Cinefluorographic evaluation of patients with velopharyngeal dysfunction in the absence of overt cleft palate.
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Cineradiography↗
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Management of velopharyngeal dysfunction in cerebral palsy.
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Cerebral Palsy↗
Velopharyngeal function and dysfunction.
The concepts regarding velopharyngeal function, the production of disordered nasalization, and the management of velopharyngeal dysfunction can be summarized as follows: 1. Although the function of the velopharyngeal mechanism is critical to the control of oral-nasal balance, the configuration and function of the speech articulatory system as a whole will determine the degree of nasalized speech that is produced. 2. Velopharyngeal dysfunction can be related to one or a combination of structural and motor limitations within the velopharyngeal mechanism. 3. There are two perceptual manifestations of velopharyngeal dysfunction. One is acoustic (nasality); the other is aerodynamic (nasal emission). For any given speaker, it is possible to hear both, and it is possible to hear one and not the other. 4. Velopharyngeal dysfunction can be treated in a variety of ways. The method of treatment should be determined by the structural characteristics of the velopharyngeal mechanism and the speech-motor abilities of the patient.
Child↗
Need for velopharyngeal management following palatoplasty: an outcome analysis of syndromic and nonsyndromic patients with Robin sequence.
The Robin sequence is a pathogenetically and etiologically heterogeneous condition that can be a nonsyndromic anomaly or one feature of many syndromes. Little information is available regarding the distribution of patients having Robin sequence, with or without associated syndromes, who develop velopharyngeal dysfunction. In order to discern whether patients with Robin sequence, nonsyndromic and/or syndromic, have different velopharyngeal dysfunction rates from those observed among all patients undergoing palatoplasty during the same time period, a retrospective study was undertaken. The charts of 873 patients with overt clefts of the secondary palate managed at a single cleft center between 1978 and 1992 were reviewed. Diagnostic criteria for Robin sequence included cleft palate without cleft lip, microretrognathia, and perinatal respiratory and/or feeding difficulties; 79 such patients (9 percent) were identified from the initial group of 873. Of these, 58 patients (7 percent) were at least 3 years of age and had sufficient follow-up to allow for evaluation of speech outcome by an experienced speech pathologist through a variety of methodologies (videonasendoscopy, speech videofluoroscopy, perceptual speech characteristics). This group comprised the Robin sequence study population. All Robin sequence patients' charts were reviewed by a medical geneticist to confirm the presence or absence of a syndrome. Of the original 873 patients, there were 127 non-Robin sequence patients who were sufficiently cooperative in diagnostic testing to yield definitive information. This group comprised the non-Robin sequence study population. Among nonsyndromic Robin sequence patients, 15 of 34 (44 percent) developed velopharyngeal dysfunction and required velopharyngeal management, while 2 of 24 syndromic patients (8 percent) developed velopharyngeal dysfunction (p = 0.003). Of the 127 non-Robin sequence isolated cleft palate patients, 113 were nonsyndromic, of whom 18 percent (20 of 113) required velopharyngeal dysfunction management, and 14 were syndromic, of whom 64 percent (9 of 14) required velopharyngeal dysfunction management (p = 0.00009). We conclude that nonsyndromic Robin sequence patients have a higher rate of postpalatoplasty velopharyngeal dysfunction than the nonsyndromic non-Robin sequence cleft population. Outcome analysis of velopharyngeal function in cleft patients should take into account patients who have cleft palate in association with Robin sequence, with or without a recognizable syndrome.
Child, Preschool↗
Surgery for speech in cleft palate patients.
INTRODUCTION: Superiorly based pharyngeal flaps and sphincter pharyngoplasties are the two main possibilities for the surgical treatment of hypernasality in velopharyngeal dysfunction. Videonaspharyngoscopy and multi-view videofluoroscopy can provide anatomical and physiological data for planning these surgical procedures for correcting hypernasality. AIM: This study was undertaken to assess the planning and outcome of pharyngeal flaps and sphincter pharyngoplasties for correcting velopharyngeal insufficiency. The surgical techniques were customized according to the findings of videonasopharyngoscopy and multiview videofluoroscopy. MATERIALS AND METHODS: Seventy patients with repaired palate clefts and residual velopharyngeal dysfunction were studied. The patients were randomly divided into two groups. The first group received a pharyngeal flap. The second group received a sphincter pharyngoplasty. Both procedures were individually customized according to the findings of videonasopharyngoscopy and multi-view videofluoroscopy. RESULTS: There was a non-significant difference (P >0.05) between the mean size of preoperative velopharyngeal closure gap between the two groups of patients (mean=27.5%; S.D.=7.7% versus mean=28.3%; S.D.=5.9%). Postoperatively, velopharyngeal dysfunction was completely corrected in 89% of the cases from group 1, and in 85% of the cases from group 2. There was a non-significant difference (P >0.05) between the success rate for correcting VPI in both groups of patients. CONCLUSIONS: Pharyngeal flap and sphincter pharyngoplasty seem to be safe and reliable procedures for treating residual velopharyngeal dysfunction. Although not all the patients studied for this paper achieved complete closure after the surgical procedures, all of them showed a reduction of the size of the velopharyngeal closure defect. The planning of the surgical procedure, in order to match the postoperative structure to the preoperative velopharyngeal dimensions and movements visualized through Videonasopharyngoscopy and videofluoroscopy, seems to be the most important aspect of the surgery for correcting residual velopharyngeal dysfunction.
