Robotic-assisted laparoscopic pyeloplasty (RALP), for ureteropelvic junction obstruction (UPJO), is an alternative to open pyeloplasty in the pediatric population.

We aimed to evaluate the safety and efficacy of RALP for UPJO in a heterogeneous pediatric population. The medical records of all patients with UPJO who underwent RALP over the last 6 years and completed at least 6 months of follow-up, were retrospectively reviewed. Data included age, sex, laterality, weight, preoperative and postoperative ultrasound and renal scintigraphy results, operation time, complications, length of hospital stay. We separately examined two groups: low-weight children (< 10 kg) and those who underwent RALP after failed pyeloplasty. One hundred patients with a median age of 18 months (range 2-216) underwent RALP. The median weight was 10 kg (range 4-90). The median operative time, including docking and console time, was 75 min (range 40-183). The median hospital stay was one day (range: 1-3). Ninety-eight percent of the patients showed improvement or stable hydronephrosis on postoperative imaging, with a better drainage curve on dynamic radionuclide scans. In two patients, the hydronephrosis worsened. One patient's ipsilateral UVJ was obstructed, and the other patient's UPJO recurred. The operative time was shorter in the low-weight group (p < 0.001), but the length of hospital stay and success rate were not different. Neither the hospital stay nor the success rate of redo RALP patients differed significantly from a control group of primary RALP patients. Our data show that RALP might be utilized as a universal approach in pediatric patients with UPJO.

What are the predictive factors leading to ureteral obstruction following endoscopic correction of VUR in the pediatric population?

BACKGROUND: It is extremely important to not only address the short-term success following endoscopic correction of vesicoureteral reflux (VUR) but also the long-term efficacy and safety of the tissue augmenting substance utilized for endoscopic correction. OBJECTIVE: This study retrospectively evaluated all cases of ureterovesical junction (UVJ) obstruction following endoscopic treatment of VUR over the last 5 years utilizing two tissue augmenting substances, with special emphasis on the safety of Vantris®, and performed clinical and histological review of these patients. METHODS: The study population comprised 2495 patients who underwent endoscopic correction of VUR utilizing Deflux® (1790) and Vantris® (705). Tissue sections were stained with hematoxylin & eosin and trichrome, and examined under a light microscope. Nine primary obstructive megaureters after ureteral re-implantation served as controls. RESULTS: Nine (0.5%) children (three female and six male) in the Deflux group and nine (1.3%) (five females and four males) in the Vantris group developed UVJ obstruction and required ureteral re-implantation. Obstruction developed during the period ranging 2-49 months (average 16 months) following endoscopic correction. The primary reflux grade was III in seven, IV in six, and V in six children. The mean volume of the injected material in all obstructed patients was 1.2 ± 0.6 cc (mean ± SD). Histopathological analysis revealed a pseudocapsule composed of fibrous tissue and foreign-body giant cells surrounding the Vantris implant in all patients. The distal part of the ureters demonstrated significant ureteral dilatation without ureteral fibrosis. In all patients, additional biopsies from the muscularis propria adjacent to the injection site were examined and showed no significant abnormalities. There was an increased collagen deposition in the juxtavesical segment of the obstructive ureters following Deflux and Vantris injections, and of primary obstructive megaureter. No significant difference was found in the tissue response between Deflux and Vantris patients and controls. Statistical analysis of the nonhomogeneous population demonstrated higher obstruction rates in patients from the Vantris group. However, no statistical difference was demonstrated regarding the obstruction rate in the homogenous group with relation to gender, age and reflux grade group of patients. Moreover, univariate analysis revealed that Grade V reflux, the presence of beak sign on the reviewed pretreatment, and inflamed bladder mucosa upon injection were significant independent risk factors leading to obstruction. DISCUSSION: This study suggested that the underlining ureteral pathology lead to UVJ obstruction following Vantris injection. There was increased collagen deposition in the juxtavesical segment of the obstructive ureters following Vantris injection. Furthermore, these findings were similar to those discovered in patients who underwent endoscopic correction with Deflux, and in patients who required ureteral reimplantation due to primary obstructive megaureter. Additional biopsies from the muscularis propria adjacent to the injection site showed no significant abnormalities, ironing out the fact that Vantris did not led to adverse tissue reaction following injection. Univariate analysis further ironed out the hypothesis that underlying ureteral pathology was responsible for the increased incidence of UVJ obstruction and demonstrated that Grade V reflux, the presence of beak sign on the reviewed pretreatment VCUG, and inflamed bladder mucosa upon injection were significant independent risk factors leading to obstruction. CONCLUSION: Data showed that Vantris injection did not lead to any different ureteral fibrosis or inflammatory changes to the tissue augmenting substances utilized in past and present clinical practice, and therefore did not seem to increase the incidence of UVJ obstruction. High reflux grade, presence of obstructive/refluxing megaureter and inflamed bladder mucosa were the only statistically significant and independent predictive factors for UVJ obstruction following endoscopic correction of VUR.

