One-stage combined delayed bladder closure with Kelly radical soft-tissue mobilization in bladder exstrophy: preliminary results.

BACKGROUND: The radical soft-tissue mobilization (RSTM, or Kelly repair) is an anatomical reconstruction of bladder exstrophy generally performed as a second part of a two-step strategy, following successful neonatal bladder closure. OBJECTIVE: The objective of this study is to determine the feasibility of a combined procedure of delayed bladder closure and RSTM in one stage without pelvic osteotomy, in both primary and failed initial closure. DESIGN, SETTING, AND PARTICIPANTS: From 11/2015 to 01/2018, 27 bladder exstrophy patients underwent combined bladder closure with RSTM by the same surgical team at four cooperating tertiary referral centers for bladder exstrophy, including 20 primary repairs (delayed bladder closure, median age 3.0m [0.5-37m]) and seven secondary repairs after failed attempt at neonatal closure, median age 10m [8-33m]. INTERVENTION: RSTM included full mobilization of the bladder plate, urogenital diaphragm, and corpora cavernosa from the medial pelvic walls, followed by anatomical reconstruction with antireflux procedure, bladder closure, urethrocervicoplasty, muscle sphincter approximation, and penile/clitoral reconstruction. OUTCOME MEASUREMENTS: The main criteria were bladder dehiscence or prolapse. Secondary outcomes included bladder neck fistula or urethral fistula, urethral stenosis, and parietal hernia. Continence and voiding have not been addressed at this stage. RESULTS AND LIMITATIONS: All bladder exstrophy cases were successfully closed without osteotomy, with no case of bladder dehiscence after 12 m [3-30] follow-up. COMPLICATIONS: Urethral fistula or stenosis occurred in eight patients: 4/5 fistulae closed spontaneously in less than 3 months; four urethral stenoses were successfully treated with 1-3 sessions of endoscopic high-pressure balloon dilatation or meatoplasty; one patient with persistent bladder neck fistula is currently awaiting repair. Although the follow-up is short, it does allow examination of the main outcome criterion, namely bladder dehiscence, which is usually expected to happen very early after surgery. CONCLUSION: The Kelly RSTM can be safely combined with delayed bladder closure without osteotomy in both primary and redo cases in classic bladder exstrophy.

Renal outcome following heminephrectomy for duplex kidney.

PURPOSE: We evaluated functional outcome following heminephrectomy in duplex kidneys. MATERIALS AND METHODS: A total of 101 patients were identified who underwent upper or lower pole heminephrectomy between January 1995 and December 2000. We retrospectively reviewed 60 case notes with complete data. A total of 37 cases (62%) were diagnosed prenatally, while 20 (33%) presented as single or recurrent urinary tract infection and 3 (5%) as urinary incontinence. Of the patients 52 (87%) had unilateral duplex and 8 (13%) had bilateral duplex kidneys. In addition, 20 of the 60 patients (33%) had ureterocele. Mean age at surgery was 46 months (range 3 to 10 years). RESULTS: Mean function as assessed by nuclear renography of the duplex kidney before heminephrectomy was 39.9% (range 22% to 61%) and following heminephrectomy was 33.1% (13% to 60%). Mean decrease in function was 6.8%, with a decrease in function of less than 5% in 18 patients, 5% to 10% in 13 and more than 10% in 5. The functional values were increased by 1% to 5% in 9 patients and unchanged in 15 patients after heminephrectomy. CONCLUSIONS: Following heminephrectomy there was a significant decrease in 8% of patients and a small decrease in function in the remaining moiety in 51%.

Use of the silastic sheath in bladder neck reconstruction.

