ESPU Meeting on Friday 21, April 2023, 12:05 - 12:45
Peter CUCKOW, Kevin CAO, May BISHARAT, Satej MHASKAR, Sharon MOHAN-KUNNATH, Ganesh VYTHILINGAM, Karen RYAN and Navroop JOHAL
Great Ormond Street Hospital for Children, Urology, London, UNITED KINGDOM
Since 1999, consecutive patients with classic bladder exstrophy were treated by new-born primary bladder closure and Kelly soft tissue reconstruction in their second year. We report continence outcomes at five, ten and fifteen year-age milestones.
MATERIAL AND METHODS
118 new-borns had a primary closure without osteotomy (reserved only for those 7% requiring a secondary closure). All had a Kelly operation, irrespective of bladder size and/or prior complications. This was: i) bilateral Cohen ureteric re-implantation ii) full mobilisation of the pelvic floor and the penile/clitoral corpora with preservation of the pudendal pedicles iii) anatomical reconstruction of the bladder neck, urethra and sphincter iv) reconstruction of the penis and correction of chordee in males (+/- hypospadias) or midline creation of a mons pubs and hemi clitori in females.
Prospective follow up with nurse bladder function assessments, ultrasounds and clinical review. Continence scored using our continence scale.
By 5 years/school age (98 patients), 43% have been dry by day and 10% at night. Of 93 patients seen at 10 years of age, 61% have been dry by day and 27% also at night. Of 57 patients followed to 15 years, 83 % have been dry by day and 65% at night. Over 15 years, daytime continence rose to 86% and night time to 81%.
This Kelly operation enables normal daytime continence in over 40% of patients by the time they reach school age. Increasing continence is seen through childhood with most (>80%) attaining day and night time continence with puberty.
Hortense ALLIOT 1, Sébastien FARAJ 1, Thomas LOUBERSAC 1, Guillaume MEURETTE 2, Stephan DE NAPOLI COCCI 1 and Marc-David LECLAIR 1
1) CHU de Nantes Hôpital Mere Enfant, Pediatric Surgery Department, Nantes, FRANCE - 2) CHU de NANTES, Hotel Dieu, General Surgery Department, Nantes, FRANCE
During one-stage delayed Kelly's Radical Soft Tissue Mobilization (RSTM) for Bladder Exstrophy Epispadias Complex (BEEC), children underwent a pelvic floor muscles detachment. The aim of this study was to evaluate the impact of this procedure on anorectal function in children.
MATERIAL AND METHODS
Monocentric controlled study of prospectively collected data of 27 children who underwent RSTM for BEEC from 2010 to 2017, over 5 years, and 81 healthy children paired on age and sex. Patients born after 2017 were not included because under theoretical age of continency acquirement.
Fecal function was assessed using the Childhood Bladder and Bowel Dysfunction Questionnaire, and quality of life (QoL) related to fecal continence using the CINCY FIS questionnaire. Control group was paired on age and sex with a 1:3 patient/control ratio. Answers to questionnaires were collected from September 2021 to January 2022. Statistical analysis were descriptive and univariate comparing the two groups. Sub-group analysis following age were also performed.
During the period of study, 55 children with BEEC underwent Kelly's RSTM. Twenty-seven (49%) were included and paired with 81 healthy children on age and sex. Median (IQR) age at surgery was 15 months (8.5-50), with median (IQR) follow-up of 10 years (6-11). There was no difference between patient's and control group in anorectal function for both incontinence and constipation items. No significant difference was found in QoL related to fecal incontinence assessment. Sub-group analysis did not show difference.
This study suggests that the levator ani detachment during Kelly's procedure, realized in a pediatric population under the age of 8, did not impact anorectal function with a mid-term follow-up.
