Is pharyngoplasty a risk in velocardiofacial syndrome? An assessment of medially displaced carotid arteries.

The association of medially positioned internal carotid arteries and velocardiofacial (Shprintzen) syndrome was first made in 1987. This is also the most common syndrome associated with facial clefting. The potentially dangerous implications in children with this syndrome requiring pharyngoplasty for velopharyngeal incompetence and stigmatized hypernasal speech involve potential damage to these vessels. This three-part study was undertaken to analyze this anatomic variant. First, a group of 25 children with velocardiofacial syndrome, velopharyngeal incompetence, and obvious posterior pharyngeal pulsations seen on nasendoscopy was studied by CT angiography to determine the degree of this abnormal vascular pattern. This technique, together with three-dimensional reconstructions, made it possible to determine the precise location of these abnormally positioned vessels. Second, our routine superiorly based pharyngeal flap was measured by lateral cervical x-ray to show the distal tip of the flap. The variance was minimal and demonstrated the tip of most flaps to be at the disk between the C2 and C3 vertebrae. By correlating this information with the CT angiography, the risk of surgery can be determined on strict anatomic grounds, allowing customized flap design in some unilateral cases. In this series of children, routine superiorly based pharyngoplasty would be safe in 52 percent, while in 28 percent a pharyngeal flap would be safe if custom designed, and in the remaining 20 percent surgery should not be attempted because the risk of damage to the carotid arteries is too great. Third, in a double-blind study, velocardiofacial children with obvious pulsations seen on nasendoscopy were grouped with other children with palatal dysfunction. When only endoral examination was performed by plastic surgeons and plastic surgical residents, no vascular pulsations were ever seen. This indicates another important role of nasendoscopy in the preoperative assessment of children for palatopharyngoplasty.

The use of videonasopharyngoscopy for biofeedback therapy in adults after pharyngeal flap surgery.

Nasopharyngoscopy with videorecording was used as a visual feedback therapy tool to establish and promote consistent closure of the velopharyngeal ports during connected speech after pharyngeal flap surgery. Of the three adults in this report, two achieved consistent closure during connected discourse and have been discharged from therapy; the third demonstrated improved velopharyngeal closure but elected not to continue therapy. These outcomes demonstrate that nasopharyngoscopy biofeedback therapy is useful in helping the motivated adult to achieve better velopharyngeal closure during connected speech after pharyngeal flap surgery or revision. This type of therapy may also prove beneficial with children.

Comparison of multi-view videofluoroscopy and nasopharyngoscopy in the assessment of velopharyngeal insufficiency.

Ratings and drawings of multi-view videofluoroscopy and nasopharyngoscopic studies of the velopharyngeal valve in 25 subjects with velopharyngeal insufficiency (VPI) were compared. The videofluoroscopic projections assessed were lateral, base, and Towne views. The Towne view was visually compared with flexible fiber optic nasopharyngoscopic studies in the same subjects. The Towne view was found to compare well with nasopharyngoscopic examination, and lateral view alone was found to be inadequate for the description of velopharyngeal movements.

Patterns and location of velopharyngeal valving problems: atypical findings on video nasopharyngoscopy.

Patterns of velopharyngeal (VP) valving and the location of velopharyngeal gaps were investigated in 246 consecutive nasopharyngoscopy studies. The predominant pattern of closure was coronal (68 percent), followed by the circular (23 percent), circular with a Passavant's ridge (5 percent), and sagittal (4 percent) patterns. Gaps were found in 181 patients; in 121 they were considered typical (centrally located), in 28 they were in one of the lateral aspects of the valve, and in 32 they were attributed to the shape of the adenoid tissue or to abnormalities in the anatomy or function of the posterior border of the soft palate. Atypical findings occurred primarily in patients with a coronal pattern of valving. These findings raise further questions about treatment for patients with atypical valving problems.

Changes in aesthetic appearance and intelligibility of speech after partial glossectomy in patients with Down syndrome.

Eight patients with Down syndrome, aged 9 years and 10 months to 25 years and 4 months, underwent partial glossectomy. Preoperative and postoperative videotaped samples of spoken words and connected speech were randomized and rated by two groups of listeners, only one of which knew of the surgery. Aesthetic appearance of speech or visual acceptability of the patient while speaking was judged from visual information only. Judgments of speech intelligibility were made from the auditory portion of the videotapes. Acceptability and intelligibility also were judged together during audiovisual presentation. Statistical analysis revealed that speech was significantly more acceptable aesthetically after surgery. No significant difference was found in speech intelligibility preoperatively and postoperatively. Ratings did not differ significantly depending on whether the rater knew of the surgery. Analysis of results obtained in various presentation modes revealed that the aesthetics of speech did not significantly affect judgment of intelligibility. Conversely, speech acceptability was greater in the presence of higher levels of intelligibility.

The use of nasopharyngoscopy biofeedback therapy in the correction of inconsistent velopharyngeal closure.

Nasopharyngoscopy was used as a visual feedback tool in a 10-year-old girl who had a repaired bilateral cleft lip and palate and was unable to establish velopharyngeal closure during production of sibilant-fricative sounds. After one session, the patient was able to achieve velopharyngeal closure consistently during speech. Nasopharyngoscopy biofeedback therapy may be a useful tool to help children to achieve consistent velopharyngeal closure during connected discourse.

