ESPU Meeting on Friday 10, June 2022, 08:40 - 09:30
08:40 - 08:43
Tamir SHOLKLAPPER 1, Mahir MARUF 1, Jiafeng ZHU 2, Jason MICHAUD 1, Mohammad ZAMAN 1, John JAYMAN 1, Roni MANYEVETCH 1, Rachel DAVIS 1, Wayland WU 1, Thomas HARRIS 1, Heather DICARLO 1, Gayane YENOKYAN 2 and John GEARHART 1
1) Johns Hopkins Hospital, Robert D. Jeffs Division of Pediatric Urology, Brady Urological Institute, Baltimore, USA - 2) Johns Hopkins Bloomberg School of Public Health, Johns Hopkins Biostatistics Center, Baltimore, USA
Bladder capacity (BC) is an important metric in the management of patients with classic bladder exstrophy (CBE) and is used to clinically estimate likelihood of continence and determine eligibility for continence procedures, such as bladder neck reconstruction. The Koff equation, which estimates BC from patient’s age has limited applicability in the exstrophy population. We sought to develop a model of maximum bladder capacities for those with CBE using readily available clinical variables.
MATERIAL AND METHODS
An institutional database of CBE patients was reviewed for those who have undergone annual gravity cystogram 6 months after bladder closure. Linear mixed effects models with random intercept and slope were used to construct models predicting log transformed BC and were compared with adjusted R2, Akaike Information Criterion, and cross-validated mean square error. The final model includes outcome of primary closure, sex, log-transformed age at successful closure, time from successful closure, and interaction between outcome of primary closure and log-transformed age at successful closure as the fixed effects with random effect for patient and random slope for time since successful closure.
In total, 369 patients (107 female, 262 male) with CBE had at least one BC measurement after bladder closure. Patients had a median of 3 annual measurements (range 1-10). The log bladder capacity increases with the age at cystographic evaluation. Other factors associate with capacity include sex, outcome of primary bladder closure, age at successful bladder closure and age at evaluation. A web based interactive CBE bladder growth nomogram (Figure 1) may be found athttps://exstrophybladdergrowth.shinyapps.io/be_app/.
Using readily accessible patient and disease related information, the bladder capacity model in this study provides an accurate prediction of bladder capacity. Using clinical factors specific for the exstrophy population should be considered over age-based Koff equation estimates.
08:43 - 08:46
Preeya KHANDGE, Kelly HARRIS, Wayland WU, Mahir MARUF, Hiten PATEL, Heather DICARLO and John GEARHART
The Johns Hopkins Medical Institutions, Robert D. Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institute, Baltimore, USA
Following successful closure of patients with classic bladder exstrophy (CBE), the next milestone is establishing continence. However, prior to determining the appropriate continence surgery, it is imperative to reach an adequate bladder capacity of 100 cc minimum to make the decision between bladder neck reconstruction (BNR) and continent stoma with or without augmentation cystoplasty (AC). The authors sought to examine the timing of when patients achieve this threshold bladder capacity to be eligible for BNR.
MATERIAL AND METHODS
An institutional database of 1388 exstrophy patients was retrospectively reviewed for CBE patients after successful primary closure. Bladder capacity was measured via gravity cystography. The cohort was stratified by location, neonatal (≤28 days) or delayed closure period and osteotomy status. The capacities were categorized as reaching goal and a cumulative event analysis performed. The event being reaching > 100cc capacity and time being the number of years between closure and goal capacity attainment.
253 patients met criteria, majority closed at authors’ institution (52.5%), within the neonatal period (80.7%) and without osteotomy (51.7%). 64.9% reached capacity and analysis demonstrated a median time of 5.73 years corresponded with 50% event probability of reaching capacity. Cox-proportional hazards showed closure location was significantly associated with reaching capacity (HR = 0.58, CI 0.40 – 0.85, p = 0.005). The median time to event would be 5.20 years for cases done at authors’ hospital and 6.26 years if at outside hospital.
These findings help counsel families appropriately on the odds of attaining capacity at various ages. For those who do not reach 100cc by five years old, it helps further characterize the odds of requiring augmentation and the best timing for surgery to gain continence. Families may be assured that most patients will have the breadth of options when it comes to continence surgery as more than half of patients reached the bladder capacity threshold
08:46 - 08:49
Chad CRIGGER 1, Thomas HARRIS 2, Tamir SHOLKLAPPER 1, Richard REDETT III 2 and John GEARHART 1
1) Johns Hopkins Medicine, Urology, Division of Pediatric Urology, Baltimore, USA - 2) Johns Hopkins Medicine, Pediatric Surgery, Division of Plastic Surgery, Baltimore, USA
Classic bladder exstrophy (CBE) is the most common manifestation of the exstrophy-epispadias complex (EEC). Restoration of genitourinary anatomy and ultimately functional urinary continence remains the surgical goal. For patients who do not achieve continence and are not candidates for bladder neck reconstruction, bladder neck closure (BNC) remains an option. The aim of this study was to evaluate patients who underwent BNC and identify risk factors for BNC failure.
MATERIAL AND METHODS
A prospectively maintained database of 1,401 EEC patients was queried with IRB-approval to identify CBE patients who previously underwent BNC. At the time of BNC, patients underwent continent urinary stoma creation with or without augmentation cystoplasty (AC). The database was reviewed for the following: patient demographics, pre-BNC bladder mucosa violations (MVs) and all interventions required to achieve continence. Total bladder MVs including newborn exstrophy closure(s), BNR, AC and ureteral reimplantation, when applicable, were totaled. Comparison of baseline characteristics and surgical details were performed using Chi-squared and Fisher’s exact test for categorical variables, and t-test for continuous variables. Additionally, fistula-free survival after BNC was evaluated using Kaplan-Meier method and log-rank test.
A total of 195 CBE patients underwent BNC. 136 (69.7%) were male, while 59 (30.3%) were female with a mean age at BNC of 11 years. 172 patients underwent BNC at the author’s institution. Prior to BNC, 75 patients underwent BNR, 70 underwent ureteral reimplantation and 22 underwent AC with 1 patient undergoing 2 ACs and 3 patients who underwent 3 ACs. On univariate analysis, prior failed exstrophy closure (p=0.008), 3 or more bladder MVs (p=0.0018) and those treated at an outside hospital (p=0.0029) were more likely to develop a vesicourethral or vesicocutaneous fistula after BNC. On multivariate analysis each mucosal violation conferred a greater risk for fistula formation (OR=2.66 per violation, 95% CI [1.41,5.04], p =0.0021).
Increasing violations of the bladder mucosa confers increased risk of fistula formation after BNC. This highlights the importance of centers of excellence in dealing with the complex reconstruction required in EEC patients, as well as managing potential complications. Future directions include managing fistulas with interposed pedicled muscle flaps.
08:49 - 08:52
Faisal ALMUTAIRI 1, Arianna MARIOTTO 1, Ae HERNANDEZ 2, David KEENE 1 and Raimondo Maximilian CERVELLIONE 1
1) Royal Manchester Children's Hospital, Department of Paediatric Urology, Manchester, UNITED KINGDOM - 2) Hospital Universitario Virgen del Rocio, Paediatric urology unit, Seville, SPAIN
This study aims to report the authors’ experience with preputial and para-exstrophy skin urethral grafting to augment a short or deficient exstrophy urethral plate.
MATERIAL AND METHODS
Among the exstrophy patients treated at the authors’ institutions (2018-2021), those with a short or inadequate urethral plate were prospectively included. A short urethral plate was defined as a distance between the verumontanum and the base of the glans <10mm. The urethral grafting was performed electively before the exstrophy closure. The urethral plate was divided just distal to verumontanum and a thin inner preputial or para-exstrophy-skin graft was harvested and deployed to cover the defect. Exstrophy closure was subsequently performed. The study assesses the following outcomes: age at grafting, type of graft, age at exstrophy closure, success of closure, and complications. Data is presented as median (range).
Five patients were included in the study: 3 male classic bladder exstrophy (CBE) and 2 cloacal exstrophy (CE). One of the CBE had a failed initial closure elsewhere and presented with a proximal urethral stricture. Age of grafting was 9 (3-18) months. Inner preputial grafts were utilized in the 3 CBE patients, and para-exstrophy skin grafts were used for the 2 CE patients. The grafts took in all cases and allowed the creation of a longer and wide urethral plate. Bladder closure with proximal urethroplasty was subsequently performed 3 (3-6) months after grafting.
Pre-closure urethral plate grafting represents a safe and effective option for exstrophy patients with a short or inadequate urethral plate.
08:52 - 08:55
Patrick GREEN, Verity HAFFENDEN, Arianna MARIOTTO, David KEENE and Raimondo Maximilian CERVELLIONE
Royal Manchester Children's Hospital, Department of Paediatric Urology, Manchester, UNITED KINGDOM
INTRODUCTION AND AIMS
Inguinal hernias are common in infants with bladder exstrophy. Recurrence rate after herniotomy has been reported to be high. This study aims to assess the recurrence rate following standard herniotomy compared to the herniotomy and Duhamel plasty of the internal inguinal ring.
MATERIAL AND METHODS
Patients born with bladder exstrophy 2010-2021 were identified from a prospectively maintained database. Those with a diagnosis of inguinal hernia were included in the study. Patients were divided in two groups: A) 2010-2016 underwent standard herniotomy; B) 2017-2021 underwent herniotomy and Duhamel plasty of the internal inguinal ring. Fisher's exact test was used to compare the recurrence rate between group A and group B. Data are presented as median (IQR).
One hundred and twenty-one exstrophy babies were identified of which 36 developed inguinal hernias : 34/78 male (44%), 2/43 female (5%). Four patients were excluded: 3 repaired elsewhere, 1 awaiting repair. Age at first repair was 80 days (47-108). Twenty-four (57%) had bilateral hernias and 2 developed contralateral hernia following initial repair. Thirty-seven hernias in 22 patients in group A developed 9 recurrences (24%). Of the 20 hernias in 11 patients in group B, none developed a recurrence (p=0.02).
