Management of neuropathic bladder secondary to spina bifida: Twenty years' experience with a conservative approach.

Introduction: Treatment of neuropathic bladder secondary to spina bifida is an ongoing challenge. Although different management strategies and protocols are available in the literature, reliance on expert opinion remains fundamental. A conservative approach can be utilized, but patients must be closely monitored throughout the management process. The objective of this study was to review the management and outcomes of neuropathic bladder in spina bifida by appraising long-term bladder and renal function in patients treated at a medical center utilizing a conservative management style. Methods: This is a single-center retrospective review of urology care for all spina bifida patients 5-19 years of age with a neuropathic bladder who attended follow-ups between April 2000 and April 2020. Only patients with more than 5 years of follow-up were included. Renal functions, continence and results of invasive video urodynamics (IUD) and any surgical interventions were recorded. Results: Seventy-one patients (mean age = 10.5 years) were identified after exclusions. Bladder compliance between first and last IUDs increased significantly (p = 0.0056). Anticholinergic treatment was started at the first outpatient appointment. Intravesical botulinum toxin injection was the second line treatment in ten patients. 94% of patients had an end fill pressure below 40 cm H2O in their last IUD. 82% were socially continent (dry or occasional damp patches) with or without catheterisations at the age of 11.5 years. One patient in the cohort had bladder augmentation. Conclusion: The optimal management of neuropathic bladder secondary to spina bifida remains controversial. Bladder and renal functional outcomes can be improved with close monitoring and less invasive management.

Post-operative steroids in boys with histologically proven balanitis xerotica obliterans reduce the need for subsequent meatal dilatation.

AIM: Post-circumcision topical steroids may reduce the number of patients with balanitis xerotica obliterans (BXO) who require subsequent meatal dilatation. However, their use has only been investigated in the presence of an abnormal urethral meatus. The aim of this study is to determine if post-operative topical steroids reduce the need for subsequent meatal dilatation in patients with histologically proven BXO independent of the appearance of the urethral meatus. To determine if post-operative topical steroids reduce the need for subsequent meatal procedures in patients with histologically proven BXO. To determine if the appearance of the meatus at circumcision affects the need for subsequent meatal procedure. METHODS: We performed a retrospective review of all paediatric patients with histologically confirmed BXO post circumcision. We excluded all those with 'chronic inflammation' or 'early BXO'. Patients were divided into groups based on whether post-operative topical steroids were prescribed. The appearance of the urethral meatus at circumcision was recorded. Primary outcome was progression to subsequent meatal dilatation. Statistical significance was calculated using Fisher's exact test. A P value < 0.05 was considered significant. RESULTS: One hundred and seventeen patients with histologically confirmed BXO were identified between October 2012 and December 2017. Fifty-nine patients were prescribed post-operative topical steroids (50%). Nine patients required subsequent meatal dilatation procedure (8%) and only one of these had been prescribed post-operative topical steroids (P = 0.0165). Abnormal meatus was noted in 24 (21%) patients. Three patients in this group required subsequent meatal dilatation compared with 6 in the group with a normal meatal appearance (P = 0.385). CONCLUSIONS: Post-operative topical steroids can reduce the need for subsequent meatal dilatation in boys with BXO. The appearance of the urethral meatus at circumcision does not predict the need for subsequent meatal dilatation.

The acute scrotum in infancy: A retrospective regional study.

INTRODUCTION: Testicular torsion during infancy (<1 year) is known to be a rare event with relatively few studies on the subject in the published scientific literature. We reviewed the experience of infant scrotal exploration within a paediatric surgical network of four centres serving an approximate paediatric population of 1.8 million. AIM: To review current practice of scrotal explorations in infancy and explore areas for improvement. METHOD: Retrospective review of emergency operations for acute scrotum between January 2016 and December 2018. Data are presented as median (range) and compared using non-parametric tests. P < 0.05 was regarded as significant. RESULTS: A total of 560 paediatric scrotal explorations were performed. Of these, 25 (4%) were under one year at the time of surgery, median 4 months (1 day-5 months) (Fig. 1). The most common diagnosis was "epididymitis" (11/25, 44%). Testicular torsion was found in 28% (7/25), increasing to 66% (4/6) in those under one month. Of those with confirmed testicular torsion, three (43%) underwent a primary orchiectomy with contralateral fixation and three (43%) detorsion and bilateral fixation, with all three cases found to have testicular atrophy at follow-up. The final case was of metachronous torsion, with the initial presentation not operated on and the subsequent presentation undergoing detorsion and fixation. This was the only case of testicular salvage in our series. There were no underlying urological issues in any patient and no reported complications or ongoing urological issues post-surgery. The rate of follow-up was 17/25 (68%). CONCLUSION: We present one of the only clinical series to focus on acute scrotum in the under one year old and not just the neonatal period. Testicular torsion remains a rare event in this age group but invariably leads to testicular loss.

