Velopharyngeal Incompetence in Children With 22q11.2 Deletion Syndrome: Velar and Pharyngeal Dimensions.

ABSTRACT: The surgical management of velopharyngeal incompetence (VPI) in children with 22q11.2 deletion syndrome (22q11.2 DS) is challenging. There are numerous approaches and children often undergo more than one operation. Our aim was to develop a method using images from routine lateral videofluoroscopy to study the dimensions of the velopharynx in this cohort.We analyzed 22 pre-operative lateral videofluoroscopy recordings of children with 22q11.2 DS and VPI. Fourteen had a submucous cleft palate (SMCP) and 8 had no obvious palatal abnormality but who were subsequently labelled as having an occult submucous cleft palate (OSMCP). The control data were 10 historic records of children with cleft lip and an intact palate. The authors identified key points on radiographs of the velum at rest and when elevated to measure the total velar length, functional velar length and pharyngeal depth and compared them ratiometrically.The intra-observer reliability was > 0.9 whereas the inter-observer reliability was > 0.74. The velopharyngeal depth/total velar length was significantly greater in 22q11.2 DS than the control group P < 0.001. There was no difference between SMCP and OSMCP patients, P = 0.556. There was no difference in the functional velar length/total velar length between 22q11.2 DS and controls (P = 0.763).In this study, the authors demonstrate a reliable method to gain useful ratiometric measurements of the velopharynx. This may help with treatment planning. Children with 22q11.2 DS and VPI have a larger velopharyngeal depth/total velar length ratio that may explain some of the difficulty in management.

Surgical management of velopharyngeal incompetence in velocardiofacial syndrome.

OBJECTIVES: To analyze the results of surgery for velopharyngeal incompetence (VPI) in velocardiofacial syndrome. DESIGN: Prospective data collection, with randomized, blind assessment of speech and velopharyngeal function on lateral videofluoroscopy and nasendoscopy. SETTING: Two-site, tertiary referral cleft unit. PATIENTS: Forty-two consecutive patients with the 22q11 deletion underwent surgery for symptomatic VPI by a single surgeon. INTERVENTIONS: Intraoral examinations, lateral videofluoroscopy (+/- nasendoscopy) and intraoperative evaluation of the position of the velar muscles through the operating microscope. Based on these findings, either a radical dissection and retropositioning of the velar muscles (submucous cleft palate [SMCP repair]) or a Hynes pharyngoplasty (posterior pharyngeal wall augmentation pharyngoplasty) was performed. As anticipated, a proportion of patients undergoing SMCP repair subsequently required a Hynes. The aim of this staged approach was to maximize velar function, thereby enabling a less obstructive pharyngoplasty to be performed. Thus, there were three surgical groups for analysis: SMCP alone, Hynes alone, and SMCP+Hynes. MAIN OUTCOME MEASURES: Blind perceptual rating of resonance and nasal airflow; blind assessment of velopharyngeal function on lateral videofluoroscopy and nasendoscopy; and identification of predictive factors. RESULTS: Significant improvement in hypernasality in all three groups. The SMCP+Hynes group also showed significant improvement in nasal emission. There were significant improvements in the extended and resting velar lengths following SMCP repair and a trend toward increased velocity of closure. CONCLUSIONS: Depending on velopharyngeal anatomy and function, there is a role for SMCP repair, Hynes pharyngoplasty, and a staged combination of SMCP+Hynes, all of which are procedures with a low morbidity.

Submucous cleft palate: a grading system and review of 40 consecutive submucous cleft palate repairs.

OBJECTIVES: This study was designed to determine whether velar surgery was worthwhile for submucous cleft palate (SMCP) and evaluate whether results were dependent on the degree of the anatomical abnormality. DESIGN: A prospective study of a consecutive series of patients fulfilling the entry criteria, assessed blindly from records arranged randomly. PATIENTS: Fifty-eight patients diagnosed with SMCP and operated on by a single surgeon between June 1991 and April 1997 were reviewed. Forty patients fulfilled the entry criteria. Minimum follow-up was 6 years. INTERVENTION: Radical reconstruction of the soft palate musculature was performed by one surgeon using the operating microscope. A scoring system was devised for grading the anatomical severity of submucous cleft (SMCP score). MAIN OUTCOME MEASURES: Postoperative hypernasality and nasal emission scores and the degrees of improvement were considered the primary outcome measures, and the degree of velopharyngeal closure was also assessed. RESULTS: There were highly significant improvements in hypernasality, nasal emission, and velopharyngeal closure. A preoperative gap size of more than 13 mm was associated with less satisfactory outcomes, but gap size was not predictive of improvement. Severity of the SMCP did not correlate with the degree of preoperative speech abnormality but was a significant predictor of outcome of surgery, with the less severe (total SMCP score of 0 to 3) having less satisfactory end results and lesser degrees of improvement. Patients with less abnormal muscle anatomy had lesser degrees of improvement. CONCLUSION: Repair of the muscle abnormality in SMCP is recommended as the first line of treatment in most cases.

Palate re-repair revisited.

OBJECTIVE: To analyze the results of a consecutive series of palate re-repairs performed using the operating microscope and identify predictive factors for outcome. DESIGN: Prospective data collection, with blind assessment of randomized recordings of speech and velar function on lateral videofluoroscopy and nasendoscopy. PATIENTS: One hundred twenty-nine consecutive patients with previously repaired cleft palates and symptomatic velopharyngeal incompetence (VPI) and evidence of anterior insertion of the levator veli palatini underwent palate re-repairs by a single surgeon from 1992 to 1998. Syndromic patients, those who had significant additional surgical procedures at the time of re-repair (23 patients), and all patients with inadequate pre- or postoperative speech recordings were excluded, leaving a total of 85 patients in the study. INTERVENTIONS: Palate re-repairs, with radical dissection and retropositioning of the velar muscles, were performed using the operating microscope with intraoperative grading of anatomical and surgical findings. MAIN OUTCOME MEASURES: Pre- and postoperative perceptual speech assessments using the Cleft Audit Protocol for Speech (CAPS) score, measurement of velar function on lateral videofluoroscopy, and assessment of nasendoscopy recordings. RESULTS: There were significant improvements in hypernasality, nasal emission, and nasal turbulence and measures of velar function on lateral videofluoroscopy, with improvement in the closure ratio, velopharyngeal gap at closure, velar excursion, velar movement angle, and velar velocity. CONCLUSIONS: Palate re-repair has been shown to be effective in treating VPI following cleft palate repair, both in patients who have not had an intravelar veloplasty and those who have had a previous attempt at muscle dissection and retropositioning. Palate re-repair has a lower morbidity and is more physiological than a pharyngoplasty or pharyngeal flap.