Submucous Cleft Palate Research Articles

Velopharyngeal Muscle Morphology in Children With Unrepaired Submucous Cleft Palate: An Imaging Study.

To identify quantitative and qualitative differences in the velopharyngeal musculature and surrounding structures between children with submucous cleft palate (SMCP) and velopharyngeal insufficiency (VPI) and noncleft controls with normal anatomy and normal speech. Magnetic resonance imaging was used to evaluate the velopharyngeal mechanism in 20 children between 4 and 9 years of age; 5 with unrepaired SMCP and VPI. Quantitative and qualitative measures of the velum and levator veli palatini in participants with symptomatic SMCP were compared to noncleft controls with normal velopharyngeal anatomy and normal speech. Analysis of covariance revealed that children with symptomatic SMCP demonstrated increased velar genu angle (15.6°, P = .004), decreased α angle (13.2°, P = .37), and longer (5.1 mm, P = .32) and thinner (4 mm, P = .005) levator veli palatini muscles compared to noncleft controls. Qualitative comparisons revealed discontinuity of the levator muscle through the velar midline and absence of a musculus uvulae in children with symptomatic SMCP compared to noncleft controls. The levator veli palatini muscle is longer, thinner, and discontinuous through the velar midline, and the musculus uvulae is absent in children with SMCP and VPI compared to noncleft controls. The overall velar configuration in children with SMCP and VPI is disadvantageous for achieving adequate velopharyngeal closure necessary for nonnasal speech compared to noncleft controls. These findings add to the body of literature documenting levator muscle, musculus uvulae, and velar and craniometric parameters in children with SMCP.

Speech Outcomes Comparison Between Adult Velopharyngeal Insufficiency and Patients With Unrepaired Cleft Palate.

This study compared the speech outcomes of adult velopharyngeal insufficiency patients and adult cleft palate (ACP) patients, and explored whether there was any difference in the phonological level of these 2 types of patients. Perceptual evaluation was used to assess speech intelligibility, hypernasality and compensatory articulation in 89 adult patients with velopharyngeal insufficiency and 35 adult patients with unrepaired cleft palate. Each group was divided into complete cleft palate and incomplete cleft palate (including submucous cleft palate). The phonological differences were compared between the 2 groups of patients and 2 types of cleft palate. The mean speech intelligibility was 43.04% in velopharyngeal insufficiency group and 32.87% in ACP group. There was a significant difference in speech intelligibility between the 2 groups by T test, t = 2.916 (P < 0.01), speech intelligibility between 2 types of cleft palate was no significant difference. Also, there was a significant difference between the 2 groups in the constitution of hypernasality degree by Chi-Square test, x2 = 31.650 (P < 0.01), compensatory articulation were present in 74.3% ACP patients (26/35) and 47.2% velopharyngeal insufficiency patients (42/89), x2 = 7.446 (P < 0.01), there was a significant difference in incidence of compensatory articulation between the 2 groups. Adult patients with unpaired cleft palate present an even worse speech intelligibility and hypernasality degree than velopharyngeal insufficiency patients after cleft palate repair, regardless of the cleft type. Additionally, patients in ACP group have a higher incidence of compensatory articulation than that in incomplete cleft palate group. In sequenced treatments of cleft lip and palate, evaluation and treatment of speech disorders cannot be ignored.

Preoperative velopharyngeal closure ratio correlates with Furlow palatoplasty outcome among patients with nonsyndromic submucous cleft palate

This study aimed to explore the preoperative factors that are potentially associated with the outcome of Furlow palatoplasty in treating nonsyndromic submucous cleft palate (SMCP). In this study, we reviewed patients with nonsyndromic SMCP who received Furlow palatoplasty between 2008 and 2017 at our department. A comprehensive panel of preoperative variables was included for analyses including gender, age at operation, concurrence of cleft lip, preoperative hypernasality, nasal emission, velopharyngeal closure ratio (VCR), velopharyngeal closure pattern, velum and pharyngeal wall movement, presence of Passavant Ridge and articulation error. The improvement of velopharyngeal function was considered a good outcome. Both univariate and multivariate analyses were performed to screen the potential predictors of the postoperative velopharyngeal function. Multivariate regression analyses indicated preoperative VCR as the only factor that was significantly associated with surgical outcome (p = 0.025). The receiver operating characteristic curve and Youden index indicated that VCR>52.5% was the cutoff value for predicting preferable postoperative velopharyngeal function (OR, 0.240; 95% IC, 0.059–0.979; p = 0.047). In conclusion, Furlow palatoplasty was recommended for non-syndromic SMCP patient with preoperative VCR>52.5% as the primary surgical treatment.

