Abstract
The care of a newborn with the exstrophy-epispadias complex presents a formidable challenge to the pediatric surgical community. The approach to these children is a multi-disciplinary effort involving multiple subspecialties including urologists, surgeons, orthopedists, pediatric anesthesiologists and specialty nurses. Advances in the basic and clinical sciences in the past two decades have yielded insight into the embryologic, genetic, and the physiology of exstrophy. Despite multiple approaches to the surgical management of the exstrophic patients are used throughout the world, the critically important part of the surgical care is a successful primary closure. The best long-term outcomes can only be achieved with a successful primary closure, and each failure decreases a patient’s chance at voided continence. This chapter aims to examine all aspects of the exstrophy patient including the surgical management and long term prognosis.
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References
Buyukunal CS, Gearhart JP. A short history of bladder exstrophy. Semin Pediatr Surg. 2011;20(2):62–5.
Syme J. Ectopia vesicae. Lancet. 1852;2.
Murphy L. The history of urology. Springfield: Charles C. Thomas; 1972.
Trendelenburg F. De la cure operatoire de l’exstrophie vesicale et de l’epispadias. Arch Klin Chir. 1892;43:394.
Young, H., Exstrophy of the bladder: the first case in which a normal bladder and urinary control have been obtained by plastic operation. Surg Gynecol Obstet. 1942;74:729–37.
Rickham PP. Vesico-intestinal Fissure. Arch Dis Child. 1960;35(179):97–102.
Hollowell JG, Ransley PG. Surgical management of incontinence in bladder exstrophy. Br J Urol. 1991;68(5):543–8.
Lepor H, Jeffs RD. Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy. J Urol. 1983;130(6):1142–5.
Mesrobian HG, Kelalis PP, Kramer SA. Long-term followup of 103 patients with bladder exstrophy. J Urol. 1988;139(4):719–22.
Stec AA. Embryology and bony and pelvic floor anatomy in the bladder exstrophy-epispadias complex. Semin Pediatr Surg. 2011;20(2):66–70.
Ludwig M, et al. Bladder exstrophy-epispadias complex. Birth Defects Res A Clin Mol Teratol. 2009;85(6):509–22.
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):244–9.
Ricketts RR, et al. Modern treatment of cloacal exstrophy. J Pediatr Surg. 1991;26(4):444–8; discussion 448–50
Gargollo PC, Borer JG. Contemporary outcomes in bladder exstrophy. Curr Opin Urol. 2007;17(4):272–80.
Lattimer JK, Smith MJ. Exstrophy closure: a followup on 70 cases. J Urol. 1966;95(3):356–9.
Epidemiology of bladder exstrophy and epispadias: a communication from the International Clearinghouse for Birth Defects Monitoring Systems. Teratology. 1987;36(2): 221–7.
Nelson CP, Dunn RL, Wei JT. Contemporary epidemiology of bladder exstrophy in the United States. J Urol. 2005;173(5):1728–31.
Shapiro E, Lepor H, Jeffs RD. The inheritance of the exstrophy-epispadias complex. J Urol. 1984;132(2):308–10.
Wood HM, Trock BJ, Gearhart JP. In vitro fertilization and the cloacal-bladder exstrophy-epispadias complex: is there an association? J Urol. 2003;169(4):1512–5.
Hurwitz RS, et al. Cloacal exstrophy: a report of 34 cases. J Urol. 1987;138(4 Pt 2):1060–4.
Keppler-Noreuil KM. OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): a review of 14 cases. Am J Med Genet. 2001;99(4):271–9.
Diamond DA, Jeffs RD. Cloacal exstrophy: a 22-year experience. J Urol. 1985;133(5):779–82.
Caton AR, et al. Epidemiology of bladder and cloacal exstrophies in New York State, 1983–1999. Birth Defects Res A Clin Mol Teratol. 2007;79(11):781–7.
Smith NM, et al. The OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): recurrence in sibs. J Med Genet. 1992;29(10):730–2.
Botto LD, Mulinare J, Erickson JD. Occurrence of omphalocele in relation to maternal multivitamin use: a population-based study. Pediatrics. 2002;109(5):904–8.
Mills JL, et al. Folate and vitamin B12-related genes and risk for omphalocele. Hum Genet. 2012;131(5):739–46.
Reutter H, et al. Family-based association study of the MTHFR polymorphism C677T in the bladder-exstrophy-epispadias-complex. Am J Med Genet A. 2006;140(22):2506–9.
