Long-term stability of postpalatoplasty perceptual speech ratings: a prospective study.

This prospective study was undertaken to assess the long-term stability of velopharyngeal perceptual speech ratings of patients with repaired cleft palate. All patients were evaluated and managed at the Cleft Palate and Craniofacial Deformities Institute, St. Louis Children's Hospital. Patients alternately received palatoplasty with or without intravelar veloplasty. Two senior surgeons standardized their operative procedures and performed or supervised directly all operations. Perceptual speech and language evaluations were conducted by the same experienced speech pathologist when the children were 6 years old and 12 years or older. Data were analyzed from the 28 patients available for long-term follow-up. The intravelar veloplasty (N = 14) and nonintravelar veloplasty (N = 14) groups were similar with respect to cleft anatomy and mean age at palatoplasty and at the second perceptual speech evaluation. Evaluation of the 12-year-old and older ratings indicated that the overwhelming majority of patients improved or maintained clinical stability in perceptual ratings of velopharyngeal function. When assessing direction and magnitude of change (i.e., incremental improvement vs. deterioration), the intravelar veloplasty and nonintravelar veloplasty groups had a similar distribution of perceptual speech ratings at both the 6-year and 12-year or older speech evaluations. Results were consistent with previously published data from our center, that the intravelar veloplasty procedure did not affect demonstrably the incidence of postpalatoplasty auditory perceptual symptoms of velopharyngeal dysfunction.

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.

The effect of surgeon experience on velopharyngeal functional outcome following palatoplasty: is there a learning curve?

There is little information in the cleft palate literature concerning the relationship between surgeon volume and clinical outcomes. It is unknown whether such a relationship applies specifically to velopharyngeal dysfunction and the need for secondary physical management of the velopharynx. The purpose of this paper was to explore the concept of an operative learning curve for different surgeons with respect to palatoplasty. Impact of case volume and procedure type on the occurrence of secondary palatal management (the main outcome measure) was assessed. The charts of 472 consecutive palatoplasty patients were reviewed by one speech and language pathologist to determine when the palatoplasty was performed, which surgeon (n = 9) performed the palatoplasty, whether velopharyngeal status was documented at a minimum of 6 years of age, and whether secondary palatal management was prescribed. The results were analyzed by year of palatoplasty, by surgeon, and by number of operations per surgeon to determine total and individual surgeon rates of secondary palatal management. There were 401 palatoplasties (85 percent recovery) with adequate documentation of velopharyngeal status by at least 6 years of age. Palatoplasty rates ranged between 1 and 258 palatoplasties per surgeon. Over the 12 years reviewed, secondary palatal management was performed for 92 patients (23 percent) of the study population. Examination of the proportion of palatoplasty patients receiving secondary palatal management by surgeon and by year showed only one surgeon with a pattern suggesting a learning curve. The proportion of patients receiving secondary palatal management was plotted against the total number of surgeries the surgeon performed. There was a strong relationship between experience and success. The number of procedures this surgeon performed per year increased at approximately the same time as the success rate improved. The categories of "total procedures" and "procedure per year" were highly correlated with each other. Success rates were analyzed by number of procedures performed per year, and there was a clear association between the two variables. To separate the effect of the two variables, a multiple regression model was constructed. The category of "total procedures" was statistically significant in the model, whereas procedures per year was not, suggesting that the key to the dominant surgeon's improvement was cumulative experience rather than frequency of performance of the operation. Palatoplasties performed by high-volume surgeons are more likely to result in better postoperative outcomes (i.e., lower rates of secondary palatal management) as compared with palatoplasties performed by low-volume surgeons. The influence of the surgeon's cumulative experience on improvement seems to be more important than the frequency of performance of primary palatoplasty.

Does preexisting posterior pharyngeal wall motion drive the dynamism of sphincter pharyngoplasty?

Lateral speech videofluoroscopic evaluations were videotaped preoperatively and postoperatively for 20 patients who underwent sphincter pharyngoplasty. Randomized videotapes were constructed and subsequently evaluated by speech/language pathologists experienced in assessing patients with velopharyngeal dysfunction. Rating forms assessing various motion parameters of the posterior pharyngeal wall were completed and analyzed statistically. Results showed that the posterior pharyngeal wall configuration postoperatively was less likely to be rated as smooth relative to the preoperative configuration (p = 0.019). No other statistically significant data were obtained, although there was a trend for posterior pharyngeal wall movement rated as discrete preoperatively to be described as generalized postoperatively. We conclude that when comparing preoperative and postoperative parameters, sphincter pharyngoplasty does not significantly affect posterior pharyngeal wall motion. Posterior pharyngeal wall configuration is less likely to be categorized as smooth after sphincter pharyngoplasty relative to the preoperative condition. Although sphincter pharyngoplasty has been shown to improve velopharyngeal function, there is little evidence from this study to suggest that preexisting posterior pharyngeal wall motion causes sphincteric movement.

