Anatomic Severity, Midfacial Growth, and Speech Outcomes in Van der Woude/Popliteal Pterygium Syndromes Compared to Nonsyndromic Cleft Lip/Palate

Reardon, Jeff; Brustowicz, Katherine; Marrinan, Eileen M.; Mulliken, John B.; Padwa, Bonnie L.

doi:10.1597/14-132

Cited by 10 publications

(12 citation statements)

References 24 publications

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“…As with our study, the participants with VdW syndrome were significantly more likely to require secondary surgery for VPI than the nonsyndromic group. Reardon et al (2015) also found the VdW group had a significantly higher fistula rate but this was not replicated in our study.…”

Section: Comparison With Other "Sommerlad Technique" Studiescontrasting

confidence: 86%

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Benchmarking Speech, Velopharyngeal Function Outcomes and Surgical Characteristics Following the Sommerlad Protocol and Palate Repair Technique

Baillie

Sell

2020

The Cleft Palate Craniofacial Journal

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Objective: To report speech and velopharyngeal function (VPF) outcomes, and surgical characteristics, at age 5 following early complete palate closure using the Sommerlad protocol. Design: A retrospective, descriptive, cross-sectional consecutive series. Setting: A regional twin site center; a district general hospital and tertiary children’s hospital. Participants: Between 1993 and 2006, 877 participants underwent surgery; 712 (81%) were eligible for inclusion; 391 (55%) were included, 321 (45%) excluded. Thirteen percent had bilateral cleft lip and palate (CLP), 40% unilateral CLP, and 47% isolated cleft palate. Intervention: Lip and vomerine flap repair at 3 months of age (BCLP, UCLP) with soft palate closure using radical muscle dissection and retropositioning at a mean age of 6.6 months, range 4 to 23 months, as described and undertaken by Sommerlad. Outcome Measures: Velopharyngeal Composite (VPC) CAPS-A and articulation summary scores derived from analysis using the Cleft Audit Protocol for Speech-Augmented. Results: A VPC-SUM CAPS-A score of “0” was found in 97% reflecting adequate VPF; 73% had no cleft articulation difficulties. The BCLP group had the poorest articulation. Secondary speech surgery rate was 2.6% and 10.7% had fistula repair. Levator muscle quality and degree of retropositioning was associated with speech outcomes related to VPF. 2.6% had current features of VPI. Conclusions: Complete palate closure by around 6 months of age by the surgeon who described the technique led to excellent perceived VPF and very good speech outcomes at age 5, with less speech therapy and secondary speech surgery than commonly reported. These findings serve as a benchmark for the Sommerlad protocol and technique.

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Section: Comparison With Other "Sommerlad Technique" Studiescontrasting

confidence: 86%

“…Reardon et al (2015) compared patients with VdW and nonsyndromic iCP and Kitchin et al (2019) compared VdW with nonsyndromic CP±L. As with our study, the participants with VdW syndrome were significantly more likely to require secondary surgery for VPI than the nonsyndromic group.…”

Section: Discussionsupporting

confidence: 65%

Benchmarking Speech, Velopharyngeal Function Outcomes and Surgical Characteristics Following the Sommerlad Protocol and Palate Repair Technique

Baillie

Sell

2020

The Cleft Palate Craniofacial Journal

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“…While there have been prior studies investigating paramedian lip pits, dental features, facial features, family histories of VWS, and even wound healing in patients with VWS, the longterm outcome of these patients is not as well reported 13,15,17,20,22,[24][25][26][27][28][29] . Recently, Reardon et al 30 documented a unique study involving the clinical characteristics and surgical course of 28 patients with VWS/Popliteal Pterygium Syndrome (PPS) at one tertiary care center. While the study by Reardon et al (2015) is notable, it does not trace patient speech pathology outside of the need for secondary velopharyngeal repair.…”

Section: Introductionmentioning

confidence: 99%

“…Recently, Reardon et al 30 documented a unique study involving the clinical characteristics and surgical course of 28 patients with VWS/Popliteal Pterygium Syndrome (PPS) at one tertiary care center. While the study by Reardon et al (2015) is notable, it does not trace patient speech pathology outside of the need for secondary velopharyngeal repair. We therefore aim to investigate patients with VWS at our institution with an increased focus on speech pathology, specifically resonance, intelligibility, and need for speech therapy.…”

Section: Introductionmentioning

confidence: 99%

Surgical, Speech, and Audiologic Outcomes in Patients With Orofacial Cleft and Van der Woude Syndrome

Kitchin

Grames²,

Naidoo³

et al. 2019

Journal of Craniofacial Surgery

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Objective-The purpose of this study was to investigate the surgical, speech, and audiologic outcomes in patients with Van der Woude syndrome (VWS) and compare them to patients with non-syndromic cleft palate with or without cleft lip (NS-CP±L) treated at the same institution. Design-Retrospective chart review.Setting-A single children's hospital at a major academic institution.Patients-We retrospectively examined the records of 18 patients with VWS who had been treated at a single institution from 1989-2017. 38 patients with NS-CP±L who were also treated at the same institution during that same time frame were selected to closely match sex and date of birth.Main Outcome Measures-Demographic, clinical, surgical, and speech pathology data were gathered from medical charts.Result-By age four, 83% of subjects with VWS and 76% of subjects with NS-CP±L (p = 0.732) had been, or were actively involved in, speech therapy. By age ten, 100% of remaining subjects with VWS and 58% of remaining subjects with NS-CP±L remained involved in speech therapy (p = 0.027). 33% of patients with VWS and 16% of patients with NS-CP±L had a secondary procedure for velopharyngeal dysfunction (VPD) (p = 0.171).Conclusions-The VWS group had more than twice the rate of secondary procedures for VPD repair, and a higher rate of continuing involvement for speech therapy at age ten. No differences were found in the rate of participation in speech therapy at or by age four.

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“…This bilateral distribution is even more heavily skewed than in the other common cleft syndromes. In van der Woude, 46% have bilateral clefts (Rearden et al, 2015); in CHARGE, 37% have bilateral clefts (Isaac et al, 2018). A phenotypic subgroup analysis has not been reported for Trisomy 13.…”

Section: Discussionmentioning

confidence: 99%

Cleft Lip and Palate in Ectodermal Dysplasia

Ganske

Irwin

Langa

et al. 2020

The Cleft Palate Craniofacial Journal

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Objective: Ectodermal dysplasia (ED) comprises multiple syndromes that affect skin, hair, nails, and teeth, and sometimes are associated with orofacial clefting. The purpose of this study is to (1) identify the prevalence and characteristics of cleft lip and/or palate (CL/P) in patients with ED and (2) describe the management and outcomes. Design: Retrospective review from 1990 to 2019. Patients: All patients with ED treated at Boston Children's Hospital. Main Outcomes Measures: Prevalence of CL/P was calculated and clinical details recorded: phenotypic anomalies, cleft type, operative treatment, and results of repair. Results: Of 170 patients with a purported diagnosis of ED, 24 (14%) had CL/P. Anatomic categories were bilateral CL/P (67%), unilateral CL/P (8%), and cleft palate only (25%). The most common ED syndrome (37%) was ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC). