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AJP Rep 2015; 05(02): e183-e187
DOI: 10.1055/s-0035-1556759
Case Report
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Exstrophy–Epispadias Complex in a Newborn: Case Report and Review of the Literature

Enrico Valerio
1   Department of Woman and Child Health, Neonatal Intensive Care Unit, Medical School, University of Padua, Padova, Italy
,
Valentina Vanzo
1   Department of Woman and Child Health, Neonatal Intensive Care Unit, Medical School, University of Padua, Padova, Italy
,
Patrizia Zaramella
1   Department of Woman and Child Health, Neonatal Intensive Care Unit, Medical School, University of Padua, Padova, Italy
,
Sabrina Salvadori
1   Department of Woman and Child Health, Neonatal Intensive Care Unit, Medical School, University of Padua, Padova, Italy
,
Marco Castagnetti
2   Section of Pediatric Urology, Urology Unit, University Hospital of Padova, Padua, Italy
,
Eugenio Baraldi
1   Department of Woman and Child Health, Neonatal Intensive Care Unit, Medical School, University of Padua, Padova, Italy
› Institutsangaben
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Publikationsverlauf

17. Januar 2015

19. Mai 2015

Publikationsdatum:
07. September 2015 (online)

   Lizenzen und Reprints

Abstract

Aim The aim of this report is to present a brief review of the current literature on the management of EEC.

Case Report A term male neonate presented at birth with classic bladder exstrophy, a variant of the exstrophy-epispadias complex (EEC). The defect was covered with sterile silicon gauzes and waterproof dressing; at 72 hours of life, primary closure without osteotomy of bladder, pelvis, and abdominal wall was successfully performed.

Discussion EEC incidence is approximately 2.15 per 1,00,000 live births; several urological, musculocutaneous, spinal, orthopedic, gastrointestinal, and gynecological anomalies may be associated to EEC. Initial medical management includes use of occlusive dressings to prevent air contact and dehydration of the open bladder template. Umbilical catheters should not be positioned. Surgical repair stages include initial closure of the bladder and abdominal wall with or without osteotomy, followed by epispadias repair at 6 to 12 months, and bladder neck repair around 5 years of life. Those who fail to attain continence eventually undergo bladder augmentation and placement of a catheterizable conduit.

Conclusion Modern-staged repair of EEC guarantees socially acceptable urinary continence in up to 80% of cases; sexual function can be an issue in the long term, but overall quality of life can be good.

Keywords

exstrophy–epispadias complex - neonatology - newborn - congenital disease - urology
 

