5th Joint Meeting of ESPU-SPU - Virtual

S13: EXSTROPHY-EPISPADIAS COMPLEX

Moderators: Marc-David Leclair (France)

ESPU-SPU Meeting on Saturday 25, September 2021, 10:00 - 10:42


10:00 - 10:03
S13-1 (SO)

DELAYED PRIMARY CLOSURE OF CLASSIC BLADDER EXSTROPHY: WHEN IS IT TOO LATE?

Wayland WU, Mahir MARUF, Rachel DAVIS, Roni MANYEVITCH, Kelly HARRIS, Hiten PATEL, Heather DICARLO and John GEARHART
Johns Hopkins Hospital, Urology, Baltimore, USA

PURPOSE

With current trends delaying the closure of classic bladder exstrophy (CBE) may impact bladder growth rate or ultimate capacity. 

MATERIAL AND METHODS

A retrospective review was performed using an institutional exstrophy patient database. Inclusion criteria were: CBE, successful neonatal (i.e. ≤28 days old) or delayed (i.e. >28 days old) primary closure, at least three consecutive bladder capacities or two measures taken 18 months apart, and first bladder capacity measured at least three months after closure. Only capacities measured prior to continence surgery and before 14 years of age were considered. Linear mixed model evaluated the affects of age and length of delay on bladder capacity.

RESULTS

Cohort included 128 patients in the neonatal and 38 patients in the delayed group. Median age at closure for the delayed group was 193 days (IQR 128 – 299). For the first three capacity measurements, the delayed group had significantly lower capacities despite having similar age when the measurements were taken. A linear mixed effects model showed significantly decreased total bladder capacity in delayed closure compared to neonates. The 2nd and 4th quartile groups had the most significant decreases in capacity.

CONCLUSIONS

All patients in the delayed bladder closure group demonstrated a decline in bladder capacity, with significant differences in the 2nd and 4th quartiles. Thus, closing the bladder prior to nine months of age offers the best chance for greatest bladder capacity is recommended.


10:03 - 10:06
S13-2 (SO)

DELAYING RECLOSURE OF BLADDER EXSTROPHY LEADS TO GRADUAL DECLINE IN BLADDER CAPACITY

Wayland WU, Mahir MARUF, Kelly HARRIS, Hiten PATEL, Heather DICARLO and John GEARHART
Johns Hopkins Hospital, Urology, Baltimore, USA

PURPOSE

After unsuccessful repair of bladder exstrophy, when to repeat surgical intervention is unclear. The authors aim to study whether a relationship exists between bladder growth/capacity and time till eventual successful closure.

MATERIAL AND METHODS

An institutional database of exstrophy-epispadias complex patients was queried for failed exstrophy closure with successful repeat reconstruction, at least three consecutive bladder capacity measurements, and measurements obtained at least three months following successful closure. Patients closed successfully in the neonatal period were used as a comparative group. Linear mixed effects models were used to study the effect of time and age on bladder capacity.

RESULTS

Forty-seven patients requiring reclosure and 117 who had successful neonatal closures were included. Two models were created. The first linear mixed effects model found that for a given age, the bladder capacity declined approximately 9.6 mL per year (p = 0.016). The second model found that when time to successful closure was grouped by quartiles, compared to neonates, those in the fourth quartile had significantly decreased bladder capacity of 28.8 cc (p = 0.042). An interaction model comparing neonates and those requiring reclosure did not demonstrate a significant change in bladder growth rate (p = 0.098). A model stratified by quartiles similarly did not find any significant impact to bladder growth rate.

CONCLUSIONS

There is a demonstrable significant impact on overall bladder capacity with increasing delay to successful reclosure. One should be cautious when prolonging reconstruction of the bladder as these data demonstrate a time dependent decline in overall capacity.


10:06 - 10:09
S13-3 (SO)

INTRAOPERATIVE LASER ANGIOGRAPHY TO EVALUATE PENILE BLOOD FLOW DURING BLADDER EXSTROPHY CLOSURE

Martin KAEFER 1, Kahlil SAAD 2, Patricio GARGOLLO 3, Benjamin WHITTAM 2, Richard RINK 2, Molly FUCHS 4, Diana BOWEN 5 and Rama JAYANTHI FOR THE PEDIATRIC UROLOGY MIDWEST ALLIANCE (PUMA) 4
1) Riley Hospital for Children, Urology, Indianapolis, USA - 2) Indiana University, Pediatric Urology, Indianapolis, USA - 3) Mayo Clinic, Pediatric Urology, Rochester, USA - 4) Nationwide Children's Hospital, Pediatric Urology, Columbus, USA - 5) Lurie Children's Hospital, Pediatric Urology, Chicago, USA

PURPOSE

The decision to proceed with a staged repair vs. complete primary repair of exstrophy (CPRE) is one of the most debated issues in Pediatric Urology. Each approach has its advantages. Proponents of staged repair argue that the CPRE carries a higher chance of penile injury. We hypothesize that quantitative assessment of penile perfusion with indocyanine green at various points in the procedure is a feasible technique and may assist in decision making during the repair of this complex condition.

