John E. Riski

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 suggeest 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.

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.

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

&NA; Recent studies have shown that the Furlow doubleopposing 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. (Plast. Reconstr. Surg. 106: 539, 2000.)

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.

Parameters of Care for Craniosynostosis

Background A multidisciplinary meeting was held from March 4 to 6, 2010, in Atlanta, Georgia, entitled “Craniosynostosis: Developing Parameters for Diagnosis, Treatment, and Management.” The goal of this meeting was to create parameters of care for individuals with craniosynostosis. Methods Fifty-two conference attendees represented a broad range of expertise, including anesthesiology, craniofacial surgery, dentistry, genetics, hand surgery, neurosurgery, nursing, ophthalmology, oral and maxillofacial surgery, orthodontics, otolaryngology, pediatrics, psychology, public health, radiology, and speech-language pathology. These attendees also represented 16 professional societies and peer-reviewed journals. The current state of knowledge related to each discipline was reviewed. Based on areas of expertise, four breakout groups were created to reach a consensus and draft specialty-specific parameters of care based on the literature or, in the absence of literature, broad clinical experience. In an iterative manner, the specialty-specific draft recommendations were presented to all conference attendees. Participants discussed the recommendations in multidisciplinary groups to facilitate exchange and consensus across disciplines. After the conference, a pediatric intensivist and social worker reviewed the recommendations. Results Consensus was reached among the 52 conference attendees and two post hoc reviewers. Longitudinal parameters of care were developed for the diagnosis, treatment, and management of craniosynostosis in each of the 18 specialty areas of care from prenatal evaluation to adulthood. Conclusions To our knowledge, this is the first multidisciplinary effort to develop parameters of care for craniosynostosis. These parameters were designed to help facilitate the development of educational programs for the patient, families, and health-care professionals; stimulate the creation of a national database and registry to promote research, especially in the area of outcome studies; improve credentialing of interdisciplinary craniofacial clinical teams; and improve the availability of health insurance coverage for all individuals with craniosynostosis.

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), Nagers 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.

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

&NA; The purpose of this investigation was to evaluate the prevalence of Chiari malformation, cervical spine anomalies, and neurologic deficits in patients with velocardiofacial 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. (Plast. Reconstr. Surg. 106: 16, 2000.)

An outcome evaluation of sphincter pharyngoplasty for the management of velopharyngeal insufficiency

Sphincter pharyngoplasty is frequently used for the management of children with velopharyngeal insufficiency. The purpose of this study was to evaluate outcome and revision rates of sphincter pharyngoplasty at the authors’ institution. Two hundred fifty patients underwent sphincter pharyngoplasty for velopharyngeal insufficiency between January of 1987 and March of 2001. There were 117 female patients and 133 male patients, with a mean age at primary sphincter pharyngoplasty of 7.6 years (range, 1 to 45 years). Diagnoses included velopharyngeal insufficiency alone (n = 63), velopharyngeal insufficiency associated with cleft palate (n = 127), velocardiofacial syndrome (n = 32), submucous cleft (n = 15), and other (n = 13). Pharyngoplasty revision was defined as any secondary surgical revision of the sphincter as determined by clinical evaluation and objective speech assessment. The pharyngoplasty revision rate was found to be 12.8 percent (n = 32). A favorable outcome was demonstrated in 30 of these patients (93.8 percent) after pharyngoplasty revision. Two patients, one with a diagnosis of a submucous cleft and velocardiofacial syndrome and the other with a cleft palate, required a second revision because of persistent velopharyngeal insufficiency. The revision rate was highest in those patients with velocardiofacial syndrome (21.8 percent) and lowest in patients with velopharyngeal insufficiency alone (6.3 percent). Patients who required revision had significantly higher preoperative oral sentence nasometry (55.2 percent versus 46.1 percent; p < 0.01) and larger velopharyngeal areas (23.7 mm2 versus 18.9 mm2). There was no significant difference in age or sex for those patients who required a revision compared with those who did not require revision. Mean follow-up was 2.4 years (range, 4 months to 13.6 years). Sphincter pharyngoplasty is an effective procedure for the treatment of velopharyngeal insufficiency using revision rate as the standard of success. It had an 87 percent primary success rate that increased to 99 percent after a single revision. Patients with velocardiofacial syndrome, more severe preoperative hypernasal resonance, and larger velopharyngeal areas were more likely to require pharyngoplasty revision.

A rationale for modifying the site of insertion of the Orticochea pharyngoplasty

A modification of the insertion level of Orticochea flaps is proposed. The purpose of the modification is to place the pharyngoplasty at a higher site, in the area of attempted velopharyngeal contact. The site of velopharyngeal contact can be identified using lateral radiographic techniques. A success rate of 93 percent was achieved in improved oral-nasal resonance balance when the pharyngoplasty was placed at a site in the nasopharynx.

Effect of nasal decongestion on nasalance measures.

Objective The purpose of this study was to evaluate the effect of nasal decongestant on nasalance scores for a group of 100 individuals. Participants Forty-one subjects with hypernasality and 59 subjects without hypernasality underwent nasometric assessment at the Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Bauru, Brazil. Design Nasalance scores were obtained for each subject before the application of a nasal decongestant and again 10 minutes after subjects received a topical nasal decongestant applied into both nostrils. Results The nasalance scores obtained after the application of the nasal decongestant were significantly higher than those obtained before the decongestant. Conclusions Nasal decongestion had a small but statistically significant effect on nasalance scores, suggesting that in some individuals, nasal congestion should be a variable of concern when using the Nasometer. Both nasal congestion (i.e., due to nasal rhinitis) and the effects of nasal decongestant sprays may influence Nasometer test results. Interpretation of nasalance scores, therefore, should be done carefully. Furthermore, nasometry, with and without nasal decongestant, can be a valuable clinical tool for screening anterior nasal obstruction, helping to isolate obstruction due to nasal congestion from structural obstruction in the nasal cavities.