ESPU Meeting on Thursday 18, April 2024, 10:00 - 11:00
10:00 - 10:03
S08-1 (OP)
Paul MERGUERIAN 1 and Margarett SHNORHAVORIAN 2
1) Seattle Children's Hospital, Division of Urology, Seattle, USA - 2) Seattle Children's Hospital, Urology, Seattle, USA
PURPOSE
Clinical Standard Work (CSW) aims to improve quality of care through standardization based on evidence in medical literature and/or expert opinion. We hypothesize that implementing such a pathway for bladder exstrophy patients at our institution will improve outcomes.
MATERIAL AND METHODS
A comprehensive literature search was performed. Of 777 records screened, 15 papers were included in pathway development by a multidisciplinary group. High volume bladder centers were also surveyed regarding management. We compared pre implementation outcomes data for 22 patients from 2014-2018 to post implementation data for 20 patients from 2018-2023
RESULTS
CSWprotocols were developed spanning prenatal diagnosis, neonatal admission, pre-operative evaluation, intra operative management and post operative care. Average hospital stay decreased from 7.6 days to 5 days. Thirty day readmission rate decreased from 30% to 10.5%. Laboratory costs decreased from an average of $2951 to $1559. Pharmacy costs were reduced from $7646 to $4849. Moreover newborns with bladder exstrophy were successfully managed at their birthing institutions without transfer to a tertiary care center.
CONCLUSIONS
Care of bladder exstrophy patients can be standardized using evidence based and best practice pathways. These pathways decrease variation, improve patient education and result in decreased hospital stay, decreased readmissions and decreased cost.These pathways can be updated regularly based on available new evidence such as adding Enhanced Recovery Protocols that have been recently implemented in the pathway
10:03 - 10:06
S08-2 (OP)
Martin PROMM 1, Raphael HOFBAUER 2, Roland BRANDL 3, Christopher GOSSLER 4, Susanne BRANDSTETTER 5, Marco J. SCHNABEL 4, Michael KERTAI 6 and Wolfgang H. RÖSCH 7
1) Klinik St. Hedwig, Paediatric Urology, Regensburg, GERMANY - 2) Hospital St. Marien,Amberg, Germany, Pediatric Orthopedics and Neuroorthopedics section-Clinic for Orthopedics, Regensburg, GERMANY - 3) Clinic St. Hedwig, University Medical Center of Regensburg, Department of Radiology, Regensburg, GERMANY - 4) University of Regensburg, Caritas St. Josef Medical Center, Regensburg, Germany, Department of Urology, Regensburg, GERMANY - 5) University Children's Hospital Regensburg (KUNO) of the University of Regensburg and the Order of St. John at the St. Hedwig Hospital, Regensburg, Germany, Regensburg, GERMANY - 6) Hospital St. Marien,Amberg, Germany, Department of Pediatric Surgery and Orthopedics, Amberg, GERMANY - 7) Clinic St. Hedwig, University Medical Center of Regensburg, Department of Pediatric Urology, Regensburg, GERMANY
PURPOSE
To compare the orthopedic long-term outcome after primary bladder exstrophy(BE) repair with approximation of the pubic symphysis without osteotomy in immediate and delayed bladder closure.PATIENTS AND METHODS
From 03/2018-12/2020 individuals with primary exstrophy repair(PER) and approximation of the symphysis without osteotomy were recruited prospectively. Patients <12 years and surgery in the bony pelvis or lower extremities were excluded. Orthopedic examinations were performed by two pediatric orthopedists. MRI (T1- and T2-wheigted) of the bony pelvis including the hip joints was performed and pubic diastasis, the acetabulum angle(ACA) and the centre-edge-angle(CEA) were evaluated by a pediatric radiologist.RESULTS