Child↗
Revision of the failed sphincter pharyngoplasty: an outcome assessment.
This paper reports results of surgical management of failed sphincter pharyngoplasties that were performed for velopharyngeal dysfunction. Revisional surgery consisted of tightening of the sphincter pharyngoplasty port or reinsertion of sphincter pharyngoplasty flaps following dehiscence. We critique the anatomic abnormalities associated with unacceptable vocal resonance and nasal air escape following sphincter pharyngoplasty and analyze the effect of sphincter pharyngoplasty revision on ultimate speech outcome. The results of initial sphincter pharyngoplasty surgery were evaluated in 46 patients with velopharyngeal dysfunction. Nine (20 percent) of these patients were considered surgical failures because of persistent hypernasality and/or nasal turbulence on perceptual speech evaluation at least 3 months postoperatively. These patients underwent sphincter pharyngoplasty revision and form the basis of this report. All patients who failed sphincter pharyngoplasty initially underwent both preoperative and postoperative perceptual speech evaluations, lateral phonation radiographic studies with still reference views, and flexible nasendoscopic studies. Evaluations of upper airway status were conducted by the same experienced otolaryngologist. Following sphincter pharyngoplasty revision, 7 of 9 (78 percent) patients demonstrated resolution of velopharyngeal dysfunction, and to some degree, all patients managed with revision became hyponasal. The primary cause of failure was partial or complete flap dehiscence; a secondary cause was hypotonicity of the velopharyngeal mechanism. Failure was not correlated with the level of insertion of the pharyngoplasty flaps with respect to the point of attempted velopharyngeal contact. Sphincter pharyngoplasty is an effective means of management for velopharyngeal dysfunction in many patients. The objective of removing the stigmata of velopharyngeal dysfunction without causing upper airway obstruction may not be realistic in some patients with microretrognathia (i.e., Pierre Robin sequence), in whom anatomic constraints predispose to flap dehiscence. Problems with surgical technique contributing to failure appear to be related to experience of the surgeon, and improvement in outcome can be anticipated as the "learning curve" is overcome.
Child↗
Quantification of dynamic velopharyngeal port excursion following sphincter pharyngoplasty.
The sphincter pharyngoplasty is a surgical procedure designed to correct velopharyngeal dysfunction. Its advocates cite the theoretical advantage of its induction of dynamic activity of the neovelopharyngeal port, but this dynamic activity has yet to be quantitatively demonstrated in the literature. The purpose of this study was to quantify postoperative velopharyngeal dynamism and to document the results of intervention outcome on sphincteric excursion measurements from minimal-to-maximal orifice closure. We conducted a 7-year retrospective review of speech videofluoroscopy evaluations in patients who had undergone sphincter pharyngoplasty in our center. Between 1989 and 1994, there were 58 patients so treated for postpalatoplasty velopharyngeal dysfunction by two surgeons using the same operative technique. Patients for whom sphincter pharyngoplasty was recommended fulfilled both of the following criteria: (1) velopharyngeal dysfunction caused by an anatomic, myoneural, or combined deficiency of the velopharyngeal sphincter that would not be expected to be managed by speech therapy alone, and (2) preoperative videonasendoscopy and speech videofluoroscopic studies that demonstrated large-gap coronal, circular, or bow-tie closure patterns or velopharyngeal hypodynamism. Of the original 58 patients, 24 underwent postoperative speech videofluoroscopic evaluations with basal views. Of these, 20 of the evaluations (83 percent) were of adequate quality to be included in a research study. Still images showing maximum and minimum excursion of the sphincter in basal view were obtained. To test for observer reliability, the speech videofluoroscopic studies were randomized and presented for measurement to the same individual on two occasions, each session separated by a 1-month time interval. Topographic imaging software was used to obtain maximum and minimum measurements to within 0.1 mm. Partitioning the variance of the data showed that measurement variability was a very small portion of the total, and that difference between the minimum and maximum values was the largest source of variability. Of the total variability in the data, 64.0 percent originated in the minimum/maximum difference, 34.3 percent came from patient variability, and only 1.7 percent resulted from original or repeat measurements. The patient variability may be exaggerated because of variability in the scale of measurement. Results of this study indicate a quantifiable and statistically significant difference in maximum-to-minimum excursion of sphincteric closure. Sphincter pharyngoplasty appears to be dynamic in the majority of cases.
