Superiorly Based Posterior Pharyngeal Flaps: Using A Care Pathway to Optimize Speech and Airway Outcomes.

BACKGROUND: Pharyngeal flap (PF) surgery is effective at improving velopharyngeal sufficiency, but historical literature shows a concerning prevalence rate of obstructive sleep apnea (OSA), reported as high as 20%. Our institution has developed a protocol to minimize risk of postoperative obstructive complications and increase safety of PF surgery. We hypothesize that (1) preoperative staged removal of significant adenotonsillar tissue along with (2) multiview videofluoroscopy to guide patient-specific surgical approach via appropriately sized PFs can result in excellent speech outcomes while limiting occurrence of OSA. METHODS: This was a retrospective chart review of all patients with velopharyngeal insufficiency (VPI) (aged 2-20 years) seen at the University of Rochester from 2015 to 2022 undergoing PF surgery to correct VPI. Nasopharyngoscopy was used for surgical planning and airway evaluation. Patients with tonsillar and adenoid hypertrophy underwent staged adenotonsillectomy at least 2 months before PF. Multiview videofluoroscopy was used to identify anatomic causes of VPI and to determine PF width. Patients underwent polysomnography and speech evaluation before and at least 6 months after PF surgery. RESULTS: Forty-one children aged 8.5 ± 4.1 years (range, 4 to 18 years) who underwent posterior PF surgery for VPI were identified. This included 10 patients with 22q11.2 deletion and 4 patients with Pierre Robin sequence. Thirty-nine patients had both pre- and postoperative speech data and underwent both a pre- and postoperative sleep study. Polysomnography showed no significant difference in obstructive apnea-hypopnea index after posterior PF surgery (obstructive apnea-hypopnea index preop, 1.3 ± 1.2 events per hour; postop, 1.7 ± 2.1 events per hour; P = 0.111). Significant improvements in speech outcome were seen in patients who underwent PF (modified Pittsburgh score preop, 11.52 ± 1.37; postop, 1.09 ± 2.35; P < 0.05). CONCLUSIONS: Use of preoperative staged adenotonsillectomy as well as patient-specific PF dimensions results in effective resolution of VPI and a low risk of OSA.

Simultaneous Furlow Palatoplasty and Tonsillectomy for the Treatment of Velopharyngeal Insufficiency and Tonsillar Hypertrophy.

OBJECTIVE: To determine whether performing tonsillectomy at the time of Furlow palatoplasty for the treatment of cleft palate related velopharyngeal insufficiency (VPI) incurs increased surgical complications or compromises speech outcomes. DESIGN: A retrospective review of patients who had Furlow palatoplasty and the outcomes of surgery in the treatment of cleft palate related VPI. SETTING: A single academic center between January 2015 and January 2022. PARTICIPANTS: Patients with submucous cleft (SMC) palate or patients with prior straight line primary palatoplasty presenting with VPI. INTERVENTIONS: Simultaneous conversion Furlow palatoplasty and tonsillectomy. MAIN OUTCOME MEASURE(S): Primary outcome measures include preoperative and postoperative Modified Pittsburgh Weighted Speech Scale (mPWSS), and postoperative surgical complications. RESULTS: Eight patients (25%) underwent Furlow palatoplasty and concomitant tonsillectomy, while 24 patients (75%) underwent Furlow palatoplasty alone. A significantly lower median postoperative mPWSS score, corresponding to better velopharyngeal function, was reported for patients in the Furlow-tonsillectomy group (0, IQR 0-0) compared to the Furlow only group (1, IQR 0-9, p = 0.046). No surgical complications were encountered in either group. Five patients (20.8%) in the Furlow only group required subsequent surgery for persistent VPI. No patients in the Furlow-tonsillectomy group required additional surgical treatment for VPI (0%, p = 0.16). CONCLUSIONS: Tonsillectomy at time of Furlow palatoplasty is utilized in patients with both VPI and baseline tonsillar hypertrophy to lessen the risk of postoperative obstructive breathing. Tonsillectomy performed concurrently with Furlow palatoplasty is safe, without increased risk of surgical complications, and does not compromise post-Furlow palatoplasty speech outcomes.

Pharyngeal Flap Is Effective Treatment for Post Maxillary Advancement Velopharyngeal Insufficiency in Patients With Repaired Cleft Lip and Palate.