A total of 29 patients were included, 11 had primary neonatal bladder closure and 18 a delayed procedure. Between the two groups no significant differences could be observed concerning the patient reported orthopaedic outcome at last follow-up, including hip pain (p=0.419), mobility impairment (p=0.543) and sports impairment (p=0.543). Furthermore, there were no significant differences with regard to the measurable orthopaedic endpoints, including hip impingement (p=1.000), leg length discrepancy (p=0.505) and width of the pubic diastasis as measured by MRI (p=0.401). Mean pubic diastasis was 47.69 mm (SD18.499 mm) for the entire cohort, 52.38 mm (SD14.793 mm) in the post-partum PER group and 45.61 (SD19.956) in the delayed PER group. There was also no significant differences with regard of CEA right (median30°, p=0.976),CEA left (median31.5°,p=0.420),ACA right (median19°,p=0.382) and ACA left (median 17°,p=0.880).CONCLUSIONS
Clinical, orthopedic and radiological long-term outcome after neonatal and delayed bladder closure was similar in our cohort. This confirms that delayed bladder closure without osteotomy is feasible and successful.10:06 - 10:09
S08-3 (OP)
Joshua ROTH 1, Diana BOWEN 2, Molly FUCHS 3, Patricio GARGOLLO 4, Harrison GOTTLICH 4, David HAINS 5, Andrew STRINE 6 and Konrad SZYMANSKI 5
1) Riley Children's Health at Indiana University, Urology, Indianapolis, USA - 2) Lurie Children's Hospital, 60611, USA - 3) Nationwide Children's Hospital, Columbus, USA - 4) Mayo Clinic, Rochester, USA - 5) Riley Children's Health at Indiana University, Indianapolis, USA - 6) Cincinnati Children's Hospital, Cincinnati, USA
PURPOSE
Some with classic bladder exstrophy (CBE) experience renal deterioration in adulthood. Little is known about the incidence of end-stage and chronic kidney disease (ESKD/CKD) in this population. Our aim was to describe the incidence of ESKD and prevalence CKD in a CBE cohort.
MATERIAL AND METHODS
We retrospectively reviewed records of patients with CBE followed at five tertiary care centers. The primary outcome was incidence of ESKD, defined as permanent peritoneal/hemodialysis or renal transplantation. The secondary outcome was prevalence of CKD stage 3 or higher (CKD3+, estimated glomerular filtration rate [eGFR]<60ml/min/1.73m2). Creatinine-based eGFRs were calculated using CKD-EPI (adults) and Schwartz (children). Survival analysis and Fisher's exact test were used.
RESULTS
A total of 201/216 patients (93%) had renal function data available (63% male). 197 patients had a primary bladder closure. At a median of 18.8 years old, 12 were diverted and 108 were augmented. Three developed ESKD (1.5%) at a median age of 23.4 years (1 hemodialysis, 2 transplantation). On survival analysis, the risk of ESKD was 0% at 10 years, 1% at 20 years and 5% at 30 years, higher than the 0.003% risk at 21-year-olds in the general population (p<0.001). Median age of 147 individuals with eGFR data was 20.5 years old (65% male). One child (2%) and 8 adults (8%) had CKD3+ (p=0.16).
CONCLUSIONS
The risk of ESKD among patients with CBE appears to be more common than the general population. Reliable long term follow up is needed in this population to monitor for ESKD.
10:21 - 10:24
S08-4 (OP)
Irene PARABOSCHI 1, Gianluca SAMPOGNA 2, Massimo DI GRAZIA 3, Dario Guido MINOLI 1, Michele GNECH 4, Erika Adalgisa DE MARCO 1, Francesca MITZMAN 5, Gianantonio MANZONI 5, Waifro RIGAMONTI 6 and Alfredo BERRETTINI 1
1) Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Pediatric Urology, Milano, ITALY - 2) ASST Grande Ospedale Metropolitano Niguarda, Spinal Unit, Milano, ITALY - 3) Infermi Hospital, AUSL Romagna, Rimini, ITALY - 4) Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinicoione, Pediatric Urology, Milano, ITALY - 5) Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Pediatric Urology, Milano, ITALY - 6) Urology Clinic-Pediatric Urology Unit, Department of Surgery, Oncology and Gastroenterology, Padova, ITALY
PURPOSE
To examine long-term psychosocial and psychosexual outcomes of adult patients with classic bladder exstrophy (BE).