OBJECTIVE: The study compared two populations of patients undergoing bladder neck reconstruction using the silastic sheath in two major pediatric centers. The success with this technique was markedly different in the two centers. The purpose of the study was to determine factors that might explain the divergent results. PATIENTS AND METHODS: Fifteen patients treated in Indianapolis were compared with 94 patients treated in London with the silastic sheath technique of bladder neck reconstruction. Eighty-seven percent of the Indianapolis patients had myelomeningocele whereas 86% of the London group had exstrophy/epispadias. Median age of the Indianapolis patients was 11 years whereas it was 8.4 years in London. Seventy-three percent of patients in Indianapolis were female and 79% in London were male. Patients were followed for a minimum of eight years in Indianapolis and a mean of seven years in London. Similar surgical technique was employed in the two centers but, over time, the London approach included use of a non-reinforced silastic wrapped loosely around the bladder neck with the interposition of omentum. RESULTS: Both groups achieved continence rates exceeding 90%. Of the Indianapolis patients, two-thirds experienced erosion of the silastic at a mean of 48 months. With modifications in the London technique, the erosion rate of silastic was lowered from 100% to 7%. CONCLUSION: Direct, snug wrap of silastic without omentum around the Young-Dees tube as well as simultaneous bladder augmentation placed patients at increased risk for erosion. The silastic sheath technique may be less applicable to myelomeningocele patients. It seems most applicable to older male patients with exstrophy or epispadias undergoing Young-Dees bladder neck reconstruction who have the ability to void.

[The effect of enterocystoplasty in childhood on linear growth]. / Längenwachstum nach Harnblasenaugmentation mit Darmsegmenten.

OBJECTIVE: The assumption that enterocystoplasty in children has a detrimental effect on linear growth has been based almost exclusively upon a chance finding in a retrospective study 10 years ago. We re-evaluated the same research question in a larger cohort and with a longer follow-up. PATIENTS AND METHODS: Between 1982 and 1997, 242 children and adolescents underwent enterocystoplasty. Patients with conditions involving organ systems apart from the urinary tract, and those with myelomeningocele, malignant diseases, reduced glomerular filtration rate and incomplete notes were excluded. In the definitive study cohort (123; mean age at operation 8.6 years; mean age at investigation 16.8 years), enterocystoplasty had been undertaken using colon in 70, ileum in 37, a combination of both in 11, ileocaecal segments in three and stomach in two patients. RESULTS: In all, 1215 height and weight measurements had been recorded. The distribution of percentile positions before and after enterocystoplasty showed a normal configuration, with 83 % and 80 % of patients growing within two standard deviations of the 50th percentile. After surgery, 85 % either remained the same or reached a higher percentile. Nineteen (15.5 %) were in a lower position, with a similar tendency in the weight percentile. A clinically relevant growth disorder was recognized in four patients with a complete endocrinological evaluation; in none of these was enterocystoplasty thought to be a causal factor. CONCLUSIONS: It is very unlikely that loss of the preoperative percentile position on the growth curve in 15 % of children after enterocystoplasty is a consequence of that particular surgery. Rather, it is a non-specific phenomenon that has to be considered in any clinical population of the same size and age distribution after the same length of time.

Long-term gynecological outcome of patients with persistent cloaca.

PURPOSE: Persistent cloaca is a complex malformation that remains a difficult reconstructive challenge, and data on long-term outcome are scarce. Gynecological abnormalities are common with cloaca but may remain asymptomatic until puberty or adult life. We evaluate long-term gynecological sequelae in these patients with persistent cloaca. MATERIALS AND METHODS: The records and radiographs of postpubertal patients (mean age 16.8 years, range 10 to 32) treated for cloacal malformation at 1 institution from 1970 to 2001 were retrospectively reviewed. Outcome data at puberty were available in 41 patients. Of the patients 24 are currently older than 16 years and outcome data for sexual activity were available in 21 with 3 lost to followup. RESULTS: All 41 patients were evaluated at puberty, and 28 (68%) had uterine function, 13 (32%) were menstruating normally and 15 (36%) presented with hematometra/hematocolpos. All 15 girls with an obstructed uterus required surgery, which included hysterectomy in 2, partial hysterectomy with vaginoplasty in 3 and vaginoplasty in 9. There was 1 complex case of fistula. Etiology of the obstructed uterus was vaginal stenosis after reconstruction in 3 cases, stenosis of persistent urogenital sinus (no previous reconstruction) in 11 and cervical stenosis in 1. Ten patients experienced primary amenorrhoea, which was confirmed in 8 (20%) while 2 (5%) continue to be followed for possible cryptic obstruction. In 10 girls the diagnosis of absent/vestigial uterus was made at early laparotomy but this was erroneous in 6 in whom uterine function developed at puberty. Of the 21 older girls (age at review 17 to 32 years, mean 24) 12 are or have been sexually active and 6 have been examined by a gynecologist and have an adequate vagina but are not sexually active. To date 4 patients have required revision vaginal surgery in adulthood to facilitate intercourse (re-do vaginoplasty in 3, introitoplasty in 1). One woman has postponed vaginal reconstructive procedures and 2 others are currently being followed. There have been no pregnancies in this series to date. CONCLUSIONS: Patients born with persistent cloaca have a high incidence of gynecological problems at the onset of menses and in early adult life. Therefore, it is necessary to reassess these girls at early puberty by ultrasound/magnetic resonance imaging and vaginoscopy. Additional surgery may then be necessary to create a vagina for menstruation and sexual intercourse.

