Sphincterplasty for Velopharyngeal Insufficiency in the Child Without a Cleft-Palate: Etiologies and Speech Outcomes

J Craniofac Surg. 2015 Oct;26(7):2067-71. doi: 10.1097/SCS.0000000000001967.

Abstract

The aim of this study is to report on speech outcomes following surgery for velopharyngeal insufficiency (VPI) on a broad spectrum of patients without a cleft palate. Inclusion criteria included patients without a cleft palate operated on by a single surgeon (JKW) over a 10-year period and postoperative speech evaluation within 1 year. All patients underwent a sphincter pharyngoplasty. The main outcome measures were perceptual speech assessment using a 6-point scale (1 = none or normal, 6 = severe); velopharyngeal function (VPF) (1 = adequate, 2 = marginal, 3 = dequate); and quantitative nasalance score. Forty children (mean age 7.5 y) were included. Eight unique conditions were identified; the most common was 22q deletion syndromes (27.5%). All patients had a deep nasopharynx, mean nasopharyngeal depth >0.67. Two novel cases are presented in each child with mosaic Trisomy 14 and ring chromosome 18 abnormality. Of all patients, 87.5% improved their postoperative hypernasality score. Preoperatively, all patients had either marginal or inadequate VPF (2 or 3). Postoperatively, 90% of patients (n = 36) achieved adequate velar function, the remainder did not improve at the first postoperative evaluation. Intelligibility and audible nasal emissions improved in between 57% and 65% of patients. Articulation proficiency was the only perceptual rating not to improve initially, but then did so on the most recent postoperative speech evaluation. This study demonstrates successful speech outcomes in a diverse group of patients. It also increases awareness of noncleft VPI amenable to surgical correction.

MeSH terms

  • 22q11 Deletion Syndrome / complications
  • Cephalometry / methods
  • Child
  • Chromosomes, Human, Pair 14 / genetics
  • Chromosomes, Human, Pair 18 / genetics
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Mosaicism
  • Nasopharynx / diagnostic imaging
  • Palate, Soft / physiology
  • Pharynx / physiology
  • Radiography
  • Ring Chromosomes
  • Speech / physiology*
  • Speech Intelligibility / physiology
  • Treatment Outcome
  • Trisomy / genetics
  • Velopharyngeal Insufficiency / diagnostic imaging
  • Velopharyngeal Insufficiency / etiology
  • Velopharyngeal Insufficiency / surgery*
  • Velopharyngeal Sphincter / diagnostic imaging
  • Velopharyngeal Sphincter / physiology
  • Velopharyngeal Sphincter / surgery*
  • Voice Quality / physiology

Supplementary concepts

  • Chromosome 14, trisomy mosaic
  • Chromosome 18 ring