title: Urinary incontinence - an unusual indication for early vaginoplasty in late presenting congenital adrenal hyperplasia.
abstract: BACKGROUND
  Congenital adrenal hyperplasia (CAH) is an autosomal recessive condition leading to deficient cortisol with an incidence of 1/16,000. Patients with CAH typically present early with ambiguous genitalia or as an emergency with adrenal crisis.
  
  
  METHODS
  We report an atypical late presentation of a 4-year-old girl with pubertal-like symptoms and urinary incontinence, due to a persistent urogenital sinus (UGS). An early vaginoplasty was successfully performed allowing the patient to achieve continence.
  
  
  CONCLUSIONS
  Literature describing the symptoms of CAH with UGS is scarce. The case is unusual in demonstrating pubertal-like symptoms and urinary incontinence due to the late presentation of a persistent UGS, highlighting the need for an open mind in assessment of children with urinary incontinence. Timing of surgery is controversial, and cases need to be considered on an individual basis.
authors.affiliation: Department of Paediatric Surgery, Alder Hey Children's Hospital, Eaton Road, Liverpool L12 2AP, United Kingdom. Electronic address: benjaminkychan@gmail.com.
authors.forename: B K Y
authors.initials: BK
authors.lastname: Chan
authors.forename: R R
authors.initials: RR
authors.lastname: Turnock
authors.forename: H F
authors.initials: HF
authors.lastname: McAndrew
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000150:complications*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D011629:Puberty, Precocious / Q000150:complications; D014521:Urethra / Q000002:abnormalities / Q000601:surgery*; D014549:Urinary Incontinence / Q000209:etiology / Q000601:surgery*; D014621:Vagina / Q000002:abnormalities / Q000601:surgery*
keywords: Congenital adrenal hyperplasia; Genitoplasty; Incontinence; Urogenital sinus; Vaginoplasty