Readmission within 30 days of discharge (ReAd): a quality-of-care indicator in paediatric surgery.

PURPOSE: Following a previously published 1 year audit of readmissions, this is a reaudit of our readmission rate (ReAd) in paediatric surgery, asking: is ReAd reproducible, can it be an indicator of quality of care in paediatric surgery, and can it be improved? METHOD: Prospectively collected Hospital Episode statistics were used to identify readmissions over 1 year. Patients were subdivided into emergency vs elective regarding the first admission and outcomes compared including with our previously published ReAd data. RESULTS: 2616 children (67% male) were admitted during 2016: 1398 (53%) elective and 1218 (47%) emergency admissions. The overall ReAd was 0.9%, comparable to and lower than our previously published rate of 2%. The commonest cause for readmission was appendicitis-related (22%). The emergency cohort ReAd was 1.5% (18/1218) compared to 0.4% (5/1398) in the elective cohort, 4× higher (p = 0.002). In the emergency cohort, the commonest causes for readmission were abdominal pain and perforated appendicitis. 80% of elective group readmissions were related to urological procedures. More of these required surgical intervention to treat (80% vs 22%) (p = 0.03). CONCLUSION: (1) ReAd is a reproducible and reducible quality-of-care indicator in paediatric surgery. (2) Emergency admission is a risk factor for readmission. (3) Appendicectomy was associated with the highest ReAd.

Presence of viable germ cells in testicular regression syndrome remnants: Is routine excision indicated? A systematic review.

There is no consensus in the literature about the necessity for excision of testicular remnants in the context of surgery for an impalpable testis and testicular regression syndrome (TRS). The incidence of germ cells (GCs) within these nubbins varies between 0 and 16% in previously published series. There is a hypothetical potential future malignancy risk, although there has been only one previously described isolated report of intratubular germ-cell neoplasia. Our aim was to ascertain an accurate incidence of GCs and seminiferous tubules (SNTs) within excised nubbins and hence guide evidence-based practice. The systematic review protocol was designed according to the PRISMA guidelines, and subsequently published by the PROSPERO database after review (CRD42013006034). The primary outcome measure was the incidence of GCs and the secondary outcome was the incidence of SNTs. The comprehensive systematic review included articles published between 1980 and 2016 in all the relevant databases using specific search parameters and terms. Strict inclusion and exclusion criteria were ultilised to identify articles relevant to the review questions. Twenty-nine paediatric studies with a total of 1455 specimens were included in the systematic review. The mean age of the patients undergoing nubbin resection was 33 months and the TRS specimen was more commonly excised from the left (68%). The incidence of SNTs was 10.7% (156/1455) and the incidence of GCs, 5.3% (77/1455). Histological analysis excluding the presence of either SNTs or GCs was consistent with TRS, fibrosis, calcification or haemosiderin deposits. There is limited evidence on subset analysis that GCs and SNTs may persist with increasing patient age. This systematic review has identified that 1 in 20 of resected testicular remnants has viable GCs and 1 in 10 has SNTs present. There is insufficiently strong evidence for the persistence of GCs and SNTs with time or future malignant potential. Intra-abdominal TRS specimens may contain more elements and, therefore, require excision, although this is based on limited evidence. However, there is no available strong evidence to determine that a TRS specimen requires routine excision in an inguinal or scrotal position.

British Association of Paediatric Urologists consensus statement on the management of the primary obstructive megaureter.