Adolescent↗
Clinical features in 130 patients with the velo-cardio-facial syndrome. The Leuven experience.
The velo-cardio-facial syndrome (VCFS) is a leading cause of velopharyngeal dysfunction and cleft palate and caused by a submicroscopic deletion in the long arm of chromosome 22 (band 22q11). During the last 5 years, 130 patients with a 22q11 deletion were diagnosed in Leuven. Most patients presented a wide variety of the classical features of the velo-cardio-facial syndrome. Velopharyngeal dysfunction was almost always present whereas an isolated cleft lip/palate was observed in a minority of patients. The velopharyngeal function can be evaluated by the classic combination of indirect and direct techniques. Because of the frequent occurrence of the velo-cardio-facial syndrome, estimated at around 1/4000 live births, and given the extremely broad clinical spectrum which makes clinical diagnosis difficult, screening of patients with velopharyngeal dysfunction for a deletion 22q11 is indicated.
Abnormalities, Multiple↗
Clinical results of pharyngeal flap surgery: the Iowa experience.
Sixty-five patients with cleft palate, with or without cleft lip, who received previous pharyngeal flap surgery for chronic velopharyngeal dysfunction in our department, were examined for velopharyngeal status, speech production patterns, and evidence of nasal airway obstruction. Of the 65 subjects, 54 (83.1 percent) showed velopharyngeal function within normal limits, 43 (66.1 percent) showed normal or near-normal speech production, and 58 (89.2 percent) reported snoring sometimes or often. Of the 58 reporting snoring, electrocardiogram (ECG) data for 33 were examined for evidence of right ventricular hypertrophy. Only one (3 percent) of the 33 showed such possible indication. We conclude that by our methods, pharyngeal flap surgery is an effective treatment for velopharyngeal dysfunction. After surgery, patients may report symptoms of nasal airway obstruction during sleep but are not expected to show ECG changes in cardiac function resulting from oxygen deprivation.
Adolescent↗
Salvaging the failed pharyngoplasty: intervention outcome.
OBJECTIVE: This paper reports on the rates of failure of operations (pharyngeal flap and sphincter pharyngoplasty) performed for management of velopharyngeal dysfunction, and outcome following their revision. DESIGN: Anatomic abnormalities associated with unacceptable vocal resonance and nasal air escape following pharyngeal flap and sphincter pharyngoplasty were critiqued. The results of primary pharyngeal flap were evaluated for 65 patients, and the results of primary sphincter pharyngoplasty were evaluated for 123 patients. All patients were treated for velopharyngeal dysfunction. The definition of surgical failure was based on persistent hypernasality and/or nasal turbulence on perceptual speech evaluation, and incomplete velopharyngeal closure on instrumental evaluation, at least 3 months postoperatively. SETTING: All patients were evaluated and managed at the Cleft Palate and Craniofacial Deformities Institute, St. Louis Children's Hospital, a tertiary cleft care center. PATIENTS, PARTICIPANTS: All patients had failed surgical management initially, either with pharyngeal flap or sphincter pharyngoplasty, and all underwent repeat preoperative and postoperative perceptual speech evaluations; real-time lateral phonation fluoroscopy including still reference views; and flexible nasendoscopy of the velopharynx using standard speech protocols. INTERVENTIONS: Revisional surgery for both procedures consisted of either tightening of the sphincter pharyngoplasty or pharyngeal flap port(s) or reinsertion of the sphincter pharyngoplasty or pharyngeal flaps following dehiscence. MAIN OUTCOME MEASURES: The main outcome measure was normalcy of velopharyngeal function, i.e., elimination of perceptual hypernasality and instrumental evidence of complete velopharyngeal closure. The rates of pharyngeal flap failure and sphincter pharyngoplasty failure were determined for those patients requiring surgical revision. RESULTS: Thirteen of 65 patients (20%) who underwent primary pharyngeal flap required revisional surgery. Of these 13 patients, eight were managed successfully with a single revisional operation. The remaining five patients (38%) continued to exhibit velopharyngeal dysfunction and underwent a second revision consisting of tightening or augmentation of the lateral ports. Speech results were satisfactory in all patients so treated; however, hyponasality with no other airway morbidity occurred in all five. Twenty of 123 patients (16%) who underwent primary sphincter pharyngoplasty required surgical revision. Of these 20 patients, 17 were managed successfully. For both procedures, the principal cause of failure was partial or complete flap dehiscence. CONCLUSIONS: Rates of primary pharyngeal flap failure are roughly equivalent to rates of primary sphincter pharyngoplasty failure. Pharyngeal flap and sphincter pharyngoplasty failures can be salvaged with revisional surgery, which can provide a velopharyngeal mechanism capable of complete closure. Revisional surgery is usually associated with denasal speech.
