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.

Radiographic and aerodynamic measures of velopharyngeal anatomy and function following Furlow Z-plasty.

Recent studies have shown that the Furlow double-opposing Z-plasty has several advantages that make it an attractive procedure for cleft palate repair and treatment of velopharyngeal insufficiency in selected cases. The anatomic changes associated with this procedure have never been documented prospectively. The purpose of this study was to describe radiographic dimensions of the velopharynx and aerodynamic measures of velopharyngeal function in a group of patients before and after Furlow Z-plasty for the treatment of velopharyngeal insufficiency. Twelve consecutive patients with cleft palate and velopharyngeal insufficiency, ranging in age from 3 to 19 years, were selected as candidates for Furlow Z-plasty based on perceptual, endoscopic, and radiographic findings. Eight patients had repaired cleft palate with a residual muscle diastasis and four patients had unrepaired submucous cleft palate. Subjects received aerodynamic and cephalometric assessments before and after Z-plasty. Cephalometric x-rays were measured for velar length, thickness, and pharyngeal depth. Mean nasal airflow during pressure consonants (Vn) was calculated from pressure/flow studies, and patients were categorized as having complete closure (<10 cc/sec Vn) or incomplete closure (>10 cc/ sec Vn). After Z-plasty, there was a significant increase in velar length (p = 0.002) and velar thickness (p = 0.001). After surgery, patients with complete velopharyngeal closure had significantly greater velar length than the incomplete closure group (p = 0.05) with nearly twice the increase in length. Similarly, following surgery, the complete closure group had significantly greater thickness than the incomplete closure group (p = 0.01), with a greater postoperative increase in velar thickness (p = 0.005). Finally, there was a significant negative correlation between percent increase in length and percent increase in thickness for patients in the complete closure group (r = -0.91, p = 0.03). Findings demonstrate that following Furlow Z-plasty, patients with cleft palate and velopharyngeal insufficiency obtained significant increases in velar length and thickness. Greater velar length and greater velar thickness both were associated with complete velopharyngeal closure. Patients in the complete closure group tended to demonstrate large percent gains in either length or thickness or moderate gains in both. Patients in the incomplete closure group tended to demonstrate relatively small percent gains in both dimensions. Results suggest there may be important anatomic features (such as pharyngeal depth/velar length ratio) that can be evaluated before surgery to predict which patients may be most likely to benefit from Furlow Z-plasty as a form of treatment for velopharyngeal insufficiency.

Chiari malformation, cervical spine anomalies, and neurologic deficits in velocardiofacial syndrome.

The purpose of this investigation was to evaluate the prevalence of Chiari malformation, cervical spine anomalies, and neurologic deficits in patients with velocardio-facial syndrome. This study was a prospective evaluation of 41 consecutive patients with velocardiofacial syndrome, documented by fluorescence in situ hybridization, between March of 1994 and September of 1998. The 23 girls and 18 boys ranged in age from 0.5 to 15.2 years, with a mean age of 6.7 years. Nineteen patients were assessed with magnetic resonance imaging, 39 underwent lateral cephalometric radiography, and all patients were examined for neurologic deficits. Eight of 19 patients (42 percent) had anomalies of the craniovertebral junction, including Chiari type I malformations (n = 4), occipitalization of the atlas (n = 3), and narrowing of the foramen magnum (n = 1). One patient with Chiari malformation required suboccipital craniectomy with laminectomy and decompression. Fourteen of 41 patients (34 percent) had demonstrated neurologic deficits; 10 patients (24 percent) had velar paresis (6 unilateral and 4 bilateral). Chiari malformations, cervical spine anomalies, and neurologic deficits are common in velocardiofacial syndrome. Because these findings may influence the outcome of surgical intervention, routine assessment of patients with velocardiofacial syndrome should include careful orofacial examination, lateral cephalometric radiography, and magnetic resonance imaging of the craniovertebral junction.

An anatomic evaluation of the furlow double opposing Z-plasty technique of cleft palate repair.

