Revision Pharyngoplasty in Cleft Palate and Velopharyngeal Insufficiency: Management and Outcomes.

INTRODUCTION: Velopharyngeal insufficiency (VPI), a stigmatizing hallmark of palatal dysfunction, occurs in a wide spectrum of pediatric craniofacial conditions. The mainstays for surgical correction include palate repair and/or pharyngeal surgery. However, primary pharyngoplasty has a failure rate of 15% to 20%. Although revision pharyngoplasty may be necessary in those with persistent VPI, little is known regarding the indications for and outcomes after such procedures. The purpose of this study is to describe the authors' experience with indications for and outcomes after revision pharyngoplasty. METHODS: A single-center retrospective review was performed of all patients undergoing revision pharyngoplasty between 2002 and 2019. Demographic data and Pittsburgh Weighted Speech Scores, diagnoses, comorbidities, and complications were tabulated. Two-tailed Student t test was used, and a P value of 0.05 or less was considered statistically significant. RESULTS: Thirty-two patients (65.6% male) met inclusion criteria for this study. The most common diagnoses included cleft palate (68.8%), submucous cleft palate (SMCP, 18.8%), and congenital VPI (6.3%, likely occult SMCP). Most patients (84.4%) underwent palatoplasty before their initial pharyngoplasty. The primary indication for initial pharyngoplasty was VPI (mean age 7.1 ± 4.6 years). The most common indication for revision pharyngoplasty (mean age 11.2 ± 5.1 years) included persistent VPI (n = 22), followed by obstructive sleep apnea (OSA) (n = 11). Persistent VPI (n = 8) and OSA (n = 6) were the most common complications after secondary pharyngoplasty. Thirteen patients (40.6%) within the revision pharyngoplasty cohort required additional surgical intervention: 4 underwent tertiary pharyngoplasty, 4 underwent takedown for OSA (n = 3) or persistent VPI (n = 1), 3 underwent takedown and conversion Furlow for persistent VPI (n = 2), OSA (n = 2) and/or flap dehiscence (n = 1), and 2 underwent palatal lengthening with buccal myomucosal flaps for persistent VPI. Of the 4 patients who required a tertiary pharyngoplasty, the mean age at repair was 6.6 ± 1.1 years and their speech scores improved from 13.5 to 2.3 after tertiary pharyngoplasty (P = 0.11). The overall speech score after completion of all procedures improved significantly from 19 to 3.3. CONCLUSION: Patients who fail primary pharyngoplasty represent a challenging population. Of patients who underwent secondary pharyngoplasty, nearly half required a tertiary procedure to achieve acceptable speech scores or resolve complications.

Nonsyndromic Craniosynostosis and Associated Abnormal Speech and Language Development.

BACKGROUND: Although many metrics for neurodevelopment in children with nonsyndromic craniosynostosis have been analyzed, few have directly examined early language acquisition and speech development. The authors characterized language acquisition and speech development in children with nonsyndromic craniosynostosis. METHODS: The authors' institutional database was queried for nonsyndromic craniosynostosis from 2000 to 2014. Patients with an identified syndrome were excluded. Specific data elements included age, gender, velopharyngeal adequacy by means of the Pittsburgh Weighted Speech Scale, evaluation for anatomical motor delay, language acquisition delay/disorder, articulation or speech sound production delays/disorders, and whether speech therapy was recommended. Diagnosis of a submucous cleft palate was noted. RESULTS: One hundred one patients met inclusion criteria, of which 57.4 percent were male. Average age at the time of the most recent speech evaluation was 6.1 years (range, 2.31 to 17.95 years); 43.6 percent had normal speech/language metrics and 56.4 percent had one or more abnormalities, including anatomical motor delay/disorder (29.7 percent), language acquisition delay/disorder (21.8 percent), articulation or speech production delay/disorder (4.0 percent), hypernasality (15.8 percent), and velopharyngeal insufficiency or borderline competency (23.8 percent). Average Pittsburgh Weighted Speech Scale score was 1.3 (range, 0 to 5), and 29.7 percent (n = 30) of patients were recommended to have speech therapy. In addition, 25.8 percent of patients were diagnosed with a submucous cleft palate. CONCLUSIONS: One in four patients with nonsyndromic craniosynostosis carried a diagnosis of submucous cleft palate. The authors found that abnormal speech and language development occurs in one in 1.7 patients with nonsyndromic craniosynostosis, and that speech therapy for such abnormal development is warranted in one in 3.4 of them-a prevalence two to five times higher compared with the general pediatric population.