MATERIAL AND METHODS
The validated Sexrelation Evaluation Schedule Assessment MOnitoring (SESAMO) questionnaire was used to assess the psychosocial and psychosexual adjustment of adult BE patients. Section I investigated domains common to all patients, section II singles, section III couples. Z-scores were calculated for each item and compared in relation to patients’ gender, relationship status, and the voiding technique used to empty the bladder.
RESULTS
A total of 33 (F:M 12:21; singles:couples 11:22) BE patients were enrolled in the study at a median age of 39 (32-47) years. The results of the questionnaire showed mild to moderate dysfunctions in all the domains investigated, with no significant differences between the different voiding techniques used to empty the bladder. Lower z-scores were recorded for psychosexual identity (z-score:-1.282), areas of pleasure (z-score:-0.915) and desire (z-score:-0.583); singles for relational attitude (z-score:-1.751) and imaginative eroticism (z-score:-0.806); couples for extramarital sexuality (z-score:-1.175) and communicativeness sexual sphere (z-score:-0.524). Overall women performed significantly worse than men regarding psychosexual identity (p-value: <0.0001) and sphere of pleasure (p-value: <0.001), single women on present masturbation (p-value: <0.05), single men on relational attitude (p-value: <0.05), coupled women on present masturbation (p-value: <0.05), coupled men on sexual intercourses (p-value: <0.01).
CONCLUSIONS
Several psychosocial and psychosexual outcomes were affected in BE adults, regardless of the voiding technique used to empty the bladder. A long-term psychosexuological follow-up is required to help them cope with their past medical experience and actual clinical condition.
10:24 - 10:27
S08-5 (OP)
Sarah ABDELLAOUI 1, Nicolas MOREL JOURNEL 2, Fabiana CAZZORLA 3, Valeska BIDAULT 1, Pierre MOURIQUAND 1 and Delphine DEMEDE 1
1) Hospices Civils of Lyon, Pediatric Surgery, Lyon, FRANCE - 2) Hospices Civils of Lyon, Urology, Lyon, FRANCE - 3) University of Grenoble-Alpes, Infectious disease control Departement, Grenoble, FRANCE
PURPOSE
Genital reconstructive surgery and the aesthetic appearance of the external genitalia (EG) may affect classic bladder exstrophy (CBE) patients sex life. To understand surgical outcomes, standardised sexuality scores are necessary. We sought to assess sexual outcomes and fertility in CBE patients using validated questionnaires.
MATERIAL AND METHODS
QUALEXSTRO was a retrospective, non-interventional, single-center cohort study of 15 years and older CBE patients using questionnaires: the FSFI in women and the IIEF-5 in men.
RESULTS
Of the 63 eligible patients, 20 women and 22 men responded (response rate 66.7%). For women, with a median age and follow-up of 28 and 26 years respectively, the median FSFI score was 28.4 [14.2-30.1] with sexual dysfunction for 42.6%. 66.7% reported satisfaction with their sex life. 65% of patients had subsequent gynecological surgery with 37.5% satisfied with the aesthetic appearance of their EG. Pregnancy rate was 66.7%. For men, with a median age and follow-up of 23 years and 21 years respectively, the median IIEF-5 score was 23 [16,5-23] with no erectile dysfunction for 66.7%. 41.7% reported sexual satisfaction. After initial repair mostly with the Cantwell-Ransley technique (21/22), 63.6% had subsequent urethroplasty with 12% satisfied with the aesthetic appearance of their EG. One case of paternity was recorded.
CONCLUSIONS
Long-term sexual outcomes in CBE patients show high rates of sexual dysfunction in women, despite high sexual satisfaction. In men, we report few cases of erectile dysfunction with the Cantwell-Ransley technique, but a low rate of sexual satisfaction. Specific CBE scores need to be developed.