Skeletal growth and long-term bone turnover after enterocystoplasty in a chronic rat model.

OBJECTIVE: To investigate skeletal growth and bone metabolism in a chronic animal model of urinary diversion. MATERIALS AND METHODS: Young male Wistar rats (120) were allocated randomly to four groups undergoing: ileocystoplasty, ileocystoplasty and resection of the ileocaecal segment, colocystoplasty, and controls. All animals received antibiotics for 1 week after surgery; half of each group remained on oral antibiotics. Bone-related biochemistry was measured in serum and urine. Dual-energy X-ray absorptiometry and peripheral quantitative computed tomography (pQCT) were used to determine bone mass ex vivo. RESULTS: Most (90%) of the rats survived the study period (8 months); six rats died from bowel obstruction at the level of the entero-anastomosis and four had to be killed because of persistent severe diarrhoea. Vital intestinal mucosa was found in all augmented bladders. There were no differences in bone length and volume. Loss of bone mass was almost exclusively in rats with ileocystoplasty and resection of the ileocaecal segment (-37.5%, pQCT, P < 0.01). There was no hyperchloraemic metabolic acidosis or gross impairment of renal function. Hypomagnesaemia, hypocalcaemia and decreased insulin-like growth factor-binding protein 3 were the only significant findings on blood analysis. Deoxypyridinoline crosslinks in urine were higher in rats with an enterocystoplasty than in controls. CONCLUSIONS: Enterocystoplasty in rats neither impairs skeletal growth nor bone quantity, but leads to significant loss of bone mass when combined with resection of the ileocaecal segment. Rarefaction of the trabecular network is confined to the metabolically highly active cancellous compartment, most likely as a consequence of intestinal malabsorption.

Management of urinary extravasation in 18 boys with posterior urethral valves.

PURPOSE: Urinary extravasation with associated posterior urethral valves is uncommon and published articles are restricted to case reports. We propose a management algorithm on the basis of presentation of 18 boys with this condition. MATERIALS AND METHODS: In this retrospective review we analyze the clinical features, diagnostic radiological imaging (voiding cystourethrogram and (99m)technetium mercaptoacetyltriglycine scan) and measured glomerular filtration rate (GFR) for assessment of renal function in boys presenting with posterior urethral valves and urinary extravasation. Indications for surgical intervention and long-term outcome were evaluated. Results are presented as mean +/- SD. RESULTS: Patient age at presentation was 4 days (range 1 to 28). Salient features at presentation were abdominal distention in 55.5% of cases, flank mass in 55.5%, respiratory distress in 16.6% and septicemia in 22.2%. Patients were categorized into group 1 with ascites (6 boys) and group 2 without ascites (12). Followup was 70.8 +/- 5.6 months. In group 1 the 3 boys with ascites alone had a followup GFR of 29 versus 74 ml. per minute per 1.73 m.2 in 3 with urinoma plus ascites (p = 0.001) at 64 +/- 4.9 months. In group 2 the GFR in 3 boys with bilateral urinoma was 104.7 +/- 30.4 versus 54.3 +/- 29.4 ml. per minute per 1.73 m.2 in the other 9 with unilateral urinoma (p = 0.001) at 36 +/- 4.2 and 81.3 +/- 4.7 months, respectively. The ipsilateral kidney showed reduced function in 6 of 12 boys with unilateral urinoma from both groups. CONCLUSIONS: Renal function on the side of the urinoma is impaired, and the majority of patients have impaired global renal function. A management algorithm is proposed.

