Literature DB >> 19015444

Outcome of velopharyngoplasty in patients with velocardiofacial syndrome.

Josine C C Widdershoven1, Bart M Stubenitsky, Corstiaan C Breugem, Aebele B MinkvanderMolen.   

Abstract

OBJECTIVE: To compare the outcomes of surgical correction of velopharyngeal insufficiency (VPI) in patients with velocardiofacial syndrome (VCFS) and a non-VCFS group.
DESIGN: Twenty-five patients with VCFS (16 girls and 9 boys) underwent palatal lengthening for VPI between 1986 and 2001. The mean age at surgery was 6.4 years. Revision was defined as the need for secondary sphincter pharyngoplasty as determined by speech investigation, nasal endoscopy, and acoustic nasometry. A comparison was made to a control group made up of a randomized group of patients without VCFS who underwent palatal lengthening for VPI (32 patients: 10 girls and 22 boys).
SETTING: Wilhelmina Children's Hospital, a tertiary referral center in Utrecht, the Netheralands. PATIENTS: A total of 57 patients who underwent palatal lengthening for VPI, 25 with VCFS and 32 without VCFS.
INTERVENTIONS: Primary surgery consisted of a palatal lengthening technique. If revision was needed, a sphincter pharyngoplasty was carried out. MAIN OUTCOME MEASURES: Pharyngeal function was assessed using perceptual speech investigation, nasal endoscopy, and acoustic nasometry.
RESULTS: In the VCFS group, 16% of the patients required surgical revision (4 of 25). These patients were slightly older at the time of primary surgery than those who did not require surgical revision (mean age, 6 vs 5.5 years). In the control group, no patients required revision. Preoperative speech analysis showed a more pronounced VPI in the VCFS group than in the control group. Outcomes of endoscopy and speech hypernasality improved significantly more in the control group than in the VCFS group. Improvement in the results of acoustic nasometry did not differ significantly between the 2 groups.
CONCLUSIONS: Treatment of VPI using palatal lengthening in children with VCFS is both safe and effective. The discrepancy in improvement between the speech analysis and the nasal endoscopy results within the VCFS group indicates that mechanical improvement does not necessarily correspond to an improvement in speech and emphasizes the complexity of speech disorders found in VCFS.

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Mesh:

Year:  2008        PMID: 19015444     DOI: 10.1001/archotol.134.11.1159

Source DB:  PubMed          Journal:  Arch Otolaryngol Head Neck Surg        ISSN: 0886-4470


  8 in total

Review 1.  Velopharyngeal insufficiency: diagnosis and management.

Authors:  Robert J Shprintzen; Eileen Marrinan
Journal:  Curr Opin Otolaryngol Head Neck Surg       Date:  2009-08       Impact factor: 2.064

Review 2.  Hard to swallow: Developmental biological insights into pediatric dysphagia.

Authors:  Anthony-Samuel LaMantia; Sally A Moody; Thomas M Maynard; Beverly A Karpinski; Irene E Zohn; David Mendelowitz; Norman H Lee; Anastas Popratiloff
Journal:  Dev Biol       Date:  2015-11-07       Impact factor: 3.582

3.  Histology of the pharyngeal constrictor muscle in 22q11.2 deletion syndrome and non-syndromic children with velopharyngeal insufficiency.

Authors:  Josine C C Widdershoven; Nicole E Spruijt; Wim G M Spliet; Corstiaan C Breugem; Moshe Kon; Aebele B Mink van der Molen
Journal:  PLoS One       Date:  2011-06-28       Impact factor: 3.240

Review 4.  In search of the optimal surgical treatment for velopharyngeal dysfunction in 22q11.2 deletion syndrome: a systematic review.

Authors:  Nicole E Spruijt; Judith Reijmanhinze; Greet Hens; Vincent Vander Poorten; Aebele B Mink van der Molen
Journal:  PLoS One       Date:  2012-03-28       Impact factor: 3.240

5.  Obstructive sleep apnea syndrome in children with 22q11.2 deletion syndrome after operative intervention for velopharyngeal insufficiency.

Authors:  David Jeffrey Crockett; Steven L Goudy; Sivakumar Chinnadurai; Christopher Todd Wootten
Journal:  Front Pediatr       Date:  2014-08-11       Impact factor: 3.418

6.  General approach to velocardiofacial anomalies: a pediatric case presenting with Fallot tetralogy.

Authors:  Aysu Turkmen Karaagac; Ayse Inci Yildirim
Journal:  North Clin Istanb       Date:  2015-01-24

7.  Surgical interventions in velopharyngeal dysfunction: comparative perceptual speech and nasometric outcomes for three techniques.

Authors:  Ryan Instrum; Agnieszka Dzioba; Anne Dworschak-Stokan; Murad Husein
Journal:  J Otolaryngol Head Neck Surg       Date:  2022-02-04

8.  Characteristics of velopharyngeal dysfunction in 22q11.2 deletion syndrome: a retrospective case-control study.

Authors:  Sebastiano Failla; Peng You; Chandheeb Rajakumar; Anne Dworschak-Stokan; Philip C Doyle; Murad Husein
Journal:  J Otolaryngol Head Neck Surg       Date:  2020-07-31
  8 in total

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