Francisca Yankovic

Current practice in paediatric hypospadias surgery; A specialist survey

AIM To undertake an online survey of current hypospadias surgery practice among those specialists attending the IVth World Congress of the International Society for Hypospadias and Disorders of Sex Development (ISHID), 2011. MATERIALS AND METHODS An online survey covering 22 separate questions relating to proximal and distal hypospadias surgery was set up, and all delegates registered for the conference were invited to complete this questionnaire anonymously. The data was analysed by three of the authors. RESULTS A total of 162 delegates registered for the conference of whom 74% were paediatric surgeons, paediatric urologists, plastic surgeons and adult/adolescent urologists. 93 delegates completed the online survey, and most of them (57%) were from Europe. The majority of surgeons see over 20 new patients/year (90%) and perform primary hypospadias surgery in over 20 patients/year (76%). The tubularized incised plate (TIP) repair is the most frequent technique used for the management of distal hypospadias (59%); other techniques used included Mathieu, onlay and TIP with graft. A variety of techniques are used for proximal hypospadias, but nearly half of the respondents (49%) preferred a staged approach. Self reported complication rates for distal hypospadias surgery are favourable (less than 10%) for 78% of the respondents. However, proximal hypospadias complication rates are higher. CONCLUSIONS With a majority of paediatric urologists and European delegates responding to our survey, the results suggest that there are differences in the management of proximal and distal hypospadias between surgeons, yet no differences were observed according to the region of their practice. Variations in long-term outcomes appear to be in keeping with the current literature.

Surgical technique: Retroperitoneoscopic approach for adrenal masses in children

Laparoscopic adrenalectomy is considered to be the standard of care for the surgical excision of adrenal masses. The transperitoneal laparoscopic and retroperitoneoscopic approaches are described. Both are safe and as effective as open adrenalectomy, with the added benefit of the minimally invasive approach. It can be utilized for patients requiring surgery for a phaeochromocytoma, adrenal adenoma, adrenal adenocarcinoma, Cushings syndrome, neuroblastoma, and an incidentaloma. Relative contraindications include previous surgery of the liver or kidney, large tumours (>8-10 cm in diameter) or coagulation disorders. Although the transperitoneal route is used more widely, the retroperitoneal approach provides direct access to the adrenal gland and easy visualization of the adrenal vein. It avoids also colonic mobilization, minimizes the risk of injury to hollow viscera, and the potential risk of adhesion formation. However, the reversed orientation of the kidney and hilum, combined with a significantly smaller working space, may make this approach difficult to master.

Primary obstructive megaureter: Cutting balloon endo-ureterotomy

OBJECTIVE In this video we will demonstrate endo-ureterotomy using a cutting balloon for vesico-ureteric junction (VUJ) dilatation and stenting of the primary obstructive megaureter. METHOD For the technique, a 0.014 inch guide-wire is endoscopically inserted through the VUJ and allowed to curl in the megaureter. A 3 mm atherotome-bladed cutting balloon is inflated with iohexol contrast solution. Under fluoroscopy or cystoscopically, the stenotic VUJ segment is observed to open and post-dilated with a 4 mm simple balloon before JJ stent placement for six weeks. RESULTS This video demonstrates the equipment and technique of VUJ endo-ureterotomy using a cutting balloon and stenting of the primary obstructive megaureter. CONCLUSION Where intervention for the primary obstructive megaureter is indicated, we propose VUJ endo-ureterotomy as the first line treatment.

