Feilim Murphy

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.

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.

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.

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.

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.

Is routine excision of testicular remnants in testicular regression syndrome indicated

BACKGROUND Undescended testicles are a common finding in full-term male infants. In the majority of these infants, the testicle spontaneously descends in the first year of life. However, in others, it remains impalpable in an abnormal position or there may only be a small abnormal testicular remnant present. For these infants there is still controversy surrounding inguinal exploration and/or excision of these testicular remnants at the time of operative intervention. The controversy centres on their potential future malignant potential. AIM The aim of the study was to ascertain the incidence of the presence of either germ cells (GCs) or seminiferous tubules (SNTS) in the excised testicular remnants. This was performed at a paediatric surgical tertiary centre and contributes to the evidence base for this condition. METHOD A retrospective data analysis occurring over a 15-year period of all excised testicular remnants. The testicular remnants were analysed for age, laterality, histological analysis and clinical diagnosis. Subset analysis included subdivision into both intra-abdominal or inguinal positions, and age ranges. Statistical analysis was using Fishers exact test and a P-value of <0.05 was considered to be significant. RESULTS A total of 140 paediatric male patients were identified as having had a testicular remnant excised during the study period. Their demographics and also the main results are summarised in the overall summary Table. The mean age at intervention was 3.5 years (range: 3 months to 17 years). A total of 132/140 of the boys underwent excision of an inguinal testicular regression syndrome (TRS) remnant and 8/140 an intra-abdominal remnant. Comparison of these two groups revealed no significant difference for the presence of GCs (12 (9%) vs 2 (25%), P = 0.18). However, intra-abdominal TRS remnants were much more likely to contain SNTs (27 (21%) vs 7 (88%), P = 0.0002). There was no decreased incidence of either GCs or SNTs with increased patient age. DISCUSSION The main reason for the debate over the management of boys with TRS is the variable incidence of viable germ cells reported in different studies: it has been reported between 0 and 16%. The incidence of GCs (10%) and also SNT (24%) in the present series therefore contributes to this evidence base and is in the middle of this range. It is still unclear as to whether these remnants have a future malignancy risk, as there is only one case of intratubular germ cell neoplasia (ITGCN) in a testicular remnant reported in the literature and this was not immunohistochemically supported. The presence of ITGCN, although considered as a precursor to the development of a testicular germ cell tumour in adult patients, has also not been established in paediatric patients. The natural history of the GCs in TRS specimens is also unknown. In the present series, however, there was no decreased incidence demonstrated with increased patient age, although older patient numbers limited this subset analysis. Despite this controversy, as these patients were already under general anaesthetic, an inguinal exploration and excision of any TRS remnant that was present did not significantly increase the operative procedure or time, and removed any potential malignancy risk. CONCLUSION This evidence supports the exploration and excision of inguinal testicular remnants, as one in ten boys have GCs present and one in four have SNTs, which may have a potential future malignant transformation risk.

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

BackgroundThe 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.PurposeIn order to clarify the situation in our centre, we reviewed our own indications for nephrectomy over a 20-year period.MethodsMedical 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.ResultsA 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.ConclusionsThis 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.

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.

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.

Response to letter to the editor re ‘Is routine excision of testicular remnants in testicular regression syndrome indicated?’

We read the response to our article from our esteemed colleagues with gratitude, as one of the main reasons for the publication of our series was to initiate debate within the paediatric surgical and urological community about this controversial topic. We are also grateful for the opportunity to answer their concerns. For the first concern that they raise about the lack of adult urological case reports, we couldn’t agree more, as we state in our discussion: ‘It is still unclear as to whether these remnants have a future malignancy risk, as there is only one case of intratubular germ cell neoplasia (ITGCN) in the literature and this was not immune-histochemically supported’. For their second concern there are a number of different points raised. The first is the use of an inguinal approach for our explorations. In all of our patients there was a clinically impalpable testicle pre-operatively; we feel this is different from the subgroup who have a scrotal remnant that can be palpated and who may indeed not even undergo surgical intervention. The scrotal approach to an orchidopexy has been described with great results for many years, and so the use of a scrotal approach for testicular regression syndrome (TRS) exploration is probably a valid approach, just not one that we use. The second is that all TRS specimens are within the scrotum, as they are secondary to a perinatal torsion event. We agree that the descended testicle does not become fixed in the scrotum until 2e4 months postnatally, and we have discussed this in our manuscript. The prenatal torsion theory for TRS is supported by the presence of haemosiderin-laden macrophages in the resected specimens, but it is worth noting that this is not the only proposed mechanism. In our series, 35% of specimens had this histological finding, including some of