Child↗
Acute obstructive sleep apnea as a complication of sphincter pharyngoplasty.
This report describes postoperative airway compromise following sphincter pharyngoplasty (SP) for treatment of post-palatoplasty velopharyngeal dysfunction. A retrospective review of 58 SPs performed for post-palatoplasty velopharyngeal dysfunction, on 30 male, and 28 female patients, over a 5-year study period was undertaken at a tertiary referral academic institution (Washington University School of Medicine), at the St. Louis Children's Hospital, Cleft Palate and Craniofacial Deformities Institute. Eight patients were identified who had the following inclusion criteria: overt perioperative and/or postoperative airway dysfunction, identifiable syndromes, or microretrognathia. Items reviewed were patient demographic factors, associated medical problems, genetics evaluations, nasendoscopic characteristics of velopharyngeal closure, anesthetic evaluation of the patients, and the incidence and severity of perioperative complications. Particular attention was paid to factors contributing to the airway obstruction. Of the eight subjects with perioperative and/or postoperative upper airway dysfunction following SP, five patients had Pierre Robin sequence/micrognathia, while three patients had a history of perinatal respiratory and/or feeding difficulties without micrognathia or an identified genetic disorder. All but two episodes of airway dysfunction resolved within 3 days postoperatively. These patients were discharged home with apnea monitors; both were readmitted with recurrent airway dysfunction. Continuous positive airway pressure (CPAP) was utilized successfully in all instances, and no patients required take-down of the SP to relieve airway dysfunction. CPAP is an effective, noninvasive treatment strategy for management of iatrogenically induced apnea following SP, without sacrificing the surgical benefit of improved speech intelligibility.
Adolescent↗
The velo-cardio-facial syndrome: the otorhinolaryngeal manifestations and implications.
The velo-cardio-facial syndrome (VCFS), due to a deletion in chromosome 22 on its long arm (22q11), is a leading cause of velopharyngeal dysfunction and cleft palate. With the recent finding of a deletion on chromosome 22q11 in these patients with velopharyngeal dysfunction, a routine test is available making the diagnosis of VCFS much more frequent than previously thought.
Child↗
Sphincter pharyngoplasty for the surgical management of speech dysfunction associated with velocardiofacial syndrome.
There are no reports in the literature that document the effectiveness of sphincter pharyngoplasty as a surgical alternative to pharyngeal flap for management of velopharyngeal dysfunction in patients with velocardiofacial syndrome. A retrospective review of patients with velocardiofacial syndrome was undertaken at our tertiary cleft care centre. All patients were managed between 1984 and 1996 at the Cleft Palate and Craniofacial Deformities Institute, St Louis Children's Hospital. Subjects (n = 19) underwent velopharyngeal surgical management on the basis of perceptual speech evaluations and instrumental assessments of inadequate velopharyngeal closure. All patients had a molecular diagnosis of velocardiofacial syndrome based on fluorescent in situ hybridisation analysis of peripheral blood lymphocytes and independent evaluation by a medical geneticist. Surgical outcome was classified as successful if perceptual speech assessment indicated elimination of hypernasality, nasal emission and turbulence, and instrumental assessment indicated 100% velopharyngeal closure. Results showed that 18 of 19 patients were managed successfully with sphincter pharyngoplasty. Our data corroborate that sphincter pharyngoplasty is a reasonable alternative to pharyngeal flap in patients with velopharyngeal dysfunction secondary to velocardiofacial syndrome.
Adolescent↗
The evolution of velopharyngeal imaging.