Irene PARABOSCHI 1, Dario Guido MINOLI 1, Erika Adalgisa DE MARCO 1, Michele GNECH 1, Giovanni PARENTE 2, Filippo MOLINARI 1, Carolina BEBI 1, Gianantonio MANZONI 1, Marc-David LECLAIR 3 and Alfredo BERRETTINI 1
1) Fondazione IRCCS Ca Granda -Ospedale Maggiore Policlinico, Pediatric Urology, Milan, ITALY - 2) Fondazione IRCCS Ca Granda -Ospedale Maggiore Policlinico, Pediatric Surgery, Milano, ITALY - 3) CHU Nantes, Pediatric surgery, Nantes, FRANCE
The vascular supply of the pelvic structures and the external genitalia can be easily injured during the one-stage delayed bladder closure and radical soft-tissue mobilization (Kelly procedure) for bladder exstrophy/epispadias (BEE) repair. Aiming to help surgeons assessing and confirming the tissue perfusion and viability, indocyanine green (ICG)-based laser angiography was incorporated into the operative approach to reduce the risk of ischemic injuries.
MATERIAL AND METHODS
The EleVision IR system (Medtronic Ltd) was adopted to confirm the identification of the vascular pedicles and assess the tissue perfusion in real-time in a 5-month-old with BEE undergoing the one-stage delayed bladder closure and radical soft-tissue mobilization (Kelly procedure). ICG (0.15 mg/kg) was intravenously administered at 6 key steps during surgery with the ability to be re-dosed every 15 minutes.
ICG-based laser angiography helped to confirm the correct identification of the vascular structures during surgery and to assess tissue perfusion in real-time. Blood flow did not change to any significant degree either after initial dissection or upon approximating the pubis symphysis. At the end of the procedure, a good penile perfusion was shown, proving that no direct injury or significant compression of the pudendal vessels had occurred following the mobilization and the reconstructive phase.
ICG-based laser angiography proved to be safe, effective, and easy to employ and should be considered as a reasonable adjunct for tissue perfusion assessment and operative decision-making in patients undergoing BEE Kelly repair.
Ted LEE, Briony VARDA, Alyssia VENNA, Richard LEE and Joseph BORER
Boston Children's Hospital, Urology, Boston, USA
Bladder neck reconstruction (BNR) aims to alleviate persistent incontinence after initial bladder exstrophy (BE) closure. The objective of this study was to compare outcomes of two different BNR techniques: Young-Dees-Leadbetter (YDL) technique versus modified BNR (mBNR) techniques.
MATERIAL AND METHODS
We identified BE patients undergoing BNR from 2002 to 2021. Continence outcomes included obstruction (not voiding spontaneously), controlled voiding (no pad), intermittent leakage (pad usage), and complete incontinence (no dry periods). YDL technique included detrusor wrap of the bladder neck while mBNR included a more limited dissection of the mucosa and single anterior midline tubularization of mucosa and detrusor.
22 BNRs were performed: 10 YDL and 12 mBNR. Post void residual is significantly higher in YDL group (83.3 vs 18.8mL, p=0.01). Continence outcomes are outlined in table. Notably, 50% of YDL group do not void spontaneously postoperatively while all patients who underwent mBNR are voiding spontaneously. Three patients in YDL group required additional surgeries for de novo bladder stones while none of mBNR patients formed bladder stones. 4 YDL patients underwent concomitant continent catheterizable conduit while 1 YDL patient required continent catheterizable conduit at later date for obstruction.
|YDL (n=10)||mBNR (n=12)||p-value|
|Preoperative percentage of expected bladder capacity||54%||49%||0.10|
|Intraoperative continent catheterizable conduit||4(40%)||0(0%)||0.03|
|Intraoperative bladder neck width||14.4mm||20.0mm||<0.0001|
YDL technique is associated with higher rate of obstruction compared to mBNR technique. Although this may result in improved continence compared to the mBNR technique, it comes at the cost of increased reliance on creation of continent catheterizable conduit, CIC, and higher rate of bladder stone formation.