Enterocystoplasty in childhood: a second look at the effect on growth.

OBJECTIVE: To re-evaluate the assumption that enterocystoplasty in children has a detrimental effect on linear growth (which is almost exclusively based upon a chance finding in a retrospective study 10 years ago) in a larger cohort and with a longer follow-up. PATIENTS AND METHODS: The original 12 children who had impaired linear growth in a previous study 10 years earlier were re-measured. A larger cohort was identified from the 242 children and adolescents who had undergone enterocystoplasty between 1982 and 1997. Patients with conditions involving organ systems apart from the urinary tract, and those with myelomeningocele, malignant diseases, reduced glomerular filtration rate and incomplete notes were excluded. In the definitive study cohort (123; mean age at operation 8.6 years; mean age at investigation 16.8 years) enterocystoplasty had been undertaken using colon in 70, ileum in 37, a combination of both in 11, ileocaecal segments in three and stomach in two patients. RESULTS: Of the original 12 patients, six had regained or surpassed their preoperative position on their growth charts. In all patients with a known target centile range the final height was within their genetic growth potential. In the cohort of 123 patients, 1215 height and weight measurements had been recorded. The distribution of percentile positions before and after enterocystoplasty showed a normal configuration, with 83% and 80% of patients growing within two standard deviations of the 50th percentile. After surgery, 85% either remained on the same or reached a higher centile. Nineteen (15.5%) were in a lower position, with a similar tendency in the weight centile. A clinically relevant growth disorder was recognized in four patients with a complete endocrinological evaluation; in none of these was enterocystoplasty thought to be a causal factor. CONCLUSIONS: It is very unlikely that the loss of the preoperative percentile position on the growth curve in 15% of children after enterocystoplasty is a consequence of the surgery. Rather it is a non-specific phenomenon that has to be considered in any clinical population of the same size and age distribution after the same length of time.

Long-term urological outcome of patients presenting with persistent cloaca.

PURPOSE: Persistent cloaca is a complex malformation with variable presentation that remains a difficult reconstructive challenge. Previous reviews have concentrated on surgical technique, and data on long-term outcome are scarce. We evaluate long-term outcome and when possible correlate outcome with anatomy at presentation. MATERIALS AND METHODS: The records and radiographs of 64 patients 0.5 to 25 years old with persistent cloaca treated at 1 institution from 1975 to 2000 were retrospectively reviewed to determine the outcome for urinary and fecal continence. Outcome data were available in 61 girls as 3 died. Of these patients 11 were younger than 3 years, making evaluation of continence difficult, and so the remaining 50 patients constitute the study population. Mean patient age at review was 11.3 years (range 4 to 25). RESULTS: Of the 50 patients 10 (20%) are incontinent of urine (4 with urinary diversion) and 40 (80%) are socially continent, including 11 (22%) who void spontaneously, 6 (12%) who perform clean intermittent catheterization alone and 23 (46%) who underwent reconstructive surgery. Fecal continence was achieved in 30 (60%) of patients and 40% are fecal incontinent or have a stoma. CONCLUSIONS: The majority of patients born with a cloacal malformation can achieve social fecal and urinary control, although in 46% additional reconstructive surgery was required. The presence of a good bladder neck, short common channel, a normal sacrum and 2 normal kidneys indicates improved prognosis for urinary continence. Achieving normal voiding continence in a child with a cloacal malformation still represents an enormous surgical challenge.

Renal outcome in patients with cloaca.