Gambhir L, et al. Epidemiological survey of 214 families with bladder exstrophy-epispadias complex. J Urol. 2008;179(4):1539–43.
Draaken M, et al. Genome-wide association study and meta-analysis identify ISL1 as genome-wide significant susceptibility gene for bladder exstrophy. PLoS Genet. 2015;11(3):e1005024.
Joan Ko KL, Yan G, Di Carlo H, Isaacs W, Gearhart J. Rs9291768 risk allele frequency in patients with bladder exstrophy-epispadias complex, in The Society for Pediatric Urology Annual Meeting, 2016, San Diego, CA.
Reutter H, et al. Genetics of bladder-exstrophy-epispadias complex (BEEC): systematic elucidation of Mendelian and multifactorial phenotypes. Curr Genomics. 2016;17(1):4–13.
Geoffroy-Saint-Hilaire I. Historie generale et particulaire des anomalies de lorganisation chez l’homme et les animaux, ouvrage comprenant des recherches sur les caracteres, la classification, linfluence physiologique et pathologique, les rapports generaux, les lois et les causes des monstruosities, des varietes et vices de conformation. Traite de teratologie. Paris: JP Bailliere; 1832.
Stec AA, et al. Classic bladder exstrophy in a nonhuman primate: a comparative analysis. Urology. 2002;59(2):180–3.
Muecke EC. The role of the Cloacal membrane in exstrophy: the first successful experimental study. J Urol. 1964;92:659–67.
van der Putte SC. Normal and abnormal development of the anorectum. J Pediatr Surg. 1986;21(5):434–40.
Thomalla JV, et al. Induction of cloacal exstrophy in the chick embryo using the CO2 laser. J Urol. 1985;134(5):991–5.
Slaughenhoupt BL, Chen CJ, Gearhart JP. Creation of a model of bladder exstrophy in the fetal lamb. J Urol. 1996;156(2 Pt 2):816–8.
Mildenberger H, Kluth D, Dziuba M. Embryology of bladder exstrophy. J Pediatr Surg. 1988;23(2):166–70.
Fein RL. Artificial exstrophy in the dog for separated renal function studies. J Surg Res. 1969;9(4):235–9.
Ambrose SS, O’Brien DP 3rd. Surgical embryology of the exstrophy-epispadias complex. Surg Clin North Am. 1974;54(6):1379–90.
Marshall VF, Muecke EC. Handbuch de Urologie. New York: Springer-Verlag; 1968.
Sponseller PD, et al. The anatomy of the pelvis in the exstrophy complex. J Bone Joint Surg Am. 1995;77(2):177–89.
Stec AA, et al. Evaluation of the bony pelvis in classic bladder exstrophy by using 3D-CT: further insights. Urology. 2001;58(6):1030–5.
Stec AA, et al. Fetal bony pelvis in the bladder exstrophy complex: normal potential for growth? Urology. 2003;62(2):337–41.
Stec AA, et al. Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial insights. J Urol. 2001;166(4):1444–9.
Williams AM, et al. 3-dimensional magnetic resonance imaging modeling of the pelvic floor musculature in classic bladder exstrophy before pelvic osteotomy. J Urol. 2004;172(4 Pt 2):1702–5.
Connolly JA, et al. Prevalence and repair of inguinal hernias in children with bladder exstrophy. J Urol. 1995;154(5):1900–1.
Silver RI, et al. Penile length in adulthood after exstrophy reconstruction. J Urol. 1997;157(3):999–1003.
Novak TE, et al. Polyps in the exstrophic bladder. A cause for concern? J Urol. 2005;174(4 Pt 2):1522–6; discussion 1526
Canning DA, et al. The cephalotrigonal reimplant in bladder neck reconstruction for patients with exstrophy or epispadias. J Urol. 1993;150(1):156–8.
Mathews R, et al. Cloacal exstrophy—improving the quality of life: the Johns Hopkins experience. J Urol. 1998;160(6 Pt 2):2452–6.
Diamond DA. Management of cloacal exstrophy. Dial Pediatr Urol. 1990;13:2.
McHoney M, et al. Cloacal exstrophy: morbidity associated with abnormalities of the gastrointestinal tract and spine. J Pediatr Surg. 2004;39(8):1209–13.
Lund DP, Hendren WH. Cloacal exstrophy: a 25-year experience with 50 cases. J Pediatr Surg. 2001;36(1):68–75.
Schlegel PN, Gearhart JP. Neuroanatomy of the pelvis in an infant with cloacal exstrophy: a detailed microdissection with histology. J Urol. 1989;141(3):583–5.
