Surgical Management of Submucous Cleft Palate by Radical Muscle Dissection Veloplasty: Speech Outcomes in Patients with 22q11.2 Deletion Syndrome.

OBJECTIVE: To evaluate the benefit of anatomical muscle dissection repair for velopharyngeal insufficiency (VPI) in patients with submucous cleft palate (SMCP) with 22q11.2 deletion syndrome. DESIGN: Retrospective blinded randomised analysis of a surgeon's management over 10 years. SETTING: The study was performed at a specialised Paediatric hospital in the United Kingdom. PATIENTS: Children with SMCP and 22q11.2 deletion syndrome. INTERVENTIONS: All participants underwent radical muscle dissection veloplasty. OUTCOMES MEASURED: Pre- and post- operative measurements included severity of anatomical defect, speech samples and lateral images which were digitised, randomised then externally and blindly analysed using validated techniques. Stata software was used to perform statistical analysis. RESULTS: 57 children with 22q11.2 deletion syndrome were included in this analysis. Intra-operatively, the majority of cases were identified as SMCP Grade I anomalies. Post-operatively, a statistically significant improvement in hypernasality, resting palate length, palate length at maximum closure, palate excursion and gap size at maximum closure was observed. Secondary surgery was performed for 59% of patients by ten years. CONCLUSION: Muscle dissection repair improves hypernasality, palate closure function and the closure gap in patients with 22q11.2 deletion syndrome. Although over 50% of patients may require further surgery, muscle dissection repair should be a first step due to its utility at a younger age, when invasive investigations are impossible, its lower morbidity, speech and language benefits or altering the plans for less obstructive secondary surgery when it lead to reduced velo-pharyngeal gap and improved palate mobility even when adequate velo-pharyngeal closure was not achieved.

Submucous Cleft Palate (SMCP): Indications and Outcomes of Radical Muscle Dissection Palatoplasty in Children Under 4 Years of Age.

To delineate the indications, referral sources and outcomes of radical muscle dissection palate repair in the first 4 years of life for patients with submucous cleft palate (SMCP).This study presents a retrospective analysis of a single surgeon's management.All children were treated at Great Ormond Street Hospital, United Kingdom.Twenty-three children with nonsyndromic SMCP were included in this study.All participants underwent radical muscle dissection repair before their fourth birthday.Parameters analyzed included: referral sources, indications for referral, extent of anatomical abnormality, and postoperative outcomes.Paediatricians provided the largest proportion of referrals to the cleft lip and palate clinic (39%) due to the presence of cleft lip/palate on prenatal scans or during neonatal examination. Over half (n = 12, 52%) of patients had severe anatomical anomalies being in grade IV (score 8-9), with the classical triad present to some extent in all but 2 of the patients. The main indication for surgery was nasal regurgitation of food and liquid (n = 9, 35%) followed by hypernasality (n = 6, 21%), difficulty feeding (n = 3, 8%), and severe anatomical defect (n = 2; 4%). Postoperatively, the presenting complaint improved in the majority of cases.Nonsyndromic SMCP is often missed, though may present with nasal regurgitation, feeding problems, and/or hypernasality, though may be missed in nonsyndromic children. Early radical muscle dissection repair in the first 4 years of life is safe and effective, facilitating normal speech development.

International confederation for cleft lip and palate and related craniofacial anomalies task force report: palatoplasty in the speaking individual with unrepaired cleft palate.

Introduction : The benefits or otherwise of late palate repair in older children or adults are uncertain. The outcomes, particularly without appropriate speech therapy, are often disappointing. The issue is of special importance in the poorer countries where these patients are most commonly seen and where limited capacity and facilities may have to be rationed. Method : A task force was set up to report back to the International Congress in Orlando in May 2013. The chairman and some members were nominated by the organizers and further members were added during the discussion process. Some of the members had considerable experience of late palate repair. The task force compiled a report after 9 months of e-mail correspondence. The report includes reports of some previously unpublished studies. A summary of the report was presented at Cleft 2013 in Orlando. Conclusions : There was a general consensus that late palate repair is of benefit for many patients and that, even if normal speech is not attained, outcomes are positive. Outcomes depend on the age of the patient (the younger the better), on the skill of the surgeon and, ideally, on the availability of appropriate speech therapy. A protocol for a prospective international multi-center study is proposed.

Effect of palate re-repairs and hynes pharyngoplasties on pediatric airways: an analysis of preoperative and postoperative cardiorespiratory sleep studies.

OBJECTIVES : To determine whether Sommerlad palate re-repairs and Hynes pharyngoplasties cause obstructive sleep apnea/hypopnea or increased upper airway resistance. DESIGN : Prospective before-and-after study. SETTING : Tertiary cleft unit. PATIENTS : A total of 44 patients undergoing a Sommerlad palate re-repair or a Hynes pharyngoplasty. Interventions : Preoperative and postoperative multichannel cardiorespiratory sleep studies. MAIN OUTCOME MEASURES : The main outcome measures were mean arterial oxygen saturation, desaturation index, percentage of time spent with arterial oxygen saturation <90%, mean pulse rate, number of pulse rate rises (arousals) per hour, inspiratory effort derived from pulse transit time, pulse transit time arousals, and snoring. RESULTS : No patient in either group required intervention for airway obstruction or obstructive sleep apnea/hypopnea. Re-repairs caused no significant change in any parameter. Hynes caused an increase in inspiratory effort (P = .04) and obstructive sleep apnea/hypopnea grading (P = .002). All other parameters showed no significant deterioration. No patient developed more than mild/moderate obstructive sleep apnea/hypopnea. Snoring and arterial oxygen saturation levels were not reliable indicators of increased inspiratory effort. CONCLUSIONS : A palate re-repair had no significant adverse effect on the airway. A Hynes, in patients with optimized velar function, caused a significant increase in inspiratory effort and obstructive sleep apnea/hypopnea grade. However, compared with studies on midline flaps and on sphincter pharyngoplasties, a Hynes appears to be less obstructive. Failure to study changes in inspiratory effort in patients undergoing velopharyngeal incompetence surgery may underestimate the obstructive effect of pharyngoplasties.

