33rd ESPU Congress in Lisbon, Portugal

S16: EXSTROPHY-EPISPADIAS COMPLEX 1

Moderators: Pippi Salle (Qatar), Wolfgang Roesch (Germany)

ESPU Meeting on Friday 21, April 2023, 10:50 - 11:25


10:50 - 10:53
S16-1 (OP)

COMPLICATIONS OF DELAYED AND NEWBORN PRIMARY CLOSURES OF CLASSIC BLADDER EXSTROPHY: IS THERE A DIFFERENCE?

Christian MORRILL, Roni MANYEVITCH, Ahmad HAFFAR, Wayland WU, Kelly HARRIS, Mahir MARUF, Chad CRIGGER, Heather DI CARLO and John GEARHART
The Brady Urological Institute at Johns Hopkins University, Department of Pediatric Urology, Baltimore, USA

PURPOSE

The authors aim to compare single institutional 30-day complication rates between delayed and neonatal closure of classic bladder exstrophy (CBE).

MATERIAL AND METHODS

An institutional database of 1415 Exstrophy-Epispadias patients was reviewed retrospectively for CBE patients who underwent primary closures at the authors’ institution between 1990 and 2020. Patients were identified as having received either neonatal or delayed (at age >28 days) closures. All 30-day complications were recorded, including wound infection and dehiscence, genitourinary and nongenitourinary infections, bowel obstruction, blood transfusions, and others.

RESULTS

The cohort included 145 patients: 50 delayed and 95 neonatal closures. The total complication rate was 58% in delayed closures compared to 48.4% for neonatal closures (p = 0.298), with the majority being Clavien-Dindo grade I or II. Excluding blood transfusion, complication rates fell to 26% and 34.7% in delayed and neonatal closures, respectively (p=0.349). The most common single complication was unplanned post-operative blood transfusion (38% delayed; 26.3% neonatal; p=0.34), followed by pyelonephritis (2% delayed; 8.4% neonatal), and urinary fistula (6% delayed; 1.1% neonatal). Grade III Clavien-Dindo complications occurred in 2% delayed and 7.4% neonatal groups (n=1; n= 7 respectively; p=0.263). A single delayed patient had grade IV complications compared to three neonatal patients (p=0.66).

CONCLUSIONS

Delayed primary closure has become a frequently performed alternative in the modern treatment of bladder exstrophy for patients who do not undergo newborn closure because of prohibitive circumstances or surgeon’s discretion. The majority of the complications associated with delayed closure are a low Clavien-Dindo grade and easily managed during a patient’s standard postoperative inpatient hospital stay. Families should be counseled about the possibility of minor, conservatively managed complications and likelihood of a blood transfusion with osteotomy.


10:53 - 10:56
S16-2 (OP)

FIXATION WITH LOWER LIMB IMMOBILIZATION IN THE EXSTROPHY CLOSURE: A SAVING GRACE

Ahmad HAFFAR 1, Christian MORRILL 1, Chad CRIGGER 1, Paul SPONSELLER 2 and John GEARHART 1
1) Brady Urological Institute, Johns Hopkins Medicine, Division of Pediatric Urology, Baltimore, USA - 2) Johns Hopkins Medicine, Division of Pediatric Orthopedics, Baltimore, USA

PURPOSE

The authors hypothesize that the addition of external fixation (pelvic immobilization) in patients with any form of limb immobilization will be associated with improved outcomes.

MATERIAL AND METHODS

A prospectively maintained institutional exstrophy-epispadias complex database of 1415 patients was reviewed for patients with CBE who had undergone closure with available immobilization and osteotomy data.

RESULTS

A total of 747 closure events matching the inclusion criteria were identified. Patients included 508 males and 239 females. Limb immobilization was used in 627 (83.9%) of closure events. Successful closures were associated with osteotomy use (p<0.0001) and limb immobilization (p<0.0001); specifically, the combined anterior innominate with posterior vertical iliac osteotomy and modified Buck's traction with external fixation (p<0.0001, p<0.0001). Among the group of 33 patients who received external fixation alone and no other type of immobilization, the failure rate was 33.3%, whereas patients who had any form of combined immobilization had a failure rate of 7.1%. Among patients immobilized with mummy wrap, spica casting, or knee immobilizers, external fixation was associated with 3.76 increased odds of successful closure (p=0.0005, 95% CI 1.79-7.90). In a unique group of 67 patients without pelvic osteotomy or any form of immobilization, the failure rate was 74.6%. 

CONCLUSIONS

This study confirms, in a larger series, previous findings of improved outcomes when patients are immobilized with Buck's traction and external fixation. The authors recommend this technique be utilized whenever feasible. However, regardless of the manner of lower limb immobilization, external fixation is the critical factor to optimize closures and ensure success.


10:56 - 10:59
S16-3 (OP)

IS IMMOBILIZATION TECHNIQUE ASSOCIATED WITH POSTOPERATIVE PUBIC RAMI DIASTASIS FOLLOWING BLADDER EXSTROPHY CLOSURE?

