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Erschienen in: Pediatric Nephrology 8/2023

06.02.2023 | Original Article

Exstrophy-epispadias complex: are the kidneys and kidney function spared?

verfasst von: Roxana Cleper, Danith Blumenthal, Yossi Beniamini, Shiran Friedman, Yuval Bar Yosef, Jacob Ben Chaim

Erschienen in: Pediatric Nephrology | Ausgabe 8/2023

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Abstract

Background

Exstrophy-epispadias complex (EEC) is a complex malformation of the lower abdominal wall, bladder, and pelvic floor, which necessitates multiple successive reconstruction procedures. Surgical and infectious complications are frequent. Our aim was to evaluate kidney function in these patients.

Methods

This cross-sectional study included patients with EEC, followed since birth in a pediatric urology clinic, who underwent nephrological evaluation (blood pressure (BP) measurement and blood and urine chemistries) and imaging studies (urinary tract ultrasound and DMSA kidney scan) during 2017–2020.

Results

Forty-three patients (29 males), median age 9 years (interquartile range 6–19), were included. Eleven (26%) used clean intermittent catheterization (CIC) for bladder drainage. At least one sign of kidney injury was identified in 32 (74%) patients; elevated BP, decreased kidney function (estimated glomerular filtration rate (eGFR) < 90 ml/min/1.73 m2), and proteinuria/albuminuria were detected in 29%, 12%, and 36% of patients, respectively. Urinary tract dilatation (UTD) was found in 13 (37%) ultrasound examinations. Parenchymal kidney defects were suspected in 46% and 61% of ultrasound and DMSA scintigraphy, respectively. UTD was significantly associated with DMSA-proven kidney defects (p = 0.043) and with elevated BP, 39% vs. 20% in those without UTD. Decreased eGFR and elevated BP were less frequent among patients on CIC than among patients who voided spontaneously: 10% vs. 14% and 18% vs. 36%, respectively. Recurrent UTIs/bacteriuria and nephro/cystolithiasis were reported by 44% and 29% patients, respectively.

Conclusion

The high rate of signs of kidney injury in pediatric patients with EEC dictates early-onset long-term kidney function monitoring by joint pediatric urological and nephrological teams.

