title: Bladder after total urogenital mobilization for congenital adrenal hyperplasia and cloaca--does it behave the same?
abstract: OBJECTIVE
  Followup of total urogenital mobilization for persistent urogenital sinus is well established anatomically and functionally. Nevertheless, studies comparing bladder function in different subsets of patients with urogenital sinus, such as congenital adrenal hyperplasia and cloaca, are scant.
  
  
  METHODS
  We reviewed the records of patients with congenital adrenal hyperplasia and cloaca who underwent total urogenital mobilization and urodynamics in the last 10 years. Those with a short urogenital sinus (less than 2.5 cm) not requiring an abdominal approach and without spinal dysraphism were selected for study. Urodynamics were performed postoperatively before and after toilet training, and compared between patients with congenital adrenal hyperplasia and cloaca. Methods, definitions and units conformed to International Continence Society/International Children's Continence Society standards. For the emptying phase we defined bladder outlet obstruction as maximum detrusor pressure greater than 70 cm H(2)O and underactive detrusor as maximum detrusor pressure less than 20 cm H(2)O plus post-void residual urine greater than 25 ml.
  
  
  RESULTS
  Six patients with congenital adrenal hyperplasia and 6 with cloaca met study criteria. Three patients with congenital adrenal hyperplasia and 4 with cloaca underwent urodynamics before and after toilet training at a median age of 2 (range 2 to 4) and 5 years (range 3 to 8), respectively. Urodynamics were done in 1 patient with congenital adrenal hyperplasia before toilet training, and in 2 with congenital adrenal hyperplasia and 2 with cloaca after toilet training. All patients had normal urodynamics except 1 with congenital adrenal hyperplasia and detrusor overactivity, which normalized after toilet training. In all cloaca cases urodynamics were abnormal. Before toilet training bladder outlet obstruction was found in 2 patients, detrusor underactivity was found in 1 and detrusor overactivity was found in the remaining 1. After toilet training a detrusor underactivity pattern was found in 4 patients and bladder outlet obstruction was found in 2. All patients except 1 with cloaca had post-void residual urine before and after toilet training (median 100 ml, range 25 to 200). After toilet training all patients with congenital adrenal hyperplasia became spontaneously dry and all with cloaca were placed on clean intermittent catheterization.
  
  
  CONCLUSIONS
  In the long term patients with cloaca show bladder outlet obstruction or underactive/acontractile detrusor patterns, which are not noted in patients with congenital adrenal hyperplasia. Therefore, in patients with cloaca urogenital sinus length may not be as good an indicator of functional results as it is in patients with congenital adrenal hyperplasia. Whether additional rectal dissection and repositioning surgical procedures in cloaca cases may have a role in explaining such a difference remains to be clarified.
authors.affiliation: Department of Nephrology and Urology, Bambino Gesù Children's Hospital, Rome, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Camanni
authors.forename: Antonio
authors.initials: A
authors.lastname: Zaccara
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Capitanucci
authors.forename: Giovanni
authors.initials: G
authors.lastname: Mosiello
authors.forename: Barbara D
authors.initials: BD
authors.lastname: Iacobelli
authors.forename: Mario
authors.initials: M
authors.lastname: De Gennaro
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000601:surgery*; D002675:Child, Preschool; D002988:Cloaca / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D012189:Retrospective Studies; D001743:Urinary Bladder / Q000502:physiology*; D014563:Urodynamics; D014566:Urogenital System / Q000601:surgery; D013520:Urologic Surgical Procedures / Q000379:methods