Andrew L. Freedman

Long-term outcome in children after antenatal intervention for obstructive uropathies.

BACKGROUND Antenatal intervention has been done for fetal obstructive uropathy for over a decade, yet little is known about long-term outcomes. To assess the long-term implications of fetal intervention, we reviewed the outcomes of children who underwent vesicoamniotic shunt placement. METHODS We reviewed the clinical outcomes of 14 children who underwent vesicoamniotic shunt placement at our institution and who survived beyond 2 years of age. FINDINGS In 1987-96, 34 patients underwent vesicoamniotic shunt placement. 13 died and 21 survived, of whom 17 are now more than 2 years old. Three survivors were lost to follow-up. Mean age at follow-up was 54.3 months (range 25-114). Final diagnoses included prune belly syndrome (seven cases), posterior urethral valves (four), urethral atresia (one), vesicoureteral reflux (one), and megacystis (one). Height was below the 25th percentile in 12 (86%) with seven (50%) below the 5th percentile. Five (36%) had renal failure and had successful transplantation, three (21%) have renal insufficiency, and six (43%) have normal renal function. Seven (50%) are acceptably continent, five (36%) have not yet begun toilet-training, and two (14%) are incontinent. Three of four children with valves needed bladder augmentation. INTERPRETATION Antenatal intervention may help those fetuses with the most severe forms of obstructive uropathy, usually associated with a fatal neonatal course. Intervention achieves outcomes similar to less severe cases that are usually diagnosed postnatally.

Fetal Therapy for Obstructive Uropathy: Specific Outcomes Diagnosis

AbstractPurpose: Attempts to evaluate prenatal vesico-amniotic shunt therapy have been hampered by inconsistencies in patient selection, treatment and termination criteria, and outcomes measurement. Outcomes have generally been measured against those of patients with postnatally detected posterior urethral valves. The purpose of this report was to evaluate the influence of the underlying diagnosis on the clinical outcomes of fetuses undergoing evaluation for prenatal intervention for suspected obstructive uropathy. Furthermore, specific outcomes diagnosis was compared to the published natural history of these disorders to begin to establish a basis for measuring the efficacy of prenatal intervention.Materials and Methods: We retrospectively reviewed the outcomes of 55 consecutive patients undergoing prenatal evaluation using structured outcome measures stratified by specific diagnoses to provide a comparison to the reported natural history for each underlying disorder.Results: All fetuses had early onset ...

MULTI-INSTITUTIONAL STUDY OF TESTICULAR, MICROLITHIASIS IN CHILDHOOD: A BENIGN OR PREMALIGNANT CONDITION?

PURPOSE Testicular microlithiasis, a rare ultrasonographic diagnosis in children, has been shown to coexist in benign and malignant conditions. The natural history of incidentally discovered testicular microlithiasis has not been well defined in the pediatric population. The concern that testicular microlithiasis may be a premalignant condition has been raised. Reports indicate as much as a 45% association of germ cell tumors with testicular microlithiasis at the time of tumor diagnosis and there have been 4 cases of interval testicular tumor development associated with preexisting testicular microlithiasis. To address this issue we performed a multi-institutional study to evaluate children with incidentally diagnosed testicular microlithiasis. MATERIALS AND METHODS Data on 26 patients with a mean age of 12.3 years at presentation with incidentally discovered testicular microlithiasis were collected from 7 institutions. Presenting scrotal conditions were reviewed. Two children with a previous testicular malignancy were excluded from study. RESULTS Followup ranged from 1 month to 7 years (mean 27.6 months). Testicular biopsy and tumor marker (alpha-fetoprotein and beta-human chorionic gonadotropin) determinations were performed in 9 and 15 patients, respectively. To date no testicular tumor has developed during clinical followup. CONCLUSIONS Our multi-institutional study has not yet shown a trend toward the malignant degeneration of incidentally diagnosed testicular microlithiasis in children. However, we still advocate close surveillance of patients with testicular microlithiasis, such as yearly testicular ultrasound, physical examination, and judicious tumor marker determinations. We propose that a registry be started to follow prospectively patients with testicular microlithiasis to define its significance better.