Jain M, Weaver DD. Severe lower limb defects in exstrophy of the cloaca. Am J Med Genet A. 2004;128A(3):320–4.
Gearhart JP, et al. Criteria for the prenatal diagnosis of classic bladder exstrophy. Obstet Gynecol. 1995;85(6):961–4.
Mirk P, Calisti A, Fileni A. Prenatal sonographic diagnosis of bladder exstrophy. J Ultrasound Med. 1986;5:291–3.
Verco PW, et al. Ectopia vesicae in utero. Australas Radiol. 1986;30(2):117–20.
Meizner I, Bar-Ziv J. Prenatal ultrasonic diagnosis of cloacal exstrophy. Am J Obstet Gynecol. 1985;153(7):802–3.
Hamada H, et al. New ultrasonographic criterion for the prenatal diagnosis of cloacal exstrophy: elephant trunk-like image. J Urol. 1999;162(6):2123–4.
Austin PF, et al. The prenatal diagnosis of cloacal exstrophy. J Urol. 1998;160(3 Pt 2):1179–81.
Arnaoutoglou C, et al. Outcome of antenatally diagnosed fetal anterior abdominal wall defects from a single tertiary centre. Fetal Diagn Ther. 2008;24(4):416–9.
Chan DY, Jeffs RD, Gearhart JP. Determinants of continence in the bladder exstrophy population: predictors of success? Urology. 2001;57(4):774–7.
Dodson JL, et al. The newborn exstrophy bladder inadequate for primary closure: evaluation, management and outcome. J Urol. 2001;165(5):1656–9.
Gearhart JP, et al. A combined vertical and horizontal pelvic osteotomy approach for primary and secondary repair of bladder exstrophy. J Urol. 1996;155(2):689–93.
Wild AT, et al. The role of osteotomy in surgical repair of bladder exstrophy. Semin Pediatr Surg. 2011;20(2):71–8.
Hanna MK. Reconstruction of umbilicus during functional closure of bladder exstrophy. Urology. 1986;27(4):340–2.
Gearhart JP, Jeffs RD. Management of the failed exstrophy closure. J Urol. 1991;146(2 Pt 2):610–2.
Grady RW, Mitchell ME. Complete primary repair of exstrophy. J Urol. 1999;162(4):1415–20.
Mitchell ME, Bagli DJ. Complete penile disassembly for epispadias repair: the Mitchell technique. J Urol. 1996;155(1):300–4.
Husmann DA, Gearhart JP. Loss of the penile glans and/or corpora following primary repair of bladder exstrophy using the complete penile disassembly technique. J Urol. 2004;172(4 Pt 2):1696–700; discussion 1700–1
Jarzebowski AC, et al. The Kelly technique of bladder exstrophy repair: continence, cosmesis and pelvic organ prolapse outcomes. J Urol. 2009;182(4 Suppl):1802–6.
Stolar CH, Randolph JG, Flanigan LP. Cloacal exstrophy: individualized management through a staged surgical approach. J Pediatr Surg. 1990;25(5):505–7.
Soffer SZ, et al. Cloacal exstrophy: a unified management plan. J Pediatr Surg. 2000;35(6):932–7.
Howell C, et al. Optimal management of cloacal exstrophy. J Pediatr Surg. 1983;18(4):365–9.
Phillips TM. Spectrum of cloacal exstrophy. Semin Pediatr Surg. 2011;20(2):113–8.
Sawaya D, Gearhart JP. Gastrointestinal reconstruction and outcomes for patients with the OEIS complex. Semin Pediatr Surg. 2011;20(2):123–5.
Gearhart JP, Jeffs RD. Techniques to create urinary continence in the cloacal exstrophy patient. J Urol. 1991;146(2 Pt 2):616–8.
Silver RI, Sponseller PD, Gearhart JP. Staged closure of the pelvis in cloacal exstrophy: first description of a new approach. J Urol. 1999;161(1):263–6.
Mathews R, et al. Staged pelvic closure of extreme pubic diastasis in the exstrophy-epispadias complex. J Urol. 2006;176(5):2196–8.
Thomas JC, et al. First stage approximation of the exstrophic bladder in patients with cloacal exstrophy—should this be the initial surgical approach in all patients? J Urol. 2007;178(4 Pt 2):1632–5; discussion 1635–6
Sawaya D, et al. Gastrointestinal ramifications of the cloacal exstrophy complex: a 44-year experience. J Pediatr Surg. 2010;45(1):171–5; discussion 175–6
Mitchell ME, Plaire C. Management of cloacal exstrophy. Adv Exp Med Biol. 2002;511:267–70; discussion 270–3.