Partial androgen suppression consequent to increased secretion of adrenal androgens in a patient with prostate cancer treated with long-acting GnRH agonists.

We present a case report of a patient with prostate cancer who failed to demonstrate consistent testosterone suppression to castration levels and incomplete suppression of serum prostate-specific antigen, although treated with gonadotropin releasing hormone agonists for 48 months. Serum dehydroepiandrosterone, dehydroepiandrosterone sulphate, as well as the androgen metabolite, androsterone glucuronide, were elevated compared to the other patients. The present data suggest that those prostate cancer patients who have even marginally elevated adrenal androgens may especially benefit from combined androgen blockade.

Testicular prosthesis insertion following testicular loss or atrophy during early childhood--technical aspects and evaluation of patient satisfaction.

INTRODUCTION: The absence of a testis may be a major traumatic experience with possible consequent negative behavioral impact. The current study is aimed to assess young adults who had lost their testicles early in childhood and subsequently underwent testicular prosthesis (TP) implantation at the end of their adolescence. MATERIALS AND METHODS: Nineteen patients aged 19.4 years on average underwent TP implantation following testicular loss at early childhood. A postal anonymous questionnaire was sent to the patients and 13 (68%) replied. RESULTS: Average follow-up was 50 months. Sixty-two percent of patients believed TP size were equal or close to the size of their native testicle, while 23% claimed it was too small. Similarly, 54% had the impression its weight was appropriate, while 38% claimed it was too heavy and 38% supposed its location was too high in the scrotum. However, 85% considered it "very comfortable" or "comfortable" and 77% got used to its presence within 1-4 weeks following surgery. All patients precluded any obstacle in intimate relationships. In accordance, all of them would recommend the operation to a friend with a similar problem. CONCLUSIONS: TP implantation in adolescents with an undeveloped scrotum is a highly successful surgical procedure. The operation positively influences self-confidence, inter-personal interactions and intimate relationships.

Masculinizing genitoplasty in intersex patients.

PURPOSE: We reviewed cosmetic and functional outcome of masculinizing genitoplasty (MPG) in intersex patients assigned as a male performed in our service for more than 12 years. MATERIALS AND METHODS: A total of 57 patients underwent MPG in our department during a 20-year period. Of these cases MPG was performed using modern 1-stage surgical techniques from 1991 to 2003 in 39 (68%). Of the 39 children 8 (21%) had 17beta-hydroxysteroid dehydrogenase deficiency, 2 (5%) had 3beta-hydroxysteroid dehydrogenase deficiency, 8 (21%) had 5alpha-reductase deficiency, 2 (5%) had mixed gonadal dysgenesis, 1 (2.6%) was a true hermaphrodite, 1 (2.6%) had Klinefelter's syndrome, 1 (2.6%) had partially androgen insensitivity syndrome and 16 (41%) had idiopathic male pseudohermaphroditism. The presenting disease was severe proximal penoscrotal hypospadias in 30 cases (77%), perineal hypospadias in 9 (23%) and müllerian duct opening in the perineum along with the urethral meatus in 16 (28%). Median patient age at surgery was 1.8 years. MPG was performed with a transverse pedicled preputial island flap as an onlay in 29 cases (74%). The remaining 10 patients (26%) underwent tubularization of the mucosa in the perineal area and end-to-end anastomosis to a tube made from the pedicled prepuce. Scrotal transposition as well as orchiopexy was performed in some patients as an independent operation so as not to jeopardize the perineal and preputial flaps. RESULTS: In 23 (59%) of the 39 children 1 operation achieved satisfactory cosmetic and functional results in terms of good urinary stream and straight phallus during erection. Three (7%) children presented with various degrees of breakdown of the urethroplasty and required a repeat operation. In 5 patients (12.8%) a small urethral fistula developed and closure was performed. CONCLUSIONS: A 1-stage male genitoplasty for male pseudohermaphroditism is accompanied by a reasonable incidence of major complications. It should be performed in early childhood to avoid psychological and social anxiety by the child and parents.