10:27 - 10:30
S08-6 (OP)
Samara ARKANI 1, Anna SKARIN NORDENVALL 2, Lotta RENSTRÖM KOSKELA 3, Gisela REINFELDT 4, Ann NORDGREN 5 and Agneta NORDENSKJOLD 6
1) Pediatric Surgery Unit, Department of Women's and Children's Health and Center for Molecular Medicine, and Department of Urology, Danderyds Hospital, Danderyd, Sweden, Stockholm, SWEDEN - 2) Department of Radiology, Karolinska University Hospital, Department of Molecular Medicine and Surgery, Karolinska institutet, Stockholm, SWEDEN - 3) Department of Pelvic Cancer, Karolinska University Hospital, Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, SWEDEN - 4) Department of Pediatric Surgery, Uppsala University Hospital, Women's and Children's Health, Uppsala University Hospital, Uppsala, SWEDEN - 5) Clinical Genetics, Karolinska University Hospital and Department of Clinical Genetics and Genomics, Sahlgrenska University Hospital,, 3Department of Molecular Medicine and Surgery, Karolinska Institutet and Institute of Biomedicine, Department of Laboratory Medicine, University of Gothenburg, Gothenburg, Stockholm, SWEDEN - 6) Astrid Lindgren Children's Hospital, Pediatric Surgery, Stockholm, SWEDEN
PURPOSE
The risk of urinary bladder cancer is known to be higher in individuals with classic bladder exstrophy (BE, 700 times higher) and appears at a younger age compared with the general population. The purpose of this study was to evaluate urinary bladder cancer in individuals with BE/epispadias.
MATERIAL AND METHODS
We performed two studies, a register-based case series reporting 12 novel cases and a systematic review summarizing published cases thus far. MEDLINE, EMBASE, Web of Science, and Google Scholar were searched in January 2022 following the Preferred Reporting Items for Systematic Review and Meta-Analysis statement. Publications reporting at least one case of BE with urinary bladder cancer were eligible. Ninety-seven publications were included, reporting 165 cases.
RESULTS
The main result from the register study was that the tumors were predominantly of urothelial origin (10/12). Conversely, published cases from the literature search had a non-urothelial tumor type in 95%, whereof the majority were adenocarcinomas. Both sub-studies consistently indicate a young age at cancer diagnosis, with the majority being younger than 65 years.
CONCLUSIONS
Urinary bladder cancer affects individuals with BE at a young age. The most common tumor type in the register study is urothelial, but non-urothelial in earlier publications. The divergence between the two sub-studies in tumor types could reflect a slightly older age in our case series, likewise a possible publication bias. This study implies an even higher risk for urinary bladder cancer in persons born with BE than predicted earlier.
10:30 - 10:32
S08-7 (CP)
Victoria MAXON 1, Chloe MICHEL 2, Logan GALANSKY 2, Ahmad HAFFAR 1, Alexander HIRSCH 1, Heather DICARLO 1 and Chad CRIGGER 1
1) Johns Hopkins University, Pediatric Urology, Baltimore, USA - 2) Johns Hopkins University, Urology, Baltimore, USA
PURPOSE
We present an initial case series on the use of daily intravesical n-acetylcysteine instillation to prevent bladder stones in patients with a history of bladder augmentation in the exstrophy-epispadias complex.
MATERIAL AND METHODS
NAC instillation was implemented in 4 patients who were considered high-risk for recurrent bladder stone formation (defined as having >4 prior stone surgeries) or at high-risk for complications from repeat stone surgery (history of vesicocutaneous fistula or pre-existing CKD). Patients were instructed to dilute 10ml of 20% NAC with 50ml of NS and then instill the solution into the bladder and allow to dwell for 30-60 minutes twice daily. Patients were followed with renal bladder ultrasound at 6-12 month intervals.
RESULTS
ll four patients had a history of an ileal augmentation and continent urinary stoma. Three patients had a history of at least 4 prior stone surgeries, 2 patients had a history of vesicocutaneous fistula after cystolithalopaxy and the remaining patient had a history of a transplant kidney prior to starting NAC instillations. The four patients had an average of 0.92 stone surgeries per year (range 0.38-1.2) prior to starting NAC. The mean follow up was 25.9 months (range 12.4-41). Three patients have remained stone free on ultrasound. One patient had a recurrence of a 7mm bladder stone 6 months after a previous stone surgery and is awaiting treatment.
CONCLUSIONS
NAC instillation may be useful as an adjunct to prevent bladder stone formation in high-risk stone formers. Further research will be needed to determine long-term efficacy.