Novak TE, et al. Failed exstrophy closure: management and outcome. J Pediatr Urol. 2010;6(4):381–4.
Meldrum KK, Baird AD, Gearhart JP. Pelvic and extremity immobilization after bladder exstrophy closure: complications and impact on success. Urology. 2003;62(6):1109–13.
Baker LA, Jeffs RD, Gearhart JP. Urethral obstruction after primary exstrophy closure: what is the fate of the genitourinary tract? J Urol. 1999;161(2):618–21.
Husmann DA. Surgery insight: advantages and pitfalls of surgical techniques for the correction of bladder exstrophy. Nat Clin Pract Urol. 2006;3(2):95–100.
Schaeffer AJ, et al. Complications of primary closure of classic bladder exstrophy. J Urol. 2008;180(4 Suppl):1671–4; discussion 1674
Husmann DA, McLorie GA, Churchill BM. Closure of the exstrophic bladder: an evaluation of the factors leading to its success and its importance on urinary continence. J Urol. 1989;142(2 Pt 2):522–4; discussion 542–3
Cervellione RM, et al. Penile ischemic injury in the exstrophy/epispadias spectrum: new insights and possible mechanisms. J Pediatr Urol. 2010;6(5):450–6.
Schaeffer AJ, et al. Complete primary repair of bladder exstrophy: a single institution referral experience. J Urol. 2011;186(3):1041–6.
Lazarus J. Penile loss following complete primary repair of bladder exstrophy. J Pediatr Urol. 2009;5(6):519–20.
Levitt MA, et al. Cloacal exstrophy—pull-through or permanent stoma? A review of 53 patients. J Pediatr Surg. 2008;43(1):164–8; discussion 168–70
Shnorhavorian M, et al. Long-term followup of complete primary repair of exstrophy: the Seattle experience. J Urol. 2008;180(4 Suppl):1615–9; discussion 1619–20
Surer I, et al. Combined bladder neck reconstruction and epispadias repair for exstrophy-epispadias complex. J Urol. 2001;165(6 Pt 2):2425–7.
Gearhart JP, et al. The multiple reoperative bladder exstrophy closure: what affects the potential of the bladder? Urology. 1996;47(2):240–3.
Lee RS, et al. Can a complete primary repair approach be applied to cloacal exstrophy? J Urol. 2006;176(6 Pt 1):2643–8.
Mathews R. Achieving urinary continence in cloacal exstrophy. Semin Pediatr Surg. 2011;20(2):126–9.
Miles-Thomas J, Gearhart JP, Gearhart SL. An initial evaluation of pelvic floor function and quality of life of bladder exstrophy patients after ureterosigmoidostomy. J Gastrointest Surg. 2006;10(4):473–7.
El-Hout Y, et al. Do patients with classic bladder exstrophy have fecal incontinence? A web-based study. Urology. 2010;75(5):1166–8.
Wilson CJ, et al. The psychosocial impact of bladder exstrophy in adolescence. J Adolesc Health. 2007;41(5):504–8.
Reiner WG. A brief primer for pediatric urologists and surgeons on developmental psychopathology in the exstrophy-epispadias complex. Semin Pediatr Surg. 2011;20(2):130–4.
Reiner WG, Gearhart JP, Jeffs R. Psychosexual dysfunction in males with genital anomalies: late adolescence, Tanner stages IV to VI. J Am Acad Child Adolesc Psychiatry. 1999;38(7):865–72.
Woodhouse CR, Hinsch R. The anatomy and reconstruction of the adult female genitalia in classical exstrophy. Br J Urol. 1997;79(4):618–22.
Mathews RI, Gan M, Gearhart JP. Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex. BJU Int. 2003;91(9):845–9.
Ebert AK, et al. Genital and reproductive function in males after functional reconstruction of the exstrophy-epispadias complex—long-term results. Urology. 2008;72(3):566–9; discussion 569–70
D’Hauwers KW, Feitz WF, Kremer JA. Bladder exstrophy and male fertility: pregnancies after ICSI with ejaculated or epididymal sperm. Fertil Steril. 2008;89(2):387–9.
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Stuhldreher, P.P., Gearhart, J.P. (2018). The Exstrophy Complex: Bladder and Cloacal Exstrophy. In: Losty, P., Flake, A., Rintala, R., Hutson, J., lwai, N. (eds) Rickham's Neonatal Surgery. Springer, London. https://doi.org/10.1007/978-1-4471-4721-3_48
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