Does prenatal diagnosis influence the morbidity associated with left in situ nonfunctioning or poorly functioning renal moiety after endoscopic puncture of ureterocele?

PURPOSE: We compared long-term morbidity associated with left in situ nonfunctioning or poorly functioning renal moiety of a duplex system in children with prenatal vs postnatal diagnosis of ureterocele who underwent endoscopic puncture. MATERIALS AND METHODS: A total of 48 children underwent primary endoscopic puncture of duplex system ureterocele. Of the cases 35 (73%) were diagnosed prenatally (group 1) and 13 (27%) postnatally (group 2). Median age at time of puncture was 4 months in group 1 and 3.5 years in group 2. A total of 20 patients in group 1 (57%) and 8 in group 2 (62%) presented with intravesical ureterocele, while 15 in group 1 (43%) and 5 in group 2 (38%) had ectopic ureterocele. A total of 20 children in group 1 (57%) and 7 in group 2 (54%) had a nonfunctioning renal moiety, and 15 in group 1 (43%) and 6 in group 2 (46%) had a poorly functioning ureterocele moiety. Vesicoureteral reflux (VUR) was present in 23 children in group 1 (66%) comprising 30 renal refluxing units (RRUs), and in 12 in group 2 (92%) comprising 14 RRUs. Median followup was 9 years (range 1 to 15) for both groups. RESULTS: Preoperative urinary tract infection (UTI) was common in group 2 (92%) vs group 1 (20%). No patient in group 1 had development of UTI after puncture, while 23% of the children in group 2 presented with UTI. Four children (2 from each group) with ectopic ureterocele required secondary puncture resulting in satisfactory drainage. A total of 14 RRUs (47%) showed spontaneous resolution of VUR in group 1 compared to 3 (21%) in group 2. Four RRUs (13%) required endoscopic correction due to high grade VUR in group 1. Two RRUs (17%) were treated with endoscopic correction and 2 (17%) with ureteral reimplantation due to UTI in group 2. Only 1 patient in group 1 underwent nephrectomy due to nonfunctioning kidney, while 2 patients in group 2 required partial nephrectomy due to UTI. CONCLUSIONS: Our data reveal that prenatal diagnosis of duplex system ureterocele is associated with fewer UTIs, and early endoscopic management may decrease UTI and the need for additional surgery. Nonfunctioning or poorly functioning renal moieties left in situ following successful endoscopic decompression of ureterocele are not associated with additional morbidity and do not require partial nephrectomy in the majority of the cases.

Dorsal tunica albuginea plication to correct congenital and acquired penile curvature: a long-term follow-up.

OBJECTIVE: To evaluate the long-term efficacy of a tunica albuginea dorsal plication technique for treating congenital and acquired penile curvature. PATIENTS AND METHODS: We retrospectively evaluated 83 patients (median age 1.8 years) who had their penile curvature corrected surgically using dorsal tunica albuginea plication between 1992 and 2002. The results were evaluated objectively using a pharmacological erection test or subsequently based either on the parents' reports or patients' self-assessment. The median (range) follow-up was 6 (0.7-10) years. RESULTS: Seventy (84%) patients had penile plication as an integral part of hypospadias repair, while the remaining 13 (16%) with a normal urethra had dorsal plication only. Twenty-eight (34%) of the 83 patients had an erection test during a repeat hypospadias repair or closure of a urethrocutaneous fistula; 22 of these had a straight penis, while the remaining six required additional plication for a satisfactory cosmetic outcome. Parents of 45 (54%) children reported that their child had a normal erection with no chordee during the follow-up. Ten (12%) adult patients reported straight erections enabling satisfactory penetration and sensation during sexual intercourse. None of the patients reported penile shortening or erectile dysfunction after surgery, and none had recurrent curvature during the follow-up. There was no difference in the results between patients with congenital or acquired penile curvature. CONCLUSIONS: Dorsal plication of the tunica albuginea is a simple and effective method in the long term for correcting congenital and acquired penile curvature.