PURPOSE: Patients with repaired cleft lip and cleft palate (CL/P) can develop velopharyngeal insufficiency (VPI) after Le Fort I maxillary advancement. The aim of this study was to evaluate speech outcomes in patients who required a pharyngeal flap after Le Fort I maxillary advancement. PATIENTS AND METHODS: This retrospective cohort study included all patients with repaired CL/P who underwent a Le Fort I osteotomy and subsequently required a pharyngeal flap to correct VPI. Patients were included if they had outcome measurements documented at 3 time points: 1) before Le Fort I (baseline), 2) after Le Fort I, and 3) after pharyngeal flap. Outcome measurements, including speech characteristics (resonance, nasal emission, and intraoral pressure) and velopharyngeal function, were evaluated on perceptual assessment by a speech pathologist specializing in cleft care. Velopharyngeal closure was assessed with multi-view videofluoroscopy or nasopharyngoscopy. Patient characteristics and descriptive statistics were summarized and continuous data were expressed as mean ± standard deviation. Repeated-measures analysis of variance and paired samples t test were used to measure changes in speech outcome variables between time points. All P values were 2-tailed and considered significant for values less than .05. RESULTS: There were 23 patients for analysis (13 girls [56.5%] and 10 boys [43.5%]). Two patients (9%) had cleft palate only, 9 (39%) had unilateral cleft lip and palate (CLP), and 12 (52%) had bilateral CLP. Follow-up evaluations performed on average 12 months postoperatively showed statistically meaningful improvement for all variables, including decreased hypernasality, reduced nasal emission, and increased intraoral pressure for consonant production. Patients with repaired CL/P who had VPI after Le Fort I maxillary advancement showed significant improvement in all outcome measurements after pharyngeal flap (P < .001). CONCLUSIONS: The superiorly based pharyngeal flap is highly successful in correcting VPI after Le Fort I maxillary advancement in patients with repaired CL/P.

Uvular malformation in the presence of deformational plagiocephaly.

BACKGROUND: Deformational plagiocephaly is cranial asymmetry caused by external forces on the skull. Deformational plagiocephaly is seen in 5% to 48% of healthy newborns. Incomplete uvular fusion, in contrast, is one of many uvular malformations. The incidence of all degrees of incomplete uvular fusion is approximately 1% in healthy children. Bifid uvula is a malformation that is often considered a microform cleft palate or a marker for submucous cleft palate. METHODS: This is a retrospective study of patients with deformational plagiocephaly seen at the Upstate Cleft and Craniofacial Center between January 1, 2006, and September 30, 2011. Patients were identified by the International Classification of Diseases, Ninth Revision code for plagiocephaly. Seventy-nine patients were excluded with craniosynostosis and syndromic diagnoses. One hundred forty-six patients with deformational plagiocephaly were included in the study. Data were collected for sex, age at presentation, parity, multiple births, delivery, oligohydramnios, cephalohematoma, uterine abnormalities, fetal position, and intrauterine growth restriction. Clinical findings were collected including location of cranial flattening and uvular malformations. RESULTS: Twenty-four of 146 patients with deformational plagiocephaly had incomplete fusion of the uvula ranging from complete bifid uvula to a notched uvular tip (16.4%). This association was statistically significant (odds ratio, 18; 95% confidence interval, 11.1-28.9). Most patients (62.3%) were male. We recorded primiparity (44.5%), multiple births (17.1%), vacuum-assisted delivery (6.2%), cesarean section (36.3%), oligohydramnios (4.1%), uterine abnormalities (2.1%), abnormal fetal position (3.4%), and intrauterine growth restriction (1.4%). Ten of the 24 patients with plagiocephaly and uvular malformation were seen for an initial consultation only in our chart system. Of the remaining 14 patients with follow-up, none had recorded signs or symptoms of velopharyngeal insufficiency. CONCLUSIONS: The incidence of incomplete uvular fusion in infants with deformational plagiocephaly is 16.4%, which is significantly higher than the approximate 1% incidence reported in the general population. This is the first report of uvular malformation in the presence of deformational plagiocephaly.

Anatomic Severity, Midfacial Growth, and Speech Outcomes in Van der Woude/Popliteal Pterygium Syndromes Compared to Nonsyndromic Cleft Lip/Palate.