The aim of this investigation was to examine velar anatomy following the Furlow double opposing Z-plasty in order to analyse the theoretical effects of this technique. Thirty patients with cleft lip and/or cleft palate who underwent primary Furlow palatoplasties between 1989 and 1994 were reviewed. The mean age at the time of surgery was 6.4 months. Evaluation was performed at a mean time of 2.9 years postoperatively, and consisted of oral examination of the position of the velar dimple and measurements of velar dimensions from standard lateral cephalograms. A comparative statistical analysis of velar length (n = 17) and thickness (n = 14) was performed using 2 historical control groups (non-cleft norms and non-Furlow cleft palate repairs). The Furlow procedure produced posterior dimples in 19 of 26 patients adequately rated on oral examination, suggesting successful repositioning of the velar musculature in transverse orientation. The mean velar length was not significantly different from that of norms (being 0.72 mm less), suggesting that the Furlow Z-plasty results in the attainment of near normal velar length. In contrast, the mean velar length was 0.46 mm greater compared to non-Furlow repairs. Although this difference was not statistically significant, it suggests that the Furlow Z-plasty may be more effective in increasing velar length compared to non-Furlow palatoplasty techniques. Velar thickness was significantly greater compared to both norms (P = 0.002) and non-Furlow repairs (P = 0.001). These data suggest that the Furlow double opposing Z-plasty repositions the velar muscles in transverse orientation, and increases both velar length and thickness, lending weight to the theoretical effects of this procedure. The anatomic basis of these changes and their functional implications are discussed.

Skeletal expansion combined with soft-tissue reduction in the treatment of obstructive sleep apnea in children: physiologic results.

Twenty consecutive children, ranging in age from 6 days to 18 years, were treated with skeletal expansion, in addition to soft-tissue reduction, for medically refractory obstructive sleep apnea. The underlying diagnoses were craniofacial microsomia (n = 6), Down syndrome (n = 3), Pierre Robin syndrome (n = 3), cerebral palsy (n = 3), Nager's syndrome (n = 1), Treacher Collins syndrome (n = 1), cri du chat syndrome (n = 1), juvenile rheumatoid arthritis (n = 1), and temporomandibular joint ankylosis (n = 1). Fourteen children had severe medically refractory sleep apnea and were tracheostomy candidates; in the remaining six, tracheostomies were placed shortly after birth and could not be decannulated. Overnight, 12-channel polysomnography was obtained before and after surgery. The mean apnea index improved from 7.42 to 1.26, the mean respiratory disturbance index improved from 25.24 to 1.72, and the mean lowest apnea-related oxygen saturation improved from 68% to 88%. Of the 14 children with medically refractory obstructive sleep apnea, two required tracheostomies. Of the six patients with tracheostomies, five have been decannulated at the time of this writing. Skeletal expansion in conjunction with soft-tissue reduction in the pediatric population permits substantial increases in the volume of both the nasopharynx and oropharynx. Creative use of conventional osteotomies and the application of distraction osteogenesis have enabled surgeons to apply maxillofacial and craniofacial techniques in treating children with obstructive sleep apnea.

Velopharyngeal incompetence as the presenting symptom of malignant brainstem tumor.

OBJECTIVE: When a patient presents with velopharyngeal incompetence (VPI) without an obvious structural or neurologic cause, the clinician is faced with a diagnostic challenge. We present an 11-year-old male with a long history of VPI who had been referred to our institution for evaluation and treatment. RESULTS: Detailed clinical examination and work-up revealed a malignant brainstem tumor. The presenting symptoms of breathiness associated with VPI had been overlooked by several different clinicians in the past. The patient successfully underwent a sphincter pharyngoplasty. CONCLUSIONS: A careful neurologic examination with special attention to the cranial nerves is necessary to identify subtle neurologic deficits and avoid delay in diagnosis. Differential diagnosis of neurogenic VPI is discussed.

A noninvasive technique for detecting hypernasal speech using a nonlinear operator.