Limited value of preoperative cervical vascular imaging in patients with velocardiofacial syndrome.

The purpose of this two-part study was to evaluate the safety of surgical management of speech production disorders in patients with velocardiofacial syndrome without preoperative cervical vascular imaging studies. Anomalous internal carotid arteries have been shown to be a frequent feature of velocardiofacial syndrome. These vessels pose a potential risk for hemorrhage during velopharyngeal narrowing procedures. Magnetic resonance angiography, and other forms of cervical vascular imaging studies such as computerized tomography, have been advocated as aids to surgery by defining the preoperative vascular anatomy. However, it remains unclear whether these studies alter either the conduct or outcome of operations on the velopharynx. In the first part of this study, we reviewed the charts and videonasendoscopic evaluations of 39 consecutive patients with confirmed or suspected velocardiofacial syndrome who underwent sphincter pharyngoplasty or pharyngeal flap from 1978 to 1996. The charts were reviewed to determine (1) the frequency of identification of abnormal pharyngeal pulsations; (2) whether such pulsations affected the conduct of the operative procedure; and (3) whether the presence of pulsations affected surgical morbidity and/or surgical outcome. None of the patients underwent any type of cervical vascular imaging study. In the second part of this study, we surveyed plastic surgeons with numerous years of experience participating on cleft-craniofacial teams, to ascertain practice patterns relating to the management of patients with velocardiofacial syndrome. The questions related specifically to the surgeons' behavior in relation to angiography and their awareness of any cases of surgical morbidity related to the cervical vascular system in patients with velocardiofacial syndrome. We were interested in discerning both how commonly this situation arises clinically and the distribution of the various types of operative procedures in common use. Of our 39 patients, 10 patients (26 percent) had detectable pulsations on preoperative nasendoscopy. Of these, five patients underwent sphincter pharyngoplasty and five underwent pharyngeal flap procedures. Preoperative instrumental and intraoperative clinical assessment of pulsatile vessels allowed velopharyngeal reconstruction in all patients without surgical morbidity. Results of the questionnaire indicated that most cleft surgeons do not routinely order cervical vascular imaging studies for all of their patients with velocardiofacial syndrome. About half of the respondents indicated that their operative approach was influenced by information obtained from angiographic studies. None of the surgeons queried were aware of any cases of surgical morbidity related to the cervical vascular system in patients with velocardiofacial syndrome. Nearly 50 percent of surgeons use pharyngeal flap procedures most frequently, whereas 22 percent of surgeons use sphincter pharyngoplasty most frequently. Results of this study support the safety of sphincter pharyngoplasty or pharyngeal flap procedures in patients with velocardiofacial syndrome without preparatory angiography. These procedures can be performed safely, even in patients having aberrant velopharyngeal pulsations. Given the market cost of magnetic resonance angiography ($1600), one must question the cost-efficacy of magnetic resonance angiography for routine use in the velocardiofacial syndrome population.

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.

Perception of postpalatoplasty speech differences in school-age children by parents, teachers, and professional speech pathologists.

The aims of this study were twofold: (1) to test the ability of parents and teachers to discriminate the speech of children with repaired cleft palate from that of their unaffected peers and (2) to compare these lay assessments of speech acceptability with the critical perceptual assessments of expert clinicians. The subjects for this study were 20 children of school age (age range, 8 to 12 years) who were drawn from a large population (n = 1282) of patients. All subjects had been referred for palatoplasty to the same tertiary cleft center between 1978 and 1991. There were 16 matched controls. The listening team included parents of subjects (n = 32) and teachers of age-matched school children (n = 12). Randomized master audiotape recordings of the study group were presented in blinded fashion to both groups of the adult raters, who were inexperienced in the evaluation of patients with speech dysfunction. An experienced panel of three extramural speech pathologists evaluated the same recordings. In all parameters rated, both parents and teachers showed a consistent tendency to give the subject children more negative ratings than the control children. Expert raters were sensitive to differences in resonance and intelligibility in the control and cleft palate groups. Results of this study differ from similar previous research, indicating that naive peer raters (similar-age children) were insensitive to speech differences in the cleft palate and control groups.

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.

Surgical management of velopharyngeal dysfunction: outcome analysis of autogenous posterior pharyngeal wall augmentation.

