Clinical and gonadal features and early surgical management of 45,X/46,XY and 45,X/47,XYY chromosomal mosaicism presenting with genital anomalies.

OBJECTIVE: The 45,X/46,XY and 45,X/47,XYY group of patients includes some of those previously diagnosed with 'mixed gonadal dysgenesis'. Our aim was to establish the clinical and gonadal spectrum, and early surgical management, of patients with chromosomal mosaicism presenting with genital anomalies. PATIENTS AND METHODS: We performed a retrospective review of patients with 45,X/46,XY or 45,X/47,XYY mosaicism presenting with genital ambiguity between 1988 and 2009. At least one gonadal biopsy or gonadectomy specimen was available for each patient. Gonadal histology was re-evaluated by a paediatric pathologist. RESULTS: Of 31 patients with 45,X/46,XY (n = 28) or 45,X/47,XYY (n = 3) mosaicism and genital anomalies, 19 (61%) were raised male. Histology of 46 gonads was available from patients who had undergone a gonadectomy or gonadal biopsy, at a median age of 9.5 months. 18 gonads were palpable at presentation, including 5 (28%) histologically unremarkable testes, 2 streak gonads, and 1 dysgenetic gonad with distinct areas of testicular and ovarian stroma but no oocytes. All intra-abdominal gonads were found to be dysgenetic testes (of which 2 were noted to have pre-malignant changes) or streaks, apart from 1 histologically unremarkable testis. 15 (48%) patients had other anomalies, most commonly cardiac and renal; 4 (13%) had a Turner phenotype. CONCLUSION: The anatomy and gonadal histology of 45,X/46,XY and 45,X/47,XYY individuals with genital ambiguity do not conform to a set pattern, and hence management of each patient should be individualized according to detailed anatomical and histological assessment.

Is it safe to use aluminum in the treatment of pediatric hemorrhagic cystitis? A case discussion of aluminum intoxication and review of the literature.

In pediatric oncology patients, hemorrhagic cystitis can be a life-threatening complication of bone marrow transplantation, chemotherapy, and radiation therapy. The treatment of this condition is often challenging and includes intravesical irrigation with aluminum, embolization, endoscopic laser coagulation, hydrostatic pressure, use of hyperbaric oxygen, instillation of formalin, prostaglandins, and oral sodium pentosan polysulfate. Although the efficacy of aluminum irrigation is well documented for the management of hemorrhagic cystitis in adults, there are limited reports describing its use in children. The potential multisystem toxic effects of aluminum are well described and the range and progression of aluminum toxicity can be devastating. We report a case of a 9-year-old girl suffering from acute lymphocytic leukemia with hemorrhagic cystitis. Although the symptoms resolved after intravesical aluminum treatment, she developed significant aluminum toxicity. We have reviewed the literature relating to aluminum toxicity in the pediatric age group and present our recommendations for the effective and safe use of aluminum in this cohort of patients.

An update on the management of Wilms' tumour.

AIMS: To review the management of Wilms' tumour. METHODS: A search of the literature was performed using the PubMed database (1966 to May 2006) with the search terms 'Wilms' and either 'tumour/tumor' or 'cancer' or 'carcinoma'. This was augmented by manual searches of publications. FINDINGS: The success of clinical trials in Wilms' tumour patients over the past 30 years has led to an overall survival of 85% and the introduction of less aggressive chemotherapeutic regimes for patients. Large randomised controlled trials have been published on the management of Wilms' tumour by various collaborative groups, including the National Wilms' Tumour Study Group (NWTSG) in North America and the Société Internationale d'Oncologie Pédiatrique (SIOP) plus the United Kingdom Children's Cancer Study Group (UKCCSG) in Europe. CONCLUSIONS: Controversy exists as to the best approach to the management of these children with regard to neoadjuvant chemotherapy. Challenges remain in the identification of histological and molecular risk factors for the stratification of treatment intensity.

Nephro-urological complications of epidermolysis bullosa in paediatric patients.

A small but important proportion of patients with epidermolysis bullosa (EB) may develop significant renal and urological complications which can have a major impact on their morbidity and mortality. During the last 10 years, five of a large group of children with EB under our care, with either dystrophic or junctional types of disease, experienced major nephro-urological complications. Two patients with recessive dystrophic EB (REDB) developed macroscopic haematuria - one had renal failure and underwent a renal biopsy showing IgA nephropathy. A third patient with RDEB also developed renal failure and his biopsy demonstrated postinfectious glomerulonephritis/type III membranoproliferative (mesangiocapillary) glomerulonephritis. Both patients with renal failure underwent peritoneal dialysis. Two patients with junctional EB developed obstructive uropathies, which required bladder reconstruction and the fashioning of a Mitrofanoff channel in one.