Speakers with a defective velopharyngeal mechanism produce speech with inappropriate nasal resonance (hypernasal speech). It is of clinical interest to detect hypernasality as it is indicative of an anatomical, neurological, or peripheral nervous system problem. There are various clinical techniques used to determine hypernasality. The current techniques are physically invasive or intrusive to some extent. A preferred approach for detecting hypernasality, would be noninvasive to maximize patient comfort and naturalness of speaking. In this study, a noninvasive technique based on the Teager Energy operator is proposed. Utilizing a property of the Teager Energy operator and a model for normal and nasalized speech, a significant difference between the Teager Energy profile for lowpass and bandpass filtered nasalized speech is shown. This difference is shown to be nonexistent for normal speech. A classification algorithm is formulated that detects the presence of hypernasality using a measure of the difference in the Teager Energy profiles. The classification algorithm was evaluated using a native English speaker population producing front (/i/) and mid (/A/) vowels. Results show that the presence of hypernasality in speech can be reliably detected using the proposed classification algorithm.

Speech assessment of adolescents.

The adolescent patient provides a unique opportunity for the clinician to be both retrospective and prospective. While adolescence is a period of selective and rapid oral facial growth and dental arch development, the period of rapid speech development is long past. The adolescent patient is entering a period of quiescence and is refining and adjusting existing speech skills. During the patient's adolescence, we have the opportunity to evaluate the outcome of earlier treatment and assess the results of our management strategies and techniques. We also have the opportunity to prospectively modify and improve our treatment strategies. This is a brief review of the experiences of one cleft palate center. Treatment goals and outcomes will be reviewed and areas that require continued refinement will be described.

Videofluoroscopic assessment of dysphagia in children with severe spastic cerebral palsy.

Very little has been published about the characteristics and sequelae of dysphagia in children with neurological impairment. The swallowing difficulties encountered by children with spastic cerebral palsy are particularly debilitating and potentially lethal. However, aggressive evaluation and management of their feeding is typically deferred until they are medically or nutritionally compromised. Reports of the use of videofluoroscopy to analyze the swallowing patterns and presence or absence of aspiration in such children are rare. This paper describes the histories and analyzes the videofluorographic swallow studies of 22 patients with the primary diagnosis of severe spastic cerebral palsy. The ages of the subjects ranged from 7 months to 19 years. All had severe dysphagia and were slow, inefficient eaters. Fifteen patients (68.2%) demonstrated significant silent aspiration during their swallow study. Analysis of specific features of their swallowing patterns indicated that decreased or poorly coordinated pharyngeal motility was predictive of silent aspiration. Moderately to severely impaired oral-motor coordination was indicative of severity of feeding complications. Our data suggest that early diagnostic workup, including baseline and comparative videofluoroscopic swallow studies, could be helpful in managing the feeding difficulties in these children and preventing chronic aspiration, malnutrition, and unpleasant lengthy mealtimes.

Swallowing, speech, and brainstem auditory-evoked potentials in spasmodic torticollis.

To explore the controversial "brainstem theory" of spasmodic torticollis, eight consecutively referred patients were examined. Three independent examinations were conducted on the same day: a videofluoroscopic barium swallowing examination, an instrumental speech examination, and a brainstem auditory-evoked potential (BAEP) analysis. Swallowing was normal in two patients; speech physiology, in five; and BAEPs, in all. Normal BAEPs refute the brainstem theory, while abnormalities of speech and swallowing temper this conclusion. Several alternative explanations are proposed.

Evaluation of failed sphincter pharyngoplasties.

A review of 30 failed sphincter pharyngoplasties is presented. Failure may be caused by inappropriate surgical planning, inadequate surgical technique, or inappropriate patient selection. Problems with surgical planning and technique that lead to failure were low flap placement, flap dehiscence, and flaps not approximated in midline. Problems with patient selection that lead to failure were large velopharyngeal gap on videofluoroscopy, and residual speech (articulation) deficits. Careful pre- and postoperative evaluation has led to refinement of the surgical procedure and improved outcome. Success rate improved from 67.65% in the first 5 years to 86% in the last 5 years of this 15-year series.

Evaluation of the sphincter pharyngoplasty.