Masculinizing genitoplasty in male pseudohermaphroditism.

In the last decades there is a shifting paradigm in the understanding of intersex conditions with regards to the gender assignment and the choice of the surgical technique for genitoplasty. The cosmetic and functional results of masculinizing genitoplasty are rarely assessed in the literature. We reviewed the medical literature regarding primary diagnosis, gender assignment, preoperative evaluation, goals of surgical correction, timing of surgery and surgical technique. We critically reviewed our technique of masculinizing genitoplasty in 55 patients. In conclusion, we believe that this masculinizing genitoplasty is a feasible procedure in most of the patients with ambiguous genitalia raised as males with satisfactory functional and cosmetic results.

Childhood femoral hernia: a commonly misdiagnosed condition.

Femoral hernia is a rare surgical entity in the paediatric age group. The diagnosis is still a challenging issue. The aim of our study was to review our experience with femoral hernias in children during the past 21 years. We reviewed retrospectively the medical records of all patients who underwent femoral hernia repair in our institution between 1980 and 2000. Thirty-eight children (20 females, 18 males) with the median age of 5.5 years underwent 42 femoral hernia repairs over a 21-year period. Correct preoperative diagnosis was obtained in 20 (53%) cases. Of the 18 (47%) misdiagnosed cases, 11 were found to have a femoral hernia at negative exploration for a clinically diagnosis of inguinal hernia and 7 were found to have a femoral hernia 1 week to 18 months (mean 2.5 months) post repair of a presumed inguinal hernia. Median follow-up time was 12 years (range, 6 months-22 years). Recurrent femoral hernias occurred in five (13%) patients 6 months to 3 years (mean 13.5 months) after initial hernia repair. This study shows that femoral hernia is still a commonly misdiagnosed condition. A correct preoperative diagnosis will lead to appropriate surgical management, thus avoiding unnecessary morbidity and preventing unnecessary reoperations.

Alterations in cholinergic and neuropeptide innervation of urinary bladder following partial bladder outlet obstruction.

Posterior urethral valves (PUV) are the most common cause of bladder outlet obstruction (BOO) in infancy. Bladder instability, poor compliance and myogenic failure are responsible for the poor long-term prognosis in these patients. Previous studies have reported abundance of sensory neuropeptides, e.g. substance P (SP), calcitonin gene-related peptide (CGRP), vasoactive intestinal polypeptide (VIP) and acetylcholinesterase (AchE) nerves in the urinary bladder. We hypothesized that the functional changes in the bladder following BOO are due to alteration in cholinergic and sensory neuropeptide innervation. We therefore investigated cholinergic and sensory innervation of urinary bladder following BOO. Fifteen immature male guinea pigs (Hartley strain) 3-4 weeks old and weighing approximately 250 g. underwent placement of a silk ligature around the bladder neck to induce BOO. Controls included 5 sham-operated animals. The animals were killed 1, 2 and 4 weeks following obstruction, respectively. Whole-mount preparation and conventional sections of bladder wall were performed. AchE histochemistry, and single-label immunofluorescence histochemistry for SP, CGRP and VIP were utilized. Light microscopy and laser scanning confocal microscopy were used to assess the results. AchE staining showed marked increase in cholinergic innervation density within the suburothelial region following BOO. The staining for SP, CGRP and VIP demonstrated marked reduction in sensory nerve density within the suburothelial region 1 week following BOO and the lack of sensory innervation 4 weeks after BOO. The marked reduction in sensory innervation of the bladder and simultaneous increase in cholinergic innervation following BOO may lead to bladder instability and decrease in bladder compliance.