Fetal therapy for obstructive uropathy: past, present.future?

Abstract Antenatal treatment of obstructive uropathy, although widely performed, remains controversial. An overview of prenatal therapy for obstructive uropathy, the limitations of the early published experience, advances of recent years, and future directions for treatment are reviewed. The clinical approach and outcomes of the Fetal Treatment Program of Hutzel Hospital and Wayne State University are presented. Patient selection for antenatal treatment is based on the existence of a significant threat of neonatal death due to pulmonary hypoplasia, pending exclusion criteria such as anatomical structural anomalies and chromosomal defects. Ultrasonography, karyotyping, and sequential urinary electrolyte analysis are essential. Current treatment involves the placement under ultrasonic guidance of a Rodeck vesicoamniotic shunt. Recent technical advances include the use of amnioinfusion for fetal visualization, temporary fetal paralysis, routine antibiotics, and more-precise catheter placement. The establishment of standardized short- and long-term outcome measures and the documentation of fetal and maternal complications are in progress. Procedural refinement, development of fetoscopic techniques and equipment, identification of urinary markers to aid patient selection, and the collection of multicenter outcome data will assist the future management of prenatally detected obstructive uropathy.

Is There a Best Alternative to Treating the Obstructed Upper Pole

PURPOSE We addressed whether salvage of upper pole renal units in comparison to partial nephrectomy affects differential renal function in patients with a duplicated obstructed upper pole. MATERIALS AND METHODS We retrospectively reviewed the records of all children who underwent surgery for a unilateral obstructed duplicated kidney at our institution from 1988 to 1995. Patients were evaluated with respect to postoperative complications, reoperation rate and percent change in differential renal function of the obstructed duplicated kidney, as determined by nuclear renography. RESULTS We identified 46 patients with a unilateral obstructed upper pole of a duplicated kidney who were divided into 2 groups. Group 1 (12 patients) underwent an upper pole salvage procedure, that is ureteropyelostomy or ureteroureterostomy, and group 2 (31 patients) underwent partial nephrectomy. Three reoperations (25%) were performed in group 1 and 1 (4%) was done in group 2. Postoperative symptomatic urinary tract infections were diagnosed in 3 group 1 patients (25%) and in 2 (8%) in group 2. Average change in ipsilateral renal function in the 8 patients who underwent upper pole salvage procedures was 2.25 +/- 2.34% (range -6 to 12). In the 8 patients who underwent upper pole nephrectomy and who also had postoperative renal scans average change in function was -1.25 +/- 4.51% (range -23 to +16). CONCLUSIONS There was no statistically significant loss of relative renal function in patients treated with partial nephrectomy and no significant gain in relative renal function in those treated with an upper pole salvage procedure. The reoperation rate was higher in the upper pole salvage than in the partial nephrectomy group (25 versus 4%). While not statistically significant, we believe that this rate is clinically important. We think that partial nephrectomy should remain the preferred treatment for most patients with obstructed duplicated kidneys.

Renal Cell Carcinoma in Children: The Detroit Experience

PURPOSE We analyzed the presentation, treatment and survival of 6 children with renal cell carcinoma. MATERIALS AND METHODS We retrospectively reviewed the pathological and hospital records of 6 children diagnosed with renal cell carcinoma at Childrens Hospital of Michigan (5) and Henry Ford Hospital (1) from 1980 to 1995. RESULTS The most common presenting complaints were flank pain (50%) and a palpable abdominal mass (50%), while gross hematuria was present in only 1 patient (16%). No patient had the classic triad of flank pain, hematuria and palpable mass. Only 1 patient had localized disease (stage II), while 5 of the 6 presented with stage III or IV disease. While followup is limited, all patients with stage III disease are without evidence of recurrence at a mean 38.5 months and 1 of 2 with stage IV disease is without evidence of disease at 22 months. CONCLUSIONS Although renal cell carcinoma in childhood often presents at an advanced stage, the prognosis for those with isolated regional lymph node involvement appears to be encouraging.