  • References

  • 1 Meldrum KK, Baird AD, Gearhart JP. Pelvic and extremity immobilization after bladder exstrophy closure: complications and impact on success. Urology 2003; 62 (6) 1109-1113
    CrossrefPubMedGoogle Scholar
  • 2 Castagnetti M, Berrettini A, Beniamin F, Rigamonti W. Thoraco-pelvic orthosis: a portable and removable bassinet for secure patient immobilization after primary bladder exstrophy closure. Pediatr Surg Int 2010; 26 (12) 1229-1232
    CrossrefPubMedGoogle Scholar
  • 3 Stec AA. Embryology and bony and pelvic floor anatomy in the bladder exstrophy-epispadias complex. Semin Pediatr Surg 2011; 20 (2) 66-70
    CrossrefPubMedGoogle Scholar
  • 4 Inouye BM, Tourchi A, Di Carlo HN, Young EE, Gearhart JP. Modern management of the exstrophy-epispadias complex. Surg Res Pract 2014; 2014: 587064
    PubMedGoogle Scholar
  • 5 Nelson CP, Dunn RL, Wei JT. Contemporary epidemiology of bladder exstrophy in the United States. J Urol 2005; 173 (5) 1728-1731
    CrossrefPubMedGoogle Scholar
  • 6 Lattimer JK, Smith MJ. Exstrophy closure: a followup on 70 cases. J Urol 1966; 95 (3) 356-359
    PubMedGoogle Scholar
  • 7 Evans JA, Darvill KD, Trevenen C, Rockman-Greenberg C. Cloacal exstrophy and related abdominal wall defects in Manitoba: incidence and demographic factors. Clin Genet 1985; 27 (3) 241-251
    PubMedGoogle Scholar
  • 8 Pierre K, Borer J, Phelps A, Chow JS. Bladder exstrophy: current management and postoperative imaging. Pediatr Radiol 2014; 44 (7) 768-786 , quiz 765–767
    CrossrefPubMedGoogle Scholar
  • 9 Ebert AK, Reutter H, Ludwig M, Rösch WH. The exstrophy-epispadias complex. Orphanet J Rare Dis 2009; 4: 23
    CrossrefPubMedGoogle Scholar
  • 10 Jones D, Parkinson S, Hosalkar HS. Oblique pelvic osteotomy in the exstrophy/epispadias complex. J Bone Joint Surg Br 2006; 88 (6) 799-806
    PubMedGoogle Scholar
  • 11 Baird AD, Sponseller PD, Gearhart JP. The place of pelvic osteotomy in the modern era of bladder exstrophy reconstruction. J Pediatr Urol 2005; 1 (1) 31-36
    CrossrefPubMedGoogle Scholar
  • 12 Inouye BM, Tourchi A, Di Carlo HN , et al. Safety and efficacy of staged pelvic osteotomies in the modern treatment of cloacal exstrophy. J Pediatr Urol 2014; 10 (6) 1244-1248
    CrossrefPubMedGoogle Scholar
  • 13 Massanyi EZ, Gearhart JP, Kost-Byerly S. Perioperative management of classic bladder exstrophy. Res Rep Urol 2013; 5: 67-75
    PubMedGoogle Scholar
  • 14 Mitchell ME. Bladder exstrophy repair: complete primary repair of exstrophy. Urology 2005; 65 (1) 5-8
    CrossrefPubMedGoogle Scholar
  • 15 Woodhouse CR, North AC, Gearhart JP. Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder. World J Urol 2006; 24 (3) 244-249
    CrossrefPubMedGoogle Scholar
  • 16 Stewart D, Inouye BM, Goldstein SD , et al. Pediatric surgical complications of major genitourinary reconstruction in the exstrophy-epispadias complex. J Pediatr Surg 2015; 50 (1) 167-170
    CrossrefPubMedGoogle Scholar
  • 17 Inouye BM, Shah BB, Massanyi EZ , et al. Urologic complications of major genitourinary reconstruction in the exstrophy-epispadias complex. J Pediatr Urol 2014; 10 (4) 680-687
    CrossrefPubMedGoogle Scholar
  • 18 Ebert AK, Schott G, Bals-Pratsch M, Seifert B, Rösch WH. 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 2010; 6 (1) 6-10
    CrossrefPubMedGoogle Scholar
  • 19 Novak TE, Costello JP, Orosco R, Sponseller PD, Mack E, Gearhart JP. Failed exstrophy closure: management and outcome. J Pediatr Urol 2010; 6 (4) 381-384
    CrossrefPubMedGoogle Scholar
  • 20 Mouskou S, Dionysis TT, Aivazoglou T, Kapouleas GP. Long-term follow up of complete bladder exstrophy repair. A case report. Folia Med (Plovdiv) 2014; 56 (1) 60-63
    PubMedGoogle Scholar
  • 21 Gearhart JP, Mathews RI. Exstrophy-epispadias complex. In: Wein AJ, Kavoussi LR, Novick AC, Partin AW, Peters CA, eds. Campbell-Walsh Urology, vol. 4, 10th edition. Philadelphia, PA: Elsevier; 2012: 3325-3378
    CrossrefGoogle Scholar
  • 22 Woodhouse CR. Sexual function in boys born with exstrophy, myelomeningocele, and micropenis. Urology 1998; 52 (1) 3-11
    CrossrefPubMedGoogle Scholar
  • 23 Castagnetti M, Berrettini A, Zhapa E, Rigamonti W, Zattoni F. Issues with the external and internal genitalia in postpubertal females born with classic bladder exstrophy: a surgical series. J Pediatr Adolesc Gynecol 2011; 24 (1) 48-52
    CrossrefPubMedGoogle Scholar
  • 24 Gupta AD, Goel SK, Woodhouse CR, Wood D. Examining long-term outcomes of bladder exstrophy: a 20-year follow-up. BJU Int 2014; 113 (1) 137-141
    CrossrefPubMedGoogle Scholar
  • 25 Castagnetti M, Gigante C, Perrone G, Rigamonti W. Comparison of musculoskeletal and urological functional outcomes in patients with bladder exstrophy undergoing repair with and without osteotomy. Pediatr Surg Int 2008; 24 (6) 689-693
    CrossrefPubMedGoogle Scholar
  • 26 Kouame BD, Kouame GS, Sounkere M , et al. Aesthetic, urological, orthopaedic and functional outcomes in complex bladder exstrophy-epispadias's management. Afr J Paediatr Surg 2015; 12 (1) 56-60
    CrossrefPubMedGoogle Scholar