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  Many of the anomalies encountered in pediatric urologic gynecology result from problems related to abnormal embryogenesis of the urinary and genital tracts. Given the close association between the development of the urinary and genital systems, disorders observed in one may be a harbinger for an anomaly of the other.
  Today, most urogynecologic disorders are detected even prior to birth with antenatal ultrasonography (US). Most of the remaining abnormalities are detected during infancy or childhood as the result of urinary tract infections (UTIs), urinary incontinence, or abnormalities (eg, interlabial mass, otherwise abnormal-appearing external genitalia) detected on physical examination. [1] Some urogenital malformations, however, are more elusive and only become evident later in life when a clinical problem arises.
  Because congenital abnormalities result from abnormal embryogenesis, beginning with a brief overview of healthy female embryology is appropriate.
  Congenital abnormalities commonly involve the urinary and genital tracts. An understanding of normal embryogenesis is a prerequisite for understanding congenital abnormalities.
  Marshall [2] and Stephens et al [3] provided a detailed discussion of the embryology of the lower urinary tract. The divergence of normal sexual differential into male and female phenotypes begins in week 9 of gestation. In the absence of a Y chromosome, the gonads develop into ovaries rather than testes. As a result, the wolffian system regresses because of an absence of androgen production, and the müllerian ducts form the fallopian tubes, the uterus, and the proximal part of the vagina. Formation of the female genital tract also depends on the partition of the cloaca, first by the urorectal septum and then by the urogenital septum.
  The incidence of bladder exstrophy is 1 per 30,000 live births and is lower in females than in males. It is part of a continuum of developmental anomalies that result from a persisting cloaca or an abnormally large cloaca that does not retract normally toward the perineum. Bladder exstrophy is a more severe anomaly, whereas epispadias is a less severe variant of the exstrophy-epispadias complex.
  The initial management of bladder exstrophy involves covering the exposed bladder with a nonocclusive dressing (eg, plastic food wrap) to protect the bladder plate. To prevent unnecessary trauma, ligating the umbilical cord with silk ties rather than a plastic or metal clamp is best. Some children with bladder exstrophy develop functional bladder capacities, whereas others go on to develop small and poorly compliant bladders. The reason for this is not completely understood.