What's New in Cleft Palate and Velopharyngeal Dysfunction Management?

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Have a clear understanding of the evolution of concepts of velopharyngeal dysfunction, especially as it relates to patients with a cleft palate. 2. Explain the subjective and objective evaluation of speech in children with velopharyngeal dysfunction. 3. On the basis of these diagnostic findings, be able to classify types of velopharyngeal dysfunction. 4. Develop a safe, evidence-based, patient-customized treatment plan for velopharyngeal dysfunction founded on objective considerations. SUMMARY: Velopharyngeal dysfunction is improper function of the dynamic structures that work to control the velopharyngeal sphincter. Approximately 30 percent of patients having undergone cleft palate repair require secondary surgery for velopharyngeal dysfunction. A multidisciplinary team using multimodal instruments to evaluate velopharyngeal function and speech should manage these patients. Instruments may include perceptual speech analysis, video nasopharyngeal endoscopy, multiview speech videofluoroscopy, nasometry, pressure-flow, and magnetic resonance imaging. Velopharyngeal dysfunction may be amenable to surgical or nonsurgical treatment methods or a combination of each. Nonsurgical management may include speech therapy or prosthetic devices. Surgical interventions could include palatal re-repair with repositioning of levator veli palatini muscles, posterior pharyngeal flap, sphincter pharyngoplasty, or soft palate or posterior wall augmentation. Treatment interventions should be based on objective assessment and rating of the movement of lateral and posterior pharyngeal walls and the palate to optimize speech outcomes. Treatment should be tailored to specific anatomical and physiologic findings and the overall needs of the patient.

Speech Outcomes After Clinically Indicated Posterior Pharyngeal Flap Takedown.

BACKGROUND: Velopharyngeal insufficiency affects as many as one in three patients after cleft palate repair. Correction using a posterior pharyngeal flap (PPF) has been shown to improve clinical speech symptomatology; however, PPFs can be complicated by hyponasality and obstructive sleep apnea. The goal of this study was to assess if speech outcomes revert after clinically indicated PPF takedown. METHODS: The cleft-craniofacial database of the Children's Hospital of Pittsburgh at the University of Pittsburgh Medical Center was retrospectively queried to identify patients with a diagnosis of velopharyngeal insufficiency treated with PPF who ultimately required takedown. Using the Pittsburgh Weighted Speech Score (PWSS), preoperative scores were compared to those after PPF takedown. Outcomes after 2 different methods of PPF takedown (PPF takedown alone or PPF takedown with conversion to Furlow palatoplasty) were stratified and cross-compared. RESULTS: A total of 64 patients underwent takedown of their PPF. Of these, 18 patients underwent PPF takedown alone, and 46 patients underwent PPF takedown with conversion to Furlow Palatoplasty. Patients averaged 12.43 (range, 3.0-22.0)(SD: 3.93) years of age at the time of PPF takedown, and 58% were men. Demographics between groups were not statistically different. The mean duration of follow-up after surgery was 38.09 (range, 1-104) (SD, 27.81) months. For patients undergoing PPF takedown alone, the mean preoperative and postoperative PWSS was 3.83 (range, 0.0-23.0) (SD, 6.13) and 4.11 (range, 0.0-23.0) (SD, 5.31), respectively (P = 0.89). The mean change in PWSS was 0.28 (range, -9.0 to 7.0) (SD, 4.3). For patients undergoing takedown of PPF with conversion to Furlow palatoplasty, the mean preoperative and postoperative PWSS was 6.37 (range, 0-26) (SD, 6.70) and 3.11 (range, 0.0-27.0) (SD, 4.14), respectively (P < 0.01). The mean change in PWSS was -3.26 (range, -23.0 to 4.0) (SD, 4.3). For all patients, the mean preoperative PWSS was 5.66 (range, 0.0-26) (SD, 6.60) and 3.39 (range, 0.0-27) (SD, 4.48), respectively (P < 0.05). The mean change in PWSS was -2.26 (range, -23.0 to 7) (SD, 5.7). There was no statistically significant regression in PWSS for either surgical intervention. Two patients in the PPF takedown alone cohort demonstrated deterioration in PWSS that warranted delayed conversion to Furlow palatoplasty. Approximately 90% of patients, who undergo clinically indicated PPF takedown alone, without conversion to Furlow Palatoplasty, will show no clinically significant reduction in speech. CONCLUSIONS: Although there is concern that PPF takedown may degrade speech, this study finds that surgical takedown of PPF, when clinically indicated, does not result in a clinically significant regression of speech.