Graphical Abstract

A higher resolution version of the Graphical abstract is available as Supplementary information
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Literatur
2.
Zurück zum Zitat Reinfeldt Engberg G, Mantel Ä, Fossum M, Nordenskjöld A (2016) Maternal and fetal risk factors for bladder exstrophy: a nationwide Swedish case-control study. J Pediatr Urol 12:304.e1-304.e7CrossRefPubMed Reinfeldt Engberg G, Mantel Ä, Fossum M, Nordenskjöld A (2016) Maternal and fetal risk factors for bladder exstrophy: a nationwide Swedish case-control study. J Pediatr Urol 12:304.e1-304.e7CrossRefPubMed
3.
Zurück zum Zitat Reutter H, Boyadjiev SA, Gambhir L, Ebert AK, Rösch WH, Stein R, Schröder A, Boemers TM, Bartels E, Vogt H, Utsch B, Müller M, Detlefsen B, Zwink N, Rogenhofer S, Gobet R, Beckers GM, Bökenkamp A, Kajbafzadeh AM, Jaureguizar E, Draaken M, Lakshmanan Y, Gearhart JP, Ludwig M, Nöthen MM, Jenetzky E (2011) Phenotype severity in the bladder exstrophy-epispadias complex: analysis of genetic and nongenetic contributing factors in 441 families from North America and Europe. J Pediatr 159:825–831 Reutter H, Boyadjiev SA, Gambhir L, Ebert AK, Rösch WH, Stein R, Schröder A, Boemers TM, Bartels E, Vogt H, Utsch B, Müller M, Detlefsen B, Zwink N, Rogenhofer S, Gobet R, Beckers GM, Bökenkamp A, Kajbafzadeh AM, Jaureguizar E, Draaken M, Lakshmanan Y, Gearhart JP, Ludwig M, Nöthen MM, Jenetzky E (2011) Phenotype severity in the bladder exstrophy-epispadias complex: analysis of genetic and nongenetic contributing factors in 441 families from North America and Europe. J Pediatr 159:825–831
4.
Zurück zum Zitat Woolf AS, Stuart HM, Newman WG (2014) Genetics of human congenital urinary bladder disease. Pediatr Nephrol 29:353–360CrossRefPubMed Woolf AS, Stuart HM, Newman WG (2014) Genetics of human congenital urinary bladder disease. Pediatr Nephrol 29:353–360CrossRefPubMed
5.
Zurück zum Zitat Gargollo PC, Borer JG (2007) Contemporary outcomes in bladder exstrophy. Curr Opin Urol 17:272–280CrossRefPubMed Gargollo PC, Borer JG (2007) Contemporary outcomes in bladder exstrophy. Curr Opin Urol 17:272–280CrossRefPubMed
6.
Zurück zum Zitat Husmann DA (2006) Surgery Insight: advantages and pitfalls of surgical techniques for the correction of bladder exstrophy. Nat Clin Pract Urol 3:95–100CrossRefPubMed Husmann DA (2006) Surgery Insight: advantages and pitfalls of surgical techniques for the correction of bladder exstrophy. Nat Clin Pract Urol 3:95–100CrossRefPubMed
7.
Zurück zum Zitat Stec AA, Baradaran N, Gearhart JP (2012) Congenital renal anomalies in patients with classic bladder exstrophy. Urology 79:207–209CrossRefPubMed Stec AA, Baradaran N, Gearhart JP (2012) Congenital renal anomalies in patients with classic bladder exstrophy. Urology 79:207–209CrossRefPubMed
9.
Zurück zum Zitat Ebert AK, Schott G, Bals-Pratsch M, Seifert B, Rösch WH (2009) Long-term follow-up of male patients after reconstruction of the bladder-exstrophy-epispadias complex: psychosocial status, continence, renal and genital function. J Pediatr Urol 6:6–10CrossRefPubMed Ebert AK, Schott G, Bals-Pratsch M, Seifert B, Rösch WH (2009) Long-term follow-up of male patients after reconstruction of the bladder-exstrophy-epispadias complex: psychosocial status, continence, renal and genital function. J Pediatr Urol 6:6–10CrossRefPubMed
10.
Zurück zum Zitat Hernandez DJ, Purves T, Gearhart JP (2008) Complications of surgical reconstruction of the exstrophy-epispadias complex. J Pediatr Urol 4:460–466CrossRefPubMed Hernandez DJ, Purves T, Gearhart JP (2008) Complications of surgical reconstruction of the exstrophy-epispadias complex. J Pediatr Urol 4:460–466CrossRefPubMed
11.
Zurück zum Zitat O’kelly F, Keefe D, Herschorn S, Lorenzo AJ (2018) Contemporary issues relating to transitional care in bladder exstrophy. Can Urol Assoc J 12(4 Suppl 1):S15–S23CrossRefPubMedPubMedCentral O’kelly F, Keefe D, Herschorn S, Lorenzo AJ (2018) Contemporary issues relating to transitional care in bladder exstrophy. Can Urol Assoc J 12(4 Suppl 1):S15–S23CrossRefPubMedPubMedCentral
12.
Zurück zum Zitat El-Sherbiny MT, Hafez AT, Ghoneim MA (2002) Complete repair of exstrophy: further experience with neonates and children after failed initial closure. J Urol 168(4 Pt 2):1692–1694CrossRefPubMed El-Sherbiny MT, Hafez AT, Ghoneim MA (2002) Complete repair of exstrophy: further experience with neonates and children after failed initial closure. J Urol 168(4 Pt 2):1692–1694CrossRefPubMed
