Brian C. Sommerlad

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.

The Nature of Feeding in Infants With Unrepaired Cleft Lip and/or Palate Compared With Healthy Noncleft Infants

Objective: Feeding difficulties are reported widely in infants with cleft lip and/ or palate. There is, however, a paucity of objective information about the feeding patterns of these infants. This study compared patterns of feeding in infants with unrepaired cleft lip and palate with healthy noncleft infants of a similar age. Setting: North Thames Regional Cleft Centre. The noncleft cohort was recruited from West Middlesex University Hospital, a general hospital with similar demographics. Participants: Fifty newborn infants with nonsyndromic complete unilateral cleft lip and palate or a cleft of the soft and at least two thirds of the hard palate who were referred to the North Thames Regional Cleft Centre participated. Parents of 20 randomly selected, noncleft infants agreed to participate. Main Outcome Measures: Feeding patterns were rated using the Neonatal Oral Motor Assessment Scale. Additional objective information was collected using the Great Ormond Street Measurement of Infant Feeding (Masarei et al., 2001; Masarei, 2003). Results: Infants with nonsyndromic complete unilateral cleft lip and palate or a cleft of the soft and at least two thirds of the hard palate had less efficient sucking patterns than their noncleft peers had. They used shorter sucks (mean difference, 0.30 second; p < .0005), a faster rate of sucking (mean difference, 34.20 sucks/second; p < .0005), higher suck-swallow ratios (mean difference, 1.87 sucks/swallow; p < .0005), and a greater proportion of intraoral positive pressure generation (mean difference, 45.97% positive pressure; p < .0005). Conclusions: This study demonstrated that the sucking patterns of infants with nonsyndromic complete unilateral cleft lip and palate or a cleft of the soft and at least two thirds of the hard palate differ from those of their noncleft peers.

Cleft palate re-repair—a clinical and radiographic study of 32 consecutive cases

The results of clinical and radiographic assessment of palate re-repair (by a single operator) in 32 patients are presented. This has shown that radical muscle correction as a secondary procedure (following limited or no muscle correction in primary repair) has produced measurable improvement in velar function and should be considered as the first option in many patients with velopharyngeal incompetence. The results also support the concept of muscle dissection and retropositioning in primary cleft palate repair.

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

Objective 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. Interventions 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.

Surgical management of velopharyngeal incompetence in velocardiofacial syndrome

Objective 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.

A Randomized Control Trial Investigating the Effect of Presurgical Orthopedics on Feeding in Infants With Cleft Lip and/or Palate

Objective: To investigate the controversial assertion that presurgical orthopedics (PSO) facilitate feeding in infants with cleft lip and palate. Design: Randomized control trial of 34 infants with nonsyndromic complete unilateral cleft lip and palate and 16 with cleft of the soft and at least two thirds of the hard palate. Allocation to receive presurgical orthopedics or not used minimization for parity and gender. Other aspects of care were standardized. Setting: The North Thames Regional Cleft Centre. Main Outcome Measures: Measurements were made at 3 months of age (presurgery) and at 12 months of age (postsurgery). Primary outcomes were anthropometry and oral motor skills. Objective measures of sucking also were collected at 3 months using the Great Ormond Street Measure of Infant Feeding. Twenty-one infants also had videofluoroscopic assessment. Results: At 1 year, all infants had normal oral motor skills and no clear pattern of anthropometric differences emerged. For both cleft groups, infants randomized to presurgical orthopedics were, on average, shorter. The presurgical orthopedics infants were, on average, lighter in the unilateral cleft and lip palate group, but heavier in the isolated cleft palate group. Infants with complete unilateral cleft and lip palate randomized to presurgical orthopedics had lower average body mass index (mean difference PSO-No PSO: −0.45 (95% confidence interval [−1.78, 0.88]), this trend was reversed among infants with isolated cleft palates (mean difference PSO-No PSO: 1.98 [−0.95, 4.91]). None of the differences were statistically significant at either age. Conclusions: Presurgical orthopedics did not improve feeding efficiency or general body growth within the first year in either group of infants.

