title: Reconstructive surgery for females with congenital adrenal hyperplasia due to 21-hydroxylase deficiency: a review from the Prince of Wales Hospital.
abstract: OBJECTIVE
  To present the results of feminising genitoplasty done in female patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
  
  
  METHODS
  Case series.
  
  
  METHODS
  A tertiary referral centre in Hong Kong.
  
  
  METHODS
  Female patients with congenital adrenal hyperplasia undergoing corrective surgery for virilisation between 1993 and 2012.
  
  
  METHODS
  The operative result was judged with a scoring system (1-3) for four areas: appearance of clitoris, labia and vagina, plus requirement for revision surgery.
  
  
  RESULTS
  A total of 23 female patients with congenital adrenal hyperplasia with a median age of 17.5 (range, 1.5-33.8) years were identified. Of these individuals, 17 presented in the neonatal period and early infancy, of which four had an additional salt-losing crisis. Six patients-including four migrants from mainland China-were late presenters at a median age of 2 (range, 0.5-14) years. Twenty-two patients had corrective surgery at a median age of 2 (range, 1-14) years. Clitoral reduction was performed in all, and further surgery in 21 patients. The additional surgery was flap vaginoplasty in 10 patients, a modified Passerini procedure in six, and a labial reconstruction in five; one patient with prominent clitoris was for observation only. Minor revision surgery (eg mucosal trimming) was required in three patients; a revision vaginoplasty was done in one individual. Of the 23 patients, 18 (78%) with a median age of 20 (range, 9.3-33.8) years participated in the outcome evaluation: a 'good' outcome (4 points) was seen in 12 patients and a 'satisfactory' (5-9 points) result in five patients.
  
  
  CONCLUSIONS
  Nearly three quarters of our cohort (n=17) presented with classic virilising form of 21-hydroxylase deficiency. Only four (25%) patients experienced a salt-losing crisis. Female gender assignment at birth was maintained for all individuals in this group. 'Good' and 'satisfactory' outcomes of surgery were reported in nearly all participants.
authors.affiliation: Division of Paediatric Surgery and Paediatric Urology, Department of Surgery, Prince of Wales Hospital, Shatin, Hong Kong.
authors.forename: C H
authors.initials: CH
authors.lastname: Houben
authors.affiliation: Division of Paediatric Surgery and Paediatric Urology, Department of Surgery, Prince of Wales Hospital, Shatin, Hong Kong.
authors.forename: S Y
authors.initials: SY
authors.lastname: Tsui
authors.affiliation: Division of Paediatric Surgery and Paediatric Urology, Department of Surgery, Prince of Wales Hospital, Shatin, Hong Kong.
authors.forename: J W
authors.initials: JW
authors.lastname: Mou
authors.affiliation: Division of Paediatric Surgery and Paediatric Urology, Department of Surgery, Prince of Wales Hospital, Shatin, Hong Kong.
authors.forename: K W
authors.initials: KW
authors.lastname: Chan
authors.affiliation: Division of Paediatric Surgery and Paediatric Urology, Department of Surgery, Prince of Wales Hospital, Shatin, Hong Kong.
authors.forename: Y H
authors.initials: YH
authors.lastname: Tam
authors.affiliation: Division of Paediatric Surgery and Paediatric Urology, Department of Surgery, Prince of Wales Hospital, Shatin, Hong Kong.
authors.forename: K H
authors.initials: KH
authors.lastname: Lee
mesh_terms: D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000601:surgery*; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006723:Hong Kong; D006761:Hospitals; D006801:Humans; D007223:Infant; D019651:Plastic Surgery Procedures; D013255:Steroid 21-Hydroxylase; D016896:Treatment Outcome
chemical_list: D013255:Steroid 21-Hydroxylase
keywords: Adrenal hyperplasia, congenital; Female; Steroid 21-hydroxylase