INTRODUCTION: It is well-known that the majority of congenital megaureters may be managed conservatively, but the indications and surgical options in patients requiring intervention are less well defined. Hence this topic was selected for discussion at the 2012 consensus meeting of the British Association of Paediatric Urologists (BAPU). Our aim was to establish current UK practice and derive a consensus management strategy. METHODS: An evidence-based literature review on a predefined set of questions on the management of the primary congenital megaureter was presented to a panel of 56 Consultant Surgeon members of the British Association of Paediatric Urologists (BAPU), and current opinion and practice established. Each question was discussed, and a show of hands determined whether the panel reached a consensus (two-thirds majority). RESULTS: The BAPU defined a ureteric diameter over 7 mm as abnormal. The recommendation was for newborns with prenatally diagnosed hydroureteronephrosis to receive antibiotic prophylaxis and be investigated with an ultrasound scan and micturating cystourethrogram, followed by a diuretic renogram once VUR and bladder outlet obstruction had been excluded. Initial management of primary megaureters is conservative. Indications for surgical intervention include symptoms such as febrile UTIs or pain, and in the asymptomatic patient, a DRF below 40% associated with massive or progressive hydronephrosis, or a drop in differential function on serial renograms. The BAPU recommended a ureteral reimplantation in patients over 1 year of age but recognized that the procedure may be challenging in infancy. Proposed alternatives were the insertion of a temporary JJ stent or a refluxing reimplantation. CONCLUSION: A peer-reviewed consensus guideline for the management of the primary megaureter has been established. The guideline is based on current evidence and peer practice and the BAPU recognized that new techniques requiring further studies may have a role in future management.

Balanitis xerotica obliterans in children and adolescents: a literature review and clinical series.

OBJECTIVE: Balanitis xerotica obliterans (BXO) is a chronic inflammatory disease that is considered as male genital variant lichen sclerosis. The incidence varies greatly in different series; diagnosis is mostly clinical but histopathological confirmation is mandatory. Various treatments are described, but there is no consensus that one is the best. MATERIALS AND METHODS: A literature review was made of BXO and lichen sclerosis in boys under 18 years of age, between 1995 and 2013, analyzing demographic dates, treatments and outcomes. In addition to that, we reviewed BXO cases treated in our centers in the last 10 years. RESULTS: After literature review, only 13 articles matched the inclusion criteria. Analyzing those selected, the global incidence of BXO is nearly 35% among circumcised children. Described symptoms are diverse and the low index of clinical suspicion is highlighted. The main treatment is circumcision, with use of topical and intralesional steroids and immunosuppressive agents. CONCLUSION: BXO is a condition more common than we believe and we must be vigilant to find greater number of diagnoses to avoid future complications. The main treatment for BXO is circumcision, but as topical or intralesional treatments are now available with potentially good outcomes, they may be considered as coadjuvants.

Air enema reduction of intussusception: a registrar-led, protocol-driven service is safe and effective.

PURPOSE: In the UK, air enema reduction of intussusception is predominantly performed by consultant radiologists. At our institution, it is usually performed by a team involving radiology and surgical registrars. The purpose of this study was to assess who performs air enemas in the UK and the efficacy and safety of our registrar-led service. METHODS: A telephone survey of paediatric surgical centres in the UK was performed asking who performs air enema in these institutions. Following this, a retrospective review of all air enemas at our institution between January 2005 and 2011 was performed. Cases were identified from radiology databases and reviewed for grade of radiologist, perforation and outcome. RESULTS: At all 25 centres, consultant radiologists performed air enemas. At our institution 145 enemas were performed in 6 years. 141 were analysed (54 girls, 87 boys). Median age was 9 months (range 3-107 months). 82 % were performed by the registrar-only team. Over 6 years the registrar-led reduction rate was 77.5 % and in the last 3 years 84 %. The perforation rate was 0.9 %. CONCLUSION: A paediatric surgical registrar-led service for air enema reduction can be safe and effective ensuring a team approach is adopted, equipment is efficient and a strict protocol is applied.

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.

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.

Paediatric sutureless circumcision and modified circumcision: video demonstration.

OBJECTIVE: Circumcision is undertaken using a variety of surgical methods. Modified circumcision for the hooded prepuce or mild glanular hypospadias mandates an excellent cosmetic result. Tissue glue is an alternative to sutures for circumcision in children and has significant advantages in terms of cosmesis and postoperative pain. We present two detailed videos demonstrating our technique for sutureless circumcision and modified circumcision. METHOD: The incisions were marked on the outer and inner preputial skin. Incisions were made with a scalpel. Complete haemostasis was ensured. Tissue adhesive was placed onto the wound using a 32-gauge needle to allow controlled application. It is important to ensure that the coronal sulcus is free of glue at the end of the procedure. Once the circumcision is complete the penis can be cleaned and temporarily dressed. RESULTS: Between November 2008 and September 2011, we have used this technique for 57 circumcisions and 30 modified circumcisions. No complications occurred. All were followed up for a minimum of 3-6 months. CONCLUSION: The technique allows reproducible excellent cosmetic results, can be easily taught to junior surgical staff and should be considered as an alternative to a standard sutured circumcision.

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.

Neonatal testicular torsion: a systematic literature review.