13.
Zurück zum Zitat Bolduc S, Capolicchio G, Upadhyay J, Bagli DJ et al (2002) The fate of the upper urinary tract in exstrophy. J Urol 168:2579–2582CrossRefPubMed Bolduc S, Capolicchio G, Upadhyay J, Bagli DJ et al (2002) The fate of the upper urinary tract in exstrophy. J Urol 168:2579–2582CrossRefPubMed
14.
Zurück zum Zitat Borer JG, Gargollo PC, Hendren WH, Diamond DA et al (2002) Early outcome following complete primary repair of bladder exstrophy in the newborn. J Urol 174(4 Pt2):1674–1678 Borer JG, Gargollo PC, Hendren WH, Diamond DA et al (2002) Early outcome following complete primary repair of bladder exstrophy in the newborn. J Urol 174(4 Pt2):1674–1678
15.
Zurück zum Zitat Gargollo PC, Borer JG, Diamond DA, Hendren WH, Rosoklija I, Grant R, Retik AB (2008) Prospective followup in patients after complete primary repair of bladder exstrophy. J Urol 180(4 Suppl):1665–1670CrossRefPubMed Gargollo PC, Borer JG, Diamond DA, Hendren WH, Rosoklija I, Grant R, Retik AB (2008) Prospective followup in patients after complete primary repair of bladder exstrophy. J Urol 180(4 Suppl):1665–1670CrossRefPubMed
16.
Zurück zum Zitat Dickson AP (2014) The management of bladder exstrophy: the Manchester experience. J Pediatr Surg 49:244–2450CrossRefPubMed Dickson AP (2014) The management of bladder exstrophy: the Manchester experience. J Pediatr Surg 49:244–2450CrossRefPubMed
17.
Zurück zum Zitat Ellison JS, Ahn J, Shnorhavorian M, Grady R, Merguerian PA (2017) Long-term fate of the upper tracts following complete primary repair of bladder exstrophy. J Pediatr Urol 13:394.e1-394.e6CrossRefPubMed Ellison JS, Ahn J, Shnorhavorian M, Grady R, Merguerian PA (2017) Long-term fate of the upper tracts following complete primary repair of bladder exstrophy. J Pediatr Urol 13:394.e1-394.e6CrossRefPubMed
18.
Zurück zum Zitat Joshi RS, Shrivastava D, Grady R, Kundu A et al (2018) A model for sustained collaboration to address the unmet global burden of bladder exstrophy-epispadias complex and penopubic epispadias: the International Bladder Exstrophy Consortium. JAMA Surg 153:618–624CrossRefPubMedPubMedCentral Joshi RS, Shrivastava D, Grady R, Kundu A et al (2018) A model for sustained collaboration to address the unmet global burden of bladder exstrophy-epispadias complex and penopubic epispadias: the International Bladder Exstrophy Consortium. JAMA Surg 153:618–624CrossRefPubMedPubMedCentral
19.
Zurück zum Zitat Hammouda HM, Kotb H (2004) Complete primary repair of bladder exstrophy: initial experience with 33 cases. J Urol 172(4 Pt 1):1441–1444CrossRefPubMed Hammouda HM, Kotb H (2004) Complete primary repair of bladder exstrophy: initial experience with 33 cases. J Urol 172(4 Pt 1):1441–1444CrossRefPubMed
20.
Zurück zum Zitat Shoukry AI, Ziada AM, Morsi HA, Habib EI et al (2009) Outcome of complete primary bladder exstrophy repair: single-center experience. J Pediatr Urol 5:496–499CrossRefPubMed Shoukry AI, Ziada AM, Morsi HA, Habib EI et al (2009) Outcome of complete primary bladder exstrophy repair: single-center experience. J Pediatr Urol 5:496–499CrossRefPubMed
21.
Zurück zum Zitat Gobet R, Weber D, Renzulli P, Kellenberger C (2009) Long-term follow up (37–69 years) of patients with bladder exstrophy treated with ureterosigmoidostomy: uro-nephrological outcome. J Pediatr Urol 5:190–196CrossRefPubMed Gobet R, Weber D, Renzulli P, Kellenberger C (2009) Long-term follow up (37–69 years) of patients with bladder exstrophy treated with ureterosigmoidostomy: uro-nephrological outcome. J Pediatr Urol 5:190–196CrossRefPubMed
22.
Zurück zum Zitat Schaeffer AJ, Stec AA, Baradaran N, Gearhart JP, Mathews RI (2013) Preservation of renal function in the modern staged repair of classic bladder exstrophy. J Pediatr Urol 9:169–173CrossRefPubMed Schaeffer AJ, Stec AA, Baradaran N, Gearhart JP, Mathews RI (2013) Preservation of renal function in the modern staged repair of classic bladder exstrophy. J Pediatr Urol 9:169–173CrossRefPubMed
23.
Zurück zum Zitat Arab HO, Helmy TE, Abdelhalim A, Soltan M et al (2018) Complete primary repair of bladder exstrophy: critical analysis of the long-term outcome. Urology 117:131–136CrossRefPubMed Arab HO, Helmy TE, Abdelhalim A, Soltan M et al (2018) Complete primary repair of bladder exstrophy: critical analysis of the long-term outcome. Urology 117:131–136CrossRefPubMed
24.
