The use of magnetic resonance angiography prior to pharyngeal flap surgery in patients with velocardiofacial syndrome.

Twenty consecutive patients with velocardiofacial syndrome underwent magnetic resonance angiography (MRA) to determine if abnormalities of the neck arteries would contraindicate pharyngeal flap surgery. All 20 patients were found to have anomalies to the carotid arteries, vertebral arteries, medially placed internal carotids, low carotid bifurcations, and tortuous or kinked internal carotids. The internal carotids were found to be almost directly under the mucous membrane of the pharynx in two patients. In these two patients, the arteries were close to the pharyngeal midline at the base of the first cervical vertebra and might easily be severed during the raising of a pharyngeal flap. Hypoplastic vertebral arteries also were found. One patient had an extra neck vessel. The anomalies of the internal carotids did not have a strong correlation with endoscopically observed pulsations in the position affected the location of the internal carotids did not have a strong posterior pharyngeal wall. It also was found that head position affected the location of the internal carotid arteries when they were located close to the pharyngeal mucous membrane. The information provided in the MRA studies allowed assessment of the arterial anomalies in relation to the flap donor site so that the patients in the sample who underwent pharyngeal flap surgery using a short superiorly based flap had no major bleeding complications.

Treatment of asymmetric velopharyngeal insufficiency with skewed pharyngeal flap.

Twenty-two patients, with hypernasal speech and asymmetric velopharyngeal insufficiency (VPI) identified preoperatively by multi-view video-fluoroscopy and nasopharyngoscopy, were managed with superiorly based pharyngeal flaps skewed to the side with reduced lateral pharyngeal wall movement. Patient age ranged from 5 to 58 years. The etiology of the VPI included cleft palate with or without cleft lip, neurogenic VPI, velocardiofacial syndrome, tumor resection or iatrogenic causes, submucous cleft palate, neurofibromatosis, and hemifacial microsomia. Follow-up, at 1 year and thereafter, showed resolution of VPI in all but two patients. An auxiliary flap to augment the primary flap was added on the side of diminished lateral pharyngeal wall motion which corrected the residual VPI. Three patients developed hyponasality. One was a child whose symptoms improved with time and growth. Two were adults, but the hyponasal resonance was mild and required no further intervention. The advantage of skewing flaps is that at least one port functions adequately for ease in respiration and for drainage of secretions, thus reducing the risk of nasal obstruction. One open port also allows access for nasoendotracheal intubation should anesthetic be required for future operations.

Effect of glossopexy on speech sound production in Robin sequence.

The management of Robin sequence may include glossopexy, in which the tongue is attached to the lower lip and mandible for anterior lingual positioning to resolve upper airway obstruction. The procedure is performed early in infancy and reversed at the time of palatal repair near 1 year of age. This period is critical to prespeech vocalization. The effect of glossopexy on speech sound production was investigated in 17 children with Robin sequence who had glossopexies. Analysis suggested that glossopexy may temporarily affect the development of prespeech skills and sound production. The onset of babbling and initiation of first words was often delayed in the subjects in comparison with syndrome-matched children who had not undergone glossopexy. Tongue-tip sounds were often produced with the tongue blade, but production was perceptually normal. The early delays were temporary and the only long-term effect of glossopexy was a tendency to produce tongue-tip sounds with the tongue blade.

Pharyngeal flap surgery in adults.

The elimination of hypernasal speech in patients with cleft palate following pharyngeal flap surgery in childhood is well established. However, pharyngeal flaps in adults have been considered to yield more modest results. This study reports on 20 adult patients with cleft palate-related hypernasality who underwent pharyngeal flap surgery. Normal nasal resonance was achieved in 15 cases, hyponasality occurred in 3 cases, and hypernasality persisted in 2 cases. However, speech intelligibility was not always dramatically improved. Indications and outcome were found to be highly dependent on preoperative articulation.

Standardization for the reporting of nasopharyngoscopy and multiview videofluoroscopy: a report from an International Working Group.

A multidisciplinary International Working Group of scientists was assembled to address the question of standardizing reporting techniques for multiview videofluoroscopy and nasopharyngoscopy, the generally accepted standards for direct observation of the velopharyngeal valve. This report is a first attempt to propose standards while seeking feedback from the readership in order to further develop a common methodology.

Evaluation of velopharyngeal insufficiency.

To summarize briefly, in our opinion, the combined use of end-viewing flexible fiber optic nasopharyngoscopy and multiview videofluoroscopy (which must include lateral and frontal views) and a behavioral assessment of the patient's speech provides the examiner with all of the information necessary to select the appropriate treatment for VPI. Indirect assessment procedures, such as airflow studies, do not enhance the decision making process even though they may provide what would appear to be objective data. Treatment decisions depend on the ability to confirm the type of speech abnormality present (by behavioral assessment) followed by a direct view of the defect (by endoscopy and fluoroscopy).

Velo-cardio-facial syndrome: language and psychological profiles.

The velo-cardio-facial syndrome (VCF) has been delineated as a relatively common recurrent-pattern malformation syndrome of clefting. As originally described, the syndrome's major features are clefting of the secondary palate, cardiac anomalies, characteristic facies, learning disabilities, and speech disorders. As characteristic of the syndrome, however, is a rather distinctive pattern of language disorders and personality characteristics that has not been previously described. The purpose of this report is to add to the phenotype of the velo-cardio-facial syndrome by providing a description of the language, academic, and psychological profiles of 26 patients. This information would prove useful in the process of differential diagnosis. Striking observations concerning changes manifest during the course of development from initial language acquisition through childhood and adolescence will also add to the information regarding this syndrome's natural history and prognosis.