MATERIAL AND METHODS

Consecutive patients presenting with exstrophy were evaluated at four stages of their operation (following induction of anesthesia, after bladder mobilization, following internal rotation of the pubis and at the end of the procedure) by infusing indocyanine green (IG). Measurements were taken 80 seconds post infusion. The medial thigh served as control. Penile viability was assessed three months postoperatively.

RESULTS

Seven consecutive patients (4 CPRE, 3 Staged) were enrolled in this prospective study. Penile perfusion changed little after bladder dissection. However, penile perfusion decreased by a mean of 67% (range 45-85%) following simple internal rotation and approximation of the symphysis pubis. Patients undergoing CPRE experienced an additional mean 15% drop in blood flow following the penile repair. Overall the CPRE group experienced a significantly greater mean drop in blood flow (CPRE 77% vs Staged 47%, p<0.05, Student's t-test). In all 7 cases the penis was symmetric and healthy at three months.

CONCLUSIONS

The measurement of penile perfusion with intraoperative laser angiography is easy to employ. Marked reduction in penile blood flow may occur without any outward clinical signs. Penile perfusion is reduced by 2/3rds after apposition of the pubis and, in the immediate postoperative period, further declines in patients who undergo CPRE. Future correlation with measures of penile viability and function are needed to define the clinical utility of this modality in guiding surgical decision making.


10:09 - 10:12
S13-4: Withdrawn (author request)
 
10:12 - 10:24
Discussion
 

10:24 - 10:27
S13-5 (SO)

MALE PROXIMAL EPISPADIAS: VOIDING OUTCOME

Sajid SULTAN 1, Philip G RANSLEY 1, Sadaf ABA UMER KODWAVWALA 1, Bashir AHMED 2 and Syed Adib Ul Hassan RAZVI 1
1) Sindh Institute of Urology & Transplantation, Philips G. Rensley Department of Paediatric Urology, Karachi, PAKISTAN - 2) Sindh Institute of Urology & transplantation, Philip G. Rensley Department of Paediatric Urology, Karachi, PAKISTAN

PURPOSE

To evaluate the voiding outcome of bladder neck reconstruction in (incontinent) male proximal epispadias.

MATERIAL AND METHODS

Records of 29 male patients who underwent proximal epispadias repair and bladder neck reconstruction (BNR) from 2008 to 2018 were retrospectively reviewed for the degree of pubic diastasis, pre and post operative early morning (AM) voided volume, details of cystogram and cystoscopy especially bladder capacity. The epispadias repair was performed by Cantwell Ransley technique and BNR by modified Young's technique. Voiding outcome was evaluated by voided volume, dry intervals, presence of urge, stress incontinence and nocturnal enuresis.

RESULTS

Mean age at epispadias presentation was 7.4 +/- 3.3 years. Of the twenty nine patients, no pubic diastasis (Group A) was found in 5 (17.25%), mild diastasis (Group B) in 11 (38%), moderate (Group C) in 8 (27.5%) and severe diastasis (Group D) in 5 (17.25%). Pre BNR mean early morning voided volume was 225+/-125mls (52% of EBC)in Group A, 210+/-67 mls (60% EBC) in Group B,176+/-82mls (53% EBC)in Group C and 92+/-46mls (25% EBC) in Group D. Pre operative cystoscopic bladder capacity correlated closely with AM volume. Combined single stage epispadias repair and BNR was performed in 9 (31%) whereas 20 (69%) underwent staged procedure for epispadias and BNR. Post bladder neck reconstruction AM volume increased to 90% in group A, 72% in group B, 67% in group C and 39% in group D. Dry interval ranged 3 -5 hours in 25 /29 (86%). Urge incontinence was present in 4/ 29 (13.8%), stress incontinence in 15/29 (51.7%) and nocturnal enuresis in 11/29 (38%).
Follow up period 14 to102 months

CONCLUSIONS

Overall success is satisfactory with 86% having dry interval more than three hours.
This is one of the largest and unique series of a rare abnormality, i.e. voiding outcome of isolated epispadias of late presentation from a developing country.


10:27 - 10:30
S13-6 (SO)

IS THE BLADDER EXSTROPHY-EPISPADIAS COMPLEX A RISK FACTOR FOR CONGENITAL HIP DYSPLASIA? : A SURVEY OF THE MULTICENTER GERMAN CURE-NET

Anne-Karoline EBERT 1, Ekkehart JENETZKY 2, Lisa DLASK 3, Kathi THIELE 3, Michael KERTAI 4, Matthias SCHAAL 5, Frank-Mattias SCHÄFER 6, Eberhard SCHMIEDEKE 7, Raimund STEIN 8, Wolfgang H. RÖSCH 9, Heiko REUTTER 10 and Nadine ZWINK 11
1) University of Ulm, Department of Urology and Pediatric Urology, Ulm, GERMANY - 2) University Medical Center of the Johannes Gutenberg University Mainz & Private University Witten/Herdecke gGmbH, Department of Child and Adolescent Psychiatry & Institute for Integrative Medicine, Faculty for Health, Mainz & Witten, GERMANY - 3) University Hospital Ulm, Department of Urology and Pediatric Urology, Ulm, GERMANY - 4) Klinik St. Hedwig, University Medical Center Regensburg, Department of Pediatric Surgery and Pediatric Orthopedics, Regensburg, GERMANY - 5) University Hospital Ulm, Department of Radiology, Ulm, GERMANY - 6) Cnopf'sche Kinderklinik, Department of Pediatric Surgery and Urology, Nürnberg, GERMANY - 7) Klinikum Bremen-Mitte, Department of Pediatric Surgery and Pediatric Urology, Bremen, GERMANY - 8) University Hospital Mannheim, Department of Pediatric and Adolescent Urology, Mannheim, GERMANY - 9) Klinik St. Hedwig, University Medical Center Regensburg, Department of Pediatric Urology, Regensburg, GERMANY - 10) Children's Hospital, University of Bonn & University of Bonn, Department of Neonatology and Pediatric Intensive Care & Institute of Human Genetics, Bonn, GERMANY - 11) University Medical Center of the Johannes Gutenberg University Mainz, Department of Child and Adolescent Psychiatry, Mainz, GERMANY

PURPOSE

In literature, hip dysplasia and coxarthrosis have been described in adolescents and adults with EEC before. Although pelvic abnormality and biomechanical stress might be causative, hip dysplasia might also be congenital in EEC.

MATERIAL AND METHODS

In the German multicenter network for congenital uro-rectal malformations (CURE-Net) database clinical and sonographic hip data of 37 prospectively observed EEC newborn (78% male, 70% exstrophy) were analyzed, derived at 4 weeks of age according the nationwide hip screening program, and compared to two control groups (215 newborn at risk for congenital hip dysplasia (49% male); 2550 newborn according general hip screening).

RESULTS

73% EEC hips were normal (95%-CI: 61%; 83%), 20% were physiologically delayed (95%-CI: 12%; 31%) and 1% dysplastic (95%-CI: 0%; 7%). 5% were unknown (95%-CI: 0%; 13%). Therefore, congenital hip dysplasia in EEC was equally to the screening sample (1% vs 1.4%; p=0.54). Risk factors for congenital hip dysplasia are comparable between EEC and the screening sample (16% vs 19%, p=0.66). However, EEC patients had more delayed hip development compared to the reference sample (20% vs 14.4%, p=0.18), and less delayed hips than a newborn control sample (20% vs 27%, p=0.31) with higher incidence  of hip dysplasia risk factors (16% vs 81%, p=0.0001). 10 EEC patients (27%) had "preventive" hip treatment such as wrapping and abduction braces. EEC individuals reached milestones of motor development according to WHO significantly later (p=0.001; except crawling p=0.16)

CONCLUSIONS

There was no evidence for an increased incidence of congenital hip dysplasia in the current EEC CURE-Net cohort. In addition to anatomical pelvic abnormalities delayed motor development due to early surgeries and precautious wrapping techniques may be responsible for increased hip development delay. However, higher sample sizes would be desirable to verify these first confident results.


10:30 - 10:33
S13-7 (SO)

10-YEAR EXPERIENCE WITH URETERIC REIMPLANTATION AT THE TIME OF EXSTROPHY CLOSURE

Abdulrahman ALSHAFEI 1, Arianna MARIOTTO 1, Salvatore CASCIO 2, David KEENE 1 and Raimondo Maximilian CERVELLIONE 1
1) Royal Manchester Children's Hospital, Paediatric Urology, Manchester, UNITED KINGDOM - 2) Children's Health Ireland, Paediatric Urology, Dublin, IRELAND

PURPOSE

The timing of ureteric reimplantation in patients with bladder exstrophy is still debated. We report a 10-year experience with primary bilateral ureteric reimplantation at the time of exstrophy closure focusing on its ability to prevent vesico-ureteric reflux (VUR), preserving kidney function and related complications.

MATERIAL AND METHODS

A prospectively maintained database for exstrophy was used to select patients with classic bladder exstrophy who underwent closure with ureteric reimplantation between 2009 and 2019.  The following outcomes were measured: age at closure, length of follow-up, upper urinary tract dilatation on ultrasound, VUR on cystogram, renal scarring on DMSA at the age of 5 years, estimated GFR and complications.

RESULTS

Sixty-six patients (46 males) were included. The median age at operation was 6 months (4-7). The median follow-up is 56 months (34-76). 7% had a renal pelvis measuring 10-15mm. VUR was found in 21% of renal units, equally split into dilating and non-dilating reflux. Twenty patients were > 5 years and had a DMSA, of which 15% showed scarring in one renal pole. Estimated GFR was normal in all. One required lithotripsy for a unilateral ureteric stone. Three patients developed bladder trabeculation.

CONCLUSIONS

Primary bilateral ureteric reimplantation at the time of exstrophy closure can prevent reflux in 4/5 of the patients. It is associated with minimal risk of mild hydronephrosis and protects the upper urinary tracts from renal scarring in 85% of the patients at the age of 5 years.  Complications are unlikely but bladder trabeculation was seen in 4.5% of the patients.


10:33 - 10:42
Discussion