title: Velopharyngeal dysfunction and 22q11.2 deletion syndrome: a longitudinal study of functional outcome and preoperative prognostic factors.
abstract: OBJECTIVE
  To describe the effect of time after velopharyngoplasty on outcome and to search for preoperative prognostic factors for residual hypernasality in patients with 22q11.2 deletion syndrome.
  
  
  METHODS
  Retrospective chart review.
  
  
  METHODS
  Tertiary hospital.
  
  
  METHODS
  Patients with 22q11.2 deletion syndrome and velopharyngeal dysfunction who underwent a primary (modified) Honig velopharyngoplasty between 1989 and 2009.
  
  
  METHODS
  Clinically obtained perceptual and instrumental measurements of resonance, nasalance, and understandability before and after velopharyngoplasty.
  
  
  RESULTS
  Data were available for 44 of 54 patients (81% follow-up), with a mean follow-up time of 7.0 years (range, 1.0 to 19.4 years). During follow-up, 24 (55%) patients attained normal resonance and 20 (45%) had residual hypernasality or underwent revision surgery. Mean postoperative nasalance and understandability scores were closer to the norm than mean preoperative scores were (2.0 versus 5.5 standard deviations for the normal passage, 1.3 versus 8.1 standard deviations for the nonnasal passage, and score 2.3 versus 4.1 understandability). Serial measurements revealed that hypernasality only resolved an average of 5 years after surgery, and three patients whose resonance initially normalized later relapsed to hypernasality. Gender, age at surgery, lateral pharyngeal wall adduction, velar elevation, presence of a palatal defect, previous intravelar veloplasty, nasalance, understandability, adenoidectomy, hearing loss, and IQ were not able to predict poor outcome following primary velopharyngoplasty (all p > .05).
  
  
  CONCLUSIONS
  In this chart review of patients with 22q11.2 deletion syndrome and velopharyngeal dysfunction, residual hypernasality persisted in many patients after velopharyngoplasty. None of the preoperative factors that were studied had prognostic value for the outcome.
authors: Department of Plastic Surgery, University Medical Center Utrecht, KE 04.140.0, Postbus 85090, 3508 AB Utrecht, The Netherlands.
authors: N E
authors: NE
authors: Spruijt
authors: J C C
authors: JC
authors: Widdershoven
authors: C C
authors: CC
authors: Breugem
authors: L
authors: L
authors: Speleman
authors: I L M
authors: IL
authors: Homveld
authors: M
authors: M
authors: Kon
authors: A B
authors: AB
authors: Mink van der Molen
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics*; D005260:Female; D017353:Gene Deletion; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D013064:Speech Disorders / Q000503:physiopathology*; D013577:Syndrome; D016896:Treatment Outcome; D014681:Velopharyngeal Insufficiency / Q000235:genetics* / Q000503:physiopathology* / Q000601:surgery