Currently, functional visualization of the velopharynx requires tests that are either invasive (endoscopy) or that impart ionizing radiation (speech videofluoroscopy). The overall intrusiveness of endoscopy may limit its clinical utility, especially in young children. As a resut of growing awareness of the long-range effects of radiation exposure associated with X-ray imaging, radiographic research on subjects and studies not judged to be clinically necessary have been all but abandoned. The static nature of lateral radiographs precludes temporal assessment, and the two dimensionally of images derived from both of these diagnostic modalities may limit understanding of spatial anatomic relationships and may preclude quantitative analysis. The need for a noninvasive, rapid, and easily repeatable method for examination of the velopharynx has fomented the innovative application of existing technologies, especially magnetic resonance imaging. We present an updated overview of techniques for imaging the velopharyngeal mechanism, with a focus on residual velopharyngeal dysfunction after initial palatoplasty. We provide a comprehensive perspective of the role of currently available instrumentation, summarize the work in our center regarding the technological developments of magnetic resonance imaging, and speculate about future applications of magnetic resonance imaging systems for evaluation of velopharyngeal dysfunction. The limitations of each of these measures discussed are emphasized.
Diagnosis, Differential↗
[Velopharyngeal closure dysfunction in various neurological disorders examined by using the flow-nasality graph].
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Adult↗
Navigation of the nose with flexible fiberoptic endoscopy.
The introduction of flexible fiberoptics into medicine revolutionized the evaluation and treatment of velopharyngeal dysfunction. In this paper, rigid endoscopy and flexible fiberoptic scopes are discussed, including their respective advantages and disadvantages. Anesthetic and anatomic considerations relative to the endoscopic procedure are presented. Transnasal endoscopy permits documentation of static and dynamic anatomy, information that may be fundamental for the understanding and treatment of patients with velopharyngeal dysfunction.
Anesthesia, Local↗
A Genetic Study of 66 Individuals With Syndromic Velopharyngeal Insufficiency.
ObjectiveVelopharyngeal insufficiency (VPI) is a form of velopharyngeal dysfunction caused by anatomical anomalies in the velopharyngeal sphincter. Although genetic causes such as 22q11 deletion syndrome are recognised, the broader genetic basis remains poorly understood. This study investigated the genetic aetiology of VPI.DesignWe conducted a phenotypic search on the DECIPHER database using the term 'Velopharyngeal Insufficiency' and identified genetic variants in these patients. These were classified using ACMG guidelines. Literature searches and network analyses examined gene roles and their contribution to sphincter development.PatientsWe identified 66 patients on DECIPHER with VPI.ResultsNinety-five percent of patients presented with syndromic VPI, commonly observed phenotypes included neurodevelopmental abnormalities and facial dysmorphology. Five patients (7.6%) had cleft palate. Pathogenic or likely pathogenic variants were identified in 56.1% of those with reported genetic variants (32/57); 26.3% through copy number variants and 29.8% through sequence variants (SVs). Chromosome 22q11.2 aberrations were the most frequently observed finding in the cohort; 7 patients carried deletions and 2 carried duplications. Independent truncating SVs in KMT2A and CAMTA1 were observed in multiple individuals. Network analyses and literature review of 26 genes prioritised for potential relevance to VPI revealed 2 broad functions: regulating gene expression and signalling pathways, contributing to palatogenesis and cranial-base development.ConclusionThis study demonstrates a high rate of pathogenic or likely pathogenic genetic findings in a syndromic VPI cohort. The findings highlight several recurrent genomic regions and biologically plausible genes that may contribute to VPI beyond the well-known 22q11 deletion syndrome.
development↗
Positive narrowing pharyngoplasty with forearm flap for functional restoration after extensive soft palate resection.
The restoration of velopharyngeal function after extensive soft palate resection to treat malignant oropharyngeal tumors is a major challenge to reconstructive surgeons. The authors had previously reconstructed soft palatal defects routinely with the folded forearm flap. A patient who had more than half of the soft palate excised experienced postoperative velopharyngeal dysfunction. To restore efficient velopharyngeal function, pharyngoplasty was additively applied where the folded ridge of the forearm flap was sutured to the posterior pharyngeal wall in an inverse manner of the pharyngeal flap technique. The essence of the procedure was positive narrowing of the nasopharyngeal space. Five patients who underwent this pharyngoplasty and another five who did not were evaluated for postoperative functions of speech intelligibility and of nasal regurgitation during oral feeding. The velopharyngeal movements of all patients were examined under a nasopharyngeal endoscope. The evaluations demonstrated that this surgical procedure afforded satisfactory results. This positive narrowing pharyngoplasty technique is simple, easy, and minimally invasive to the remaining healthy tissue, and it is the method of choice for the reconstruction of the soft palate after malignant tumor resection.
Aged↗