Cleft lip, cleft palate, and velopharyngeal insufficiency.

This article provides an introduction to the anatomical and clinical features of the primary deformities associated with unilateral cleft lip-cleft palate, bilateral cleft lip-cleft palate, and cleft palate. The diagnosis and management of secondary velopharyngeal insufficiency are discussed. The accompanying videos demonstrate the features of the cleft lip nasal deformities and reliable surgical techniques for unilateral cleft lip repair, bilateral cleft lip repair, and radical intravelar veloplasty.

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.

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.

The use of the operating microscope for cleft palate repair and pharyngoplasty.

Since 1991, the author has used the operating microscope for all palate and pharyngeal operations. There are benefits for the surgeon, who operates in a more comfortable position with reliable lighting and variable magnification; for trainees and operating room staff, who have the same view as the surgeon, either through a teaching arm or on a video screen; and, it is hoped, for the patient, as the anatomy is more clearly displayed and potentially, therefore, more accurately reconstructed.

A technique for cleft palate repair.

The author has developed a technique of palate repair that combines minimal hard palate dissection with radical retropositioning of the velar musculature and tensor tenotomy. The repair is performed under the operating microscope. Results are reported for 442 primary palate repairs performed between 1978 and 1992 inclusive, with follow-up of at least 10 years. In 80 percent of these palate repairs, repair was carried out through incisions at the margins of the cleft and without any mucoperiosteal flap elevation or lateral incisions. Secondary velopharyngeal rates have decreased from 10.2 to 4.9 to 4.6 percent in successive 5-year periods within this 15-year period. Evidence from independent assessment of speech results in palate re-repair and submucous cleft palate repair suggests that this more radical muscle dissection improves velar function.

Unilateral Moore pharyngoplasty in the treatment of unilateral or asymmetric velopharyngeal incompetence.

OBJECTIVE: To describe the use of a unilateral modification of the Moore pharyngoplasty in the treatment of unilateral or asymmetric velopharyngeal incompetence (VPI) and analyze the results in a consecutive series of patients operated on by a single surgeon. DESIGN: Blind assessment of randomized speech and nasendoscopy recordings. SETTING: A two-site tertiary referral cleft unit. PATIENTS: Eighteen consecutive patients with asymmetrical or unilateral VPI of varying etiology. INTERVENTIONS: A unilateral Moore pharyngoplasty was performed in all patients. Three patients underwent radical dissection and retropositioning of the velar muscles at the same time as the unilateral Moore pharyngoplasty. MAIN OUTCOME MEASURES: Pre- and postoperative nasality and nasal airflow using the CAPS score, assessment of nasendoscopy recordings, and the rate of further surgery. RESULTS: There was a significant improvement in hypernasality (p =.014). There was a highly significant decrease in the size of the velopharyngeal gap on the side on which the Moore pharyngoplasty was performed (p =.004) as well as a highly significant decrease in the total gap size (p =.003). The Moore flap was effective in obliterating the lateral pharyngeal recess in 11 of 12 patients (p =.004). Three patients required further velopharyngeal surgery. CONCLUSIONS: In appropriately selected patients, a unilateral Moore pharyngoplasty is a safe and effective treatment for unilateral or asymmetric VPI. If indicated, a radical dissection and retropositioning of the velar muscles may be combined with a Moore pharyngoplasty.

Submucous cleft palates presenting with a perforation.

OBJECTIVE: The development of a perforation in a submucous cleft palate (SMCP) is a rare occurrence, with only a few cases reported in the literature. We describe and illustrate four cases of SMCPs with a perforation in the palate. RESULTS AND CONCLUSIONS: Recognition of the symptoms and signs of an SMCP should enable early diagnosis and treatment of this condition in symptomatic patients. However, in patients in whom the diagnosis has been missed or in those who have been asymptomatic, the development of a perforation may be the presenting feature of an SMCP. A history of possible trauma to the palate was found in two cases. Relatively minor trauma may cause perforation of the thin translucent central mucosa in an SMCP. In neonates, perforations surrounded by very thin mucosa may increase in size. The perforation can be closed at the same time as the SMCP repair and does not significantly alter the surgical treatment of an SMCP.

Gross unilateral abnormalities of the velum and pharynx.

OBJECTIVE: Velopharyngeal incompetence because of gross unilateral velopharyngeal hypoplasia is rare, particularly in patients with no significant hemifacial microsomia or facial asymmetry. We describe the abnormal anatomy and treatment of three patients with gross congenital velopharyngeal asymmetry. RESULTS AND CONCLUSIONS: Following surgery, all three patients showed a good improvement in velopharyngeal function and speech. Nasendoscopy and lateral videofluoroscopy were important in confirming the abnormal anatomy and pathology and in evaluating the degree of movement of the affected side of the velum and pharyngeal walls as well as the size and location of the defect. Muscles from the normal side were radically dissected and mobilized across the midline to reconstruct the hypoplastic hemivelum. Mucosal lengthening was achieved by suturing the normal mucosa to the mucosa of the hypoplastic hemivelum and the lateral pharyngeal wall or by insetting a posterolateral pharyngeal flap (modified Moore pharyngoplasty) into the nasal surface of the hypoplastic side.

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.