Management Of Boys Research Articles

Introduction of a modified single stage reconstruction technique of male penopubic epispadias

ObjectiveTo represent the long-term outcomes of our modified single-stage technique for the reconstruction of isolated penopubic epispadias in male patients.Patients and methodsData from 113 patients were obtained from bladder-exstrophy-epispadias database of our tertiary center. A total of seven boys with isolated penopubic epispadias with no prior history of surgery and any other anomaly underwent our modified surgical approach from February 1997 to September 2019. The mean ± SD age at surgery was 6.5 ± 2.4 years. Volitional voiding status and cosmetic appearance were evaluated at each follow-up interval. Postoperative follow-up was performed at quarterly intervals in the first year and once a year in subsequent years.ResultsThe mean ± SD of follow-up was 8.5 ± 6 years. All boys who were incontinent achieved urinary control and the ability of normal transurethral micturition following the surgery. Four boys became completely dry, and the other three attained social dryness. Postoperative mean (SD) bladder capacity was significantly increased from 54.5 (11) to 124 (40.0) within 6 months, and to 194 (47.5) at 18 months after surgery. Dorsal curvature has been resolved in all cases, and no postoperative complications were noted except for surgical site infection in one patient treated with antibiotics and bilateral vesicourethral reflux resolved after injection of bulking agents. Four patients had normal erectile function and ejaculation, while the others have not reached puberty yet. Moreover, none of the patients developed urethrocutaneous fistula, stricture, or penile ischemia.ConclusionThe present findings suggest the safety and effectiveness of the combination of single-stage urethro-genitoplasty, bladder neck plication, and fat pad pedicled flap in management of boys with isolated penopubic epispadias that can lead to the achievement of urinary control, acceptable sexual function, and cosmetically satisfactory genitalia. Minimal morbidity, low complication rate, and promising outcomes are essential factors, supporting the notion of introducing this technique as a valid option for management of this entity.

Current practice and evolution of pediatric respiratory management of Duchenne muscular dystrophy in Canada

RATIONALE: Respiratory management practices for Duchenne muscular dystrophy (DMD) vary across Canada, according to a web-based survey conducted in 2010. Since then, new guidelines for respiratory management of DMD have been published. OBJECTIVES: This study aimed to describe current respiratory management practices of Canadian Pediatric Respirologists for children with DMD across the country. METHODS: A web-based survey on timing and indications for respiratory diagnostic tests and interventions was sent to all practicing Pediatric Respirologists at tertiary care centers in Canada who are involved in the longitudinal management of boys with DMD (N = 20). MEASUREMENTS AND MAIN RESULTS: Responses were received from 17 (85%) Pediatric Respirologists. Pulmonary function testing is regularly performed at least twice yearly after 5-6 years of age or after a patient becomes nonambulatory (13/17, 76%). Maximal expiratory pressures (15/17, 88%) and peak cough flow (12/17, 71%) are used to assess respiratory muscle strength. Sleep evaluation is most commonly performed with polysomnography (14/17, 82%), and is requested with the onset of symptoms (14/17, 82%) or with a forced vital capacity (FVC) < 60% predicted (6/9, 67%). At least once-daily lung volume recruitment exercises are prescribed by 94% (16/17) of respondents during periods of clinical stability, with the most common indication being an FVC < 80% predicted (9/17, 53%). CONCLUSIONS: The majority of Canadian Pediatric Respirologists are providing respiratory care to boys with DMD according to the current guidelines. The greatest change over time is the increased usage of regular LVR/airway clearance during periods of clinical stability.

Resisting the Demand to Stand

How sex-segregated bathrooms negatively impact trans, genderqueer, nonbinary, queer, and gender-nonconforming people has been extensively studied, yet few have considered how intersex people are subjected to bathroom violence. To begin broadening this conversation, I focus on the medical management of boys with the intersex variation hypospadias and demonstrate that anxieties around bathrooms extend beyond the bathroom walls—into surgical theaters—and are not simply a trans or queer issue. Anxieties about bathrooms and hegemonic urinary masculine behavior inform the violent medical maltreatment of intersex boys with hypospadias; they are subjected to shaming, disabling, and invasive procedures in the hope they will reinforce compulsory dyadism and able-bodiedness, as well as exhibit hegemonic heteromasculine behaviors, namely standing to urinate. Because of discriminatory, gratuitous surgical interventions, the bathroom and urination become sites of pain and trauma for these boys. In turn, these boys’ sense of masculine belonging are undermined or destroyed.

