Journal of Pediatric Urology最新文献

Introduction: The modified staged repair, or Toronto approach to reconstruct classic bladder exstrophy, involves bladder neck (BN) tailoring and bilateral ureteral reimplantation during primary closure, and later epispadias repair using external corpora rotation and a rotational penile skin flap. It aims to incorporate the advantages of complete primary repair while minimizing risks of upper tract deterioration and penile ischemia and improve cosmetic appearance of the genitalia. We present long-term outcomes for our initial patient series.

Methods: All patients with initial operation between 2000 and 2014 were reviewed. Data on demographics, continence, erectile and ejaculatory function, cosmetic appearance of the genitalia, and upper tract status were collected.

Results: Twelve male and four female patients were identified, with median follow-up of 12.7 (IQR 10.9-15.4) and 12.5 years (IQR 10.6-15.6), respectively. Full continence (voiding with no leaks, dry periods ≥3 h) was achieved in two of 12 males and two of four females. Five of 12 males and all four females had dry periods longer than 1 h. Nine of 12 males and all females attained volitional voiding. Three of 12 males and one of four females underwent additional continence procedures. None have undergone augmentation cystoplasty or bladder neck closure. Of seven males with preliminary sexual function data, all experienced erections, straight in five, with recurrent dorsal curvature and ventral curvature in one patient each. Four of seven ejaculate and none have attempted penetrative intercourse. All seven males reported satisfactory cosmetic appearance despite a subjectively shorter penis. Although transitory dilations of the ureters were seen immediately post op, none had scarring, hydronephrosis, or febrile urinary tract infections at latest follow-up. One patient had an eGFR on the upper range of CKD 2, while the rest of the cohort had eGFR ≥ 90 mL/min/1.73m². Mean bladder capacity on ultrasound was 145 mL for males and 97 mL for females.

Conclusion: The present data suggests that the modified staged repair of exstrophy (Toronto approach) is associated with acceptable continence outcomes while minimizing escalation to augmentation cystoplasty and bladder neck closure. Most patients void volitionally and stay dry for 1-3 h, but few are fully continent or able to remain dry for 3 h or more. No patients in the cohort had CKD3 or worse, and none had hydronephrosis or history of febrile urinary tract infections. Most males experience ejaculation and straight erections. There was no glanular or corporal tissue loss.

Background: We hereby report ventral flap aided glans urethral disassembly (V- GUD), a modification of GUD technique for distal hypospadias repair.

Methods/technique: Children with distal hypospadias and mobile meatus/urethra were selected. Ventral Mathieu-type perimeatal skin flap was marked around hypospadiac meatus and a stay stitch was placed to aid in distal urethral mobilisation by release of dartos bands holding it. Glans wings were raised and the urethra was disassembled from the glans. The neourethral meatus was fashioned at the advanced position in glans. An 8Fr silastic Foley's catheter was left indwelling for 7 days.

Results: Between 2021 and 2024, 58 boys with coronal hypospadias; median age 18 months (15-24 months), underwent this procedure. The mean operative time was 75 min (60-90). There were no urethrocutaneous fistula/glans dehiscence/meatal retraction. Three (5.1 %) developed meatal stenosis, who responded to meatal dilatation performed on an out-patient basis. Uroflowmetry in older children (n = 24) at 4-year follow-up showed bell-shaped curve with excellent paediatric penile perception score (95 %).

Conclusion: Our technique uses a ventral peri-meatal flap to hold the distal urethra during mobilisation, reducing mechanical trauma/vascular compromise to urethra. It has excellent functional and cosmetic outcomes in short-term. Larger studies with comparator are warranted.

Introduction: Duchenne muscular dystrophy (DMD), a severe, progressive disease, causes loss of function of skeletal muscles. Urological symptoms in DMD include detrusor overactivity, bladder hypocontractility, urinary incontinence, detrusor-sphincter dyssynergia and urolithiasis. This narrative review summarises available evidence and provides recommendations for bladder dysfunction management.

Materials and methods: A literature search was performed using the PubMed, Scopus, Embase, Cochrane and Web of Science search engines. The objectives of the review were: to investigate the incidence of lower urinary tract symptoms (LUTS), defecation problems, urinary tract infections (UTIs) and urolithiasis and the impact of urological problems on the quality of life of children with DMD. Following PRISMA guidelines and applying the Patient, Intervention, Comparison, Outcome (PICO) framework, nine non-randomised studies were selected, and MINORS criteria were used to validate their quality.

Results: All selected studies included DMD patients alone or in a mixed cohort with Becker Muscular Dystrophy (BMD) patients (DMD: 76-84 %). Diagnosis was mostly confirmed clinically. A single institute study design was noted for alle publications. Only one study incorporated short follow-up. Inclusion of a urologist in multidisciplinary teams was uncommon. Definitions and assessments of LUTS varied widely, and their prevalence ranged from 32 % to 85 %. Most frequently reported LUTS were hesitancy, urgency, day-time or night-time urinary incontinence and frequency. The prevalence of concomitant defecation problems ranged from 32 % to 70 %. Information on UTIs, urolithiasis, quality of life and intervention outcomes, for example, anticholinergics use was sparse.

Conclusions: DMD is a progressive muscle disease with urological complications. This review revealed a high prevalence of LUTS, including hesitancy, urgency, and incontinence, along with significant defecation issues. However, limited information is available on UTI, urolithiasis, quality of life, and treatment outcomes for these symptoms. Special attention is warranted for LUTS and bowel function. Multidisciplinary treatment including a paediatric urologist is beneficial.