Rafal CHRZAN 1, Barbara DOBROWOLSKA-GLAZAR 1, Ireneusz HONKISZ 1, Janusz SULISLAWSKI 1, Michal WOLNICKI 1, Aneta PIOTROWSKA-GALL 1 and Tom Pvm DE JONG 2
1) Jagiellonian University Medical College and University Children's Hospital Krakow, Pediatric Urology, Krakow, POLAND - 2) Wilhelmina Children's Hospital UMC Utrecht, Pediatric Urology, Utrecht, NETHERLANDS
To present a novel technique of the bladder neck suspension for the 1 stage female epispadias repair and to assess the primary outcome
MATERIAL AND METHODS
Between 2016 and 2021 4 girls had epispaadias repair as described by de Jong et al in 2000. The procedure begins with a U-shape incision around the external meatus and the urethral plate. The plate is freed and tubularized around a 10 Fr silicone catheter. Then the bladder neck is released from the pelvic floor muscules and the posterior surface of the symphysis. The port-site closure device with a blunt stylet mechanism is inserted through a suprapubic incision and introduced towards the bladder neck. A polyglactin suture is attached to the bladder neck, pulled out by means of the device and fasten to the fascia of the rectus muscle. Finally, reconstruction of the clitoris with construction of a clitoral hood and mount veneris are done. The catheter is left for 2 weeks.
Two girls were operated at the age of 1 year, 1 girl at 3.5 years and 1 at 4 years. The operative time was between 130 and 160 minutes. In one girl the bladded catheter had to be repositioned on the first day due to its displacement. Three patients are already toilet trained are 2/3 are dry day and night. One out of those 2 developed an external meatal stenosis and required meatoplasty and meatus dilation with a good final outcome. One girl was still wet during and she was lost for the follow up. One girl is less than 2 years old and not yet eligible for assessment of continence.
One stage technique for female epispadias repairs results in a reasonable success rate in this small series. A port-site closure devise is an easy and safe way to complete the suspension of the bladder neck during the procedure.
Nora HANEY, Ahmad HAFFAR, Christian MORRILL, Chad CRIGGER, Andrew GABRIELSON, Logan GALANSKY and John P GEARHART
Johns Hopkins Hospital, Urology, Baltimore, USA
Cloacal exstrophy (CE) is the most severe malformation of the exstrophy-epispadias complex. This study uniqurely aims to discuss long-term problems in a single major institution with a high volume of CE patients.
MATERIAL AND METHODS
A prospectively maintained database was reviewed for CE patients with >10 years of follow-up. Urinary, renal, gastrointestinal, orthopedic, psychosocial, and independence attributes were evaluated.
Out of 149 CE patients followed, there were 63 patients who met inclusion. Median age was 20.9 years [10.2-59.3]. Thirty-seven (58.7%) were >18 years. Twenty-one (33.3%) were born female and 39 (61.9%) born male, 14 of whom were gender converted at birth. Gender identity was self-reported 26 males, 36 females, and 1 non-binary. There were two deaths, one cancer and another associated with ESRD. Two females conceived naturally, and two patients adopted.
Catheterizable channels were the most common method of voiding (45/63, 71.4%). Of those, 88.9% (40/45) were continent. Forty-six patients (73.0%) had no CKD while 4 (6.3%) progressed to renal replacement therapy (RRT). Gastrointestinal diversion was managed by colostomy (37/63, 58.7%) and ileostomy (17/63, 27.0%). Most patients underwent osteotomy (47/63, 74.6%). Thirty-eight percent (24/63) required a wheelchair. Psychosocial diagnoses included 19/63 (30.2%) patients with anxiety and/or depression and 17/63 (27.0%) patients with chronic pain. Out of the 52 patients who were evaluated by physical therapy, 46 (88.5%) were independent. Two patients (3.2%) had cognitive delay.
Improvements in intensive care, gastrointestinal, orthopedic, and urologic management have resulted in survival rates approaching 100%. Yet CE children face long-term problems across multi-disciplinary fields.