Ted LEE 1, Lucas ANNABELL 2, Richard LEE 2, Joseph BORER 2 and Young Jo KIM 2
1) Boston Children's Hospital, Urology, Boston, USA - 2) Boston Children's Hospital, Boston, USA

PURPOSE

It is unknown whether the type of immobilization technique influences the degree of pubic rami diastasis following pelvic osteotomy and bladder exstrophy closure. In this study, we aimed to assess whether Bryant’s traction or spica cast immbolization is associated with short or long-term change in postoperative pubic rami diastasis.

MATERIAL AND METHODS

We performed a single-institutional retrospective review of bladder exstrophy patients <1.5 years who underwent posterior pelvic osteotomy and exstrophy closure from 04/2005 to 04/2020. Primary outcomes included short-term (6 months) postoperative change in diastasis compared to baseline. Secondary outcomes included length of stay and skin/wound complications. Multivariable logistic regression assessed for an association between immobilization type and degree of diastasis while controlling for age at time of diastasis measurement and sex.

RESULTS

15 patients underwent Bryant’s traction and 36 patients underwent spica cast immobilization. After adjustments, there were higher odds of having smaller pubic rami diastasis in both the short-term (OR 2.71, 95%CI 1.52–4.86,p=0.001) and long-term (OR 2.41, 95%CI 1.00–5.80,p=0.05) postoperative periods. Wound or skin complications did not differ between the two groups (53% vs 42%, p=0.46). 

Bryant’s Traction (n=15) Spica Cast (n=36)  p-value
Age at surgery(months) 3.47  3.47  0.99
Female 26%  36%  0.51
Preopeative diastasis(cm) 3.33  3.91 0.27
Short-term diastasis(cm) –0.95  –1.98  0.002
Mean time of short-term diastasis measurement(days) 14.20  8.14  0.22
Long-term diastasis(cm) +0.84  +0.08  0.05
Mean time of long-term diastasis measurement(months) 66.46  37.47  <0.001
Length of stay(days) 25.60  18.97 <0.001
Wound/skin complication 53% 42%  0.46

CONCLUSIONS

Spica cast immobilization technique following pelvic osteotomy and bladder exstrophy closure was associated with smaller postoperative pubic rami diastasis both short and long-term. This could have implications for postoperative healing under reduced abdominal tension. Further investigation with larger sample size is warranted to further elucidate our findings.


10:59 - 11:10
Discussion
 

11:10 - 11:13
S16-4 (OP)

ORTHOPEDIC COMPLICATIONS AFTER OSTEOTOMY IN PATIENTS WITH CLASSIC BLADDER EXSTROPHY AND CLOACAL EXSTROPHY: A COMPARATIVE STUDY

Tamir SHOLKLAPPER 1, Chad CRIGGER 1, Ahmad HAFFAR 1, Nora HANEY 1, Preeya KHANDGE 1, Wayland WU 1, Paul SPONSELLER 2 and John GEARHART 1
1) Brady Urological Institute, Johns Hopkins Medicine, Division of Pediatric Urology, Baltimore, USA - 2) Johns Hopkins Medicine, Division of Pediatric Orthopedics, Baltimore, USA

PURPOSE

The addition of pelvic osteotomy to the armamentarium for correction of classic bladder exstrophy (CBE) and cloacal exstrophy (CE) has been pivotal in effectuating successful primary bladder closures. The authors aim to compare orthopedic complications in patients with CBE and CE who underwent primary bladder closure with osteotomy.

MATERIAL AND METHODS

A prospectively maintained, IRB-approved database of 1,401 exstrophy-epispadias patients was reviewed for patients with CBE or CE after primary closure and pelvic osteotomy performed at a single institution from 1975-2021. Failed closure was defined as dehiscence, bladder prolapse, or vesicocutaneous fistula at any point. Surgery or anesthesia-related complications were captured within 6 weeks of osteotomy or closure.

RESULTS

146 patients were included in the analysis with 109 and 37 patients with CBE and CE, respectively. Between the CBE and CE cohorts, there were significant differences in median age at primary closure (68 days [IQR 10-260] vs 597 [448-734]; p<0.001), diastasis width (4 cm IQR [3.8-4.6] vs 6.1 [5.0-7.2]; p<0.001), osteotomy at time of closure (99.1% vs 75.7%; p<0.001), and utilization of 1 external hip fixation (67.9% vs 89.2%; p=0.011). There was no significant difference by gender, osteotomy technique, or immobilization technique. Regarding exstrophy closure outcomes, there were 5 failures in the CBE group and 1 in the CE group (p=1.000). Complications were experienced in 38.5% and 56.8% of CBE and CE patients (p=0.054) with a significant difference in orthopedic complications (i.e. superficial pin-site infections) between the cohorts (4.6% vs 16.2%, p=0.031). There was no significant difference in grade 3 or higher complications between cohorts (5.5% vs 13.5%, p=0.147).