Effects of benzalkonium chloride treatment on the intramural innervation of the upper urinary tract.

OBJECTIVE: To establish a model for investigating the pathophysiology of pelvi-ureteric junction (PUJ) obstruction, using benzalkonium chloride (BCI) treatment of the upper urinary tract of rabbits, and thus further elucidate the pathophysiology of PUJ obstruction, the most common urinary tract obstruction in children. MATERIALS AND METHODS: Although various histological abnormalities have been described, PUJ obstruction may be functional. Defective innervation in PUJ has been suggested to be a major factor in the failure to transmit peristaltic waves across the PUJ. Previously established animal models of hydronephrosis deal mostly with surgical obstruction of the PUJ, which does not correlate with human congenital hydronephrosis. BCl has been used to ablate selectively neurones of the gastrointestinal myenteric plexus, which generated spastic segments with impaired peristalsis. Thus 12 rabbits were treated with BCl at the PUJ; the right upper urinary tract was dissected extraperitoneally and treated with a local application of 0.1% or 0.5% BCl (six each) for 15 min. The controls were four sham-operated animals treated with saline. The animals were assessed by intravenous urography (IVU) at 4 and 8 weeks after treatment, after which the animals were killed, the upper urinary tracts removed and whole-mounts prepared. Acetylcholinesterase (AChE) histochemistry, and neurofilament and tyrosine hydroxylase (TH) single-enzyme immunohistochemistry were used to detect the intrinsic innervation. RESULTS: None of the animals had hydronephrosis on the IVU or at death. AChE histochemistry, TH and neurofilament immunohistochemistry showed no or very few nerve fibres within the BCl-treated PUJs in both (0.1% and 0.5%) groups. After saline treatment there was normal development of the neuronal plexus within the submucosal, muscular and adventitial layers of the upper urinary tract. CONCLUSION: These results suggest that treatment with BCl is useful for ablating the intrinsic innervation in the upper urinary tract. Defective intrinsic innervation of the upper urinary tract did not lead to clinically or radiologically evident hydronephrosis. Further physiological studies using this model are needed to further elucidate the neuronal and myogenic influence on the development of PUJ obstruction.

Alterations in smooth muscle contractile and cytoskeleton proteins and interstitial cells of Cajal in megacystis microcolon intestinal hypoperistalsis syndrome.

BACKGROUND/PURPOSE: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is characterized by decreased or absent peristalsis. Gastrointestinal motility depends on the enteric nervous system, smooth muscle cells (SMCs), and the interstitial cells of Cajal (ICCs). Contractile and cytoskeleton proteinase are important structural and functional components of SMCs. The aim of study was to examine the expression of contractile and cytoskeleton proteins in SMCs and distribution of ICCs in MMIHS bowel. METHODS: Full-thickness bowel specimens were obtained from 4 infants with MMIHS and 4 controls. Specimens were processed as whole-mount preparations and frozen and paraffin sections. Combined staining of NADPH-d histochemistry/c-kit immunohistochemistry, single and double immunohistochemistry using alpha-smooth muscle actin (alpha-SMA), calponin (CALP), caldesmon (CALD), desmin (DES), protein gene product 9.5 (PGP 9.5) and c-kit antibodies were performed and examined using light and confocal scanning microscopy. RESULTS: alpha-SMA, CALP, CALD, and DES immunoreactivity were reduced markedly in MMIHS bowel compared with controls. Combined NADPH/c-kit staining showed dense network of ICCs around myenteric plexus in MMIHS bowel. In contrast, the intramuscular ICCs either were absent or reduced in MMIHS bowel. CONCLUSIONS: Marked reduction of contractile and cytoskeleton proteins in SMCs combined with reduced expression of intramuscular ICCs in the gut may be responsible for the motility dysfunction in MMIHS.

Renal parenchymal damage in sibling vesicoureteric reflux.