Minimal standards for reporting the results of surgery on patients with cleft lip, cleft palate, or both: a proposal.

This article proposes a set of minimal standards for reporting the results of surgery on patients with cleft lip with or without cleft palate and cleft palate only. These standards do not represent what is technically possible, only what is considered minimally acceptable for presentation in a public forum. They have a clinical focus and should be attainable by any well-constituted cleft palate team. As the title indicates, this document is a proposal. It is hoped that the readership will respond to the recommendations presented so that some set of standards can be adopted in the near future. Once adopted, these standards should be reviewed and updated periodically.

Velopharyngeal insufficiency due to hypertrophic tonsils. A report of two cases.

Two children in whom idiopathic hypernasality was diagnosed were referred for investigation of velopharyngeal function. Multiview videofluoroscopic assessments showed the tonsils prolapsing posteriorly during speech, preventing the palate from fully approximating the posterior pharyngeal wall. Tonsillectomy was recommended. Postoperative evaluations found that the hypernasal resonance was eliminated in both children.

Development of articulation before delayed hard-palate closure in children with cleft palate: a cross-sectional study.

The development of articulation before surgical closure of the hard palate was compared in 75 preschool children with cleft lip and palate and 40 preschool children born without clefts. The children were aged 2 years to 5 years 11 months. The patients had significantly poorer articulation skills than the controls at each age level. Substitutions were the most frequent error, and they did not decrease with age in the patients. Fistula size and a history of speech therapy were significant factors in the articulation error scores only in 5-year-olds. No advantage in articulation proficiency was found for those who had worn a prosthesis to occlude the hard-palate defect.

Abnormal carotid arteries in the velocardiofacial syndrome: a report of three cases.

Internal carotid arteries of unusual size and tortuosity were found before or at the time of pharyngeal flap surgery in three children who had the velocardiofacial syndrome with velopharyngeal insufficiency. In two cases, medial displacement of the arteries prevented surgery, and in the other, hypernasality persisted because only a narrow, asymmetrical flap could be raised. Medial displacement of the internal carotid arteries inhibits surgical treatment of velopharyngeal insufficiency, necessitating treatment with a prosthetic speech device in such children. Since displacement and tortuosity may be associated findings in the velocardiofacial syndrome, the exact location of the internal carotids should be ascertained when pharyngeal flap surgery is planned.

Speech intelligibility after partial glossectomy in children with Down's syndrome.

Twenty-three children with Down's syndrome, aged between 3.7 and 17.5 years, underwent partial glossectomy for improvement of cosmetic appearance. Improved speech was also expected. Preoperative and postoperative audiotaped samples of spoken words and connected speech on a standardized articulation test were rated by three lay and three expert listeners on a five-point intelligibility scale. Five subjects were eliminated from both tasks and another four from connected-speech testing because of inability to complete the experimental tasks. Statistical analyses of ratings for words in 18 subjects and connected speech in 14 of them revealed no significant difference in acoustic speech intelligibility preoperatively and postoperatively. The findings suggest that a wedge-excision partial glossectomy in children with Down's syndrome does not result in significant improvement in acoustic speech intelligibility; in some patients, however, there may be an aesthetic improvement during speech.

Velopharyngeal insufficiency after adenoidectomy: an 8-year review.

The characteristics of 137 patients referred to the Speech Clinic at The Hospital for Sick Children, Toronto, Canada during the past 8 years for the investigation of persistent hypernasality after adenoidectomy are reported. Over 30% of the patients had preoperative factors that are thought to increase the risk, such as submucous cleft palate, fluid regurgitation through the nose, a family history of velopharyngeal insufficiency or clefting, or hypernasality. Fifty percent of the patients required pharyngoplasty to correct hypernasality, 37% required speech therapy alone, and 13% improved with no treatment.

Velopharyngeal insufficiency on videofluoroscopy: comparison of projections.

A prospective study of multiview videofluoroscopy was performed on 50 patients with clinically suspected velopharyngeal insufficiency to determine which views are helpful, so that radiation dosage might be reduced. The lateral view alone was insufficient: In 11 patients, velopharyngeal insufficiency was demonstrated on the basal or Towne view when the lateral view appeared normal. The Towne view demonstrated the velopharyngeal portal better than the basal projection when the adenoids were large. The basal view should be reserved for the few cases in which the Towne and lateral projections give insufficient information.

Delayed hard palate closure: the philosophy revisited.

Delaying surgical closure of the hard palate in children with cleft lip and palate has become increasingly popular. This essay is a review of the rationale and supporting evidence for this procedure, with emphasis on its effect on speech, particularly articulation and velopharyngeal function. We have concluded that the assumptions on which this method is based have never been proven, and that the deleterious effects on speech often noted have not received appropriate attention.

The physiologic basis for hypernasality during connected speech in cleft palate patients: a nasendoscopic study.

A characteristic pattern of velopharyngeal movement is associated with hypernasality. Nasendoscopic examination can identify the patterns of movement of the velopharynx during connected speech. The nasendoscopic observations provide a safe, objective way of assessing the anatomic dysfunction that causes hypernasality. These observations provide a clearer rationale for the design and placement of pharyngoplasty surgery. Recognition of distinct dysfunctional patterns opens the door to nonsurgical correction of hypernaslity in selected patients using the nasendoscope and video display.