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1/98. Pharyngeal flap for velopharyngeal incompetence in patients with myotonic dystrophy.

    Velopharyngeal incompetence (VPI) has been associated with neuromuscular disorders. Only 4 patients with myotonic dystrophy (MD) who underwent pharyngeal flap elevation for VPI have been reported in the literature. In 3 patients, surgery preceded the diagnosis of MD. Cardiorespiratory complications characterized the postoperative period of 3 patients. The authors present 3 patients with VPI and an established diagnosis of MD (by molecular genetics) who underwent pharyngeal flap elevation. The operation resulted in a major improvement in speech in all patients, although some relapse was noted later in 1 patient. Contrary to previous reports, none had peri- or postoperative cardiorespiratory complications. MD, although an uncommon etiology, should be considered in cases of late-onset VPI. Owing to differences between the authors' findings and previous reports, additional studies are needed before final conclusions can be reached regarding the benefit and safety of pharyngeal flap surgery in MD patients. At present, MD should not be considered a contraindication for this procedure, although close perioperative monitoring is indicated. ( info)

2/98. Results with sphincter pharyngoplasty and pharyngeal flap.

    OBJECTIVE: To evaluate speech outcomes and complications of sphincter pharyngoplasty and pharyngeal flap performed for management of velopharyngeal insufficiency (VPI). DESIGN: Case series. SETTING: Tertiary care children's hospital. patients: All patients who underwent pharyngeal flap or sphincter pharyngoplasty from 1990 to 1995. methods: Perceptual speech analysis was used to assess severity of VPI, presence of nasal air emissions and quality of nasal resonance (hyper, hypo, or normal). Pre-operative measures of velopharyngeal function were based upon nasendoscopy and videofluoroscopic speech assessment. Recommendations for management were made by the attending surgeon. Complications of hyponasality and obstructive sleep symptoms (OSS) were noted. Patient characteristics were compared using univariate analysis. RESULTS: Sixteen patients underwent sphincter pharyngoplasty and 18 patients underwent superiorly based pharyngeal flap. patients were similar in terms of lateral pharyngeal wall medial motion and palatal elevation. The groups were also similar with regard to VPI severity, though there was a trend for more severe VPI in patients undergoing sphincter pharyngoplasty than pharyngeal flap (50 vs. 33.3%, respectively). patients with pharyngoplasty had a higher rate of resolution of VPI than those who had pharyngeal flap (50 vs. 22.2%, respectively), although this was not statistically significant. Post-operative hyponasality and obstructive sleep symptoms were present in both groups. However, only patients who underwent PF and had postoperative OSS had obstructive sleep apnea (OSA). CONCLUSIONS: There were no detectable anatomic differences between treatment groups implying that treatment selection during the study period was not guided by strict anatomic criteria. Sphincter pharyngoplasty may have a higher success rate with a lower risk of OSS. ( info)

3/98. Familial deletion of 22q11.2.

    We present a mother and her son, both carrying a deletion of chromosome 22q.11.2. They manifest clinical heterogeneity. The mother has schizophrenia, an IQ of 70. tetralogy of fallot, a hypernasal voice, but does not have the characteristic facies. Her son has mild psychomotor developmental delay. tetralogy of fallot and mild facial features characteristic of VCFS. ( info)

4/98. De novo direct duplication of 15q15-->q24 in a newborn boy with mild manifestations.

    Duplication of distal 15q results in a recognizable clinical phenotype. We report here on a 25-day-old boy with a de novo interstitial duplication of chromosome region 15q15-q24. The manifestations in this patient are milder than those of previously described patients and include minor facial anomalies, velopharyngeal insufficiency, branchial cleft cyst, and hydronephrosis. fluorescence in situ hybridization (FISH) using a chromosome 15 painting probe confirmed that the extra material is of chromosome 15 origin. Further analysis with the SNRPN probe demonstrated that the duplication is telomeric to the Prader-Willi/angelman syndrome critical region. This case delineates a broader spectrum for patients with duplication 15q syndrome. ( info)

5/98. Velocardiofacial syndrome in childhood-onset schizophrenia.

    OBJECTIVES: Deletion of chromosome 22q11 (velocardiofacial syndrome) is associated with early neurodevelopmental abnormalities and with schizophrenia in adults. The rate of 22q11 deletions was examined in a series of patients with childhood-onset schizophrenia (COS), in whom early premorbid developmental and cognitive impairments are more pronounced than in adult-onset cases. METHOD: Through extensive recruiting and screening, a cohort of 47 patients was enrolled in a comprehensive study of very-early-onset schizophrenia. All were tested with fluorescence in situ hybridization for deletions on chromosome 22q11. RESULTS: Three (6.4%) of 47 patients were found to have a 22q11 deletion. All 3 COS patients with 22q11 deletions had premorbid impairments of language, motor, and social development, although their physical characteristics varied. brain magnetic resonance imaging revealed increased midbody corpus callosum area and ventricular volume in relation both to healthy controls and to other COS patients. CONCLUSIONS: The rate of 22q11 deletions in COS is higher than in the general population (0.025%, p < .001) and may be higher than reported for adult-onset schizophrenia (2.0%, p = .09). These results suggest that 22q11 deletions may be associated with an earlier age of onset of schizophrenia, possibly mediated by a more salient neurodevelopmental disruption. ( info)

6/98. 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. ( info)

7/98. Furlow palatoplasty to restore velopharyngeal competence following tonsillectomy avulsion of a pharyngeal flap.

