1/47. 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.- - - - - - - - - - ranking = 1keywords = cleft (Clic here for more details about this article) |
2/47. 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.- - - - - - - - - - ranking = 91.882523927038keywords = cleft palate, palate, cleft (Clic here for more details about this article) |
3/47. 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.- - - - - - - - - - ranking = 21.636532592418keywords = palate (Clic here for more details about this article) |
4/47. Specific language impairment in children with velocardiofacial syndrome: four case studies.OBJECTIVE: To describe specific language impairment in four children with velocardiofacial syndrome (VCFS). DESIGN: A descriptive, retrospective study of four cases. SETTING: University Hospital Groningen, tertiary clinical care. patients: Of 350 patients with cleft plate, 18 children were diagnosed with VCFS. Four children are described. Interventions: In all children, cardiac and plastic surgery was carried out in the first year of life. Afterward, interventions consisted of hearing improvement, pharyngoplasty, and speech therapy. MAIN OUTCOME: Inadequate and uncharacteristic development of articulation and expressive language in four children with VCFS were observed. They differed from the majority in two ways: their nonverbal IQ was in the normal range, and their language skills were below expectations for their IQ. RESULTS: Four of 18 patients with VCFS (22%) showed poor response to therapy and did not develop language in accordance with their normal learning abilities (nonverbal learning capacities and language comprehension). Persistent hypernasal resonance and severe articulation problems remained in all four children. In two children the expressive language profile was also not in agreement with the nonverbal profile: they produced only two- and three-word utterances at the age of 6.0 and 5.3 years. The other two children at the age of 6.8 and 6.4 years produced very long sentences, but they were unintelligible. CONCLUSIONS: The speech and language impairment of the four children may be characterized as a phonological or verbal programming deficit syndrome and as such can be described as a specific language impairment in conjunction with VCFS.- - - - - - - - - - ranking = 1keywords = cleft (Clic here for more details about this article) |
5/47. Avoiding perils and pitfalls in velocardiofacial syndrome: an otolaryngologist's perspective.Velocardiofacial syndrome is classically characterized by clefting of the secondary palate, cardiac defects, learning disabilities, and facial dysmorphism. knowledge of this syndrome is of significant importance to otolaryngologists because a failure to recognize it prior to head and neck surgery can result in serious iatrogenic injury, including velopalatal insufficiency and damage to anomalous carotid arteries. To illustrate these issues, we describe the case of a 5-year-old boy with velocardiofacial syndrome. We also review the literature on velocardiofacial syndrome, which is not very extensive, perhaps because it is often difficult to recognize.- - - - - - - - - - ranking = 4.0909332274883keywords = palate, cleft (Clic here for more details about this article) |
6/47. Complete denture with a removable palatal lift prosthesis: a case report and clinical evaluation.The palatal lift prosthesis is used to alleviate rhinopharyngeal closure dysfunction. It is generally problematical to use in edentulous patients, because the palatal lift prosthesis requires secure retention of the denture base and is difficult for the patient to retain while eating. This article describes the fabrication of a complete denture with a removable palatal lift prosthesis and a clinical evaluation of the denture's stability. A sprue pin and tube were used as a connecting attachment between the removable palatal lift prosthesis and the denture base. The force required for lifting the soft palate and the denture's stability were measured in the clinical evaluation. The prosthesis required an average retentive force of 0.26 N for each 1 mm of soft palate lift. The force required to dislodge the denture was measured with and without the palatal lift prosthesis in place. The force required to dislodge the conventional complete dentures of five healthy individuals was also measured as a reference. The denture was 24.4% less stable when the palatal lift prosthesis was in place and was generally easier to dislodge than were conventional dentures. This denture with a removable palatal lift prosthesis is useful for patients with dysfunction of the soft palate involving hypernasal speech who have difficulty in retaining the prosthesis while eating.- - - - - - - - - - ranking = 9.2727996824648keywords = palate (Clic here for more details about this article) |
7/47. cleft palate with congenital midline teratoma.A 4-year-old girl presented with hypernasal speech. On examination, in addition to velopharyngeal incompetence noted by speech examination, a palatal tumor was found in between the cleft palate. The computed tomographic and magnetic resonance imaging examinations were suspicious for midline teratoma. Total excision of the nasopalatal tumor was performed, and the pathology revealed benign teratoma. After 6 months of follow-up, no recurrence was noted. A two-flap palatoplasty with a superior-based pharyngeal flap was then performed to reconstruct the palatal defect and to correct the velopharyngeal incompetence. An anterior oronasal fistula developed after the operation, but a tongue flap was transferred to cover the defect successfully. The purpose of this case report is to present the relationship between a congenital midline nasopalatal tumor and cleft palate.- - - - - - - - - - ranking = 73.618748861312keywords = cleft palate, palate, cleft (Clic here for more details about this article) |
8/47. Palatal lift prosthesis for palatopharyngeal closure in Wilson's disease.This is a report of our work on a female patient with Wilson's disease who was treated for speech improvement. A palatal lift prosthesis was constructed to displace the soft palate superiorly and posteriorly and partially restore the functional deficiencies of palatopharyngeal insufficiency. Cephalometric radiographs confirm the association between the displacement of the soft palate and speech improvement.- - - - - - - - - - ranking = 6.1818664549765keywords = palate (Clic here for more details about this article) |
9/47. A new operation for velopharyngeal insufficiency: the palatoglossus myomucosal pharyngoplasty.Myomucosal flaps employing the palatoglossi were used to correct posttonsillectomy velopharyngeal insufficiency because the palatopharyngeus were resected with the posterior tonsillar pillar. This new sphincter pharyngoplasty may have a role as a secondary option for treatment of velopharyngeal insufficiency. It should be remembered that this operation has only been performed in a single patient without cleft palate, and, therefore, its application in the cleft population and its potential complication rate are unknown.- - - - - - - - - - ranking = 31.627507975679keywords = cleft palate, palate, cleft (Clic here for more details about this article) |
10/47. Alternative impression technique for a speech-aid prosthesis.OBJECTIVE: Prosthetic treatment for speech disorders attributable to surgically acquired soft palate defects are introduced. CASE: A patient who underwent soft palate resection for cancer is presented. The resected portion of the soft palate was confined to the posterior segments. A prosthesis with a speech bulb was adapted to the patient. CONCLUSION: Excellent restoration of speech and improvement of velopharyngeal function was achieved following placement of the special prosthesis.- - - - - - - - - - ranking = 9.2727996824648keywords = palate (Clic here for more details about this article) |
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