The management of boys under 3 months of age with an inguinal hernia and ipsilateral palpable undescended testis

AimsThe optimal management for boys under 3 months of age with an indirect inguinal hernia (IIH) and ipsilateral palpable undescended testis (IPUDT) is unknown. We aimed to: 1) determine the current practice for managing these boys across the UK, and 2) compare outcomes of different treatment strategies. MethodologyWe undertook two studies. Firstly, we completed a National Survey of all surgeons on the British Association of Paediatric Surgeons email list in 2014. Subsequently, we undertook a multi-centre, retrospective, 10-year (2005–2015) review across 4 pediatric surgery centers of boys under 3months of age with concomitant IIH and IPUDT. Primary outcome was testicular atrophy. Secondary outcomes included need for subsequent orchidopexy, testicular ascent and hernia recurrence. Data are presented as median (range). Chi-squared test and multivariate binomial logistic regression analysis were used for analysis; p<0.05 was considered significant. ResultsSurvey: Consultant practice varies widely across the UK, with a tendency towards performing concurrent orchidopexy at the time of herniotomy under 3 months of age. Concurrent orchidopexy is favored less in cases where the hernia is symptomatic.Case Series Review: Forty-one boys with 43 concomitant IIH and IPUDT were identified, and all included. 32 (74%) hernias were reducible, 11 (26%) were symptomatic requiring urgent or emergency repair. Post-conceptual age at surgery was 45weeks (36–65). Primary operations included: 29 (67%) open hernia repair and standard orchidopexy, 8 (19%) open hernia repair with future orchidopexy if required, 4 (9%) laparoscopic hernia repair with future orchidopexy if required, 2 (5%) open hernia repair and suturing of the testis to the inverted scrotum without scrotal incision. Variation in atrophy rate between different surgical approaches did not reach statistical significance (p=0.42). Overall atrophy rate was 18%. If hernia repair alone was undertaken (8 open and 4 laparoscopic), the testis did not descend in 8 patients, requiring subsequent orchidopexy (67%); if orchidopexy was undertaken at the time of hernia repair, 1 in 29 required a repeat orchidopexy (3%) (p=0.0001). No hernia recurred. ConclusionThis study suggests that orchidopexy at the time of inguinal herniotomy does not increase the risk of testicular atrophy in boys under 3months of age.

G.P.35 - The evaluation of bone age in patients with Duchenne muscular dystrophy on long-term glucocorticoid treatment

of muscle strength and function in DMD. However, they have many side effects; cushingoid features, obesity, growth retardation, behavioural issues, delayed puberty. Bone demineralisation and its associated increased risk of fracture to long bones and more notably lumbar vertebrae is a major issue. Bushby et al. proposed a set of recommendations for the care and management of boys with DMD. These recommendations were adopted by NICE in 2011. Part of those recommendations was that bone mineral density needs to be closely monitored. This would allow interventions to be implemented to preserve bone integrity and mitigate the risk of fracture. We present retrospective data on our DMD population; ‘time to first DEXA scan’ and ’12 monthly interval DEXA scans’ as recommended now by the guidelines; 18% did not have a DEXA scan within 6 months of starting glucocorticoids; 100% – 1 occasion of not receiving a DEXA scan within a 12 month period; 72% – 2 occasions over the 12 month threshold;18% – 4 occasions over the 12 month threshold; the longest period between DEXA scans was 4 years and the shortest 1 year and 4 months. These results will be compared to the non-fractured group pre-guidelines.

Is routine excision of testicular remnants in testicular regression syndrome indicated?

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. 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. 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 Fisher's exact test and a P-value of <0.05 was considered to be significant. 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. 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. 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.

Laparoscopic Orchidopexy without Division of Spermatic Vessels using a Neoinguinal Canal Approach: A Single Center Experience

Introduction: About 1 to 2% of boys at age of 1 year have an undescended testis (UDT); almost 20% of UDT are nonpalpable. Several surgical procedures have been described but there are no formal guidelines for the management of boys with nonpalpable testis. We report our experience with laparoscopic orchiopexy performed without dividing the spermatic vessels in this case series over a period of 4 years stating that the Fowler-Stephens technique is no longer indicated for the treatment of the intra-abdominal testis. Materials and methods: Over a period of 7 years from 2005 to 2012, we carried out diagnostic laparoscopy on 50 nonpalpable intra-abdominal testis in 45 boys followed by laparoscopic orchiopexy without sectioning the spermatic vessels even in cases where testis was higher up (30 cases). The technique consisted in sectioning the gubernaculum, opening the peritoneum laterally to the spermatic vessels, and mobilizing the testicular vessels and the vas deferens in a retroperitoneal position for 8 to 10 cm. The testis was then brought down into the scrotum through a neo-inguinal ring created just lateral to medial umbilical ligament. Results: Out of 45 cases with 50 undescended testis, 30 testes were abdominal, away from the internal ring out of which we were able to bring 28 testis in the scrotum without dividing the spermatic vessels, using a neo-inguinal ring. The other two patients had to undergo orchidectomy because of atretic testis. In the remaining 20 cases, the testis was at the inguinal ring or close to it and mobilized easily through the neo-inguinal ring to scrotum. The mean follow-up period has been 14 months (6 months to 2 years) and all the testes were found to be in scrotum with no atrophy. Conclusion: On the basis of our experience, we believe that laparoscopic orchiopexy without division of the spermatic vessels should be the treatment of choice in the management of nonpalpable testes because it does not compromise the normal testicular vascularization. Creation of neo-inguinal canal lateral to the medial umbilical ligament has the advantage of gaining more length on the vessels and vas to bring the testis to scrotum and hence Fowler-Stephens procedure is no longer routinely indicated in management of high abdominal testis.

