Interaction of the craniofacial complex and velopharyngeal musculature on speech resonance in children with 22q11.2 deletion syndrome: An MRI analysis.

There are limited MRI studies of craniofacial and velopharyngeal features in children with 22q11.2 deletion syndrome (22q11.2DS) and to date, none have explored the potential relationship between these features and the speech phenotype. The purpose of this study was to examine the relationship between craniofacial and related velopharyngeal structures in children with 22q11.2DS and to assess their correlation to resonance features using an unsedated MRI protocol. Fifteen children with 22q11.2DS and 15 age- and sex-matched controls with normal velopharyngeal anatomy (ages 4-12 years) successfully completed the study. Analysis of covariance was used to compare differences between the experimental (22q11.2DS) and control (children with normal anatomy) groups. Correlation analyses and regression models were also utilized. The 22q11.2DS group demonstrated significantly shorter nasion-to-sella, sella-to-basion, and basion-to-opisthion distances. The anterior cranial base angle was significantly more obtuse. The levator veli palatini (levator) muscle was significantly thinner and shorter, with an obtuse angle of origin in the 22q11.2DS group. Levator length was significantly correlated with the sella-to-basion measure and hypernasality was correlated with levator origin-to-origin distance. Preliminary results from this study indicate a significant association between hypernasality and levator origin-to-origin distance. Findings from the present study, provide an insight into the pathophysiology of velopharyngeal dysfunction related to this clinically complex population.

Morphology of the Levator Veli Palatini Muscle in Adults With Repaired Cleft Palate.

The purpose of this study was to examine differences in levator veli palatini (levator) morphology between adults with repaired cleft palate and adults with noncleft anatomy. Fifteen adult participants (10 with noncleft anatomy, 5 with repaired cleft palate) underwent 3-dimensional (3D) static magnetic resonance imaging (MRI). Image analyses included measures of total muscle volume and the circumference and diameter at 6 points along the length of the muscle. Differences between groups were analyzed using independent sample Mann-Whitney U tests (α < 0.05). Significant differences between groups were noted for measures of muscle volume, circumference at the origin and midline, anterior-posterior diameter at the origin and midline, and superior-inferior diameter at the point of insertion into the velum and midline. Differences in measures at other points along the levator muscle belly were not statistically significant. Limited sample size and gender differences may have impacted statistical findings. Overall, the levator muscle in adults with repaired cleft palate is significantly different than that of adults with noncleft anatomy. This study demonstrates the successful implementation of a method for 3D analysis of velopharyngeal (VP) musculature with potential clinical utility given continued technological advancements in MRI. Continued evaluation of pre- and postsurgical anatomy and short- and long-term outcomes may contribute to a better understanding of the effects of various types of palatoplasties on levator structure, which is important to VP function for speech.

Effect of Dissection and Reconstruction of Palatal Muscles on Morphological Features and Ultrastructure of the Oral Musculature in Cats.

The study was designed to determine the effect of dissection and reconstruction of palatal muscles on muscle morphology in cats. 27 cats were randomly divided into three groups according to the extent of muscle dissection from the palatal midline. All dissections were performed from the posterior border of the hard palate, and the muscles were allowed to reconstruct over time. The morphological features were determined by hematoxylin and eosin staining of tissue sections, and ultrastructure was observed under a transmission electron microscope. As a result, no obvious differences were evident in the morphological features or ultrastructure of animals in the <1/3rd and 1/3rd-2/3rd area groups. In the >2/3rd area group, the muscles fibers were disordered and inflammatory cell infiltration and naïve muscle cells were found at one month after surgery. At the second and third month after surgery, the muscle fibers showed regular alignment, the naïve muscle fibers gradually matured, and the number of infiltrating inflammatory cells decreased. Muscle ultrastructure analysis revealed that myocommata were correctly aligned, and the Z line was more distinct. In conclusion, extensive dissection of palatal muscles does not result in fibrosis. Injury to oral musculature can be repaired and the musculature regenerated over time.

Three-dimensional imaging of palatal muscles in the human embryo and fetus: Development of levator veli palatini and clinical importance of the lesser palatine nerve.

BACKGROUND: After palatoplasty, incomplete velopharyngeal closure in speech articulation sometimes persists, despite restoration of deglutition function. The levator veli palatini (LVP) is believed to be significantly involved with velopharyngeal function in articulation; however, the development and innervation of LVP remain obscure. The development of LVP in human embryos and fetuses has not been systematically analyzed using the Carnegie stage (CS) to standardize documentation of development. RESULTS: The anlage of LVP starts to develop at CS 21 beneath the aperture of the auditory tube (AT) to the pharynx. At CS 23, LVP runs along AT over its full length, as evidenced by three-dimensional image reconstruction. In the fetal stage, the lesser palatine nerve (LPN) is in contact with LVP. CONCLUSIONS: The positional relationship between LVP and AT three-dimensionally, suggesting that LVP might be derived from the second branchial arch. Based on histological evidence, we hypothesize that motor components from the facial nerve may run along LPN, believed to be purely sensory. The multiple innervation of LVP by LPN and pharyngeal plexus may explain the postpalatoplasty discrepancy between the partial impairment in articulation vs. the functional restoration of deglutition. That is, the contribution of LPN is greater in articulation than in deglutition.

Fibrosis impairs the formation of new myofibers in the soft palate after injury.

Muscle repair is a crucial component of palatoplasty but little is known about muscle regeneration after cleft palate repair. We hypothesized that the formation of new myofibers is hampered by collagen accumulation after experimental injury of the soft palate of rats. One-millimeter excisional defects were made in the soft palates of 32 rats. The wound area was evaluated after 3, 7, 28, and 56 days using azocarmine G and aniline blue to stain for collagen and immunohistochemistry to identify myofibroblasts and to monitor skeletal muscle differentiation. To evaluate age effects, 16 unwounded animals were evaluated at 3 and 56 days. Staining was quantified by image analysis, and one-way ANOVA was used for the statistical analysis. At day 56, the area percentage of collagen-rich tissue was higher in the injured palatal muscles (46.7 ± 6.9%) than in nonwounded controls (15.9 ± 1.0%, p < 0.05). Myofibroblasts were present in the injured muscles at days 3 and 7 only. The numbers of proliferating and differentiating myoblasts within the wound area were greater at day 7 (p < 0.05), but only a few new myofibers had formed by 56 days. No age effects were found. The results indicate that surgical wounding of the soft palate results in muscle fibrosis. Although activated satellite cells migrated into the wound area, no new myofibers formed. Thus, regeneration and function of the soft palate muscles after injury may be improved by regenerative medicine approaches.

Evaluation of the levator veli palatini muscle thickness in patients with velocardiofacial syndrome using magnetic resonance imaging.

Velocardiofacial syndrome (VCFS) is associated with velopharyngeal insufficiency, which occurs in approximately 75% of VCFS patients. Surgical management of velopharyngeal insufficiency in VCFS patients is difficult with a high revision rate due to the anatomic and physiological abnormalities of the velopharynx. The aims of this study were to evaluate the thickness and symmetry of the levator veli palatini (LVP) muscle using magnetic resonance imaging (MRI), and to compare the findings in VCFS patients to those in patients with nonsyndromic submucous cleft palate. We conducted a prospective analysis of 17 VCFS patients (nine boys, eight girls; age range, 4-9 years) and nine patients with submucous cleft palate without VCFS (eight boys, one girl; age range, 4-13 years) who had undergone MRI between March 2009 and August 2013. The thickness of the LVP muscle was measured at six locations in both groups. The symmetry was determined by comparing the values between the average of the left three points and the right three points. The mean LVP muscle thickness was significantly thinner in VCFS patients (2.14 ± 0.73 mm) than in patients without VCFS (3.70 ± 1.08 mm) (p < 0.001). In addition, the difference between the left and right sides of muscle thickness in the VCFS group was larger than that in the nonsyndromic submucous cleft palate group (0.25 and 0.09 mm, respectively). The thinness and asymmetry of the LVP muscle should be considered when determining the surgical management of velopharyngeal insufficiency in VCFS patients.

Limited palatal muscle resection with tonsillectomy: a novel palatopharyngoplasty technique for obstructive sleep apnea.

OBJECTIVE: The ideal palatal surgery for obstructive sleep apnea (OSA) and snoring must maintain the airway patency and correct anatomic abnormalities without complications. The purpose of this study was to investigate the efficacy of limited palatal muscle resection (LPMR) to improve OSA severity. SUBJECTS AND METHODS: Twenty-three patients with OSA underwent LPMR. The LPMR was initiated with a bilateral tonsillectomy in patients with tonsil size 2 and 3. The LPMR consisted of partial resection of palatal muscles (levator veli palatini, palatoglossus, and musculus uvulae) with preservation of the uvula and a simple double layer suturing. The retropalatal space and the length of soft palate were evaluated by magnetic resonance imaging. Subjective outcomes using visual analog scales, Epworth Sleepiness Scale, and overnight polysomnography (PSG) data were assessed. RESULTS: Six months after the operation, there was significant symptomatic improvement in snoring, morning headaches, tiredness, and daytime sleepiness. Postoperative magnetic resonance images showed upward and forward movement of uvula and soft palate after LPMR. The length of the soft palate was significantly shortened and the retropalatal space was significantly increased. Postoperative PSG revealed significant improvement in apnea-hypopnea index (AHI) and the total sleep time spent with oxygen saturation below 90%, and reduction in AHI following PMR was found in all patients. Furthermore, no patient experienced velopharyngeal insufficiency, voice changes, and pharyngeal dryness at 6 months follow-up. CONCLUSIONS: The LPMR obtained significant improvement in subjective and objective outcomes in OSA, with preserved pharyngeal function. PMR is an effective and safe technique to treat oropharyngeal obstruction in OSA surgery.

Idiopathic cranial polyneuropathy with unilateral IX and X and contralateral XI nerve palsy in a 4-year-old boy.

A 4-year-old boy presented with a sudden onset of nasal escape of fluids, nasal speech, and difficulty placing his left arm through a sleeve. Neurologic examination indicated a unilateral cranial IX and X and contralateral XI nerve palsy that was considered idiopathic. Palsy of cranial nerves IX, X, and XI is rare in childhood, and few reports have described this condition. Our patient received prednisolone for 1 week and demonstrated complete recovery within several weeks. We suggest that aggressive therapy is unnecessary for patients with idiopathic cranial polyneuropathy. The pathogenesis of this condition may involve an immunologic mechanism.

A review of the evaluation and management of velopharyngeal insufficiency in children.

This article highlights the most common causes of velopharyngeal insufficiency (VPI), and discusses routine evaluation and treatment algorithms for the management of VPI in children. VPI is a multifactorial condition that occurs commonly in syndromic and non-syndromic children. The most common features of VPI are audible hypernasal speech, facial grimacing, decreased speech intelligibility, nasal regurgitation, and nasal emission from failure to produce oronasal separation. Work-up of VPI typically involves radiologic and endoscopic testing performed with the assistance of a speech-language pathologist. Management of VPI involves initial speech therapy followed by operative repair with sphincter or pharyngeal flap pharyngoplasty, if needed.

Abnormal mitochondria organization and oxidative activity in the palate muscles of long-term snorers with obstructive sleep apnea.

BACKGROUND: Histopathological alterations and a reduced number of capillaries have been observed in the palate muscles of snorers with obstructive sleep apnea syndrome (OSAS). These changes may create a substrate for decreased microcirculation, impaired aerobic metabolism and muscle dysfunction and contribute to upper airway obstruction during sleep. OBJECTIVES: The aim was to analyze mitochondria distribution and oxidative enzyme activity in relation to capillary supply in the palate muscles of patients with a history of long-term snoring and OSAS. METHODS: Palatopharyngeus (PP) and uvula (UV) muscle samples were obtained from 8 patients undergoing uvulopalatopharyngoplasty due to habitual snoring and OSAS. The muscles were analyzed with enzyme- and immunohistochemistry and morphometry. RESULTS: Abnormalities in the internal organization of mitochondria and oxidative activity were observed in 39 ± 15% of the fibers in the PP and 4 ± 3% in the UV, but not in control samples. The majority of these fibers had a lobulated contour and trabecular internal organization of mitochondria. The number of capillaries around abnormal fibers (PP 0.9 ± 0.3, UV 0.4 ± 0.1) was lower than in fibers of a normal appearance in both patients (PP 1.4 ± 0.6, UV 1.2 ± 0.3) and references (PP 2.7 ± 0.7, UV 1.9 ± 0.9) (p < 0.05). CONCLUSIONS: Abnormal mitochondrial distribution, a low capillary supply and signs of impaired oxidative activity suggest that muscle dysfunction of the palate muscles in long-term snorers may contribute to the upper airway obstruction during sleep. The cause of these abnormalities remains unclear, but local muscle and nerve trauma due to vibration and stretch is a possible etiology.