A comparison of speech outcomes using radical intravelar veloplasty or furlow palatoplasty for the treatment of velopharyngeal insufficiency associated with occult submucous cleft palate.

BACKGROUND: The safety, efficacy, and direct comparison of various surgical treatments for velopharyngeal insufficiency (VPI) associated with occult submucous cleft palate (OSMCP) are poorly characterized. The aim of this study was to report and analyze the safety and efficacy of Furlow palatoplasty (FP) versus radical intravelar veloplasty (IVV) for treatment of VPI associated with OSMCP. METHODS: A retrospective review of one institution's experience treating VPI associated with OSMCP using IVV (group 1) or FP (group 2) during 24 months was performed. Statistical significance was determined by Wilcoxon matched-pair, Independent-Samples Mann-Whitney U, and analysis of variance (SPSS 20.0.0). RESULTS: In group 1 (IVV), 18 patients were identified from August 2010 to 2011 (12 male and 6 female patients; average age, 5.39 years). Seven patients were syndromic and 11 were nonsyndromic. In group 2 (FP), 17 patients were identified from August 2009 to 2011 (8 male and 9 female patients; average age, 8.37 years). Three patients were syndromic and 14 patients were nonsyndromic. There was statistical significance between the average pretreatment Pittsburgh Weighted Speech Score (PWSS) of the 2 groups (group 1 and 2 averages 19.06 and 11.05, respectively, P=0.002), but there was no statistical significance postoperatively (group 1 and 2 averages 4.50 and 4.69, respectively, P=0.405). One patient from each group required secondary speech surgery. Average operative time was greater for FP (140 minutes; range, 93-177 minutes) compared to IVV (95 minutes; range, 58-135 minutes), P<0.001. Average hospital stay was 3.9 days for IVV (range, 2-9 days) and 3.2 days for FP (range, 2-6 days), with no significant difference (P=0.116). There were no postsurgical wound infections, oral-nasal fistulas, postoperative bleeding complications, or mortalities. CONCLUSIONS: Nonsyndromic patients with hypernasal speech are treated effectively and safely with either IVV or FP. Intravelar veloplasty trended toward lower speech scores than FP (76% IVV, 58% FP PWSS absolute reduction). Syndromic patients with OSMCP may be more effectively treated with FP (72% IVV vs 79% FP PWSS absolute reduction). Intravelar veloplasty is associated with shorter operative times. Both techniques are associated with low morbidity, improved speech scores, and low reoperative rates.

BAC-EDTA transepithelial riboflavin-UVA crosslinking has greater biomechanical stiffening effect than standard epithelium-off in rabbit corneas.

Studies suggest that standard corneal collagen crosslinking (CXL) is a safe and effective treatment to stiffen the cornea for keratoconus and other ectatic corneal disorders. The purpose of the present study was to compare the biomechanical effects of transepithelial benzalkonium chloride-EDTA (BAC-EDTA) riboflavin-UVA crosslinking to standard epithelium-off riboflavin-UVA crosslinking in a rabbit model. Corneal stiffness was quantified using optical coherence elastography at two months after treatment. The mean lateral-to-axial displacement ratio for the BAC-EDTA transepithelial CXL group was lower (greater stiffness) [0.062 ± 0.042, mean ± SD] than epithelium-off CXL (mean ± SD: 0.065 ± 0.045) or untreated control eyes (0.069 ± 0.044). Using ANOVA with Tukey correction, a statistically significant difference was found between the BAC-EDTA transepithelial CXL group and standard epithelium-off CXL (p = 0.0019) or the untreated control (p < 0.0001) groups. A graph of the probability density functions for biomechanical stiffness also showed a greater shift in stiffening in the BAC-EDTA transepithelial CXL group than the standard epithelium-off CXL or untreated control group. These results demonstrated that the biomechanical stiffening effect produced by BAC-EDTA transepithelial CXL was greater than that produced by standard epithelium-off CXL in a rabbit model.