Submucous Cleft Palate: Evaluation and Treatment Principles for Speech-Language Pathologists

PurposeThis clinical focus article intends to provide speech-language pathologists (SLPs) with a review of the literature and practical recommendations for the evaluation and treatment of individuals with submucous cleft palate (SMCP).MethodA review of the literature focused on definition, incidence, and diagnostic recommendations for SMCP was completed. Descriptions of physical and auditory–perceptual features common during evaluation were described in detail. Guiding principles for clinical management related to surgery and/or speech therapy were provided.ResultsSeveral discrepancies in the definition, incidence, and outcomes across SMCP studies were discovered in the literature. The importance of a comprehensive perceptual evaluation, including an oral mechanism examination by an SLP trained in the assessment of individuals with craniofacial anomalies, was emphasized.ConclusionsSMCP is a less understood subtype of cleft palate that presents a unique challenge for clinicians. A trained SLP's comprehensive perceptual evaluation and thorough oral mechanism examination are critical for diagnosis and treatment. SLPs can promote best practice for this population by initiating referrals to an accredited cleft palate–craniofacial team for further evaluation of their speech and assessment of surgical candidacy.

Submucous Cleft Palate Repair in Patients With 22q11.2 Deletion Syndrome.

To determine whether surgical intervention for submucous cleft palate (SMCP) is more common in children with 22q11.2 deletion syndrome (22q DS) compared to children without 22q DS. Retrospective chart review. Tertiary pediatric hospital and 22q11.2 DS specialty clinic. One hundred forty-two children seen at the tertiary hospital or clinic during a 20-year period (June 1999-June 2019) with documented SMCP with and without 22q DS. Percentage of children with SMCP with and without 22q DS requiring surgical intervention for velopharyngeal insufficiency. Patients with 22q DS had a significantly higher frequency of SMCP repair than those without 22q DS (89.7% vs 32.0%, P < .001, χ2 = 37.75). The odds of requiring SMCP repair were 18.6 times higher in those with 22q DS compared to those without (odds ratio = 18.6, CI = 6.1-56.6). This study provides new evidence suggesting patients with 22q DS require SMCP surgical repair for velopharyngeal insufficiency at a significantly higher rate than those without 22q DS. As the majority of patients with 22q DS with SMCP require surgical intervention, future prospective studies looking at early versus late repair of SMCP in patients with 22q DS are needed to guide the surgical repair timeline in this population.

The Correlation Between Consonant Articulation and Velopharyngeal Function in Patients With Unoperated Submucous Cleft Palate.

Submucous cleft palate (SMCP) is a congenital condition related to abnormal muscle attachments within the soft palate. Even though hypernasality and nasal emission, the primary symptom of SMCP, have been well-documented, research on articulation errors is currently lacking. The object of this study was to examine the consonant articulation in patients with unoperated SMCP and its possible correlation with the velopharyngeal function. This study analyzed the perceptual speech assessment and nasopharyngoscopy data of 338 patients with unoperated SMCP between years 2008 and 2016 retrospectively. The mean age of the patients was 13.27 years. About 125 patients showed velopharyngeal competence (VPC), 174 velopharyngeal incompetence (VPI), and 39 marginal VPI (MVPI). Among the 338 patients, 137 presented normal articulation, 124 consonant omission, 25 compensatory articulation, 36 consonant weakening, 51 consonant substitution, and 36 posterior placement. About 70.40% VPC patients, 17.24% VPI patients, and 48.72% MVPI patients demonstrated normal articulation. About 59.20% VPI patients showed consonant omission. The results demonstrated that consonant articulation varied among patients with unoperated SMCP and was correlated with their velopharyngeal functions, while consonant omission was the most common articulation error. Velopharyngeal closure pattern was not associated with either velopharyngeal function or consonant articulation.