    OBJECTIVE: We report the successful use of a Furlow palatoplasty to salvage velopharyngeal competence following iatrogenic avulsion of a pharyngeal flap that had been previously established to treat velopharyngeal insufficiency associated with a submucous cleft palate. INTERVENTION: A tonsillectomy, conducted by a surgeon unaffiliated with a cleft palate team, was used to remove enlarged tonsils that had developed after pharyngeal flap surgery and extended into the lateral ports causing nasal obstruction and hypernasality because of mechanical interference with port closure. A posttonsillectomy evaluation revealed avulsion of the pharyngeal flap, which was successfully treated using a Furlow palatoplasty. CONCLUSIONS: To our knowledge, this is the first report of iatrogenic avulsion of a pharyngeal flap caused by tonsillectomy. Based on a review of the literature and this case experience, we would conclude that tonsillectomy should not be regarded as a routine procedure in patients previously treated with a pharyngeal flap. If required, it should be performed by a skilled otolaryngologist, preferably one affiliated with a multidisciplinary cleft palate team who is familiar with pharyngoplasty surgery. Finally, our experience would suggest that the Furlow palatoplasty is sufficiently robust to be used as a secondary salvage procedure to restore velopharyngeal sufficiency following iatrogenic avulsion of a pharyngeal flap. ( info)

8/98. An endosseous, implant-retained obturator for the rehabilitation of a recurrent central giant cell granuloma: a clinical report.

    The prosthodontic rehabilitation of a patient with a recurrent maxillary CGCG has been described. The patient's immense defect originally was rehabilitated with a scapular microvascular free flap, endosseous dental implants, and an ISP that became obsolete by virtue of the resection of recurrent disease and the subsequent need for velopharyngeal obturation. The patient was provided with a surgical obturator at the time of the resection of the recurrent CGCG. The surgical obturator thereafter was modified into an interim obturator to provide velopharyngeal competence while the soft tissues around the palatal defect healed. Finally, the construction of a bar-retained definitive obturator markedly improved the patient's speech, mastication, and deglutition. ( info)

9/98. La maladie de Grisel: spontaneous atlantoaxial subluxation.

    Objective: "La maladie de Grisel" (Grisel's syndrome) is a spontaneously occurring atlantoaxial subluxation with torticollis. We present a case of atlantoaxial subluxation occurring in a 20-year period of pharyngoplasty surgery. The occurrence of a "spontaneous" atlantoaxial subluxation after oral cavity or pharynx operations is rare. Because some neck pain and stiffness are commonly seen after these kinds of operations, we would like to draw attention to this unusual complication. Symptoms associated with a torticollis after an operation in the oral cavity or pharynx requires additional investigation to exclude this rare complication. A review of the available literature concerning etiology and treatment of la maladie de Grisel is presented. ( info)

10/98. Lipoinjection augmentation of the soft palate for velopharyngeal stress incompetence.

    OBJECTIVES/HYPOTHESIS: Velopharyngeal stress incompetence in professional musicians is an uncommon but potentially career-ending problem. Pharyngeal flaps, V-Y palatal pushback procedures, Teflon or collagen injection of the posterior pharyngeal wall, and speech therapy have all been used to address this problem. The ideal procedure for this subset of patients with velopharyngeal incompetence (VPI) with high-pressure, mild VPI would be one that combines low morbidity and an expedient recovery for the busy musician. We describe an approach of endoscopically assisted autologous lipoinjection of the soft palate. STUDY DESIGN: A retrospective review of our experience treating high-pressure stress VPI in two professional musicians. methods: literature review and retrospective chart review. RESULTS: Two musicians underwent autologous lipoinjection of the soft palate for stress VPI. patients resumed full play within 2 weeks of the operation with no serious complications. There has been no recurrence of the VPI after 18 and 12 months of follow-up, respectively. CONCLUSIONS: Velopharyngeal stress incompetence in musicians is an uncommon disorder. Velopharyngeal incompetence in these patients may not present as in a typical manner with hypernasality but may go undiagnosed for years mistakenly rationalized as a declining performance ability rather than a curable structural problem. The performance demands of professional musicians necessitate a timely solution to their VPI. More precise and limited contouring of palatal bulk can be achieved through the lipoinjection technique than compared with traditional palatal V-Y pushback or a standard pharyngeal flap. Lipoinjection of the palate can be performed as an outpatient procedure with only minor discomfort and an expedient recovery for the career musician. ( info)
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