AIM: To compare the incidence of renal damage in siblings of patients with vesicoureteric reflux (VUR) who presented with a documented history of urinary tract infection (UTI) with asymptomatic siblings who were diagnosed with reflux during a screening programme for hereditary VUR. METHODS: Medical and radiological records of the VUR patients (1990-2000) were examined for age, gender, mode of presentation, reflux grade and renal damage. RESULTS: VUR was noted in 226 siblings (352 ureters) in 107 families. Of the 119 siblings of index patients, 64 were investigated for a documented UTI and 55 with no history of UTI were detected during screening for sibling reflux. Dimercaptosuccinic acid scan revealed reflux nephropathy in 25 (26%) of the 97 renal refluxing units (RRU) of siblings who presented with a UTI and in 6 (7%) of the 89 RRU of asymptomatic siblings who underwent screening voiding cystourethrography (p=0.0006). Mild renal damage was present in 20 (21%) RRU of siblings with UTI and in 2 (2%) RRU of the screened siblings (p < 0.001). Moderate to severe renal damage was present in 5 (5%) RRU of siblings with UTI and in 4 (4%) RRU of the screened siblings (p > 0.05). CONCLUSION: This study demonstrated that the incidence of mild renal scarring was much higher in siblings who presented with UTI than in asymptomatic siblings. However, the incidence of moderate and severe renal scarring among asymptomatic siblings was comparable to that in siblings with VUR and UTI.

Renal parenchymal damage in male infants with high grade vesicoureteral reflux diagnosed after the first urinary tract infection.

PURPOSE: Vesicoureteral reflux during infancy is found mainly in males, and it is of high grade and often bilateral. The higher predominance of male infants is reported in series when the reflux is diagnosed prenatally and when it is detected after urinary tract infection. Renal parenchymal damage may already be present at birth before any episode of urinary tract infection or acquired after a febrile urinary tract infection. We evaluate the incidence of renal damage in a large series of male infants with high grade vesicoureteral reflux diagnosed after the first urinary tract infection. MATERIALS AND METHODS: We reviewed the medical and radiological records of 141 consecutive male infants 3 weeks to 1 year old (mean age 5.8 months) who were diagnosed with high grade (III to V) vesicoureteral reflux on voiding cystourethrography during 1984 to 2000 following hospitalization for the first febrile urinary tract infection. A total of 127 (90%) patients underwent technetium dimercapto-succinic acid scan to evaluate renal damage 3 to 6 months after the initial infection. RESULTS: Vesicoureteral reflux was unilateral in 46 infants and bilateral in the remaining 95, comprising 236 ureters. Reflux was grade III in 79 ureters, IV in 114 and V in 43. Renal parenchymal damage was detected in 56 (44%) of the 127 infants on dimercapto-succinic acid scan, and was bilateral in 18 and unilateral in the 38, representing 74 renal refluxing units. Renal damage was mild (greater than 40% uptake) in 47 units, moderate (less than 40% and greater than 20% uptake) in 22 U and severe (less than 20% uptake) in 5 U. CONCLUSIONS: This study shows that nearly half of the male infants with high grade reflux who present with the first febrile urinary tract infection have renal parenchymal damage. This high incidence of renal damage may be explained by the coexistence of the 3 risk factors of gender, urinary tract infection and high grade vesicoureteral reflux.

Long-term outcome of the retained ureteral stump after lower pole heminephrectomy in duplex kidneys.