The Duhamel plasty of the internal inguinal ring appears to prevent recurrence compared to standard herniotomy in patients with Bladder Exstrophy undergoing inguinal hernia repair.
08:55 - 08:58
Nora HANEY 1, Chad CRIGGER 1, Tamir SHOLKLAPPER 1, Shwetha MUDALEGUNDI 1, Angelica GRIGGS-DEMMIN 1, Isam NASR 2, Paul SPONSELLER 3 and John P GEARHART 1
1) Johns Hopkins Hospital, Urology, Baltimore, USA - 2) Johns Hopkins Hospital, General Pediatric Surgery, Baltimore, USA - 3) Johns Hopkins Hospital, Pediatric Orthopedic Surgery, Baltimore, USA
The type of osteotomy and pelvic fixation in the surgical management of primary cloacal exstrophy (CE) closure is variable. The purpose of this study was to evaluate primary CE closure outcomes with pelvic osteotomy and fixation trends over time.
MATERIAL AND METHODS
A prospective database was reviewed for CE patients who underwent primary closure from 1960-2020. Demographics, outcomes, osteotomy, fixation, and complications were noted. Patients were subcategorized based on where primary closure was performed (AH=author’s hospital; OH=outside hospital).
Out of 122 patients, multi-stage procedures became more common than single-stage (p=0.0186), with multi-stage associated with higher success rates of primary closure (77.4% v 45.7%; p=0.0010). The use of any osteotomy increased over time (p=0.0067), with a posterior approach falling out of favor and increasing prevalence of a combined approach (anterior innominate and vertical iliac) (p<0.001). The use of any osteotomy compared to no osteotomy was associated with successful closure (77.6% v 41.7%; p=0.0002), with highest rates of success associated with a combined approach, followed by posterior then anterior (90%, 76.2%, 60.9%, respectively; p<0.0001). Sub-analysis for AH demonstrated similar trends while OH was more likely to perform anterior osteotomy over time (p=0.0002).
Fixation modalities changed significantly over time with Buck’s traction (p=0.0002) and external fixation (p<0.0001) becoming more popular. Spica casting fell out of fashion (p=0.0002). Immobilization type was significantly associated with success rates with Buck’s (92.1%; p<0.001) and external fixation (86.0%; p=0.0002) performing better than Spica casting (36.4%; p<0.0001).
The use of osteotomy and pelvic fixation in the CE spectrum has changed markedly. In this cohort, a staged approach with combined anterior innominate and vertical iliac osteotomy was associated with better outcomes.
08:58 - 09:03
Tom CLAEYS 1, Caroline JAMAER 2, Elise DE BLESER 2, Ellen ROETS 3, Noortje VAN OSTRUM 3, Aleksandra ZECIC 4, Koen SMETS 4, Lukas MATTHIJSSENS 5, Katrien VAN RENTERGHEM 5, Patrick WOUTERS 6, Eline VAN HOECKE 7, Frank PLASSCHAERT 8, Erik VAN LAECKE 2, Piet HOEBEKE 2 and Anne-Françoise SPINOIT 2
1) VITAZ, Urology, Sint-Niklaas, BELGIUM - 2) Ghent University Hospital, Urology, Gent, BELGIUM - 3) Ghent University Hospital, Obstetrics, Gent, BELGIUM - 4) Ghent University Hospital, Neonatal Care, Gent, BELGIUM - 5) Ghent University Hospital, Surgery, Gent, BELGIUM - 6) Ghent University Hospital, Anaesthesiology, Gent, BELGIUM - 7) Ghent University Hospital, Pediatric Psychology, Gent, BELGIUM - 8) Ghent University Hospital, Orthopaedic Surgery, Gent, BELGIUM
Given the rarity of bladder exstrophy complex (BEC), knowledge of this pathology stems from a limited amount of literature. Cloacal exstrophy as a complex form of BEC is an even rarer condition with few resources devoted to anatomic and surgical insights. This video presentation showcases the primary surgical repair of a female neonate with cloacal exstrophy.
MATERIAL AND METHODS
The full surgical procedure was documented using a 4K video camera after written consent from both parents.
The patient was referred to the prenatal diagnostic center at a gestational age of 24 weeks. Intrauterine features included absence of a bladder, no anal perforation, a perineal cleft, an omphalocele, a single umbilical artery, and the pathognomonic hindgut sign.
The patient was born through vaginal delivery at 36 weeks and 5 days and admitted to neonatal care. Additional screening for birth defects revealed spinal dysraphism and an atrial septum defect. MRI showed a doubled inferior vena cava and no spleen.
Multidisciplinary planning allowed for primary surgical reconstruction on day 5, involving pediatric urologists, surgeons and orthopedists.
The colostomy became productive after 3 days. Ureteral catheters were removed after 10 days. Postoperatively, the patient remained intubated for 14 days to avoid excessive abdominal wall tension. Cystography at 3 weeks showed an intact bladder lining. 3 months post-surgery, the abdominal wall remained intact and the colostomy showed prolapse.
This video provides anatomic and surgical insight into cloacal exstrophy cases for physicians and trainees.