Surgical Outcomes for Velopharyngeal Insufficiency in Velocardiofacial Syndrome and Nonsyndromic Patients

Abstract

Velocardiofacial syndrome patients may have comparable outcomes to nonsyndromic patients in selective surgical management of velopharyngeal insufficiency. In addition, the data demonstrate the efficacy of a single-stage combined procedure without increased morbidity.

Speech Outcomes and Velopharyngeal Function After Surgical Treatment of Velopharyngeal Insufficiency in Individuals With Signs of Velocardiofacial Syndrome

“…The literature demonstrates that the late accomplishment of primary palatoplasty in cases with VCFS is common, considering that the diagnosis and onset of treatment occur between 4 and 17 years. 9,10,12,17,36 In individuals with SMCP without syndrome, especially not associated with overt cleft palate, the palatal alteration is often mild and may remain Therefore, the patients in this study, whose mean age on the first attendance at the institution was 8 years, initiated the treatment at a similar age as reported in the literature. It should be highlighted that most cases in this sample did not have a previous diagnosis or suspicion of VCFS before this attendance.…”

“…A similar study demonstrated that surgery for VPI correction improved all aspects of speech analyzed, both in cases with VCFS and the others; however, the authors observed worse results for individuals with VCFS. 10 In general, studies aiming to analyze the surgical outcome in cases with VCFS presented some limitations, including the retrospective design, small sample size, and poor definition of criteria of surgical success. Therefore, prospective studies analyzing the surgical outcome of VPI in cases with VCFS are required, to elucidate the factors that may interfere with the surgical outcomes and consequently allow the definition of the most adequate treatment for these cases.…”

The Effect of Loudness Variation on Velopharyngeal Function in Children with 22q11.2 Deletion Syndrome: A Pilot Study

“…VP dysfunction (VPD) is characterized as the inability to open and close this valve accurately and efficiently during speech. VPD can result from structural causes (e.g., insufficient palate length after palate repair surgery), neurological incompetence (e.g., dysarthria), and/or articulatory mislearning [2,3,4,5]. Children with VPD can develop compensatory articulation errors (e.g., glottal stop substitutions), nasal air emission, and hypernasal speech, which can place them at an increased risk for communication limitations and decreased social acceptance [6].…”

“…Children with this syndrome consistently exhibit hypernasality and articulation problems that are more severe than those of nonsyndromic children with VPD or cleft palate, and they do not demonstrate as favorable postsurgical speech outcomes [2,3,20,21,22]. More research is needed to determine if other behavioral treatment approaches can improve the speech of patients with 22qDS.…”

An Algorithm for Application of Furlow Palatoplasty to the Treatment of Velocardiofacial Syndrome–Associated Velopharyngeal Insufficiency

“…[3][4][5][6][7][8][9] Palatoplasty has been mostly neglected as a means of treating VPI in VCFS patients, with the exception of a single study that combined Furlow palatoplasty with sphincter pharyngoplasty pending the result of a nasoendoscopy. 10 While successful in shrinking the VP port and therefore improving VPI, pharyngoplasties carry significant risk of obstructive sleep apnea (OSA) that must be weighed against the potential benefit. By narrowing the VP port, both PPFs and sphincter pharyngoplasties predispose patients to develop OSA, with PPFs carrying the highest risk.…”