Seizures and Cardiomyopathy in a Patient with Pallister-Killian Syndrome due to Hexasomy 12p Mosaicism

Pallister-Killian syndrome (PKS) is a rare disorder presenting with developmental delay, numerous dysmorphic features, and skin pigmentation anomalies. It is caused by mosaic tetrasomy of the short arm of chromosome 12. In most instances, tetrasomy is due to a supernumerary isochromosome i(12)(p10). Although mitotic instability is a generally accepted behavior for supernumerary chromosomes, hexasomy 12p due to a gain of an isochromosome 12p, has been hardly ever reported. We report a 10 year follow-up on a girl with 2 copies of isochromosome consisting of the short arm of chromosome 12, who has craniofacial features seen in PKS, such as sparse hair with an unusual pattern, sparse eyebrows, lacrimal duct stenosis, submucous cleft palate, Pallister lip (a relatively long philtrum continuing into the vermillion border of the upper lip), narrow palate, and wide alveolar ridges. She also has other abnormalities, including unilateral renal dysgenesis, rectovaginal fistula, pre-axial polydactyly of the right hand, severe global developmental delay, and hypotonia as well as some features suggestive of mosaicism such as bilateral asymmetry, patchy areas of rough skin, and retinal mottling. Initial cytogenetic studies from peripheral blood showed a normal female karyotype. Further cytogenetic studies on a skin biopsy showed mosaicism with 2 copies of the supernumerary isochromosome 12p.

Submucous cleft palate: Therapeutic needs at different ages

Submucous cleft palate: Therapeutic needs at different ages

Increased Risk of Velopharyngeal Insufficiency in Patients Undergoing Staged Palate Repair.

To evaluate the association of 2-stage cleft palate (CP) surgery on velopharyngeal insufficiency (VPI) incidence, speech surgeries, and cleft-related surgical burden. Retrospective cohort with follow-up of 4 to 19 years. Academic, tertiary children's hospital. Patients who underwent CP surgery between 2000 and 2017. Exclusions included submucous CP or age at last contact under 3.9. Cleft palate surgery, completed in either a single-stage or 2-stage repair. Rates of VPI diagnosis and speech surgery and total cleft surgeries; t tests, tests of proportion, and linear and logistic regression were performed. Total cleft-related surgeries were examined in a subset (n = 418) of patients with chart reviews. A total of 1047 patients were included; 59.6% had 2-stage CP repair, 40.4% had single-stage repair. Approximately 32% of children with 2-stage CP repair were diagnosed with VPI, as opposed to 22% of single-stage patients (P < .001). Children with 2-stage CP repair were 1.8 times as likely to be diagnosed with VPI (P < .001). Speech surgery rates were similar across groups. Patients who had 2-stage repair received an average of 2.3 more cleft-related procedures, when excluding prosthesis management procedures. Our data show an increased risk of VPI diagnosis and increased surgical burden among patients receiving 2-stage CP repair.

EP15.28: Two cases of occult submucous cleft palate and literature review

EP15.28: Two cases of occult submucous cleft palate and literature review

Long-term Results of Unilateral Cleft Lip Repair With Multiple Infantile Hemangiomas Including the Cleft Side of the Upper Lip

PURPOSE: Cleft lip and infantile hemangioma are relatively common congenital diseases. However, infantile hemangiomas on the cleft side, in the operative field of cleft lip, are extremely rare, and no clear guidelines have been established for their treatment. We experienced a case in which a patient with a cleft lip had infantile hemangioma on the cleft side. We performed cleft lip repair on a patient with infantile hemangioma on the cleft side of the cleft lip at 3 months after birth and have been following-up on the patient for the past 18 years. We report the results of 18-year follow-up after the surgery of this rare patient. METHODS: The male patient was diagnosed with a left unilateral complete cleft lip with alveolar cleft and a submucous cleft palate. There were multiple infantile hemangiomas throughout his body, with no notable abnormalities in the chromosomal analysis. In particular, the patient had infantile hemangiomas on the upper lip and lower lip, right ear helix, back, and left shoulder. The hemangioma on the upper lip was on the lateral segment of the cleft side. As per the general treatment guidelines, cleft repair was performed at 3 months after birth. Millard’s rotation advancement technique was used for the repair, which uses the lower small triangular flap. The patient was followed up for 18 years after surgery. RESULTS: There was no excessive bleeding during the surgery, and blood transfusion was not needed. The excised tissue was confirmed to be infantile hemangioma in the histopathologic examination. The patient did not show any abnormalities during recovery, and there was some residue of infantile hemangioma in the repair site in the upper lip vermilion. The residual infantile hemangioma in the repair site of the upper lip vermilion was involuted by 5 years old, and there was only a normal degree of scarring after cleft lip repair of the upper lip vermilion. At the same time, the infantile hemangiomas in the right ear helix, back, and left shoulder were completely involuted without any scarring. The patient has been followed up until now, and at 18 years, he has no particular problems other than the red scar on the lower lip. CONCLUSION: Infantile hemangiomas requiring lip repair for unilateral complete cleft lip are extremely rare, and there are no established surgical guidelines for this condition. In such cases, delaying lip repair until the infantile hemangioma is involuted may not be desirable for obtaining the best esthetic outcome, and it is also not desirable because it may induce psychosocial impairment in patients and caregivers. Therefore, we believe that general cleft lip repair produces good outcomes even in cases involving hemangiomas on the cleft side.

