Impact of 22q Deletion Syndrome on Speech Outcomes Following Primary Surgery for Submucous Cleft Palate

Objective: 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. Design: Retrospective chart review. Setting: Tertiary pediatric hospital and 22q11.2 DS specialty clinic. Participants: 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. Main Outcome Measure: Percentage of children with SMCP with and without 22q DS requiring surgical intervention for velopharyngeal insufficiency. Results: 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). Conclusions: 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.

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The efficacy of transnasal palatal transillumination for the diagnosis of submucous cleft palate

Annals of Pediatric Surgery ◽

10.1186/s43159-021-00093-9 ◽

2021 ◽

Vol 17 (1) ◽

Author(s):

Yasir S. Jamal ◽

Sabah S. Moshref ◽

Abeer M. Baamir ◽

Mazin O. Kurdi ◽

Doaa Y. Jamal

Keyword(s):

Cleft Palate ◽

Cleft Lip ◽

Diagnostic Techniques ◽

Lateral Release ◽

Feeding Difficulties ◽

Presenting Symptoms ◽

Submucous Cleft Palate ◽

Cheap Method ◽

Operative Findings ◽

Simple Repair

Abstract Background Submucous cleft palate (SMCP) is a congenital abnormality with various clinical and anatomical features. Submucous cleft pathologies may be unrecognized during routine examinations. Current diagnostic techniques are constrained and unrevealing in presurgical patients. This prospective study aimed to evaluate transnasal palatal transillumination technique in diagnosis of SMCP at our institute hospital, during period from 2005-2020. Patients and methods Twenty-one cases with SMCP were recruited with age range from 2-60 months. Transnasal palatal transillumination with controllable light intensity endoscope used to evaluate SMCP and cases were photo and video recorded. Results In this study, 21 cases (13 males and 8 females) with SMCP were detected or confirmed by intranasal transnasal palatal transillumination. Frequency of SMCP patients at our institute was 3.39%. All patients presented with symptomatic complaints at diagnosis time, apart from 5 patients (23.8%) were diagnosed during cleft lip repair operations. Presenting symptoms were hypernasality (23.8%), delayed speech (23.8%), perforated palate with nasal escape of milk and food (14.3%), feeding difficulties (14.3%), and otitis media (4.8%). During intra-oral examination, all cases had a bifid uvula accompanied SMCP. Submucous cleft palate appeared as thin palate with central lucency. According to operative findings, operations done for repair were mostly two long palatal flaps (n = 13, 61.9%), von Langenbeck (n = 5, 23.8%), simple repair with lateral release incisions (n = 2, 9.6%), and simple repair without lateral release incision (n = 1, 4.8%). Conclusions Intraoperative assessment of submucous cleft palate with transnasal palatal transillumination is easy and cheap method to avoid missing cases of SMCP.

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A Closer Look at Delayed Primary Cleft Surgery and Unrepaired Cleft Lip and/or Palate in 5 UK Cleft Centers

The Cleft Palate-Craniofacial Journal ◽

10.1177/10556656211021700 ◽

2021 ◽

pp. 105566562110217

Author(s):

Sophie Butterworth ◽

Clare Rivers ◽

Marnie Fullarton ◽

Colm Murphy ◽

Victoria Beale ◽

...

Keyword(s):