CONCLUSIONS

While exstrophy closure success and overall complications rates are similar in patients with CBE and CE, patients with CE experience more superficial pin-site infections after pelvic osteotomy. External hip fixation may be associated with the increase in orthopedic complications, though further research is required.


11:13 - 11:16
S16-5 (OP)

HERNIOTOMY AN ADDITIONAL PROCEDURE DURING PRIMARY REPAIR OF BLADDER EXSTROPHY

Ganesh VYTHILINGAM 1, Izzy MARKS 2, Kevin CAO 2, Naima SMEULDERS 2, Imran MUSHTAQ 2, Peter CUCKOW 2 and Navroop JOHAL 3
1) Great Ormond Street Hospital, Paediatric Urology, London, UNITED KINGDOM - 2) Great Ormond Street Children Hospital, Urology, London, UNITED KINGDOM - 3) Great Ormond Street Children Hospital, London, UNITED KINGDOM

PURPOSE

Bladder exstrophy (BE) is a rare congenital abnormality that occurs in 1/40,000 births and has a known association with inguinal hernia. Patients are at risk of presenting with incarceration and bowel obstruction. Whilst some surgeons choose to prophylactically perform bilateral herniotomies at the time of BE closure, this approach is not universal.

MATERIAL AND METHODS

Retrospective study looking at 11 years of BE patients at Great Ormond Street (GOSH). Basic demographic information and timing of herniotomies was recorded. Patients presenting with inguinal obstruction were also identified. Female patients and all of those whose initial closure was not performed at GOSH were excluded.

RESULTS

: 148 children were treated at GOSH for BE between January 2011- January 2022, of whom 102 were male and 46 were female. 14 males were excluded due to duplication or incorrect diagnosis and 31 were excluded as their initial BE closure was not performed at GOSH.

 

Total patients in study

57

Herniotomy done at BE closure

37 (70%)

No herniotomy done at BE closure

17 (30%)

 

Table 1: BE patients having prophylactic herniotomy at the time of their primary bladder closure.

 

Out of 17 patients that did not have herniotomy, 7 presented with obstruction that needed reduction, 3 were diagnosed in clinic.44% (N=10) of patients who did not have herniotomy at the time of their primary closure required subsequent surgery.

CONCLUSIONS

Performing bilateral herniotomies at the time of primary BE closure reduces the risk of patients presenting with obstruction, requiring subsequent surgical repair and future testicular atrophy.


11:16 - 11:19
S16-6 (OP)

PELVIC ECTOPIC KIDNEY PREVALENCE IN CLOACAL EXSTROPHY AND PRESSURE CHANGES DURING SECOND STAGE CLOSURE

Ted LEE 1, Elizabeth ROTH 2, Dana WEISS 3, Travis GROTH 2, John KRYGER 2, Richard LEE 4, Aseem SHUKLA 3 and Joseph BORER 4
1) Boston Children's Hospital, Urology, Boston, USA - 2) Children's Wisconsin, Milwaukee, USA - 3) Children's Hospital of Philadelphia, Philadelphia, USA - 4) Boston Children's Hospital, Boston, USA

PURPOSE

Presence of pelvic ectopic kidney (PEK) in cloacal exstrophy (CE) has important implications during second-stage repair, as permanent damage of PEK has been reported. In this study, we assessed for the prevalence of PEK within a multi-institutional CE cohort. In select CE patients with PEK, renal pressures and Doppler ultrasound parameters of the orthotopic and pelvic kidneys were monitored before and after pubic bone approximation during second-stage closure.

MATERIAL AND METHODS

A multi-institutional retrospective review was performed to identify patients with CE and presence of PEK. In four CE patients undergoing second-stage repair with known pelvic and orthotopic kidneys, 4.0 French Luer-lock catheter in the renal pelvis measured renal pelvic pressure (RPP) and Doppler ultrasound measured peak systolic velocity (PSV) and resistive index (RI). Changes in RRP, PSV, and RI following pubic bone approximation were recorded. Analysis was performed using paired t-test.

RESULTS

Among 74 CE patients, 23 (25.7%) had PEK. 7 (9.5%) had solitary kidney, 3 of which were PEK. Mean intraoperative measurement changes in RPP, PSV, and RI are outlined in the table. One patient had immediate increase in RPP of 66mmHg in pelvic kidney compared to an increase of 7mmHg in the orthotopic kidney.

Change in intraoperative renal parameters before and after pubic bone approximation 

Orthotopic kidney  Pelvic ectopic kidney  p-value
Renal pelvic pressure  13.0 mmHg  29.3 mmHg 0.53
Peak systolic velocity  -34.7 cm/s 53.8 cm/s 0.14
Resistive index  -0.14  0.012  0.42

CONCLUSIONS

Prevalence of PEK in CE population is high. Although not statistically significant, we witnessed patterns of higher change in pressure, systolic velocity, and resistive index of the PEK compared to the orthotopic kidney. The clinical significance of increased pressure of PEK needs to be further investigated, as intraoperative adjustments may be necessary to mitigate risk of renal injury.


11:19 - 11:25
Discussion