Sphincter pharyngoplasty is frequently used for the management of children with velopharyngeal insufficiency. The purpose of this study was to evaluate outcome and revision rates of sphincter pharyngoplasty at the authors' institution. Two hundred fifty patients underwent sphincter pharyngoplasty for velopharyngeal insufficiency between January of 1987 and March of 2001. There were 117 female patients and 133 male patients, with a mean age at primary sphincter pharyngoplasty of 7.6 years (range, 1 to 45 years). Diagnoses included velopharyngeal insufficiency alone (n = 63), velopharyngeal insufficiency associated with cleft palate (n = 127), velocardiofacial syndrome (n = 32), submucous cleft (n = 15), and other (n = 13). Pharyngoplasty revision was defined as any secondary surgical revision of the sphincter as determined by clinical evaluation and objective speech assessment. The pharyngoplasty revision rate was found to be 12.8 percent (n = 32). A favorable outcome was demonstrated in 30 of these patients (93.8 percent) after pharyngoplasty revision. Two patients, one with a diagnosis of a submucous cleft and velocardiofacial syndrome and the other with a cleft palate, required a second revision because of persistent velopharyngeal insufficiency. The revision rate was highest in those patients with velocardiofacial syndrome (21.8 percent) and lowest in patients with velopharyngeal insufficiency alone (6.3 percent). Patients who required revision had significantly higher preoperative oral sentence nasometry (55.2 percent versus 46.1 percent; p < 0.01) and larger velopharyngeal areas (23.7 mm2 versus 18.9 mm2). There was no significant difference in age or sex for those patients who required a revision compared with those who did not require revision. Mean follow-up was 2.4 years (range, 4 months to 13.6 years). Sphincter pharyngoplasty is an effective procedure for the treatment of velopharyngeal insufficiency using revision rate as the standard of success. It had an 87 percent primary success rate that increased to 99 percent after a single revision. Patients with velocardiofacial syndrome, more severe preoperative hypernasal resonance, and larger velopharyngeal areas were more likely to require pharyngoplasty revision.
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"Lower primary success rates for all patients with hypodynamic velopharynges, including those with 22qDS, supports the logic of segregating this group (which constitutes up to 25% of the population with VPD) from the larger cleft palate population . In general, the speech outcome after surgery has been reported to be worse in patients with 22qDS than in patients without the syndrome , , , , , , , , but some patients with 22qDS fare as well as their non-syndromic counterparts , , , , , , . "
[Show abstract][Hide abstract] ABSTRACT: Background
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.
Methodology/ Principal Findings
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).
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.
"Nevertheless , two prospective randomized trials have shown that a pharyngeal flap is more effective, although not statistically significantly, than a sphincter pharyngoplasty (Ysunza et al., 2002; Abyholm et al., 2005). Some complications of a sphincter pharyngoplasty are higher than a pharyngeal flap, with revision rates of 12% to 16% (Riski et al., 1992; Sie et al., 1998; Witt, Marsh, et al., 1998; Losken et al., 2003) and hyponasality in up to 22% of patients (Losken et al., 2003). The rate of obstructive sleep apnea is not different between the two procedures (Abyholm et al., 2005). "
[Show abstract][Hide abstract] ABSTRACT: Objective : Our purpose was to compare speech outcomes among three primary procedures for symptomatic submucous cleft palate (SMCP): two-flap palatoplasty with muscular retropositioning, double-opposing Z-palatoplasty, or pharyngeal flap. Design : Retrospective review. Setting : Tertiary hospital. Patients, Participants : All children with SMCP treated by the senior author between 1984 and 2008. Interventions : One of three primary procedures: two-flap palatoplasty with muscular retropositioning, double-opposing Z-palatoplasty, or pharyngeal flap. Main Outcome Measures : Speech outcome and need for a secondary operation were analyzed among procedures. Success was defined as normal or borderline competent velopharyngeal function. Failure was defined as persistent borderline insufficiency or velopharyngeal insufficiency with recommendation for a secondary operation. Results : We identified 58 patients with SMCP who were treated for velopharyngeal insufficiency. We found significant differences in median age at operation among the procedures (p < .001). Two-flap palatoplasty with muscular retropositioning (n = 24), double-opposing Z-palatoplasty (n = 19), and pharyngeal flap (n = 15) were performed at a median of 2.5, 3.6, and 9.5 years, respectively. There were significant differences in success among procedures (p = .018). Normal or borderline competent function was achieved in 6/20 (30%) patients who underwent two-flap palatoplasty, 10/15 (67%) following double-opposing Z-palatoplasty, and 11/12 (92%) following pharyngeal flap. Among patients treated with palatoplasty, success was independent of age at operation (p = .16). Conclusions : Double-opposing Z-palatoplasty is more effective than two-flap palatoplasty with muscular retropositioning. For children older than 4 years, primary pharyngeal flap is also highly successful but equally so as a secondary operation and can be reserved, if necessary, following double-opposing Z-palatoplasty.
"Secondary velopharyngoplasty to correct the VPI may then follow. The functional outcome has been reported to be worse in patients with 22q11DS than in patients without the syndrome , , , , , . A possible explanation is the hypotonia that is often present as part of the syndrome and which cannot be corrected by surgery. "
[Show abstract][Hide abstract] ABSTRACT: Plastic surgeons aim to correct velopharyngeal insufficiency manifest by hypernasal speech with a velopharyngoplasty. The functional outcome has been reported to be worse in patients with 22q11.2 deletion syndrome than in patients without the syndrome. A possible explanation is the hypotonia that is often present as part of the syndrome. To confirm a myogenic component of the etiology of velopharyngeal insufficiency in children with 22q11.2 deletion syndrome, specimens of the pharyngeal constrictor muscle were taken from children with and without the syndrome. Histologic properties were compared between the groups. Specimens from the two groups did not differ regarding the presence of increased perimysial or endomysial space, fiber grouping by size or type, internalized nuclei, the percentage type I fibers, or the diameters of type I and type II fibers. In conclusion, a myogenic component of the etiology of velopharyngeal insufficiency in children with 22q11.2 deletion syndrome could not be confirmed.