PURPOSE: Cloaca is a complex malformation in which the rectum, vagina and urinary tract open into a single common channel. Functional results after reconstructive surgery have been documented but the renal outcome is less clearly understood. MATERIALS AND METHODS: The records of all patients with cloacal malformation treated at our institution from 1980 to 2000 were retrospectively reviewed to determine the renal outcome. All patients underwent serial ultrasound of the urinary tract, voiding cystography, nuclear renography, lumbosacral radiography, and measurement of serum creatinine and glomerular filtration rate when appropriate. RESULTS: We identified 64 patients 0.5 to 19 years old (mean age 11.2) at the time of the study. Of the 64 patients 53 (83%) were born with a structural abnormality of the urinary tract, including renal dysplasia in 17 (27%), ectopic kidney in 9 (14%), solitary kidney in 8 (13%), duplex kidneys in 6 (9%) and ureteropelvic junction obstruction in 3 (5%). Vesicoureteral reflux was noted in 34 cases (53%) and a sacral abnormality was diagnosed in 36 (57%). The glomerular filtration rate was measured in 38 patients (60%). Chronic renal failure developed in 32 patients (50%) with a glomerular filtration rate of less than 80 ml. per minute per 1.73 m.2, including 11 (17%) who progressed to end stage renal failure, 4 who (6%) required renal transplantation and 4 who died of chronic renal failure. Of the 32 children with a glomerular filtration rate of less than 80 ml. per minute per 1.73 m.2 44% had renal dysplasia, 25% had a solitary kidney, 44% had sacral anomalies, 72% had vesicoureteral reflux and 47% had secondary renal scarring. CONCLUSIONS: Renal impairment causes significant morbidity and mortality in patients with a cloacal malformation. These patients need a complete nephrourological assessment in the neonatal period due to the high incidence of urinary tract and sacral anomalies as well as careful post-reconstruction followup.

Functioning adrenocortical neoplasms in children.

OBJECTIVE: To describe the clinical features, treatment and outcome of functioning adrenocortical tumours in children, and to assess the value of tumour size as a marker for malignant potential. PATIENTS AND METHODS: Twenty-one children (mean age at presentation 4.9 years, range 0.6-11; 12 girls and nine boys) who presented with a functioning adrenal mass over a 29-year period (1972-2000) were assessed. Clinicopathological features and outcome information were obtained from a structured review of the case-notes. Before 1980 the diagnosis and staging was mainly obtained by intravenous pyelography and plain X-ray. Since 1980, ultrasonography, computed tomography and more recently magnetic resonance imaging were the methods of choice. RESULTS: The mean (range) duration of symptoms before diagnosis was 31 (2-108) months. The symptoms and signs at presentation comprised virilization (18 children), Cushingoid features (three), precocious puberty (three) and hypertension (four). Three children had malignant neoplasms, one presented with metastasis and of the other two, one died from the disease within 6 months, despite adjuvant chemotherapy and radiotherapy. The third girl is disease-free 11 years after complete excision of the neoplasm. Eighteen children had a benign neoplasm and all are alive and free of recurrence. In most of the children surgical extirpation was relatively simple, blood loss minimal, recovery uneventful and the hospital stay short (mean 5 days). The virilizing effects of all the neoplasms gradually resolved after surgery within a median (range) of 27 (6-108) months. CONCLUSIONS: Functioning adrenocortical neoplasms in children are rare, with a peak incidence in the first decade, are predominantly unilateral and more common in girls. Most are benign, but both benign and malignant lesions can present with virilizing and Cushingoid features. The malignant neoplasms have an extremely poor prognosis, especially if excision is incomplete, despite adjuvant chemotherapy and radiotherapy. Although there is an association between tumour size and malignancy, this cannot be used as a reliable individual discriminator.

Upper pole heminephrectomy: is complete ureterectomy necessary?