Partial Adenoidectomy in Patients With Palatal Abnormalities.

Velopharyngeal insufficiency is a feared complication of adenoidectomies in patients with palatal abnormalities. Thus, the benefits of adenoidectomy in this population are less understood. The techniques of partial adenoidectomy aim to preserve velopharyngeal closure while sufficiently debulking the nasal airway to ameliorate obstruction and snoring. Systematic review of online databases using a combination of search terms including "cleft palate," "submucosal cleft palate," "short palate," "adenoidectomy," "partial adenoidectomy," "subtotal adenoidectomy," and "powered adenoidectomy." Two independent reviewers assessed the eligibility of each study based on predefined inclusion criteria. From 323 studies identified, 8 were included. All were retrospective case series, except 1 prospective study. Study period ranged from 1.1 to 14 years (median 7.5 years). There were 172 patients with a cleft palate (n = 80), submucous cleft palate (n = 67) or another defined palatal abnormality [short soft palate (n = 14), bifid uvula (n = 4), questionable palatal mobility (n = 4) or incomplete CP (n = 3)] identified. Mean age was 5.5 years and just over half were male (58%). Nearly all patients showed improvement in nasal airway obstruction and snoring. The pooled risk for velopharyngeal insufficiency across all studies was 2 out of 122, which approximates to 1.6% of patients. There were very few complications. This is the first systematic review of partial adenoidectomy in patients with palatal abnormalities. Sub-total adenoidectomy in patients with submucosal cleft palate or repaired cleft palate appears to be a safe and efficacious procedure. There is; however, a need for prospective data collection using a standardized technique in large patient series required to properly assess outcomes.

Exploring the speech and language of individuals with non-syndromic submucous cleft palate: a preliminary report.

Submucous cleft palate (SMCP) has a heterogeneous presentation and is often identified late or misdiagnosed. Diagnosis is prompted by speech, resonance or feeding symptoms associated with velopharyngeal insufficiency. However, the broader impacts of SMCP on communication have rarely been examined and therefore are poorly understood. To describe the communicative profile of individuals with non-syndromic SMCP by examining speech, language and pragmatics (social language). Fifteen participants with SMCP aged 5;1-12;8, without a genetic diagnosis, participated in the study. Participants completed standardized assessments examining language, resonance, speech and non-verbal intellect. Parents also completed the Children's Communication Checklist (CCC-2), which provided a measure of overall communicative ability, including pragmatic skills. Formal language outcomes were compared with two cohorts: 36 individuals with overt non-syndromic clefts and 129 individuals with no history of clefting. Speech intelligibility was reduced secondary to hypernasality, disordered articulation and/or impaired phonology (n = 7) in children with SMCP. Poorer overall language outcomes were observed for children with SMCP compared with both those with overt clefts and no history of clefting (p < 0.001). Language scores for children with SMCP ranged from impaired (n = 6) to above the standardized mean (n = 4). Receptive and expressive language performance were independently correlated with non-verbal IQ (p < 0.01). Those with severe language impairment (n = 4) also had borderline or impaired non-verbal IQ. Parents reported that speech and semantics were the most affected sub-domains of communication, while scores were the highest for the initiation domain. Speech and language skills were correlated strongly with pragmatics (r = 0.877, p < 0.01). Overall, performance was variable within the SMCP group across speech, language and pragmatic assessments. In addition to well-documented speech difficulties, children with SMCP may have language or pragmatic impairments, suggesting that further neurodevelopmental influences may be at play. As such, for individuals with SMCP, additional clinical screening of language and pragmatic abilities may be required to ensure accurate diagnosis and guide both cleft and non-cleft related therapy programmes.