Use of rotation flap in repair of cleft palate and velopharyngeal insufficiency.

Although cleft palate anomaly is frequent, the criterion standards in surgical treatment have not been determined yet. There are a few techniques described for cleft palate repair owing to the limited tissue in the palatal mucosa, the rigid structure of the palatal mucosa, and the limited vascularity of the hard palate. In this study, a novel cleft palate repair technique based on separating the soft palate from the hard palate as a musculomucosal flap and using it as a rotation flap has been described. The operation is evaluated individually for each anomaly because variations occur in the surgical technique according to the extension of the cleft toward the teeth in the palate. This operation was performed on a total of 28 patients (17 girls and 11 boys) aged between 1.5 and 16 years and presented to our clinic. Patients were assessed for speech analysis outcomes, tympanogram values, hearing functions, magnitude of palatal lengthening during the operation, and rate of fistulae. Statistically significant differences in values of the speech analysis and the audiometric assessment were determined between before and 6 months after surgery. Complete recovery of otitis was observed 1 month after surgery without another treatment in 9 (42.8%) of 21 patients who were detected to have serous otitis media preoperatively. Tension-free closure, lower risk of fistula, good restoration of velopharyngeal functions, ability to be performed on all types of cleft palate, ability to provide a good intraoperative exposure, and being a single stage seem to be the most important advantages of this technique.

[Magnetic resonance imaging of the levator veli palatini of the cleft palate patients after operation].

OBJECTIVE: To observe the mobility of levator veli palatini muscle during speech in patients with repaired cleft palate and cleft lip. METHODS: MRI of the levator veli palatini muscle was taken during speech and breathing in three groups of patients: control group (cleft lip only, 8 cases), velopharyngeal incompetence (VPI) group (7 cases), velopharyngeal competence (VPC) group (10 cases). The length and the angle of the levator veli palatini muscle were compared. RESULTS: The changes of the length and the angle during speech were not significantly different among the three groups (P > 0.05). The ratio of length changes when speaking "a", "i", and "m" are (13.5 ± 11.7)%, (11.1 ± 10.8)%, (8.2 ± 14.3)%. Mean angle of pronouncing "a", "i", and "m" are [(43.18 ± 4.984)°, (43.08 ± 4.879)°, (39.48 ± 5.046)°]. The levator veli palatini muscle contracted progressively form "m", "i", to "a". CONCLUSIONS: The mobility of the levator veli palatini muscle in patients with repaired cleft palate and cleft lip only is basically the same.

[Analysis on the relationship between tensor veli palatini and secretory otitis media in patients with nasopharyngeal carcinoma after radiotherapy].

OBJECTIVE: The analyze the relationship between secretory otitis media (SOM) and injury of tensor veli palatini (TVP) muscle injury after radiotherapy, then to explore the pathogenesis of SOM in patients with nasopharyngeal carcinoma (NPC) after radiotherapy. METHOD: The cross section area (CSA) of TVP and medial pterygoid (MP) muscle were measured in MRI of 32 patients with NPC before and after radiotherapy, meanwhile the incidence of SOM were surveyed after radiotherapy, then to analyze the relationship between the change of TVP and the incidence of SOM after radiotherapy. RESULT: Of 48 ears without SOM before radiotherapy, 27 ears developed post-irradiation SOM, including 24 ears with TVP atrophy and 3 ears without TVP atrophy, and 21 ears had no post-irradiation SOM, including 8 ears with TVP atrophy and 13 ears without TVP atrophy. chi2 test showed significant difference (P < 0.01). It indicated that post-irradiation SOM have correlation with TVP atrophy. The more possibility of TVP atrophy occurred after long time radiotherapy. CONCLUSION: The atrophy of TVP in patients with NPC usually occurs 6 months after radiotherapy, and this may result in the post-irradiation SOM. The pathogenesis of post-irradiation SOM need further study functionally.