Zurück zum Zitat Ben-Chaim J, Binyamini Y, Segev E, Sofer M, Bar-Yosef Y (2016) Can classic bladder exstrophy be safely and successfully reconstructed at a low volume center? J Urol 195:150–154CrossRefPubMed Ben-Chaim J, Binyamini Y, Segev E, Sofer M, Bar-Yosef Y (2016) Can classic bladder exstrophy be safely and successfully reconstructed at a low volume center? J Urol 195:150–154CrossRefPubMed
25.
Zurück zum Zitat Baird AD, Nelson CP, Gearhart JP (2007) Modern staged repair of bladder exstrophy: a contemporary series. J Pediatr Urol 3:311–315CrossRefPubMed Baird AD, Nelson CP, Gearhart JP (2007) Modern staged repair of bladder exstrophy: a contemporary series. J Pediatr Urol 3:311–315CrossRefPubMed
26.
Zurück zum Zitat Bar-Yosef Y, Binyamini J, Sofer M, Ben-Chaim J (2016) Role of routine cystoscopy and cystography in exstrophy-epispadias complex. J Pediatr Urol 12:117.e1-117.e4CrossRefPubMed Bar-Yosef Y, Binyamini J, Sofer M, Ben-Chaim J (2016) Role of routine cystoscopy and cystography in exstrophy-epispadias complex. J Pediatr Urol 12:117.e1-117.e4CrossRefPubMed
28.
Zurück zum Zitat Van der Watt G, Omar F, Brink A, McCulloch M (2016) Laboratory investigation of the child with suspected renal disease. In: Avner ED, Harmon WE, Niaudet P et al (eds) Pediatric nephrology, 7th edn. Springer-Verlag, Berlin, pp 613–636CrossRef Van der Watt G, Omar F, Brink A, McCulloch M (2016) Laboratory investigation of the child with suspected renal disease. In: Avner ED, Harmon WE, Niaudet P et al (eds) Pediatric nephrology, 7th edn. Springer-Verlag, Berlin, pp 613–636CrossRef
29.
Zurück zum Zitat Loewen J, Greenbaum LA (2016) Diagnostic imaging of the child with suspected renal disease. In: Avner ED, Harmon WE, Niaudet P et al (eds) Pediatric nephrology, 7th edn. Springer-Verlag, Berlin, pp 667–704CrossRef Loewen J, Greenbaum LA (2016) Diagnostic imaging of the child with suspected renal disease. In: Avner ED, Harmon WE, Niaudet P et al (eds) Pediatric nephrology, 7th edn. Springer-Verlag, Berlin, pp 667–704CrossRef
30.
Zurück zum Zitat Tourchi A, Di Carlo HN, Inouye BM, Young E et al (2015) Ureteral reimplantation before bladder neck reconstruction in modern staged repair of exstrophy patients: indications and outcomes. Urology 85:905–908CrossRefPubMed Tourchi A, Di Carlo HN, Inouye BM, Young E et al (2015) Ureteral reimplantation before bladder neck reconstruction in modern staged repair of exstrophy patients: indications and outcomes. Urology 85:905–908CrossRefPubMed
31.
Zurück zum Zitat Dixon WJ (1983) BMPD Statistical software. University of California Press, Los Angeles Dixon WJ (1983) BMPD Statistical software. University of California Press, Los Angeles
32.
Zurück zum Zitat Bell CS, Samuel JP, Samuels JA (2019) Prevalence of hypertension in children. Hypertension 73:148–152CrossRefPubMed Bell CS, Samuel JP, Samuels JA (2019) Prevalence of hypertension in children. Hypertension 73:148–152CrossRefPubMed
33.
Zurück zum Zitat Pereira PL, Moreno Valle JA, Espinosa L et al (2008) Entercystoplasty in children with neuropathic bladders: long-term follow-up. J Pediatr Urol 4:27–31CrossRef Pereira PL, Moreno Valle JA, Espinosa L et al (2008) Entercystoplasty in children with neuropathic bladders: long-term follow-up. J Pediatr Urol 4:27–31CrossRef
34.
Zurück zum Zitat Woodhouse CR, Neild GH, Yu RN, Bauer S (2012) Adult care of children from pediatric urology. J Urol 187:1164–1171CrossRefPubMed Woodhouse CR, Neild GH, Yu RN, Bauer S (2012) Adult care of children from pediatric urology. J Urol 187:1164–1171CrossRefPubMed
35.
Zurück zum Zitat Haddad E, Sancaktutar AA, Palmer BW, Aston C, Kropp BP (2018) Who, where, and why are patients lost to follow-up? A 20-year study of bladder exstrophy patients at a single institution. J Pediatr Urol 14:276.e1-276.e6CrossRefPubMed Haddad E, Sancaktutar AA, Palmer BW, Aston C, Kropp BP (2018) Who, where, and why are patients lost to follow-up? A 20-year study of bladder exstrophy patients at a single institution. J Pediatr Urol 14:276.e1-276.e6CrossRefPubMed
Metadaten
Titel
Exstrophy-epispadias complex: are the kidneys and kidney function spared?
verfasst von
Roxana Cleper
Danith Blumenthal
Yossi Beniamini
Shiran Friedman
Yuval Bar Yosef
Jacob Ben Chaim
Publikationsdatum
06.02.2023
Verlag
Springer Berlin Heidelberg
Erschienen in
Pediatric Nephrology / Ausgabe 8/2023
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-023-05889-y

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