Treatment of velopharyngeal insufficiency in a patient with a submucous cleft palate using a speech aid: the more treatment options, the better the treatment results

BackgroundThe submucous cleft palate (SMCP) is a type of cleft palate that may result in velopharyngeal insufficiency (VPI). Palate muscles completely separate oral and nasal cavities by closing off the velopharynx during functional processes such as speech or swallow. Also, hypernasality may arise from anatomical or neurological abnormalities in these functions. Treatments of this issue involve a combination of surgical intervention, speech aid, and speech therapy. This case report demonstrates successfully treated VPI resulted from SMCP without any surgical intervention but solely with speech aid appliance and speech therapy.Case presentationA 13-year-old female patient with a speech disorder from velopharyngeal insufficiency that was caused by a submucous cleft palate visited to our OMFS clinic. In the intraoral examination, the patient had a short soft palate and bifid uvula. And the muscles in the palate did not contract properly during oral speech. She had no surgical history such as primary palatoplasty or pharyngoplasty except for tonsillectomy. And there were no other medical histories. Objective speech assessment using nasometer was performed. We diagnosed that the patient had a SMCP. The patient has shown a decrease in speech intelligibility, which resulted from hypernasality. We decided to treat the patient with speech aid (palatal lift) along with speech therapy. During the 7-month treatment, hypernasality measured by a nasometer decreased and speech intelligibility became normal.ConclusionsSurgery remains the first treatment option for patients with velopharyngeal insufficiencies from submucous cleft palates. However, there were few reports about objective speech evaluation pre- or post-operation. Moreover, there has been no report of non-surgical treatment in the recent studies. From this perspective, this report of objective improvement of speech intelligibility of VPI patient with SMCP by non-surgical treatment has a significant meaning. Speech aid can be considered as one of treatment options for management of SMCP.

当院における 22q11.2 欠失症候群の治療について－粘膜下口蓋裂と先天性鼻咽腔閉鎖不全症－

当院における 22q11.2 欠失症候群の治療について－粘膜下口蓋裂と先天性鼻咽腔閉鎖不全症－

Mandarin Chinese consonant production of patients with cleft palate

Eighteen Mandarin Chinese consonants were recorded from 259 patients with cleft palate in a /CV/ context. The age of participants ranged from 8 to 40 years old. Participants had five types of cleft palate: submucous cleft palate, hard and soft cleft palate, unilateral complete cleft palate, bilateral complete cleft palate, and congenital velopharyngeal insufficiency. All consonants were transcribed by speech-language pathologists. Results showed that the accuracy of consonant production was significantly affected by the type of cleft palate, consonant category, and their interaction. The correctness of consonant production ranged from 45.8% for the type IV cleft palate (e.g., BCCP) to 71.5% for Submucous cleft palate. In particular, for the manner of articulation, nasals had significantly higher accuracy than stops, fricatives, and affricates, while for the place of articulation, palatal and labial sounds were problematic with low accuracy. In addition, the errors of consonant production, such as replacement, compensation, and omission, were also dependent on the type of cleft palate and consonant category and their interaction.

Submucous cleft palate: from diagnosis to management

The aim of this study was to investigate submucous cleft palate (SMCP) patients in order to document the age of diagnosis, cause of referral, symptoms and palate findings, objective evaluation of nasalance and velopharyngeal dysfunction (VPD), and intervention type. The archive of Hacettepe University Cleft Lip and Palate, Craniomaxillofacial Anomalies Research, Treatment, and Application Center was reviewed retrospectively, and 166 patients diagnosed with SMCP were documented. The mean age of the patients at the time of initial referral was 10 years and 3 months ± 8 years and 5 months with the youngest being 1 month and the oldest 44 years old. The primary complaint of 127 (76.5%) patients was speech disorder. Seventy-nine patients underwent surgery, and the mean age was determined as 10 years ± 6 years and 8 months. The diagnosis of SMCP continues to be rather late, being delayed until a prominent speech disorder is present. Studies for the awareness of health professionals are of great importance for the early diagnosis and intervention of SMCP in order to prevent adverse effects.

Velopharyngeal incompetence: role in paediatric swallowing deficits.