OBJECTIVES: Duplication of the ureter and renal pelvis is the most common upper urinary tract anomaly in childhood. The anatomical and functional divisions between upper and lower moieties of duplex kidney are extremely variable. The underlying pathological condition associated with a lower moiety is usually massive vesicoureteral reflux (VUR) to the lower collecting system and only rare obstruction. The non-functioning upper moiety is usually associated with obstructive ectopic ureter (with or without ureterocele). Most lower pole heminephrectomies are carried out for non-functioning lower moieties. In most cases, the lower defunctionalised segment of the ureter is left in situ. Complete ureterectomy is usually performed if presence of VUR into the lower end of the corresponding ureter is shown. There is little information on the long-term outcome of residual ureteral 'stumps'. The purpose of our study was to review the long-term outcome of retained ureteral stumps in children undergoing heminephrectomy for non-functioning lower pole moieties in duplex kidneys. MATERIALS AND METHODS: The medical records of 19 patients who underwent 20 lower pole heminephrectomies for a non-functioning lower pole moiety of a duplex kidney between January 1990 and December 2000 were reviewed retrospectively. Median age at heminephrectomy was 4.5 years (range: 1 month to 12 years). Indications for heminephrectomy in the 20 renal units was reflux nephropathy in 16 (80%) and obstructive nephropathy in 4 (20%). All corresponding ureters were taken down as low as possible and transfixed through the heminephrectomy incision. Median follow-up was 8.5 years (range: 1-11 years). RESULTS: Eight (40%) showed VUR into the stump after lower pole heminephrectomy. Two of these underwent subureteral endoscopic correction of VUR with polytetrafluoroethylene paste and resection of the stump was carried out in remaining two patients for recurrent urinary tract infections (UTI). Remaining four of the eight patients demonstrated spontaneous resolution of VUR during follow-up. CONCLUSIONS: Our data suggest that the vast majority of patients with residual ureteral stumps after lower pole heminephrectomy do not require stump resection at long-term follow-up.

Does delaying pyeloplasty affect renal function in children with a prenatal diagnosis of pelvi-ureteric junction obstruction?

OBJECTIVE: To evaluate retrospectively children with an antenatal diagnosis of pelvi-ureteric junction (PUJ) obstruction who required pyeloplasty for deteriorating renal function during the follow-up, specifically assessing the recovery of function after surgery. PATIENTS AND METHODS: Between 1988 and 2000, 44 consecutive patients with a prenatal diagnosis of PUJ obstruction who were initially treated by observation underwent pyeloplasty because their renal function deteriorated during the follow-up. Patients with bilateral disease, a single kidney or vesico-ureteric reflux were excluded from the study. The mean (sd) age of the patients was 13.3 (4.5) months and their mean (range) initial renal function 39.7 (30.4-45)%. In all patients the diagnostic criteria and indications for surgery were identical. The main indication for surgery was deteriorating renal function of >5%, confirmed by renal scintigraphy. Of 44 patients, 35 (77%) had severe dilatation of the renal pelvis by >3 cm. The mean (sd) deterioration in renal function during the follow-up, just before surgery, was 8.2 (2.4)%, and the mean time between the last acceptable renogram and the study showing deterioration was 8.3 (1.3) months; the mean follow-up was 5.1 (1.6) years. The results were assessed statistically using the unpaired nonparametric Mann-Whitney U-test. RESULTS: An improvement in hydronephrosis was confirmed in all patients, with renal function returning to the initial levels in 36 of 44 (81%) patients 6-12 months after surgery, although in two patients renal function did not improve after surgery. There was no significant difference between the preoperative characteristics and the degree of hydronephrosis, renal function or patient age with the level of renal functional improvement after surgery. CONCLUSION: In patients with an antenatal diagnosis of PUJ obstruction, expectant management is recommended and spares the children unnecessary surgery. Even if renal function deteriorates, delayed pyeloplasty recovers the initial functional level.

Post-chemotherapy microscopic residual prostate rhabdomyosarcoma: long-term conservative follow-up.

In spite of advances in the treatment of childhood bladder and prostate rhabdomyosarcoma (RMS), the ability to detect minimal residual disease correlates imperfectly with the ultimate outcome. We report the long-term follow-up of a child with microscopic residual RMS after chemotherapy. The correct interpretation of the histologic findings spared the child unnecessary additional therapy and raises enigmatic questions about the biology of minimal residual disease.

Effective long-term androgen suppression in men with prostate cancer using a hydrogel implant with the GnRH agonist histrelin.

