Single centre experience: long term outcomes in spina bifida patients.

OBJECTIVE: To determine our single centre experience of long term renal function and mortality rates of our cohort of spina bifida patients born since 1970. MATERIALS AND METHODS: We collected a prospective database and included all patients born with spina bifida between 1970 and 2011 managed by our centre. Data we collected included the glomerular filtration rates, results of renal tract imaging, management strategies and mortalities. RESULTS: Of 160 patients identified, 120 were included for analysis with a mean age of 20 years. In our cohort 48% (58/120) had normal renal function and only 1.6% (2/120) had severe renal impairment or end stage renal failure. There was a mortality rate of 4.4% and there were no deaths from renal failure. CONCLUSION: Spina bifida patients have been reported to have high mortality rates and high morbidity secondary to renal failure. Historical data does not appear to reflect current advancements and we would therefore be more optimistic when counselling families about long term survival into adulthood.

Cultural bias in the AAP's 2012 Technical Report and Policy Statement on male circumcision.

The American Academy of Pediatrics recently released its new Technical Report and Policy Statement on male circumcision, concluding that current evidence indicates that the health benefits of newborn male circumcision outweigh the risks. The technical report is based on the scrutiny of a large number of complex scientific articles. Therefore, while striving for objectivity, the conclusions drawn by the 8 task force members reflect what these individual physicians perceived as trustworthy evidence. Seen from the outside, cultural bias reflecting the normality of nontherapeutic male circumcision in the United States seems obvious, and the report's conclusions are different from those reached by physicians in other parts of the Western world, including Europe, Canada, and Australia. In this commentary, a different view is presented by non-US-based physicians and representatives of general medical associations and societies for pediatrics, pediatric surgery, and pediatric urology in Northern Europe. To these authors, only 1 of the arguments put forward by the American Academy of Pediatrics has some theoretical relevance in relation to infant male circumcision; namely, the possible protection against urinary tract infections in infant boys, which can easily be treated with antibiotics without tissue loss. The other claimed health benefits, including protection against HIV/AIDS, genital herpes, genital warts, and penile cancer, are questionable, weak, and likely to have little public health relevance in a Western context, and they do not represent compelling reasons for surgery before boys are old enough to decide for themselves.

Ephedrine hydrochloride: novel use in the management of resistant non-neurogenic daytime urinary incontinence in children.

OBJECTIVE: To determine whether the adrenoceptor agonist, ephedrine hydrochloride, is an effective treatment for resistant non-neurogenic daytime urinary incontinence in children. METHODS: From 2000 to 2010, eighteen children with resistant non-neurogenic daytime urinary incontinence were treated with oral ephedrine hydrochloride at our institution. Sixteen were female and two were male. Median age at treatment was 12 years (range 5-15 years). Two children had spina bifida occulta. There were no other co-morbidities. Multiple anticholinergics were prescribed and dose maximized to support a bladder and bowel training programme, without achieving continence in this resistant group of children. Pre-treatment urodynamics were normal in 10, but revealed an open bladder neck in 8 patients. None showed detrusor over-activity. Oral ephedrine hydrochloride was started at 7.5 mg or 15 mg twice daily and titrated up to a maximum of 30 mg four times daily according to response. RESULTS: Median follow-up was 7 years (range 6-8 years). Seventeen children (94%) reported improvement in symptoms and six (33%) achieved complete urinary continence. All patients maintained compliant bladders on post-treatment urodynamics. Seven of the 8 previously open bladder necks were closed. No patients reported any significant side effects. Patients with open bladder necks on pre-treatment urodynamics were more likely to show a full response to ephedrine (odds ratio 15; 95% CI 1.2-185.2). CONCLUSIONS: Oral ephedrine hydrochloride is an effective treatment for carefully selected children with resistant non-neurogenic daytime urinary incontinence.

Indications and relative renal function for paediatric nephrectomy over a 20-year period.

BACKGROUND: The indications for partial or total nephrectomy for urological reasons are not clearly documented in the paediatric surgical literature and there are only a limited number of publications on this subject. PURPOSE: In order to clarify the situation in our centre, we reviewed our own indications for nephrectomy over a 20-year period. METHODS: Medical records of patients who underwent nephrectomy for urological and oncological causes between May 1990 and October 2010 at our centre were retrospectively reviewed. Indications for nephrectomy were noted. Renal function was noted for urological cases. RESULTS: A total of 357 nephrectomies were undertaken; out of these, 241 cases were for urological causes and 116 for oncological reasons. The majority of total nephrectomies were performed for multicystic dysplastic kidneys, reflux nephropathy, pelvi-ureteric junction obstruction and dysplasia. Eighty-seven patients exhibited no function at the time of nephrectomy. Of the remainder, 17 had <5% function, 18 had 5-10% function, 5 had 10-15% function and 2 patients had 15-20% function. CONCLUSIONS: This is the largest series of nephrectomies in the paediatric surgery literature to date. The majority of nephrectomies were undertaken for urological conditions with relative renal function of <10% and could not have been usefully preserved in situ. We suggest that kidneys with >10% function should not be routinely removed.

Coming face to face with the stenotic MACE-combined Maceoscopy and colonoscopy (the rendez-vous procedure): a preliminary report.

OBJECTIVE: The aim of our study was to review the management of stomal stenosis after the Malone antegrade continence enema (MACE) procedure at our center, and to describe and assess the early outcomes of 2 novel procedures used in the management of conduit stenosis: Maceoscopy and the rendez-vous. SUBJECTS/PATIENTS AND METHODS: We retrospectively reviewed 45 consecutive MACE cases performed by 1 surgeon at St George's hospital over a 13-year period. RESULTS: Until April 2002, 5 cases presented with difficulty catheterizing their MACE. This was attributed to stenosis without further investigation, and the MACE revised surgically. Of 13 cases presenting with suspected stenosis since May 2002, 8 channels turned out to be patent when assessed and catheterized endoscopically via Maceoscopy. Two truly stenotic MACEs were revised; patency of the other 3 was reestablished by means of a new double-endoscopic procedure we have termed the rendez-vous.

Are prophylactic antibiotics necessary with clean intermittent catheterization? A randomized controlled trial.

BACKGROUND/PURPOSE: Clean intermittent catheterization has been an established practice for more than 3 decades. The validity of antibiotic prophylaxis has been questioned although not tested. METHODS: Eighty-five patients were recruited into a randomized controlled trial. The randomization involved the placement into 1 of 2 groups: (A) continuing antibiotics or (B) discontinuing antibiotics. The trial would last 4 months with the outcome being a confirmed urinary tract infection. All groups were matched for age, sex, and pathology. RESULTS: The incidence of urinary tract infections was significantly increased in the group who continued to use antibiotics (n = 20) when compared with the group who discontinued prophylaxis (n = 3). The common infecting organism was Escherichia coli. CONCLUSIONS: The use of prophylactic antibiotics for children who intermittently catheterize may not be necessary. The use of prophylactic antibiotics may result in increased rates of infection because of the development of resistant organisms.