[Research on apoptosis changes of tensor veli palatini in patients with obstructive sleep apnea-hypopnea syndrome].

OBJECTIVE: To explore apoptosis changes of dilator muscles in the upper airway by detecting the expression of Bax, Bcl-2 in tensor veli palatini in patients with OSAHS. METHOD: The expression of Bax and Bcl-2 were detected in tensor veli palatini in 30 cases with OSAHS and 10 cases chronic tonsillitis without OSAHS by immunohistochemistry and image analytical system, and the results were analyzed. RESULT: (1) The expression levels of Bax in the OSAHS group increased significantly compared to control group (P < 0.05), but there were no significant differences of Bcl-2 expression between two groups, the ratio of Bax/Bcl-2 increased significantly (P < 0.05). (2) There were positive correlations between AHI and the expression levels of Bax (r = 0.697, P < 0.01) respectively in the test group. CONCLUSION: The results indicate that apoptosis occurred in tensor veli palatini in patients with OS AHS, and the more severity of OSAHS, the more apoptosis.

Histological changes of rat soft palate with exposure to experimental laryngopharyngeal reflux.

OBJECTIVE: The possible effects of laryngopharyngeal reflux (LPR) on laryngeal and otologic disorders have been studied in the literature. There have been no reports explaining the possible effects of LPR on the soft palate. Therefore, in this study, we investigated the histopathologic changes in the rat soft palate using an experimental model of reflux. SUBJECTS AND METHODS: Eighteen healthy 200-220-g 20-week-old Sprague-Dawley rats were used. The animals were divided into three groups according to exposure time (1, 4, and 12 week exposures), and four rats were examined as controls who had undergone sham operation. An experimental model of gastroesophageal reflux was induced under general anesthesia. After exposure, the animals were sacrificed, and their soft palates were removed. The histopathological changes in the soft palates were observed under a light microscope. RESULTS: Submucous gland hyperplasia, inflammation, subepithelial edema, vascular engorgement, muscular atrophy and dilated glandular excretory duct were compared among the groups. Submucous gland hyperplasia, subepithelial edema, inflammation, vascular engorgement, muscular atrophy and dilated glandular excretory duct were significantly different in the exposure groups compared with the control group. CONCLUSION: On the basis of histopathological evaluations, our findings suggest that reflux affects the soft palate, which suggests that these pathological changes may reflect the relationship between LPR and airway obstruction.

Magnetic resonance imaging assessment of velopharyngeal structures in Chinese children after primary palatal repair.

BACKGROUND: Velopharyngeal inadequacy (VPI) after primary palatal repair remains a persistent problem despite advanced surgical techniques. Velopharyngeal inadequacy has significant negative impacts on patients' speech intelligibility and quality. Previous studies have produced conflicting results regarding the causes of VPI, which are likely due to limitations in two-dimensional imaging techniques used as well as failure to include matched groups with repaired cleft palate and differing velopharyngeal outcome. The present study aimed to investigate the characteristics of the velopharyngeal structures, including the levator veli palatini muscles, in children with repaired cleft palate and different speech outcomes. METHODS: A prescan training was performed to help the child participants to finish scans successfully without general anesthesia. Multiple high-resolution magnetic resonance imaging scans covering the whole head and focusing on the velopharyngeal region, including the levator veli palatini muscles, were acquired in 3 matched groups of young children: children with cleft palate and VPI, children with cleft palate and adequate velopharyngeal closure, and children without cleft palate. RESULTS: There were few significant differences between the 2 groups with cleft palate, although the functional pharyngeal dimensions were slightly greater in the children with VPI. More significant differences were found between the children with cleft palate and the control subjects. CONCLUSIONS: Anatomic characteristics of the velopharyngeal structures may be slightly less advantageous for velopharyngeal closure in children with VPI after palatal repair, but these structural differences alone cannot explain VPI.