Megalourethra and urethral diverticula

Megalourethra and urethral diverticula encompass a diverse group of congenital and acquired urethral defects. The appropriate management of these anomalies relies on a keen appreciation of phallic anatomy and an understanding of urethral embryology. A thorough history and physical examination--including a careful evaluation of the urinary tract--is necessary to identify associated congenital anomalies. Finally, satisfactory surgical management demands meticulous attention to surgical technique.

Smooth muscle development in the obstructed fetal bladder.

OBJECTIVES To evaluate changes in the smooth muscle and connective tissue development in the obstructed and normal fetal bladder. METHODS The smooth muscle and connective tissue composition of 19 fetal urinary bladders, including those of 9 fetuses with anatomic obstruction and 10 controls free of urologic disease, were analyzed by light microscopy and computer-assisted color image analysis. RESULTS The bladder wall thickness was markedly increased in obstructed fetuses throughout gestation as compared with that in controls. The disparity in bladder wall thickness increased rapidly during gestation. The percent area density of smooth muscle and connective tissue as well as the ratio of smooth muscle to connective tissue remained the same in the obstructed and normal control fetal bladders. CONCLUSIONS Although bladder outlet obstruction is associated with a marked increase in bladder wall thickness, the percent of smooth muscle and connective tissue comprising the mural histology remains relatively constant as compared with that of normal fetal controls. This study suggests that bladder outlet obstruction in the fetus is not associated with excess collagen deposition but rather with an increased amount of bladder with normal cellular content and a concomitant increase in smooth muscle development.

Long-Term Results of Penile Vein Ligation for Impotence from Venous Leakage

Between 1986 and 1991, 46 men with organic impotence documented by dynamic pharmacocavernosometry and cavernosography to have venous leakage underwent penile vein ligation. Despite initial improvement in erections allowing normal intercourse in 34 men (74%) within the first 6 months, long-term (more than 12 months) evaluation revealed sustained potency without adjunctive therapy in only 11 (24%). Of the remaining 35 men 6 (13%) progressed to a penile prosthesis, 8 (17%) required intracavernous vasoactive injection therapy and 21 (46%) have not sought further therapy despite continued impotence. Of the 14 patients who had isolated distal leakage 6 (43%) had sustained erectile function while only 5 of the 32 patients (16%) with proximal leakage maintained potency. Associated complications included penile shortening in 20 (43%) and penile hypoesthesia in 9 men (20%). Therefore, we conclude that the long-term success of penile vein ligation is poor, with only 24% of the patients able to have normal intercourse more than 1 year later, although those patients with distal penile shaft leakage appear to have a greater chance of success than those with more proximal leakage.

RESULTS OF THE RECTUS FASCIAL SLING AND WRAP PROCEDURES FOR THE TREATMENT OF NEUROGENIC SPHINCTERIC INCONTINENCE

PURPOSE We assessed the early results of the rectus fascial sling and modified rectus fascial wrap for treating neurogenic sphincteric incontinence in a pediatric population. MATERIALS AND METHODS We retrospectively reviewed the charts of all patients who underwent a rectus fascial sling or wrap procedure for neurogenic incontinence at our institution. Most recent status was confirmed by telephone interview as successful-complete day and night dryness, partially successful-occasional daytime wetting and/or nocturnal enuresis and failed-frequent daytime incontinence. Results were analyzed with regard to patient sex, associated bladder augmentation and previous or subsequent anti-incontinence surgery. RESULTS A total of 27 patients underwent 10 sling and 18 wrap procedures. Five patients in each group were dry after 1 to 4.5 years of followup. Six of the 7 boys were wet and 1 was partially dry postoperatively, while 10 of the 20 girls became dry (p = 0.026 Fishers exact test), resulting in an overall 36% success rate. In failed cases continence was achieved after periurethral collagen injection and artificial urinary sphincter placement in 0 of 5 and 5 of 6, respectively. CONCLUSIONS Total continence was achieved even after a brief followup in a minority of patients in whom rectus fascia was used to correct neurogenic incontinence. However, our early data suggest that cases in which these procedures fail may be salvaged by artificial urinary sphincter implantation but not by periurethral collagen injection.