affected kidneys were on the left side. Prenatal hydronephrosis had been diagnosed in 58 patients (73%), of whom 19 (33%) were observed for a variable period before pyeloplasty was performed. In all cases diuretic renography performed at the same institution using a standard protocol revealed a drainage time of 20 minutes or greater preoperatively, while in 58 cases a measurable drainage time was never achieved. As a rule, drainage improved postoperatively (mean and median 25 and 16 minutes, respectively). Open renal biopsy done at pyeloplasty in 54 patients was normal in 29. Preoperatively renal function ranged from 5 to 67% (mean and median 41 and 45, respectively). In all patients the paired t test showed no statistical difference in preoperative and postoperative renal function (p = 0.078, 95% confidence interval -3.451 to 0.185). There was no statistical change in renal function in patients with an abnormal renal biopsy regardless of the severity of renal scarring (p = 0.38) or when renal function was 40% or less (mean preoperative versus postoperative 29.7 versus 28.4%, p = 0.46). The group with greater than 40% function preoperatively had no relevant difference in function before or after surgery (mean 49.7 versus 47.8%, p = 0.065). Prenatally screened patients who were initially observed had a statistically significant difference in renal function before and after pyeloplasty (mean 45.6 versus 43%, p = 0.002). CONCLUSIONS Renal function did not improve after pyeloplasty regardless of the initial level of relative function. Renal scan revealed that differential function decreased after pyeloplasty in some patients in whom hydronephrosis was detected prenatally and who were initially followed with observation. In our opinion waiting for renal function to decrease before considering pyeloplasty is not warranted, since function does not improve even when obstruction is corrected and drainage time improves.

An Alternative Approach to Myelodysplasia Management: Aggressive Observation and Prompt Intervention

We have adopted an approach to the management of myelodysplasia patients which is contrary to that most commonly supported by the existing literature. We screen patients shortly after birth with ultrasound, urine culture and serum creatinine, and then follow patients at 3 to 6-month intervals with similar studies. Cystography and urodynamics are done only as required clinically or by a change in the sonogram. Clean intermittent catheterization is indicated for continence or medical reasons. With this approach of aggressive observation and prompt intervention, we observed a rate of renal deterioration (diminished function on renal scan or parenchymal loss on imaging studies) of 5%. Renal deterioration was associated statistically with urinary tract infections and reflux. Patients started on clean intermittent catheterization for medical indications had greater risk for renal deterioration than those started on it for continence. Renal deterioration occurred with equal frequency between patients with abnormal and normal urodynamic studies, that is urodynamics did not predict renal deterioration. Our data show a rate of renal deterioration similar to other reports. We believe that aggressive observation with prompt intervention for problems once identified represents a reasonable alternative to managing patients with myelodysplasia.

Incidence of the bell-clapper deformityin an autopsy series

OBJECTIVES Testicular torsion is seen in all age groups. The bell-clapper deformity (BCD) when present predisposes the patient to torsion. This study was undertaken to determine the incidence of the BCD. METHODS An autopsy series of 51 males with 101 testes was dissected by the senior author to determine the incidence of the BCD. RESULTS The manner of testicular investment by the tunica vaginalis was classified as normal, intermediate, or absent (BCD). Seventy-six testes were normal, 13 were intermediate, and 12 were of the bell-clapper type. CONCLUSIONS The incidence of the BCD in this autopsy series was 12%. Because the incidence of testicular torsion in the general population is far less than 12%, other factors in addition to anatomic predisposition must be involved.

Laparoscopy for Localization of Nonpalpable Testes

Laparoscopy was performed on 33 consecutive boys with 36 nonpalpable testes whenever 1 or both testes were not palpable, a patient with bilateral nonpalpable testes was known to have responded to human chorionic gonadotropin stimulation and orchiopexy or orchiectomy was planned. Anatomic localization of nonpalpable testes facilitated accurate planning of operative repair, thereby potentially improving the ultimate result. Additionally, the technique rendered exploration unnecessary in patients with the intra-abdominal vanishing testis syndrome. No complications were noted as a result of laparoscopy in these patients.

