In Search of the Optimal Surgical Treatment for Velopharyngeal Dysfunction in 22q11.2 Deletion Syndrome: A Systematic Review

<div><h3>Background</h3><p>Patients with the 22q11.2 deletion syndrome (22qDS) and velopharyngeal dysfunction (VPD) tend to have residual VPD following surgery. This systematic review seeks to determine whether a particular surgical procedure results in superior speech outcome or less morbidity.</p> <h3>Methodology/ Principal Findings</h3><p>A combined computerized and hand-search yielded 70 studies, of which 27 were deemed relevant for this review, reporting on a total of 525 patients with 22qDS and VPD undergoing surgery for VPD. All studies were levels 2c or 4 evidence. The methodological quality of these studies was assessed using criteria based on the Cochrane Collaboration's tool for assessing risk of bias. Heterogeneous groups of patients were reported on in the studies. The surgical procedure was often tailored to findings on preoperative imaging. Overall, 50% of patients attained normal resonance, 48% attained normal nasal emissions scores, and 83% had understandable speech postoperatively. However, 5% became hyponasal, 1% had obstructive sleep apnea (OSA), and 17% required further surgery. There were no significant differences in speech outcome between patients who underwent a fat injection, Furlow or intravelar veloplasty, pharyngeal flap pharyngoplasty, Honig pharyngoplasty, or sphincter pharyngoplasty or Hynes procedures. There was a trend that a lower percentage of patients attained normal resonance after a fat injection or palatoplasty than after the more obstructive pharyngoplasties (11–18% versus 44–62%, p = 0.08). Only patients who underwent pharyngeal flaps or sphincter pharyngoplasties incurred OSA, yet this was not statistically significantly more often than after other procedures (p = 0.25). More patients who underwent a palatoplasty needed further surgery than those who underwent a pharyngoplasty (50% versus 7–13%, p = 0.03).</p> <h3>Conclusions/ Significance</h3><p>In the heterogeneous group of patients with 22qDS and VPD, a grade C recommendation can be made to minimize the morbidity of further surgery by choosing to perform a pharyngoplasty directly instead of only a palatoplasty.</p> </div

Correlação entre fechamento velofaríngeo e dimensões nasofaríngeas após cirurgia de retalho faríngeo avaliados por meio da técnica fluxo-pressão Correlation between velopharyngeal closure and nasopharyngeal dimensions after pharyngeal flap surgery assessed by pressure-flow technique

OBJETIVO: Verificar se a área dos orifícios velofaríngeos obtida após o retalho faríngeo (RF) durante a respiração de repouso (AVFr) é um indicador do grau de fechamento velofaríngeo durante a fala (AVFf). MÉTODOS: Os sujeitos foram 62 pacientes com fissura de palato, associada ou não à fissura de lábio, de ambos os gêneros, com idades entre seis e 32 anos, submetidos ao RF há, pelo menos, 12 meses. AVFr e AVFf foram determinadas por meio da técnica fluxo-pressão. Valores de AVFr inferiores a 0,500 cm² foram considerados subnormais. A AVFf foi classificada como adequada (0-0,049 cm²), marginal (0,050-0,199 cm²) ou inadequada (>0,200 cm²). A associação entre os valores de AVFr e AVFf pós-cirúrgicos foi analisada por meio de um modelo de regressão logística. RESULTADOS: Após o RF, 92% dos pacientes com valores de AVFr subnormais (<0,500 cm²) apresentaram fechamento velofaríngeo adequado. A proporção de pacientes com valores de AVFr normais (>0,500 cm²) e fechamento velofaríngeo adequado foi menor, porém ainda expressiva (55%). Não houve associação estatisticamente significante entre as duas variáveis. CONCLUSÃO: A maioria dos pacientes com retalho largo apresentou fechamento velofaríngeo adequado durante a fala. Entretanto, os resultados mostraram que as dimensões dos orifícios velofaríngeos durante a respiração de repouso não podem predizer a eficácia do retalho faríngeo para a fala.<br>PURPOSE: To investigate whether postoperative velopharyngeal orifice area during nasal breathing at rest (VPAb) can predict velopharyngeal closure during speech (VPAs). METHODS: The subjects were 62 patients with cleft palate, associated or not with cleft lip, of both genders, with ages between six and 32 years, who underwent pharyngeal flap surgery (PFS) at least 12 months before the evaluation performed for this study. VPAb and VPAs were assessed using the pressure-flow technique. VPAb values below 0.500cm² were considered subnormal. VPAs was categorized as adequate (0-0.049cm²), borderline (0.050-0.199cm²) or inadequate (>0.200cm²). A logistic regression model analyzed the association between VPAb and VPAs. RESULTS: After PFS, 92% of the patients with subnormal VPAb values (<0.500cm²) presented adequate velopharyngeal closure. The percentage of patients with normal VPAb values (>0.500cm²) who also presented adequate closure was smaller, but still significant (55%). The association between VPAb and VPAs values was not statistically significant. CONCLUSION: Most patients with large flaps presented adequate velopharyngeal closure during speech. However, the findings show that the velopharyngeal orifice area during breathing is not a good predictor of the effectiveness of the pharyngeal flap for speech