OBJECTIVE: To summarize the clinical characteristics and surgical and speech outcomes for patients with Van der Woude/popliteal pterygium syndromes (VWS/PPS) and to compare them with a historic cohort of patients with nonsyndromic cleft lip/cleft palate (CL/P). DESIGN: Retrospective chart review. SETTING: Tertiary care center. PATIENTS: All patients with VWS/PPS seen at Boston Children's Hospital from 1979 to 2012: 28 patients with VWS (n = 21)/PPS (n = 7) whose mean age was 17.3 ± 10.4 years, including 18 females (64%) and 10 males (36%); 18 patients (64%) had a family history of VWS/PPS. MAIN OUTCOME MEASURES: Cleft type, operative procedures, speech, and midfacial growth. Data were compared with historic cohorts of patients with nonsyndromic CL/P treated at one tertiary care center. RESULTS: There were 24 patients (86%) with CP±L, Veau types I (n = 4, 17%), II (n = 4, 17%), III (n = 5, 21%), and IV (n = 11, 46%). Nine patients (38%) had palatal fistula after palatoplasty. Fourteen of 23 (61%) patients with CL/P age 5 years or older had midfacial retrusion, and 10 (43%) required a pharyngeal flap for velopharyngeal insufficiency. Fisher's exact test demonstrated higher frequencies of Veau type IV CP±L (P = .0016), bilateral CL±P (P = .0001), and complete CL±P (P < .0001) in VWS/PPS compared with nonsyndromic patients. Incidences of midfacial retrusion (P = .0001), palatal fistula (P < .0001), and need for pharyngeal flap (P = .0014) were significantly greater in patients with VWS/PPS. CONCLUSIONS: Patients with VWS/PPS have more severe forms of labiopalatal clefting and higher incidences of midfacial retrusion, palatal fistula, and velopharyngeal insufficiency following primary repair as compared with nonsyndromic CL/P.

Predictors of velopharyngeal insufficiency after Le Fort I maxillary advancement in patients with cleft palate.

PURPOSE: Approximately 25% to 40% of patients with cleft lip/palate develop maxillary retrusion that requires Le Fort I osteotomy. Maxillary advancement brings the soft palate forward, and this may cause velopharyngeal insufficiency (VPI). The goal of this study was to identify predictors that place patients with repaired cleft palate at risk of developing VPI after Le Fort I advancement. MATERIALS AND METHODS: This was a retrospective study of nonsyndromic patients with cleft lip/palate who had a Le Fort I osteotomy between 2000 and 2008. Charts were reviewed and data were collected on patient characteristics, preoperative speech assessments, and nasopharyngoscopic reports. Pre- and postoperative cephalometric radiographs were used to measure maxillary advancement and to assess the structure of the velopharynx. Simple logistic regression analysis examined the association between each predictive variable and postoperative VPI, as indicated by need for pharyngeal flap. Predictors with P ≤ .10 were included in the multivariate regression model. In both the univariate and the multivariate analyses, P ≤ .05 was considered statistically significant. RESULTS: Univariate analysis showed a significant association between preoperative soft palatal length and need for a pharyngeal flap (P = .005). By multivariate analysis, both preoperative soft palatal length and postoperative pharyngeal depth were associated with need for pharyngeal flap (P = .003 and P = .030). CONCLUSION: This study shows that a short soft palate is associated with VPI after Le Fort I osteotomy. Assessment of palatal length and pharyngeal depth on cephalometric radiographs is helpful in predicting postoperative VPI and need for a pharyngeal flap in patients with cleft palate after maxillary advancement.

Submucous cleft palate and velopharyngeal insufficiency: comparison of speech outcomes using three operative techniques by one surgeon.

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.

Palatoplasty outcomes in nonsyndromic patients with cleft palate: a 29-year assessment of one surgeon's experience.

The primary objective of cleft palate repair is velopharyngeal competence without fistula. The reported incidence of fistula and velopharyngeal insufficiency (VPI) is variable. Our purpose was to assess the senior surgeon's 29-year palatoplasty experience with respect to incidence of fistula and VPI. Our hypotheses were that VPI is related to (1) age at palatoplasty, (2) cleft palate type, and (3) VPI and palatal fistula incidence decrease with the surgeon's experience. We reviewed the records of all children with cleft palate treated by the senior author between 1976 and 2004. Cleft palate was categorized according to Veau. Palatoplasty was performed on 449 patients, using a 2-flap technique with muscular retropositioning. The mean age at palatoplasty was 11.6 +/- 4.9 months (range, 7.0-46.4 months). The incidence of palatal fistula was 2.9%, and velopharyngeal sufficiency was found in 85.1% of patients. We found a significant association between age at palatoplasty and VPI (P = 0.009, odds ratio, 1.06 [95% confidence interval, 1.02-1.10]). Velopharyngeal insufficiency was also associated with the Veau hierarchy (P = 0.001). Incidence of VPI was independent of surgeon experience (P = 0.2). In conclusion, the incidence of palatal fistula was low. Velopharyngeal insufficiency was associated with increasing age at palatoplasty and with the Veau hierarchy.