The results of the sphincter pharyngoplasty were evaluated in 139 patients with velopharyngeal incompetence (VPI) who demonstrated active velar elevation. All patients underwent perceptual speech evaluation and lateral phonation radiographic study; select patients underwent multiview videofluoroscopic, flexible nasendoscopic, and pressure-flow studies. All but one patient demonstrated improvement and 109/139 (78.42%) demonstrated resolution of VPI. Sixteen of thirty failed pharyngoplasties were revised. Revision was successful in 8/16 patients yielding an overall success rate of 117/139 (84.17%). Success rate was 67.65 percent for patients managed during the first 5 years and improved to 84.78 percent for patients managed during the last 5 years of this 15-year series. Analysis revealed that younger patients were treated more successfully than older patients, large velopharyngeal areas were treated as successfully as smaller ones, and circular closure patterns were treated more successfully than coronal patterns. The primary cause of failure was insertion of the flap below the point of attempted velopharyngeal contact.

Swallowing in torticollis before and after rhizotomy.

To determine risk factors for dysphagia after ventral rhizotomy, videofluoroscopic barium swallowing examinations were done on 41 spasmodic torticollis patients before and after surgery. Radiologic abnormalities were present in 68.3% of the patients before surgery, but these were only mildly abnormal in the majority. After surgery 95.1% showed radiologic abnormalities which were moderate or severe in one-third of the patients. Swallowing abnormalities correlated significantly with duration of torticollis and subjective complaints of swallowing difficulty both before and after surgery, but not with age, sex, or type of torticollis. The major acute postoperative finding was aggravation of preexisting pharyngeal dysfunction. Follow-up from about half of our original sample showed that gradual improvement occurred from 4 to 24 weeks after surgery by subjective report. We review the innervation of intrinsic and extrinsic pharyngeal musculature, and suggest that C1-3 rhizotomies and selective sectioning of the spinal accessory nerve are responsible for aggravation of pharyngeal swallowing dysfunction in the acute postsurgical period.

Parents of children with cleft lip and plate.

The preceding literature review and parent interviews are intended to provide novel insight into the stress, anxiety, and fears faced by parents of children born with a cleft lip and palate. Professionals need to be available to assist with the adjustments and transitions that the parents will face and to tailor their interactions to the specific needs of the parents and the child. Several conclusions are apparent. 1. Needs appear to be greatest at times of transition: birth, operation, and school. 2. Feelings of loss and grief may accompany the birth of a child with a cleft. The birth may be marked by the realization that the perfect child is lost and by the grief that this imperfect child must be loved and nurtured. The rearing process is complicated by the parents' own insecurities and can be assisted by positive coping strategies and the support of other parents of children with clefts. 3. Parents may feel uncomfortable discussing aspects of finance and personal fears. The parents interviewed indicated that the fears and concerns expressed here were the very concerns that they do not raise with professionals. 4. Parents are highly variable in their experience and needs. The age of the parents, the extent of the cleft, and familiarity with clefting appear to be major factors in determining how well parents deal with the birth of a child with a cleft. 5. Cleft palate team members have innumerable opportunities to assist parents.(ABSTRACT TRUNCATED AT 250 WORDS)

Swallowing function in patients with spasmodic torticollis.

We examined the oropharyngeal swallowing ability of 43 patients with spasmodic torticollis using a videofluoroscopic procedure. Twenty-two (51.2%) demonstrated objective evidence of swallowing abnormalities; 15 (34.9%) had subjective complaints. Delayed swallowing reflex and vallecular residue were more frequent (p less than 0.0046) than any other abnormality. The constellations of abnormalities were consistent with neurogenic, postural, and mixed neurogenic-postural types of dysphagia.

Macroglossia: clinical considerations.

Macroglossia is a multifactorial condition that is almost always associated with a space-occupying mass of the tongue. The usual treatment is surgical resection. The typical medical conditions that lead to macroglossia have been described. The orofacial myologist should be alert to the presence of possible pathology of the tongue during orofacial examination, and refer suspected instances of macroglossia to an appropriate medical resource for definitive diagnosis and treatment.

The role of pressure flow and endoscopic assessment in successful palatal obturator revision.

The purpose of this paper is to illustrate the value of combined aerodynamic and endoscopic examination of velopharyngeal function in the revision of prosthetic speech appliances. Use of these combined measures enables the clinician to identify accurately the site(s) of any under- or overobturation. Furthermore, any needed revision is completed accurately and efficiently.