10:47 - 10:50
S08-8 (OP)
Amilal BHAT 1, Nikhil KHANDELWAL 2, Akshita BHAT 3 and Mahakshit BHAT 4
1) Bhat Hypospadias & Reconstructive Urology Hospital and Research Centre, Urology, Jaipur, INDIA - 2) Rungta hospital Malviya Nagar Jaipur, Urology, Jaipur, INDIA - 3) SWAI MANSIGH HOSPITAL JAIPUR, SURGERY, Jaipur, INDIA - 4) N.I.M.S.JAIPUR, Jaipur, INDIA
PURPOSE TO EVALUATE THE RESULTS OF MODIFIED PARTIAL PENILE DIS-ASSEMBLY IN MANAGEMENT OF CONCEALED EPISPADIAS
In isolated epispadias, the prepuce is usually absent on the dorsal side of the penis leaving the glans uncovered. But a rare variant of epispadias is concealed epispadias where the prepuce is well formed .Less than 100 cases are reported. The diagnosis can be missed unless looked for specifically.We present the results of modified partial penile dis-assembly in managing these cases.
MATERIAL AND METHODS
In a retrospective study we evaluated isolated male epispadias patients treated at our centre between 2016 and 2023 regarding age at presentation, meatal location, incontinence, dorsal curvature, success rate, and complications.Penile degloving with the mobilization of the urethral plate from the ventral to the dorsal aspect with the preservation of blood supply at both ends, distally up to the level of mid-glans and proximally up to the pubic symphysis is done. Tubularization of urethral plate followed by spongioplasty, corporoplasty with medial rotation of corporeal bodies,and glanuloplasty with meatoplasty was done to bring the meatus ventrally.
RESULTS
We treated 46 patients with isolated male epispadias, out of these 9 (19.56%) were of concealed epispadias . Age of the patients varied from 6 months to 24 years with an average of 7 years. the type were 5 glanular, 2 coronal , one each sub coronal penopubic. All were continent except one who had partial incontinence . Dorsal chordee was seen mild in 3 moderate in 5 & severe I case and seven cases had mild to moderate torsion. Prepuce could be preserved in 3 cases and in remaining cases it was utilized for skin coverage. No complication was seen except in one partial glanular dehiscence in a follow up of 9 months to 4 years
CONCLUSIONS
Concealed epispadias represents about 20 % of isolated male epispadias cases. Presentation is late because of hidden glans .Cosmetic and functional results are good with modified partial penile dis-assembly.
10:50 - 10:53
S08-9 (OP)
Marko BENCIC, Marta BIZIC, Borko STOJANOVIC and Miroslav DJORDJEVIC
University Children's Hospital Belgrade, Urology, Belgrade, SERBIA
PURPOSE
One of the most severe congenital anomalies of the genitalia. Their correction is necessary to allow average penile growth and normal voiding function. We aimed to analyze the long-term outcome after surgical reconstruction of isolated male epispadias.
MATERIAL AND METHODS
This retrospective study involved 31 patients with isolated epispadias, surgically treated from January 2000 to January 2015. The data were collected from the medical records and outpatient examinations. The data included the age of initial surgery and type of surgical correction, epispadias grade, additional surgical reconstruction, the esthetical appearance of genitals, sexual satisfaction, and continence. Continence was defined as the ability to control urination without needing protection.
RESULTS
Follow-up ranged from 6 to 20 years (mean 14,4 years). Peno-pubic epispadias was noted in 24 (77.4%) patients, midshaft in 5 (16.1%), and distal in 2 (6.5%) patients. The mean penile length in the stretched position was 9.6 cm (range 7.1-10.2 cm). Recurrent penile curvature was seen in 6 (19.4%) patients, and 5 required surgical repair. Fistula was the most common urethral complication and was corrected with surgical intervention in 7 (22,6%) treated boys six months after the initial surgery. Complete incontinence was maintained in 9 (29%) patients, requiring additional surgical correction.
CONCLUSIONS
Treatment of isolated epispadias requires complex surgical repair in childhood, especially in severe cases. Some complications, such as residual curvature, can worsen after complete penile growth in puberty. Thus, follow-up should be extended until patients achieve their sexual growth.