Upper airway asymmetry in velo-cardio-facial syndrome.

OBJECTIVE: Various forms of asymmetry have been recognized as a feature of velo-cardio-facial syndrome (VCFS). This study was implemented to determine the frequency of anatomic and functional asymmetry of the velum, pharynx and larynx in children with VCFS. METHODS: Individuals with VCFS underwent prospective, blinded analysis by an expert panel who assessed the velum, pharynx and larynx with multi-view videofluoroscopy (MVF) and nasopharyngolaryngoscopy (NPL). The VCFS group was compared to an age-matched group of normal individuals. Eight different parameters were assessed in both groups for functional and anatomic symmetry including: velar elevation, adenoid size, posterior pharyngeal wall size, carotid pulsations, epiglottis size and shape, arytenoid size, true vocal cord size and true vocal cord motion. RESULTS: One hundred and twenty-one subjects with VCFS and 20 normal individuals underwent examination. Children with VCFS showed significantly more asymmetry compared to the normal group (69% versus 20%, P=0.01) with greatest differences seen with palatal motion, posterior pharyngeal wall size and epiglottis shape. On average, subjects with VCFS had three asymmetric parameters versus one parameter in the normal group. CONCLUSION: Asymmetric development of the pharynx and larynx in children with VCFS appears to be a distinct clinical feature of this syndrome. This finding may provide an important diagnostic clue for patients presenting with subtle features of the 22q11.2 microdeletion. These developmental abnormalities may increase the risk of speech impairment, aspiration and airway obstruction in affected individuals.

Perceptual Assessment of Velopharyngeal Dysfunction by Otolaryngology Residents.

OBJECTIVE: To assess the ability of otolaryngology residents to rate the hypernasal resonance of patients with velopharyngeal dysfunction. We hypothesize that experience (postgraduate year [PGY] level) and training will result in improved ratings of speech samples. STUDY DESIGN: Prospective cohort study. SETTING: Otolaryngology training programs at 2 academic medical centers. SUBJECTS AND METHODS: Thirty otolaryngology residents (PGY 1-5) were enrolled in the study. All residents rated 30 speech samples at 2 separate times. Half the residents completed a training module between the rating exercises, with the other half serving as a control group. Percentage agreement with the expert rating of each speech sample and intrarater reliability were calculated for each resident. Analysis of covariance was used to model accuracy at session 2. RESULTS: The median percentage agreement at session 1 was 53.3% for all residents. At the second session, the median scores were 53.3% for the control group and 60% for the training group, but this difference was not statistically significant. Intrarater reliability was moderate for both groups. Residents were more accurate in their ratings of normal and severely hypernasal speech. There was no correlation between rating accuracy and PGY level. Score at session 1 positively correlated with score at session 2. CONCLUSION: Perceptual training of otolaryngology residents has the potential to improve their ratings of hypernasal speech. Length of time in residency may not be best predictor of perceptual skill. Training modalities incorporating practice with hypernasal speech samples could improve rater skills and should be studied more extensively.

Speech outcome after palatal repair in nonsyndromic versus syndromic Robin sequence.

BACKGROUND: The authors' purpose was to document speech outcome after cleft palate repair in patients with syndromic versus nonsyndromic Robin sequence. Results of secondary correction of velopharyngeal insufficiency using a superiorly based pharyngeal flap or double-opposing Z-palatoplasty are also reported. METHODS: Charts of patients with Robin sequence and cleft palate between 1980 and 2007 were reviewed. Data collected included date of birth, sex, syndrome/association, cleft palatal type (Veau I or II), age at palatoplasty, incidence of palatal fistula, postoperative speech assessment, videofluoroscopic results, need for secondary operation for velopharyngeal insufficiency, and type of secondary operation (pharyngeal flap or double-opposing Z-palatoplasty). RESULTS: The authors identified 140 patients with Robin sequence who had palatal closure. Postoperative speech evaluation was available for 96 patients (69 percent). A syndrome or association was identified in 42 patients (30 percent). Primary palatoplasty was successful in 74 patients (77 percent); speech was characterized as competent and competent to borderline competent. The authors found a significantly higher incidence of velopharyngeal insufficiency following palatal repair for syndromic (38 percent) than nonsyndromic Robin sequence (16 percent). (p = 0.039). In patients with velopharyngeal insufficiency, competent or borderline competent speech was determined after double-opposing Z-palatoplasty (two of five patients) or pharyngeal flap (eight of 10 patients). CONCLUSIONS: The rate of velopharyngeal insufficiency in syndromic Robin sequence is significantly greater than in nonsyndromic Robin sequence. The authors prefer pharyngeal flap for velopharyngeal insufficiency in patients with Robin sequence, whether syndromic or nonsyndromic, without retrognathism or signs/symptoms of obstructive sleep apnea.