Posterior pharyngeal wall augmentation has been advocated for patients having velopharyngeal dysfunction with a small coronal gap. Nonautogenous augmentation has not been accepted widely because of migration or extrusion of alloplastic implants and resorption of injected materials. Autogenous posterior pharyngeal wall augmentation has been performed for decades by Italian surgeons. A retrospective study was conducted to evaluate the efficacy of this procedure. Autogenous posterior pharyngeal wall augmentation, using a rolled superiorly based pharyngeal myomucosal flap, was performed on 14 patients, between November of 1989 and June of 1992, who fulfilled two criteria: velopharyngeal dysfunction unresponsive to speech therapy and a small (< 20 percent) coronal gap on velopharyngeal nasendoscopy. Of these, 3 patients had prior prosthetic velopharyngeal management, including 2 patients with Robin sequence. All patients were evaluated preoperatively and 3 months postoperatively with recorded (audio-videotape) perceptual, nasendoscopic, and fluoroscopic standardized speech and airway evaluations. The tapes were used for construction of a randomized master tape that was presented in blinded fashion and random order to three skilled raters for independent assessment of numerous perceptual and instrumental parameters of speech. The raters were uninvolved in the care of the patients or this study, and their intraobserver and interobserver reliabilities were known. Preoperatively, the majority of patients had nasal turbulence. All patients had variable degrees of hypernasality ranging from intermittent to pervasive. Parameters rated included (1) resonance (hypernasality, hyponasality, mixed), (2) auditory nasal emission (including nasal turbulence), and (3) visual characteristics regarding velopharyngeal closure. The visual parameters consisted of questions about whether a pharyngeal bulge was present or absent, descriptions of posterior pharyngeal wall movements with speech, level of closure, completeness of velopharyngeal closure, and quantitative descriptions of the percentage of velopharyngeal closure postoperatively. Examiners were instructed to look for a static and/or dynamic projection or bulge (i.e., Passavant's ridge) and, if a bulge was present, whether the level of velopharyngeal closure was on the same plane as the neoposterior pharyngeal bulge. Results of the extramural judgments of these parameters showed that there was no statistically significant tendency for patients' speech to be rated as more normal after the augmentation procedure than before it. We conclude that (1) autogenous posterior pharyngeal wall augmentation does not result in speech improvement and (2) autogenous posterior pharyngeal wall augmentation does not impair the nasal airway.

Speech outcome following palatoplasty in primary school children: do lay peer observers agree with speech pathologists?

The aim of this study was twofold: (1) to test the ability of normal children to discriminate the speech of children with repaired cleft palate from the speech of unaffected peers and (2) to compare these naive assessments of speech acceptability with the sophisticated assessments of speech pathologists. The study group (subjects) was composed of 21 children of school age (aged 8 to 12 years) who had undergone palatoplasty at a single cleft center and 16 matched controls. The listening team (student raters) was composed of 20 children who were matched to the subjects for age, sex, and other variables. Randomized master audio-tape recordings of the children who had undergone palatoplasty were presented in blinded fashion and random order to student raters who were inexperienced in the evaluation of patients with speech dysfunction. The same sound recordings were evaluated by an experienced panel of extramural speech pathologists whose intrarater and interrater reliabilities were known; they were not direct care providers. Additionally, the master tape was presented in blinded fashion and random order to the velopharyngeal staff at the cleft center for intramural assessment. Comparison of these assessment methodologies forms the basis of this report. Naive raters were insensitive to speech differences in the control and cleft palate groups. Differences in the mean scores for the groups never approached statistical significance, and there was adequate power to discern a difference of 0.75 on a 7-point scale. Expert raters were sensitive to differences in resonance and intelligibility in the control and cleft palate groups but not to other aspects of speech. The expert raters recommended further evaluation of cleft palate patients more often than control patients. Speech pathologists discern differences that the laity does not. Consideration should be given to the utilization of untrained listeners to add real-life significance to clinical speech assessments. Peer group evaluations of speech acceptability may define the morbidity of cleft palate speech in terms that are most relevant to the patients themselves and may safeguard against the possibility of offering treatment that may be unnecessary.

Standardization for the reporting of nasopharyngoscopy and multiview videofluoroscopy: a report from an International Working Group.

A multidisciplinary International Working Group of scientists was assembled to address the question of standardizing reporting techniques for multiview videofluoroscopy and nasopharyngoscopy, the generally accepted standards for direct observation of the velopharyngeal valve. This report is a first attempt to propose standards while seeking feedback from the readership in order to further develop a common methodology.

Intravelar veloplasty: a prospective study.

Preliminary results are reported from a prospective, alternated, single-institution study of the effect of intravelar veloplasty (IVV) upon postpalatoplasty velopharyngeal incompetence. Two senior surgeons standardized their operative procedures and then performed or directly supervised all surgery. Patients alternately had palatoplasty with or without IVV. One speech pathologist conducted all perceptual speech and language evaluations without knowledge of the surgical procedure when the children were 3 years of age. The IVV and non-IVV groups were similar with respect to cleft anatomy, mean age at palatoplasty, and audiograms at 1 and 3 years of age. The findings from a sample of 51 patients were that surgical retropositioning and approximation of the levator muscles during initial palatoplasty (1) did not demonstrably affect the incidence of postpalatoplasty auditory perceptual symptoms of velopharyngeal incompetence; (2) required a significantly longer operating time; (3) were costlier than the control procedure; and (4) did not have greater morbidity than the control procedure. These findings suggest either that there is no beneficial effect of IVV upon postpalatoplasty velopharyngeal competence or that the effect, if present, is of small magnitude.