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Long-Term Fate of the Upper Tracts Following Complete Primary Repair of Bladder Exstrophy
Introduction and Objectives:
Complete primary repair of bladder exstrophy (CPRE) is widely used for classic bladder exstrophy (CBE) closure. The long-term status of renal health in these patients is poorly characterized and may be impacted by bladder storage parameters or recurrent urinary tract infections (UTI). We present our long-term experience with CPRE hypothesizing that long-term renal function would be influenced by receipt of ureteral reimplantation and degree of hydronephrosis (HN).
Methods
A retrospective review of patients at our institution with CBE undergoing CPRE from 1989-2015 was performed. Patients were considered included if age at last renal ultrasound was at least 5 years. Outcomes based on renal imaging and renal function measured by serum creatinine and estimated glomerular filtration rate (eGFR, Schwartz formula) were reviewed. Serum creatinine after 6 months of age was available in 20 patients and eGFR could be calculated in 14. A pediatric urologist reviewed imaging studies to retrospectively assign Society of Fetal Urology (SFU) and Upper Tract Dilation (UTD) grades to all available ultrasounds with HN. Additionally, surgical outcomes for vesicoureteral reflux (VUR) were assessed. Descriptive and comparative statistical analysis was performed to assess factors influencing HN and renal function at follow-up.
Results:
Thirty patients (57% male) included had a median follow-up of 9.7 (3.9 to 22.3) years. HN was present in 16 (53%) patients, with the majority being SFU 1-2 (12, 75%) or UTD P1-2 (13, 81%). Mean creatinine was 0.50 mg/dL (0.2 to 1.0) and mean eGFR was 106.8 mL/min/1.73m². No patient had greater than stage 2 chronic kidney disease and no patient developed end stage renal disease in follow-up. As shown in the Table, male gender was associated worse renal outcomes, including overall rate of HN (p < 0.001), severity of HN (p = 0.004) and worse eGFR (p = 0.05). Neither age at initial operation, complications related to the initial closure, nor receipt of osteotomy appeared to impact long-term HN rates. Likewise, no differences were noted on urodynamic parameters in patients with or without HN. Ureteral reimplantation was performed in 22 patients (73%) at a mean age of 22 months for indications of persistent VUR (10), worsening HN (1), or recurrent UTI (11). Receipt of ureteral reimplantation did not impact renal function or persistence of HN in follow-up.
Conclusions:
HN is common following CPRE, though severe HN is seen infrequently. Male patients may be at higher risk for long-term upper tract deterioration and warrant close follow-up, especially if high-grade HN is present. Ureteral reimplantation does not appear to impact long-term rates of HN or renal function though prospective studies to further assess benefit of ureteral reimplant on renal function and UTI rates in this population.
Table: Renal Outcomes Stratified by Gender and Receipt of Ureteral Reimplant

	Gender			Reimplant
Upper Tract Outcome	Female	Male	P Value	No	Yes	P value
Renal Function (Mean, SD)
Creatinine (mg/dL)	0.44 (0.15)	0.52 (0.20)	0.38	0.40 (0)	0.51 (0.20)	0.36
eGFR (mL/min/1.73m2)	123 (24)	98 (18)	0.05	112 (23)	105 (24)	0.68
Hydronephrosis (N, %)
Yes	2	14	< 0.001	4 (50)	12 (55)	0.83
No	11	3		4 (50)	10 (45)
SFU, N (%)
- 1	0	6 (43)	0.004	4 (100)	3 (27)	0.25
- 2	1 (100)	3 (21)		0	4 (36)
- 3	0	3 (21)		0	2 (18)
- 4	0	2(14)		0	2 (18)
- Not Available	1	0		0	1
UTD, N (%)
- P1	0	6 (43)	0.002	3 (75)	3 (27)	0.48
- P2	1 (100)	5 (36)		1 (25)	5 (45)
- P3	0	3 (21)		0 (0)	3 (27)
- Not available	1	0		0	1
Renal Length, cm (Mean, SD)
- Left	10.1 (1.4)	9.4 (1.5)	0.26	9.2 (1.1)	9.9 (1.6)	0.27
- Right	9.9 (1.4)	9.4 (1.6)	0.37	8.9 (1.3)	9.9 (1.6)	0.12