Speech outcomes following pharyngeal flap in patients with velocardiofacial syndrome.

BACKGROUND: Velocardiofacial syndrome is the most common defined disorder associated with palatal insufficiency. The authors' purpose is to evaluate one surgeon's experience with correction of velopharyngeal insufficiency in velocardiofacial syndrome using a tailored pharyngeal flap. METHODS: The authors reviewed the records of all children with velocardiofacial syndrome and velopharyngeal insufficiency who were managed with a pharyngeal flap between 1983 and 2009. Data collected included age at operation, preoperative videofluoroscopic findings, speech outcomes, complications, and need for a secondary operation. RESULTS: The authors identified 33 patients with velocardiofacial syndrome and velopharyngeal insufficiency who had postoperative speech evaluations. Velopharyngeal insufficiency was diagnosed at a median age of 5 years. Palatal findings were: Veau type I (n = 4), overt submucous (n = 6), or occult submucous (n = 23). Median preoperative lateral pharyngeal wall movement was 22 percent (range, 0 to 90 percent). Successful correction of velopharyngeal insufficiency was achieved in 29 of 33 patients (88 percent). One patient had a medially displaced right internal carotid artery, and evidenced intraoperative bleeding and required a blood transfusion. One patient developed obstructive sleep apnea. CONCLUSION: A tailored pharyngeal flap is highly effective for correction of velopharyngeal insufficiency in velocardiofacial syndrome with few complications.

Pharyngeal flap outcomes in nonsyndromic children with repaired cleft palate and velopharyngeal insufficiency.

BACKGROUND: Velopharyngeal insufficiency occurs in 5 to 20 percent of children following repair of a cleft palate. The pharyngeal flap is the traditional secondary procedure for correcting velopharyngeal insufficiency; however, because of perceived complications, alternative techniques have become popular. The authors' purpose was to assess a single surgeon's long-term experience with a tailored superiorly based pharyngeal flap to correct velopharyngeal insufficiency in nonsyndromic patients with a repaired cleft palate. METHODS: The authors reviewed the records of all children who underwent a pharyngeal flap performed by the senior author (J.B.M.) between 1981 and 2008. The authors evaluated age of repair, perceptual speech outcome, need for a secondary operation, and complications. Success was defined as normal or borderline sufficient velopharyngeal function. Failure was defined as borderline insufficiency or severe velopharyngeal insufficiency with recommendation for another procedure. RESULTS: The authors identified 104 nonsyndromic patients who required a pharyngeal flap following cleft palate repair. The mean age at pharyngeal flap surgery was 8.6 +/- 4.9 years. Postoperative speech results were available for 79 patients. Operative success with normal or borderline sufficient velopharyngeal function was achieved in 77 patients (97 percent). Obstructive sleep apnea was documented in two patients. CONCLUSION: The tailored superiorly based pharyngeal flap is highly successful in correcting velopharyngeal insufficiency, with a low risk of complication, in nonsyndromic patients with repaired cleft palate.

Velopharyngeal insufficiency: diagnosis and management.

PURPOSE OF REVIEW: Journal articles relevant to the diagnosis and treatment of velopharyngeal insufficiency were reviewed. All studies ascertained by PubMed search were included. RECENT FINDINGS: Studies reported on the application of magnetic resonance scanning, reliability tests of the International Working Group diagnostic protocol, the use of nasometry, and techniques designed to assess the function of the velopharyngeal mechanism. Treatment studies focused on outcomes in small samples of cases and complication rates from pharyngeal flap. One study discussed ineffective speech therapy procedures. SUMMARY: There were relatively few studies this past year. Those that were published were hindered by small and heterogeneous sample sizes and occasionally by inappropriate methods for assessing outcomes. None of the findings will have a major impact on the current state-of-the-art for diagnosis of velopharyngeal insufficiency. The speech therapy study has a very important message that should be taken to heart by all clinicians involved in the management of children with clefts and craniofacial disorders.