Management of boys and men with disorders of sex development

This review of disorders of sex development (DSDs) in boys and men will outline the conditions that may lead to this phenotype, present some guidance on how to evaluate and investigate affected cases and then review the medical and surgical management and subsequent outcome. DSDs are a wide range of relatively rare conditions which need multidisciplinary input. The underlying cause is clearer in girls with DSDs, but the actual diagnosis remains unclear in the majority of boys with DSDs. There is a need to improve the diagnostic yield and develop standardized methods for assessing, describing and investigating DSDs as well as for reporting outcome. This will lead to improved clinical management and genetic counselling.

Comment on: Management of boys with abnormal appearance of meatus at circumcision for balanitis xerotica obliterans

Comment on: Management of boys with abnormal appearance of meatus at circumcision for balanitis xerotica obliterans

Management of boys with abnormal appearance of meatus at circumcision for balanitis xerotica obliterans

The aim of this study was to develop a standardised management plan for boys with abnormal appearance of meatus at circumcision for balanitis xerotica obliterans (BXO). Between 1995 and 2008, 107 boys underwent circumcision for BXO (confirmed on histology). Of these, 23 had abnormal appearance of the meatus at operation; their case notes were reviewed for age, presenting symptoms, management, outcome and follow up. The age range at operation was 3-15 years (mean: 9 years). Patients commonly presented with phimosis and balanitis. Seven patients had an additional procedure at circumcision: six had meatotomy, one had meatal dilatation. Thirteen were treated with topical steroid cream post-operatively. Eight of these (62%) subsequently required meatotomy. Three patients were observed and did not require further intervention. Meatotomy was required in 9 patients, 6-29 months after circumcision (mean: 11 months). Two patients required dilatation, including one with a previous intraoperative meatotomy, who required multiple dilatations. We propose the following standardised management plan: 1. With clinical evidence of BXO at circumcision, prepuce should be sent for histology. 2. If BXO is confirmed but the meatus appears normal, patients should be seen once post-operatively to give information about meatal stenosis. 3. When the meatus appears scarred with a narrowed lumen at operation, a meatotomy should be performed, with follow up for at least two years. 4. If the lumen is scarred but adequate, patients should be followed up in clinic for the same period for possible development of stenosis. 5. Topical steroid cream can be considered for voiding discomfort without decreased urine stream.

Posterior urethral valve

Posterior urethral valve (PUV) is a significant cause of morbidity, mortality and ongoing renal damage in children. It accounts for end-stage renal disease in a proportion of children. This article aims at highlighting the current trend in the management of boys with posterior urethral valve. PubMed/Medline and bibliographic search for posterior urethral valve was done. Relevant literatures on presentation, pathology, evaluation, management and outcomes of PUV were reviewed. PUV which is increasingly diagnosed prenatally presents a spectrum of severity. The varied severity and degree of obstruction caused by this abnormality depend on the configuration of the obstructive membrane within the urethra. The decision to intervene prenatally is dependent on gestational age, amniotic volume, and renal function of fetal urine aspiration. Identification of the patients who may benefit from early intervention remains inconclusive. Endoscopic ablation of the valve is the gold standard of treatment but use of Mohan's valvotome and other modalities are invaluable in developing countries where endoscopic facilities are limited. Proximal urinary diversion may result in poor bladder compliance and should be reserved for patients with persisting or increasing upper urinary tract dilatation, increasing serum creatinine or inappropriate instruments. The behavior of the bladder and its subsequent management after valve ablation may influence the long-term renal outcome in PUV patients. The care of children with PUV continues to improve as a result of earlier diagnosis by ultrasound, developments in surgical technique and meticulous attention to neonatal care. The ultimate goal of management should be to maximize renal function, maintain normal bladder function, minimize morbidity and prevent iatrogenic problems.