Neonatal testicular torsion (NTT) is rare and reported salvage rates vary widely both in their cited frequency and plausibility. The timing and necessity of surgery is controversial with different centers arguing for the conservative management of all cases while others argue for prompt exploration for all. Confusion also reigns over the need to fix the contralateral testis. In order to clarify the issue the authors reviewed the literature and found 18 case series of NTT, containing 268 operated cases suitable for analysis. This paper reviews the literature on NTT specifically regarding salvage rates and timing/necessity of surgery. Its primary aim is to produce an overall salvage rate in the operated group. Overall salvage rate was 8.96%, 24 testes. When operation is specified as an emergency, salvage may be as high as 21.7%. While salvage of a testis torted at birth is rare, it is reported. Early asynchronous torsion is also rare but reported. Worryingly, bilateral torsion can present with unilateral signs.Given these findings, we would suggest early surgery with fixation of the contralateral side.

Botulinum toxin in paediatric urology: a systematic literature review.

The use of botulinum toxin in paediatric urology has become increasingly widespread during recent years; however, the evidence base for its clinical use has been from adult urology centres and trials. The mechanism of action of this potent neurotoxin is discussed and evidence for its efficacy and safety is reviewed in both the adult and paediatric literature. Second, an electronic literature search was performed using the keywords paediatric urology, botulinum toxin, bladder, sphincter, and voiding dysfunction (excluding abstracts, letters and case reports). A total of 225 children in 10 peer-reviewed publications were evaluated with 165 paediatric patients with neurogenic overactivity (6 studies), 21 patients with idiopathic overactivity (1 study) and 39 patients with voiding dysfunction (3 studies). Specifically, we discuss the main indications of intravesical injections for detrusor overactivity and of intrasphincteric injections for refractory voiding dysfunctions in the paediatric population.

The role of dynamic magnetic resonance urography in complex neonatal hydrometrocolpos.

Urinary hydrometrocolpos is rarely diagnosed and is most often attributed to a persistent urogenital sinus or a cloaca. We report the case of a neonate in which the use of dynamic MRI allowed accurate preoperative diagnosis of urinary hydrometrocolpos secondary to ectopic drainage of a small left pelvic kidney, associated with a bicornuate uterus.

Vesical prolapse in an apparently "normal" bladder.

Bladder prolapse is extremely rare in children. Reports have been limited to pathologic bladder conditions such as the exstrophy-epispadias complex. We report an interesting presentation of bladder prolapse in a child with Williams syndrome with an apparently normal bladder.

Orchidopexy and its impact on fertility.

We critically analysed the factors known to influence the fertility potential after orchidopexy in children. A comprehensive literature review of all publications in the English language listed in Medline using the words cryptorchidism, undescended testis, orchidopexy, fertility, semen analysis and surgery from 1970 to 2005. In unilateral ectopic, canalicular and emergent testes, as long as the surgery is carried out in early childhood, fertility outcome is good (>90%). The majority of the bilateral abdominal testes are infertile. While unilateral abdominal testes and unilateral absent or vanishing testes have favourable fertility potential, quantification has proven difficult. Despite multiple studies, fertility in crypt orchid tests is still an uncertain issue. Hormonal treatment in conjunction with early surgical correction has not been fully explored. We recommend orchidopexy soon after 6-7 months of age, corrected for term, to maximise the future fertility potential.

The novel use of small intestinal submucosal matrix for chest wall reconstruction following Ewing's tumour resection.

Chest wall reconstruction following major tumour resection remains a challenging problem in the paediatric population due to the need for growth and development following the repair. Synthetic non-biodegradable grafts and materials have led to asymmetrical growth and significant functional and cosmetic compromise. The ideal graft material should be biological non-immunogenic which enables natural healing and facilitates further growth. Ewing's sarcoma is an uncommon primitive neuroectodermal tumour which rarely occurs in the ribs. However, recent improvements in management have produced a significantly improved survival; increasing the importance of chest wall reconstruction. We prospectively assessed the utilisation of Surgisis (an acellular collagen matrix) as a replacement graft in two children undergoing primary chest wall reconstruction following tumour resection. Surgisis is derived from porcine small intestinal submucosa (SIS) that serves as a template for tissue reconstruction and remodeling. The graft was well tolerated; there was no herniation and it allowed normal symmetrical chest movement and growth to occur. The properties of SIS allows natural collagen growth and compares favourably with marlex mesh and other forms of chest wall reconstruction. We believe its unique properties allow it to act as a suitable framework for chest wall reconstruction allowing chest wall growth and development.