The purpose of this manuscript is to highlight the latest advances in diagnosis and management of velopharyngeal incompetence (VPI) as it pertains to swallowing deficits in children. This is timely and relevant as otolaryngologists are often amongst the first to diagnose and treat VPI. Although nasal regurgitation of a bolus is frequently transient, persistent problems can be associated with other swallowing problems and other significant medical problems. Furthermore, velopharyngeal incompetence has implications for speech production. Persistent VPI associated with a swallowing deficit can be an isolated anomaly with or without a cleft palate or submucous cleft palate. VPI may be secondary to a cranial neuropathy, esophageal abnormality or associated with another airway anomaly, any of which may further contribute to dysphagia. Findings of additional anomalies may be suggestive of a syndrome. Workup should explore these potential causes. When velopharyngeal incompetence is associated with dysphagia, fiberoptic endoscopic evaluation of swallow (FEES) and videofluoroscopic swallow study (VFSS) can be helpful in diagnosis and management. The advantages and disadvantages of FEES and VFSS have been well delineated over the past few years. Similarly, nasopharyngoscopy and fluoroscopy are increasingly used in diagnosis and management of VPI that is associated with hypernasal resonance disorders. Concurrent medical diagnoses or syndrome manifestations are often associated with or contribute significantly to the cause of dysphagia in children with VPI. As VPI can be a sign of brainstem vagal neuropathy, the clinician should investigate by imaging the CNS if other correlative symptoms of dysphagia and examination findings are present. Endoscopy is advocated for evaluation of vocal fold function. Fluoroscopy is best for further assessment of airway protection or safety of swallow. And, whenever indicated, additional workup is recommended to determine an underlying cause of the swallowing disorder.

Prevalence of bifid uvula in primary school children

BackgroundA bifid uvula is an anatomic variation that can be predictive of sub-mucous cleft palate, which may cause velopharyngeal insufficiency (VPI). Bifid uvula prevalence in the literature ranges from 0.18% to 10.3%, depending on the population studied. The aim of this study is to determine the prevalence of bifid uvula in the Geneva's school children population. MethodsA cross-sectional study was conducted in Geneva's primary school children, from September 2014 to June 2015. An examination of the uvula was performed by dentists working for the Scholastic Dental Service, after a specific training in diagnosing bifid uvulas. The dentists recorded their findings on a standardized form. ResultsThe total number of school children in Geneva in the school year 2014–2015 was 30,375. 23,961 children had their uvula examined, representing 79% of the total population of school children. Among them, a hundred school children had a cleft uvula. One schoolgirl had no uvula. The prevalence of bifid uvula is 0.42%. Sex ratio (M/F) is 0.96. DiscussionThis large study, the second in literature for number of patients examined, identified a prevalence of bifid uvula of 0.42%. This result is in agreement with previous studies.

Management of velopharyngeal dysfunction in patients with 22q11.2 deletion syndrome: A survey of practice patterns

ObjectiveTo determine demographics and practice patterns of surgeons treating velopharyngeal dysfunction (VPD) in patients with 22q11.2 deletion syndrome (22q11.2DS). MethodsAn anonymous electronic survey study was administered to the surgical membership of the American Cleft Palate-Craniofacial Association and the Society for Ear Nose and Throat Advances in Children. The survey queried surgeon demographics and differences in management practices for submucous cleft palate (SMCP), pharyngoplasty algorithms, and self-reported complications for nonsyndromic versus 22q11.2DS patients. Results126 surveys were returned from 9 international regions with the majority from the United States (73%), followed by Western Europe (9.5%) and Canada (7.9%). Plastic surgery was the most common specialty (61.9%), followed by otolaryngology (27.8%). 88.1% reported fellowship training, and 33% completed multiple fellowships. Prior to proceeding with pharyngoplasty in 22q11.2DS patients, surgeons required the following assessments: speech evaluation (79.4%), velopharyngeal imaging (51.6%), cardiac evaluation (50.0%), carotid artery MRI (29.4%), and cervical spine x-rays (11.1%). Nasoendoscopy was the most common modality used for imaging the velopharynx. Overall, providers managed patients with 22q11.2DS similarly to nonsyndromic patients, with several significant exceptions including that they were more likely to perform SMCP repair alone as a first approach in nonsyndromic patients (p = 0.031) and posterior pharyngeal flap without SMCP repair in those with 22q11.2DS (p = 0.017). ConclusionsPractice patterns for the management of VPD in patients with 22q11.2DS vary across providers. Further collaborative studies are needed to develop optimal treatment paradigms for VPD in patients with 22q11.2 DS.