Management of boys with nonpalpable undescended testis

Cryptorchidism is one of the most common genitourinary disorders in young boys. Although the management of boys with palpable testis is standardized, there are no formal guidelines for the management of boys with nonpalpable testis. In this Review we look at the current trends in the diagnosis and treatment of this disorder, as well as the indications for therapy and surgical procedures. On the basis of current evidence, we find that there is no optimum orchidopexy technique for the treatment of intra-abdominal testis, although it is preferable to adopt techniques that preserve the spermatic vessels. We also briefly examine the follow-up of patients with this disorder and its common complications. As yet, there are no data that assess the potential of laparoscopic orchidopexy being a risk factor for impaired fertility later in life.

A review of urodynamic evaluation in children and its role in the management of boys with posterior urethral valves

Posterior urethral valves are the commonest cause of lower urinary tract outflow obstruction in male infants with an estimated incidence of 1:5000 male infants and 1:25,000 live births. Despite treatment with fulguration of the obstructing valves, bladder function is abnormal in up to 70% of older children and adolescents. Bladder dysfunction causes morbidity e.g. urinary incontinence and has been implicated in the late deterioration of renal function in this population. A poor understanding and inappropriate management of bladder dysfunction can result in unnecessary morbidity, which can handicap a child for life. Any method that measures function or dysfunction of the lower urinary tract constitutes a urodynamics investigation. Broadly, the investigations can be classified into simple or noninvasive urodynamics and invasive urodynamics. The objective of urodynamics assessments in children is to reproduce the patient's complaints or symptoms. Video urodynamics can provide additional information that may contribute to a further understanding of the problem under investigation. Urodynamics provides a useful tool to test the efficacy of treatment as well as determine any refinements necessary to improve the outcome of such treatment.

The ascending testis: is late orchidopexy due to failure of screening or late ascent?

It is widely recommended that infant boys with undescended testes (UDT) should be referred for surgical opinion before the age of 18 months. To attempt to identify the reasons why the mean age at orchidopexy in our institution was as high as 5.5 years, a retrospective review of the screening history, examinations, and management of boys over the age of 3 years at the time of orchidopexy was undertaken by reference to community, general practitioner, and hospital records. In 36 children where hospital and community records giving information prior to referral were available, the UDT had previously been documented on at least one occasion as descended in 24 children or retractile in 10. In 1 child there was delayed referral, and in another, there was operative delay. It is concluded that there is either frequent failure of the Child Health Surveillance screening programme, or that late ascent of a testis previously sited in the scrotum is a common occurrence. We recommend further prospective studies to clarify this latter phenomenon.

A study of clinical opinion and practice regarding circumcision

AIMTo establish clinical opinion regarding appropriate indications for circumcision and to examine actual clinical practice.METHODSA questionnaire was sent to all NHS hospital consultants in the Yorkshire region of the UK...

Molecular detection of acute lymphoblastic leukaemia in boys with testicular relapse.

AIMS: To determine the role of polymerase chain reaction (PCR) based minimal residual disease (MRD) detection of leukaemia specific DNA in testicular relapse in childhood acute lymphoblastic leukaemia. METHODS: DNA...

Bilateral undescended testes.

The management of boys with undescended testes appears to be an increasingly common problem. While Scorer (1964) [11] reported an incidence of 0.8% of undescended testes in boys of one year of age, a recent survey (Radcliffe Hospital, Oxford, 1986) [18] suggests the incidence has risen to as high as 1.6%. While the etiology may be varied, it is quite clear that many cases represent disturbances of the hypothalamic-pituitary-gonadal axis and may represent a forme fruste of hypogonadotropic hypogonadism [3]. Bilateral undescended testes might logically appear to represent the worst end of such an endocrinopathy. Indeed, a review of the experience at The Children's Hospital of Philadelphia [12] of boys with bilateral undescended testes indicated a greater number of CNS abnormalities (P = 0.0000) than in boys with unilateral undescended testes (Table 1). Also consistent with bilateral cryptorchid testes representing the most severe end of the spectrum was the reported findings from that study that bilateral undescended testes also exhibit a significantly greater number of associated G-U anomalies (P = 0.0004).

The Treatment and Management of Duchenne Muscular Dystrophy at Regency Park Centre, South Australia

This paper describes the treatment and management of boys with Duchenne Muscular Dystrophy at the Regency Park Centre for Young Disabled in Adelaide, South Australia. We have not been convinced that some of the active management programmes advocated else-where are effective. Our philosophies and practices reflect a relaxed environment which emphasises achievement and freedom of choice.

The ureter in posterior urethral valves.

Summary A main problem in the management of boys after ablation of posterior urethral valves is the diagnosis of ureteric obstruction. An accurate diagnosis of obstruction is essential if unnecessary operations are to be avoided and intravenous and retrograde urography in this respect have their limitations. Perfusion studies as discussed here have proved valuable and the results of 17 studies in 12 boys illustrate that there is a wide variation in degrees of obstruction which often correlate poorly with urographic assessment. These studies have helped to plan a more logical approach to ureteric surgery in these boys.