Myogenin and desmin immunohistochemistry in the assessment of post-chemotherapy genitourinary embryonal rhabdomyosarcoma: prognostic and management implications.

PURPOSE: Posttreatment genitourinary embryonal rhabdomyosarcoma often shows well differentiated rhabdomyoblasts, which are detectable on routine histological staining. Definite areas of residual undifferentiated rhabdomyosarcoma indicate residual/recurrent disease. However, the recent use of immunohistochemical staining with desmin and myogenin in resected specimens and surveillance biopsies following adjuvant therapy may demonstrate scant positive staining cells that appear undifferentiated on light microscopy. To our knowledge the clinical significance of this finding is currently unknown. Therefore, we reviewed our retrospective experience with genitourinary embryonal rhabdomyosarcoma to examine the relationship between immunostain positive undifferentiated cells and subsequent clinical outcome. MATERIALS AND METHODS: A total of 14 children with a median age of 2.75 years (range 8 months to 7 years) with genitourinary embryonal rhabdomyosarcoma were identified in the histopathology database. All had biopsy confirmation of the diagnosis, followed by multi-agent chemotherapy. Two children in whom there was obvious residual active tumor at the resection margins were excluded from further analysis. Histopathological findings in all patients on the resection/posttreatment biopsy were reviewed. All specimens were immunostained with desmin and myogenin to detect residual undifferentiated rhabdomyoblasts. The relation between histopathological findings and outcome was determined. RESULTS: There were 14 cases of genitourinary embryonal rhabdomyosarcoma. In 2 cases (14%) residual embryonal tumor was pathologically confirmed following initial treatment. In 12 cases no obvious residual tumor was present following initial therapy. Rhabdomyosarcoma affected the bladder in 10 cases and the vagina in 2. There were no distant metastases in any child. Ten patients underwent local resection following chemotherapy and 2 underwent followup biopsies only without resection. A total of 11 cases showed well differentiated, posttreatment rhabdomyoblasts that was identifiable on routine hematoxylin and eosin staining with margins apparently free of tumor and 1 showed no morphological evidence of residual rhabdomyosarcoma. However, all cases demonstrated at least scant abnormal desmin and myogenin positive cells in the specimens. Four patients had no further treatment and none had clinical recurrence. All were well 10 years (range 8 to 13) after treatment. Eight patients received further treatment (chemotherapy and/or radiotherapy) based on clinical and pathological findings, followed by further resection in 3. One patient died of disease but 7 were well a median of 7.2 years (range 8 months to 13 years) after treatment. CONCLUSIONS: The significance of undifferentiated myogenin/desmin positive cells in genitourinary embryonal rhabdomyosarcoma in the absence of morphological residual/recurrent embryonal rhabdomyosarcoma remains unclear since such cells can be detected in all cases of posttreatment embryonal rhabdomyosarcoma. In some cases findings are associated with clinical disease recurrence, while others with identical histopathological findings following initial treatment have no clinical sequelae even in the absence of further treatment. In genitourinary embryonal rhabdomyosarcoma close and regular clinical surveillance is essential. Desmin/myogenin immunohistochemistry to detect scattered undifferentiated cells does not appear to provide useful prognostic information.

Laparoscopic transposition of lower pole vessels--the 'vascular hitch': an alternative to dismembered pyeloplasty for pelvi-ureteric junction obstruction in children.

OBJECTIVE: Dismembered pyeloplasty is the traditional technique in the management of ureterovascular pelvi-ureteric junction obstruction (PUJO) in children. Controversy remains regarding the role of lower pole vessels as the sole aetiology for PUJO. Endopyelotomy and concomitant laparoscopic transposition of lower pole vessels for PUJO has been described in adults. We describe our technique of laparoscopic transposition of lower pole vessels in children with PUJO, leaving the PUJ intact. PATIENTS AND METHODS: Thirteen patients (seven boys and six girls) with a mean age of 10.2 years (range 7-16 years) underwent laparoscopic transposition of lower pole vessels. Surgery was indicated on the basis of intermittent pain and ultrasound/MAG3 appearance of obstruction with or without reduced function. The technique involved laparoscopic transperitoneal mobilization of the lower pole vessels from the region of the PUJ thereby freeing the junction and transposing them superiorly onto the anterior wall of the pelvis. The main outcome measures were relief of pain and improvement in ultrasound appearance or drainage parameters on a postoperative MAG3 renogram performed within 4-6 weeks of surgery. RESULTS: Median operating time was 92 min. All patients were discharged within 36 h of surgery. All patients remain pain free at a median of 6 months (range 3-18 months). Twelve patients showed good drainage on the postoperative MAG3 renogram and improvement in ultrasound appearance. One patient had recurrent symptoms requiring insertion of a JJ stent. She has undergone further laparoscopic exploration. The vessels were in their transposed position and there was a kink at the PUJ which was released. She had a vertical pyelotomy and transverse closure over the JJ stent with good results. CONCLUSION: This technique is simple and requires less operating time. No anastomosis or temporary JJ stent is required. Our early results are very encouraging with no serious complications.

Wilms' tumour with persistent intravascular extension: a review of the surgical aspects of management.

AIM: To review the surgical management of Wilms' tumour with persistent intravascular (vena caval +/- atrial) tumour extension. PATIENTS AND METHODS: Data were collected regarding operative details, tumour and 'thrombus' histology, and long-term outcome for patients with Wilms' tumour with cavo-artial extension. RESULT: From 1988 to 2004, 13 patients underwent treatment for Wilms' tumour with persistent intravascular extension. Preoperative chemotherapy was administered in 11/13 patients and postoperative radiotherapy in eight patients. Intravascular involvement was upto IVC (5), and right atrium (8) patients. Techniques employed for excision of intra-vascular component were: local cavotomy (3), extensive infra-diaphragmatic cavotomy without cardiopulmonary bypass (CPB) (1), and excision of cavo-atrial tumour with CPB (+/- hypothermia and cardiac arrest) (9). Mean time on CPB was 90 min. Caval repair was accomplished by primary repair (6) and pericardial graft in (7) patients. There were no intraoperative deaths and few major complications. Tumour thrombus contained malignant cells in 10/13 cases. Mean follow up has been for 55.4 months. To date, seven patients remain disease-free (one lost to follow up), disease recurred in five patients, three of whom have died. There were no symptoms related to the graft. CONCLUSIONS: Surgery for Wilms' tumour with persistent intravascular extension despite chemotherapy is technically challenging. CPB +/- hypothermia and cardiac arrest and extensive caval repair with a graft is safe and reliable in the long term.

Primary intrarenal neuroblastoma: a rare, aggressive tumour of childhood mimicking Wilms' tumour.

Primary intrarenal neuroblastoma is an extremely rare and aggressive tumour of the kidney. We report on a case that mimicked Wilms' tumour. Differentiating primary intrarenal neuroblastoma from Wilms' tumour is important both for management and prognosis.

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%.

Isolated unilateral tuberous sclerosis-associated renal cystic disease in a neonate.

We present a male infant with antenatally detected, focal, unilateral apparently isolated renal cystic disease with morphological features of renal involvement in tuberous sclerosis. Only one previous case with similar presentation has been described. Most affected children present with either diffuse bilateral renal cystic disease or extrarenal manifestations. The major genes involved in tuberous sclerosis are now well described, and early onset of severe renal cystic disease in affected children often is related to the presence of a contiguous gene deletion syndrome involving TSC2 and PKD1 on chromosome 16.

Long-term outcome of Fowler-Stephens orchiopexy in boys with prune-belly syndrome.

PURPOSE: Intra-abdominal testes in boys with prune-belly syndrome have been conventionally managed by 1 or 2-stage orchiopexy with division of the gonadal vessels. We reviewed a series of adults with prune-belly syndrome to assess the morphological and functional outcome of orchiopexy in childhood with specific reference to the spontaneous onset of puberty, hormonal profiles and sexual function. MATERIALS AND METHODS: A total of 41 boys were divided into 3 groups depending on the type of orchiopexy performed, namely group 1-20 with bilateral 1-stage orchiopexy, group 2-10 with unilateral 1-stage and contralateral 2-stage orchiopexy, and group 3-11 with bilateral 2-stage orchiopexy. RESULTS: In group 1 9 of 20 patients had good scrotal testes bilaterally, 6 had a good scrotal testis on 1 side and 3 had small testes on each side. Two boys required testosterone supplementation but 18 had normal hormonal and sexual function. In group 2 6 of 10 patients had good scrotal testes bilaterally and 4 had a good scrotal testis on 1 side. All patients underwent spontaneous puberty with good sexual function. In group 3 7 of 11 boys had good scrotal testes bilaterally and 3 had 1 good testis with normal puberty and sexual function. These 10 patients underwent spontaneous puberty with good sexual function. CONCLUSIONS: The majority of boys with prune-belly syndrome had a satisfactory outcome after orchiopexy with division of the gonadal vessels with testicular function sufficient to induce puberty and maintain satisfactory sexual function in adult life.