The Relationship Between Arch Dimensions and the 5-Year Index in the Primary Dentition of Patients With Complete UCLP

OBJECTIVE To compare dental arch dimensions of children in the primary dentition with repaired unilateral clefts of the lip and palate (UCLP) to a noncleft group of a similar age and determine how the dimensions of the cleft arches relate to an index of treatment outcome. METHOD Dental study casts of 44 5- to 6-year-olds with complete UCLP (22 boys and 22 girls) from a single center, whose primary surgery had been carried out by one surgeon, were matched for age, sex, and ethnicity with dental study casts from a longitudinal growth study. Analysis of variance was used to ascertain differences in arch dimensions between the two groups. The cleft group casts were then assessed with an established index of surgical outcome, the 5-year-old index. Spearmans rank correlation coefficient was used to see how the arch dimensions of the cleft group related to the categories of the index. RESULTS AND CONCLUSIONS Maxillary arch dimensions were significantly smaller in the cleft group than in the noncleft group, irrespective of sex (p < .05). In the mandibular arch, there was no difference between the cleft and noncleft groups (p > .05). Maxillary arch dimensions of the cleft group correlated significantly with the 5-year-old index for arch length and intercanine width (p < .05) but not intermolar width (p = .842). This would suggest that the 5-year-old index is a suitable tool for assessing the outcome of treatment in the primary dentition for anteroposterior and anterior transverse arch dimensions.

Musculus uvulae and levator palati: their anatomical and functional relationship in velopharyngeal closure

The morphological relationship between the musculus uvulae and levator palati muscles and their importance in velopharyngeal closure was studied in cadavers by simulation of levator action, palate serial section and dissection, and in various subjects by nerve stimulation and blockade. These studies support the cardinal importance of the levator muscles in velopharyngeal closure. The significance of musculus uvulae activity is less clear. While lesser palatine nerve stimulation evoked a response from the musculus uvulae, a nerve block produced no detrimental effect on speech or nasendoscopic appearance in normal subjects.

Surgical Significance of Abnormal Internal Carotid Arteries in Velocardiofacial Syndrome in 43 Consecutive Hynes Pharyngoplasties

Objectives To determine: (1) the incidence of surgically significant, abnormal internal carotid arteries (ICAs) in velocardiofacial syndrome (VCFS); (2) the implications for a Hynes pharyngoplasty; (3) the reliability of preoperative investigations in detecting surgically significant abnormal ICAs. Design Prospective data collection with blind reassessment of nasendoscopy recordings. Setting Two-site, tertiary referral cleft unit. Patients Forty-three consecutive patients with VCFS who underwent a Hynes pharyngoplasty (six had a subsequent revision). Interventions Intraoral examinations, lateral videofluoroscopy, nasendoscopy when possible, and intraoperative palpation of the posterolateral pharyngeal walls. Only one patient had a magnetic resonance angiography (MRA). Main Outcome Measures Incidence of surgically significant pulsations; modifications to surgical procedure; and correlation of surgical findings with preoperative nasendoscopy and MRA. Results Five patients (11.6%) had abnormal pulsations noted at the time of the Hynes. In no patient was the decision to perform a Hynes altered as a result of abnormal pulsations. Two patients had minor adjustments to the Hynes flaps to avoid exposing/damaging the ICA. In one patient an abnormal ICA was exposed during elevation of the left Hynes flap. This was covered uneventfully by routine closure of the secondary defect. Pulsations were noted in only 3 of 24 assessable preoperative nasendoscopies. Conclusions A Hynes pharyngoplasty is not contraindicated in VCFS, even if abnormal pulsations are present. Examination and palpation of the pharyngeal walls after the patient is positioned for surgery appear to be reliable in detecting abnormal pulsations and allow accurate surgical planning. Routine vascular imaging, even in patients with pulsations on preoperative nasendoscopy is not essential and may not always be reliable, as shown by the variation in endoscopic, MRA, and intraoperative findings. This further re-emphasizes the importance of palpating the pharyngeal walls once the patient is positioned for surgery.

Increasing accuracy of antenatal ultrasound diagnosis of cleft lip with or without cleft palate, in cases referred to the North Thames London Region

To determine the accuracy of antenatal ultrasound diagnosis of cleft lip with or without cleft palate (CL ± P) and isolated cleft palate (CP).