Spontaneous Bladder Rupture Following Enterocystoplasty

We treated 2 male patients with spontaneous bladder rupture following enterocystoplasty. Both adolescents had neurogenic bladders that were managed with enterocystoplasty and an artificial urinary sphincter. With prompt aggressive therapy including intravenous antibiotics, laparotomy and closure of the perforation serious sequelae were averted. Cystography failed to demonstrate the lesion in either case. The failure of cystography to diagnose these ruptures is especially disturbing. These patients demonstrate that in augmented bladders a high index of suspicion may be necessary to diagnose ruptures clinically. Early diagnosis is critical so that aggressive therapy may be instituted.

Prepubertal Yolk Sac Testicular Tumors - Report of the Testicular Tumor Registry

In 1980 the Section on Urology of the American Academy of Pediatrics established a registry of prepubertal testicular tumors. A total of 181 yolk sac tumors has been reported to the registry. Complete followup is available for 154 of these 181 patients. Most of the patients presented with low stage disease. Radical orchiectomy without adjunctive retroperitoneal lymphadenectomy, chemotherapy or radiotherapy was sufficient therapy for most stage 1 cancer patients. alpha-Fetoprotein is a reliable marker for this tumor. Chest x-rays and abdominal computerized tomography are reliable for staging. When metastases appeared they did so within 14 months of presentation so that 2 years of followup after any evidence of disease seems to be adequate. Approximately two-thirds of the patients with metastases were salvaged by chemotherapy, radiotherapy and/or an operation.

Hypertension in infants—a complication of umbilical arterial catheterization

Hypertension was observed in ten infants. Seven had thrombosis either of one renal artery or of both. The seven infants had previously had an indwelling catheter in the umbilical artery. One infant who had not had an indwelling umbilical arterial catheter had stenosis of a renal artery. No etiology for the hypertension was found in two infants, one of whom had had an umbilical arterial catheter. Response to antihypertensive medication was generally poor. Five of the infants died. We recommend prompt diagnostic evaluation in hypertensive infants. Nephrectomy should be considered, if the renal artery is occluded. Hypertension appears to be a life-threatening complication of umbilical arterial catheterization.

The cystic renal lesion in tuberous sclerosis

A characteristic cystic renal lesion is described in three infants with tuberous sclerosis. Large cystic kidneys were present at birth in one child. The other two patients were discovered to have cystic kidneys and hypertension at 3 months of age. Development delay and seizures were ultimately present in two infants; typical cutaneous lesions of tuberous sclerosis developed in only one child. The roentgenographic appearance of the kidneys in the two older children was similar to that of adult-type polycystic kidney disease. Cysts were lined by a distinctive, hyperplastic epithelium in each patient. The diagnosis of tuberous sclerosis may be determined by renal biopsy in infants presenting with cystic kidneys.

Genitourinary trauma in the pediatric patient

Trauma is the major source of mortality in the pediatric population. A retrospective review was performed on patients admitted to the Childrens Hospital and Health Center Trauma Program, San Diego, California, from August 1984 to May 1990. The purpose of this review was to evaluate pediatric trauma and to determine the best treatment and evaluation for genitourinary injuries. Blunt trauma was responsible for 98 percent of the injuries, with renal injuries being the most common. Bladder (7) and male urethral (2) injuries, and vaginal lacerations (8) also occurred. The most severe renal injuries (70%) and all significant bladder and urethral injuries were associated with gross hematuria. Hypotension was present in 31 percent of patients but rarely required surgical exploration for correction. Eighty-six patients underwent radiographic imaging. Computerized tomography (CT) scans demonstrated the most information about intra-abdominal solid organ injuries but was inaccurate in detecting bladder or urethral injuries. Genitourinary injury is common in children but rarely requires surgical management. CT scan is the best study to determine extent of solid-organ injury but is inferior to cystourethrography to diagnose bladder or urethral injuries.