Epidemiology of paediatric renal stone disease in the UK.

BACKGROUND: The previous epidemiological study of paediatric nephrolithiasis in Britain was conducted more than 30 years ago. AIMS: To examine the presenting features, predisposing factors, and treatment strategies used in paediatric stones presenting to a British centre over the past five years. METHODS: A total of 121 children presented with a urinary tract renal stone, to one adult and one paediatric centre, over a five year period (1997-2001). All children were reviewed in a dedicated stone clinic and had a full infective and metabolic stone investigative work up. Treatment was assessed by retrospective hospital note review. RESULTS: A metabolic abnormality was found in 44% of children, 30% were classified as infective, and 26% idiopathic. Bilateral stones on presentation occurred in 26% of the metabolic group compared to 12% in the infective/idiopathic group (odds ratio 2.7, 95% CI 1.03 to 7.02). Coexisting urinary tract infection was common (49%) in the metabolic group. Surgically, minimally invasive techniques (lithotripsy, percutaneous nephrolithotomy, and endoscopy) were used in 68% of patients. CONCLUSIONS: There has been a shift in the epidemiology of paediatric renal stone disease in the UK over the past 30 years. Underlying metabolic causes are now the most common but can be masked by coexisting urinary tract infection. Treatment has progressed, especially surgically, with sophisticated minimally invasive techniques now employed. All children with renal stones should have a metabolic screen.

[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.

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.

Detrusor myotomy: a 5-year review in unstable and non-compliant bladders.

OBJECTIVE: To identify urodynamic factors that might determine the clinical outcome of detrusor myotomy in incontinent children. PATIENTS AND METHODS: Six girls and three boys (aged 5-14 years) underwent detrusor myotomy for severe urinary incontinence. Seven children had spina bifida, one had traumatic paraplegia and one had low bladder compliance. The patients were followed for a minimum of 5 years. RESULTS: Urodynamic studies before surgery showed that three patients had normal compliance with grossly unstable detrusor contractions, and six had low bladder compliance with few phasic detrusor contractions. Detrusor leak-point pressures were > 40 cmH2O in five patients and < 40 cmH2O in four. Only two patients, both with grossly unstable detrusor contractions and leak-point pressures of > 40 cmH2O, had a successful 5-year outcome. The other seven patients remained incontinent; six underwent further surgery and one died from unrelated causes. CONCLUSION: Detrusor myotomy appears to have the best outcome in those patients with marked phasic unstable detrusor contractions with a competent urethral sphincter. In this group it may have distinct advantages over more commonly used procedures.

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.

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.

Endopyelotomy in the symptomatic older child.

OBJECTIVE: To evaluate the results of endopyelotomy in children, an established method in adult practice as a treatment for pelvi-ureteric junction (PUJ) obstruction. PATIENTS AND METHODS: Endopyelotomies undertaken between 1992 and 1999 by one surgeon in an established endourology unit were reviewed retrospectively. Children aged > 5 years presenting with pain and obstruction on isotope renography were selected for endopyelotomy. Patients with crossing vessels detectable on spiral computed tomography were treated by open pyeloplasty. Access to the renal pelvis was provided by a uroradiologist. Endopyelotomy was carried out through an Amplatz sheath of (median) 26 F. After applying traction to invaginate the PUJ an incision was made postero-laterally using electrocautery via an 11 F paediatric resectoscope. Stenting was maintained for 6 weeks. In all, 13 patients (median age 10 years, range 5-14) were treated; two had associated calculi. RESULTS: The symptoms resolved and the obstruction was relieved in only six patients, with a median (range) follow-up of 50 (26-68) months. The seven patients in whom endopyelotomy failed, as indicated by persistent pain, proceeded to open pyeloplasty at a median (range) of 4 (1.3-79) months. Of these, two had presented with associated multiple calculi and significant hydronephrosis (one with an associated duplex system) and three had crossing lower pole vessels at open operation. One developed a urinoma after the original endopyelotomy and one had a retained stent fragment removed at the time of pyeloplasty. CONCLUSIONS: Endopyelotomy in the symptomatic child requires a careful preoperative evaluation. Crossing lower pole vessels warrant an open pyeloplasty.