OBJECTIVE: To determine the re-operation rate on the distal ureter after upper pole heminephrectomy with incomplete ureterectomy. PATIENTS AND METHODS: The case notes from one institution were reviewed retrospectively; 60 upper pole heminephrectomies with incomplete ureterectomy were undertaken in 39 girls and 16 boys (mean age at primary surgery 27 months, range 3--88). RESULTS: Thirty-two children (58%) had an antenatal diagnosis while 12 (22%) presented with a urinary tract infection (UTI) and six (11%) with urinary incontinence. Twenty-nine of the 60 renal units (48%) had an associated ureterocele and in nine (15%) the ureter was ectopic. Ten infants (18%) underwent initial puncture of a ureterocele. Five patients (8%), all females, required lower urinary tract re-operation. The indications for secondary surgery were recurrent UTIs in all and a prolapsed ureterocele in one. All five had ultrasonographic evidence of a dilated ureteric stump. Reflux into the retained stump was detected in one child. CONCLUSIONS: The re-operation rate for a redundant ureteric stump in this series was 8%. The risk of injury to the good ureter may outweigh the benefits of a complete ureterectomy.

The Yang-Monti ileovesicostomy: a problematic channel?

OBJECTIVE: To compare the differences in the quality of Mitrofanoff channels created using appendix and re-tubularized small bowel (the Yang-Monti ileovesicostomy). Patients and methods The case-notes were reviewed retrospectively for all patients who underwent a Mitrofanoff procedure using either appendix or small bowel, over a 5-year period from June 1994 to July 1999. RESULTS: In all, 92 patients underwent 94 Mitrofanoff procedures; the appendix was used in 69 and small bowel in 25. The underlying diagnoses were exstrophy-epispadias complex (38), neuropathic bladder (21), anorectal malformations and cloacal anomalies (15), posterior urethral valves (nine) and miscellaneous (nine). The mean (range) age at operation was 9.2 (1.1-18.3) years. The mean (range) follow-up for the appendix group was 37 (6.7-65) months and for the Monti group 25 (6-66) months. Catheterization problems occurred in 18 (27%) patients from the appendix group; two needed an adjustment of technique, six dilatation and 10 revision. Stomal stenosis occurred in 10 (15%) patients, bladder level stenosis in four (6%) and conduit necrosis in two. Catheterization problems were reported in 15 (60%) patients from the Monti group; five needed revision, three dilatation and seven are being managed conservatively. The incidences of stomal stenosis (four, 16%) and bladder level stenosis (two, 8%) were comparable with the appendix group. In addition, two patients had distal channel (sub-stomal) stenosis and two had mid-channel stenosis. The problem unique to the Yang-Monti channel was a pouch-like dilatation in seven patients (28%), all of whom presented with catheterization problems; five are being managed conservatively and two have needed pouch resection. Stomal prolapse occurred in five (7%) patients in the appendix group, but in none of the Monti group. CONCLUSIONS: The appendix is the conduit of choice for a Mitrofanoff procedure. Re-tubularized small bowel conduits have a considerably higher incidence of catheterization problems. Anatomical factors may contribute to the unique incidence of pouch formation.

Medical versus surgical treatment in children with severe bilateral vesicoureteric reflux and bilateral nephropathy: a randomised trial.

BACKGROUND: Nephropathy associated with vesicoureteric reflux (VUR) and urinary tract infection can result in end-stage renal failure, hypertension, or both. Whether long-term VUR contributes to these outcomes is unknown. We compared, in a randomised trial, medical with surgical management of children with bilateral severe VUR and bilateral nephropathy. METHODS: We stratified by age and glomerular filtration rate (GFR) 25 boys and 27 girls aged 1-12 years and randomly assigned them to medical or surgical management. At enrolment and 4 years' follow-up we estimated GFR from the plasma clearance of 51Cr-labelled edetic acid (EDTA), and did intravenous urography. We also did a metastable 99mTc-labelled dimercaptosuccinic acid (DMSA) assay and contrast cystography. The change in GFR at 4 years, expressed as a percentage change between enrolment and 4 years, was available for 26 of 27 patients in the medical and 24 of 25 in the surgical group. We assessed GFR in 48 patients 10 years after enrolment. FINDINGS: Mean GFR at enrolment was 72.4 mL/min per 1.73 m(2) (SD 24.1) in the medical and 71.7 mL/min per 1.73 m(2) (22.6) in the surgical group. The mean percentage change in GFR at 4 years was 2.4% (SE 4.5) versus 4.7% (5.0) in the medical and surgical groups, respectively. The difference in change in GFR at 4 years between the two groups was not significant (7.1%, 95% CI 6.4% to 20.6%). INTERPRETATION: Our data do not lend support to the view that the outcome for renal function is improved by surgical correction of VUR in children with bilateral disease.