Paediatric cystolitholapaxy through the Mitrofanoff/Monti channel

INTRODUCTION Bladder calculi are a known complication of bladder augmentation. Open cystolithotomy remains the preferred option for treating large or multiple stones. Increasingly, however, minimal access techniques have been used. Reports of Mitrofanoff cystolitholapaxy are rare and have been limited to adults. This study presented a two centre series of children treated by cystolitholapaxy via the Mitrofanoff/Monti channel. MATERIALS AND METHODS With institutional approval the current study retrospectively reviewed and identified 14 patients, on a prospective database, who underwent Mitrofanoff cystolitholapaxy to treat bladder calculi at two independent institutions in the UK and Chile between 2004 and 2016. It looked at patient demographics, surgical technique, stone clearance and recurrence, as well as leak or catheterisation difficulties of the Mitrofanoff/Monti channel post-procedure. RESULTS Fourteen patients underwent Mitrofanoff cystolitholapaxy during the period 2004-2016. One patient was excluded due to lack of follow-up. The remaining 13 patients were aged 5-22 (median 14) years at the time of the procedure. Their underlying diagnoses were four neuropathic bladders, four bladder exstrophy, four cloacal exstrophy and one posterior urethral valve. Patients underwent augmentation cystoplasty at a median age of 5 (range 1-15) years, using ileum in 10 and sigmoid colon in three. The channel for clean intermittent catheterisation was an appendix Mitrofanoff in nine and a Monti channel in four. An Amplatz sheath was placed through the Mitrofanoff to allow safe access to the bladder for treating the stones (see Summary Table). Recurrent stones were treated using the same technique. Stone and channel outcomes were analysed for each procedure. There were 22 procedures in 13 patients; five (38%) patients had recurrent stones. Median time to recurrence was 6 months. There were no immediate complications. Stone clearance was confirmed by ultrasound and abdominal x-ray at 3-6 months after the procedure. Median follow-up was 15 (range 3-53) months. There were no leaks or difficulties catheterising the channel on follow-up. DISCUSSION This was the first series of Mitrofanoff/Monti cystolitholapaxy for the treatment of calculi in augmented bladders of paediatric patients. Previous concerns about damaging the continence mechanism of the conduit appeared to be unwarranted. The use of an Amplatz sheath protected the continence system from repeated instrumentation, and permitted free backflow of irrigation and rapid clearance of stone fragments. Recurrence of stones occurred in 38%, which was in keeping with rates reported in the wider literature. CONCLUSION Mitrofanoff cystolitholapaxy was safe, and with appropriate care did not result in leakage or difficulty catheterising.

Undescended Testis: Laparoscopic Fowler-Stephens Orchidopexy

Undescended testis is the most frequent congenital genitourinary anomaly, affecting 1–2 % of boys [1]. About 20 % of them will have an impalpable testicle (IPT) [1, 2], situated intra-abdominally in nearly half. In the remainder, the testis may be absent, may have atrophied, or may be hidden in the inguinal canal or fat [2, 3]. For patients with IPT, laparoscopy is the gold standard for diagnosis, and it allows the surgical treatment to be performed in the same setting [4]. The laparoscopic surgical options for the intra-abdominal testis include single-stage orchidopexy, single-stage Fowler-Stephens procedure (FSP), and two-stage FSP. Intra-abdominal testes with sufficient vessel length may be mobilised into the scrotum in one procedure, but the gonadal vessels are too short in the vast majority of patients. In 1959, Fowler and Stephens [5] described division of the testicular vasculature to aid mobilisation, thereby leaving the testes to rely on collateral blood supply along the vas deferens. Twenty-five years later, Ransley et al. [6] advised a two-stage procedure with an interval between vessel ligation and testicular mobilisation to allow time for enhancement of the collateral blood supply along the vas deferens. Laparoscopy for division of the vessels was introduced 20 years ago, and more recently, it has been used for mobilisation during the second stage [7–9]. The FSP has been demonstrated to be an effective and safe technique with reported success rates consistently above 80 %; the complications of testicular atrophy and ascent are documented in about 10 % and 5 %, respectively. A systematic review comparing single-stage and two-stage FSP concluded that the staged approach has a higher rate of success, with better testicular viability [10]. This chapter describes the surgical technique of first- and second-stage Fowler-Stephens orchidopexy.

Surgical technique: Utriculus masculinum excision by a laparoscopic-cystoscopic approach

OBJECTIVE A utriculus masculinum is encountered in 12% of hypospadias. However, patients rarely present with symptoms, such as post-void incontinence or infection. We describe a combined laparoscopic-cystoscopic approach to overcome the challenge posed by the location of the utriculus deep within the pelvis and to accurately identify the confluence of the utriculus with the urethra. METHODS A 12-year-old boy with Williams syndrome and previous hypospadias surgery presented with new-onset progressive day-time incontinence. Investigations (cystoscopy, urodynamics, MCUG) demonstrated a normal caliber urethra and the incontinence to result from a large utriculus masculinum. This video demonstrates the surgical technique: simultaneous utriculoscopy enabled the dome of the utriculus to be identified, laparoscopic dissection to progress under guidance of transillumination, and the urethral-utricular convergence to be clarified. RESULTS Histology demonstrated an inflamed utriculus with squamous metaplasia and cystitis glandularis. Symptoms resolved post-operatively and at 4 months follow-up, the patient remains dry by day. CONCLUSIONS A utriculus masculinum rarely requires excision. A combined laparoscopic-cystoscopic approach facilitates the delineation of the utriculus and allows its confluence to the urethra to be accurately determined.