United Kingdom ◽

Cleft Palate ◽

Cleft Lip ◽

Medical Problems ◽

Primary Surgery ◽

The United Kingdom ◽

Number Of Patients ◽

Primary Palate ◽

Palate Repair ◽

Cleft Surgery

Background: There may be many reasons for delays to primary cleft surgery. Our aim was to investigate the age of children undergoing primary cleft lip or primary cleft palate repair in 5 cleft centers within the United Kingdom. Identify the reasons for delayed primary cleft lip repair (beyond 6 months) and delayed primary palate repair (beyond 13 months). Identify children who had a cleft lip and/or palate (CL±P) that was intentionally unrepaired and the reasons for this. Methods: A retrospective, multicenter review of patients born with a CL±P between December 1, 2012, and December 31, 2016. Three regional cleft centers, comprising of 5 cleft administrative units in the United Kingdom participated. Results: In all, 1826 patients with CL±P were identified. Of them, 120 patients had delayed lip repair, outside the expected standard of 183 days. And, 178 patients in total had delayed palate repair, outside the expected standard of 396 days. Twenty (1%) patients had an unrepaired cleft palate. Conclusions: This large retrospective review highlights variations between centers regarding the timing of lip and palate surgery and details the reasons stated for delayed primary surgery. A small number of patients with an unrepaired cleft palate were identified. All had complex medical problems or comorbidities listed as a reason for the decision not to operate and 50% had a syndromic diagnosis. The number of patients receiving delayed surgery due to comorbidities, being underweight or prematurity, highlights the importance of the cleft specialist nurse and pediatrician within the cleft multidisciplinary team.

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Dental anomalies frequency in submucous cleft palate versus complete cleft palate

European Journal of Orthodontics ◽

10.1093/ejo/cjab003 ◽

2021 ◽

Author(s):

João Paulo Schwartz ◽

Daniela Gamba Garib

Keyword(s):

Cleft Palate ◽

Permanent Teeth ◽

Tooth Agenesis ◽

Dental Anomalies ◽

Supernumerary Teeth ◽

Chi Square ◽

Intergroup Comparison ◽

Significant Difference ◽

Submucous Cleft Palate ◽

Isolated Cleft Palate

Summary Background/Objectives This retrospective study evaluated the prevalence of dental anomalies of number in different subphenotypes of isolated cleft palate. Materials/Methods The sample comprised 26 individuals with submucous cleft palate (group S) and 68 individuals with complete cleft palate (group C) aged between 9 and 12 years from a single centre. Panoramic radiographs were evaluated regarding the presence of dental anomalies of number in permanent teeth. Intergroup comparison was performed using chi-square tests (P < 0.05). Results Tooth agenesis was found in 34.61 and 36.76 per cent of group S and group C, respectively. The most commonly missing teeth were the maxillary second premolar, maxillary lateral incisor, and mandibular second premolar. Supernumerary teeth were found in none and 1.47 per cent of the individuals with submucous and complete cleft palate, respectively. No statistically significant difference was found between groups for the frequency of tooth agenesis and supernumerary teeth. Limitations Only dental anomalies of number were evaluated. Conclusions/Implications Individuals with submucous and complete cleft palate showed similar prevalence for tooth agenesis and supernumerary teeth. Dental anomalies frequency seems not to be a discriminator for subphenotypes of cleft palate.

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Impact of cleft palate repair on speech and development of dentoalveolar deformities

Voprosy praktičeskoj pediatrii ◽

10.20953/1817-7646-2021-3-47-53 ◽

2021 ◽

Vol 16 (3) ◽

pp. 47-53

Author(s):

Yu.V. Stebeleva ◽

◽

Ad.A. Mamedov ◽

Yu.O. Volkov ◽

A.B. McLennan ◽

...

Keyword(s):

Cleft Palate ◽

Cleft Lip ◽

Cleft Lip And Palate ◽

Surgical Techniques ◽

Normal Functioning ◽

Primary Surgery ◽

Cleft Palate Repair ◽

Congenital Cleft ◽

Palate Repair ◽

Late Postoperative Period

Surgical repair of cleft palate is quite difficult because it aims not only to eliminate the anatomical defect of the palate, but also to ensure normal functioning, including speech. Moreover, successful surgery implies no or minimal deformation of the middle face that can be corrected in the late postoperative period. No doubt that primary surgery (both in terms of technique and time) is crucial for further growth and development of the maxilla. However, surgical techniques and the age of primary cleft palate repair vary between different clinics, which makes this literature review highly relevant. Key words: cleft palate repair, cleft palate, congenital cleft lip and palate

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Phonological Profile of Patients With Velopharyngeal Dysfunction and Palatal Anomalies