OBJECTIVES: To evaluate the effectiveness of a hydrogel implant containing the gonadotropin-releasing hormone (GnRH) agonist histrelin in suppressing testosterone production in men with prostate cancer and to determine the effective dose (one, two, or four implants). METHODS: Forty-two men with prostate cancer and indications for androgen ablation were treated with one, two, or four implants. In two of the clinics, comprising 27 subjects, the treatment period was 12 months, with replacement with the same number of implants at 12-month intervals. In a third clinic, which treated 15 subjects, the implants were left in place for up to 30 months. The total experience was 605 treatment months. RESULTS: The histrelin levels were detected in serum proportional to the number of implants placed. The response, however, was similar among all three dose levels, with testosterone and luteinizing hormone essentially completely suppressed. Serum testosterone levels decreased from 21.9 +/- 17.6 nmol/L to 0.93 +/- 1.57 nmol/L within 1 month and were maintained at 0.55 +/- 0.24 nmol/L at 6 months and 0.60 +/- 0.28 nmol/L after 12 months of treatment. Of the 38 assessable patients, 35 (92%) had castrate levels of testosterone within 4 weeks of the initial implant placement. All patients followed for up for 12 months after placement of the initial set of implants maintained suppression of testosterone production while the implant was in place. CONCLUSIONS: The histrelin hydrogel implant provided adequate and reliable delivery of the potent GnRH agonist histrelin during at least 1 year using a single implant in men with prostate cancer. No apparent advantages were found in using more than one implant, and the question of the possible effectiveness of even lower doses remains open. This treatment modality appears to be both safe and effective.

Feminizing genitoplasty in patients with 46XX congenital adrenal hyperplasia.

Congenital adrenal hyperplasia (CAH) is the most common cause of ambiguous genitalia in newborns. This paper is based upon review of the literature and personal experience. We focus upon the surgical anatomy, pre-operative evaluation, including imaging, mainly by transabdominal ultrasound, and upon the goals and the history of surgical reconstruction. The various surgical techniques are mentioned with a detailed description of our technique used in 52 patients. The timing and staging of the operation and the implications of prenatal therapy are discussed. In conclusion, we believe that infants with 46XX CAH can undergo one-stage feminizing genitoplasty very early in life with satisfactory cosmetic and functional results.

1-Stage feminizing genitoplasty: 8 years of experience with 49 cases.

PURPOSE: We retrospectively evaluated the results of a modified technique for 1-stage feminizing genitoplasty in children with congenital adrenal hyperplasia (CAH) and other rare intersex conditions. MATERIALS AND METHODS: Between 1991 and 1998, 47 infants and 2 adolescents underwent 1-stage feminizing genitoplasty at our hospital. Of the patients 44 had CAH, 2 had the partial androgen insensitivity syndrome and 3 were true hermaphrodites. In 3 patients we used the Passerini-Glazel technique, while in 46 our technique of feminizing genitoplasty was used, which consists of maximal mobilization of the urogenital sinus en bloc including the vagina and urethra via the perineal approach. Two lateral plates built from dorsal split phallic and preputial skin together with the dorsal split urogenital sinus are used to construct the vaginal introitus and labia minora, and the scrotal folds are used to construct the labia majora. Reduction clitoroplasty is done according to the technique of Kogan et al. Mean age plus or minus standard deviation was 0.9 +/- 0.3 years of the patients with CAH and 13 +/- 2.3 of the remainder. Preoperative information, including status of the internal genitalia, anatomical length of the vagina site of communication between the vagina and urogenital sinus, in relation to the pelvic floor, was obtained by transabdominal pelvic ultrasound. RESULTS: Operating time ranged between 120 and 180 minutes, average hospitalization period was 4 to 5 days and mean followup was 4.7 +/- 2.6 years. Preoperative ultrasound provided the correct data regarding the vaginal and internal genitalia anatomy in all cases, and the exact communication site between the vagina and urogenital sinus was demonstrated in 93%. Complications consisted of intraoperative rectal injury in 1 case and mild wound infection of the buttocks area in 3. In 1 case total clitoris loss was observed. All patients who underwent modified genitoplasty have had successful cosmetic and early functional results. Two patients presented with repeat clitoromegalia due to inadequate adrenal suppression. CONCLUSIONS: Our adapted technique of 1-stage feminizing genitoplasty provides good cosmetic and functional results. The functional results of this operation should be reevaluated after puberty and in adulthood.