The relationship between early renal status, and the resolution of vesico-ureteric reflux and bladder function at 16 months.

OBJECTIVE: To examine, in infants presenting with vesico-ureteric reflux (VUR), the relationship between the presence of initial renal abnormalities with the outcome of VUR and bladder function at 16 months of age. PATIENTS AND METHODS: The study group comprised 40 infants (32 boys) presenting consecutively (29 after prenatal detection) with VUR grade III or greater (bilateral in 29) on the initial micturating cystogram (median age 8 weeks). The initial presence of abnormal kidneys was determined from isotopic renography and/or ultrasonography. These data were correlated with the outcome of VUR, from direct isotope cystography, and bladder function assessed by natural filling urodynamics, examined at age 16 months (mean 16.4 months, SD 2.1). RESULTS: Three groups were identified. Group 1 (eight boys and six girls) had normal kidneys bilaterally; initially grade III VUR was common. At 16 months bladder function was normal in 10 children and none had VUR (complete resolution). Group 2 (14 boys and two girls) had unilateral renal abnormalities; initially VUR was predominantly grade IV or grade V. At 16 months bladder function was normal in eight children and VUR resolved in eight, five of these with normal bladder function. Group 3 (10 boys) had bilateral renal abnormalities. Initially grade V VUR predominated; at 16 months the bladder function was normal in only one, and in the rest the emptying dynamics were abnormal. All 10 boys had persisting VUR (no resolution). CONCLUSIONS: In infants with moderate or severe VUR, resolution at 16 months old is associated with normal kidneys in a similar proportion of boys and girls. Resolution also correlates well with normal bladder function. Presentation in infancy with bilateral abnormal kidneys, associated with severe VUR in boys, is a poor prognostic sign for the early outcome of VUR and for bladder function.

Long-term renal function in the posterior urethral valves, unilateral reflux and renal dysplasia syndrome.

PURPOSE: The syndrome of posterior urethral valves, persistent unilateral reflux and renal dysplasia (VURD) is said to be protective of the contralateral nonrefluxing kidney and the outcome for renal function is reported to be excellent. We tested this hypothesis in our patients by replicating previous studies but with longer followup and glomerular filtration rate data. MATERIALS AND METHODS: We retrospectively reviewed the records of 183 boys presenting with posterior urethral valves between 1980 and 1989, including 12 who underwent nephrectomy for ipsilateral nonfunction and fulfilled all criteria for the VURD syndrome. Mean age at the most recent followup was 8.5 years. Serial serum creatinine levels and glomerular filtration rates were analyzed and compared to age matched normal values. RESULTS: Histological evaluation revealed dysplasia in all kidneys, confirming the VURD syndrome. Followup plasma creatinine was normal 67% of the patients during year 2 of life, 50% between ages 4 and 5 years, and only 30% between ages 8 and 10 years. Glomerular filtration rate was within the normal range in 25% of boys tested in year 2 of life, and between ages 5 and 8 years. CONCLUSIONS: Our data do not support the protective effect of the VURD syndrome on long-term renal function. All patients with posterior urethral valves require diligent long-term followup.

Bladder hemangioma as a cause of massive hematuria in a child. A case report and literature review.

A 12-year-old girl with bladder hemangioma presenting with massive hematuria is described. The patient was previously operated for removal of cystic hygroma from the chest wall. The association of those two pathological entities suggests possible common etiology and pathogenesis and the various therapeutic options are reviewed.