Journal of Speech Language and Hearing Research ◽

10.1044/2021_jslhr-20-00652 ◽

2021 ◽

pp. 1-15

Author(s):

Ariela Nachmani ◽

Muhamed Masalha ◽

Firas Kassem

Keyword(s):

Cleft Palate ◽

Cleft Lip ◽

Cleft Lip And Palate ◽

Age Groups ◽

Phonological Process ◽

Phonological Processes ◽

Process Error ◽

Submucous Cleft Palate ◽

Different Types

Purpose This purpose of this study was to assess the frequency and types of phonological process errors in patients with velopharyngeal dysfunction (VPD) and the different types of palatal anomalies. Method A total of 808 nonsyndromic patients with VPD, who underwent follow-up at the Center for Cleft Palate and Craniofacial Anomalies, from 2000 to 2016 were included. Patients were stratified into four age groups and five subphenotypes of palatal anomalies: cleft lip and palate (CLP), cleft palate (CP), submucous cleft palate (SMCP), occult submucous cleft palate (OSMCP), and non-CP. Phonological processes were compared among groups. Results The 808 patients ranged in age from 3 to 29 years, and 439 (54.3%) were male. Overall, 262/808 patients (32.4%) had phonological process errors; 80 (59.7%) ages 3–4 years, 98 (40, 0%) ages 4.1–6 years, 48 (24.7%) 6.1–9 years, and 36 (15.3%) 9.1–29 years. Devoicing was the most prevalent phonological process error, found in 97 patients (12%), followed by cluster reduction in 82 (10.1%), fronting in 66 (8.2%), stopping in 45 (5.6%), final consonant deletion in 43 (5.3%), backing in 30 (3.7%), and syllable deletion and onset deletion in 13 (1.6%) patients. No differences were found in devoicing errors between palatal anomalies, even with increasing age. Phonological processes were found in 61/138 (44.20%) with CP, 46/118 (38.1%) with SMCP, 61/188 (32.4%) with non-CP, 70/268 (26.1%) with OSMCP, and 25/96 (26.2%) with CLP. Phonological process errors were most frequent with CP and least with OSMCP ( p = .001). Conclusions Phonological process errors in nonsyndromic VPD patients remained relatively high in all age groups up to adulthood, regardless of the type of palatal anomaly. Our findings regarding the phonological skills of patients with palatal anomalies can help clarify the etiology of speech and sound disorders in VPD patients, and contribute to general phonetic and phonological studies.

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The Pattogenesis of Submucous Cleft Palate

Scandinavian Journal of Plastic and Reconstructive Surgery ◽

10.3109/02844317409084368 ◽

1974 ◽

Vol 8 (1-2) ◽

pp. 34-41 ◽

Cited By ~ 9

Author(s):

David Poswillo

Keyword(s):

Cleft Palate ◽

Submucous Cleft Palate

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Cleft Palate in Kabuki Syndrome: A Report of Six Cases

The Cleft Palate-Craniofacial Journal ◽

10.1597/05-174 ◽

2006 ◽

Vol 43 (6) ◽

pp. 756-761 ◽

Cited By ~ 11

Author(s):

Takuya Iida ◽

Susam Park ◽

Kogo Kato ◽

Ichiko Kitano

Keyword(s):

Cleft Palate ◽

Clinical Features ◽

Congenital Anomalies ◽

Kabuki Syndrome ◽

Characteristic Appearance ◽

Submucous Cleft Palate ◽

Velopharyngeal Function

Kabuki syndrome is a syndrome of rare congenital anomalies that was named after its characteristic appearance, a face resembling that of an actor in a Kabuki play. Although cleft palate is a feature that is sometimes observed in patients with Kabuki syndrome, there are few clinical reports of cleft palate associated with Kabuki syndrome. This report presents six cases of Kabuki syndrome with cleft palate and reviews their clinical features. Our results suggest that (1) patients with cleft palate in Kabuki syndrome tend to fail in acquiring normal velopharyngeal function and (2) submucous cleft palate might be more common in patients with Kabuki syndrome than previously was reported.

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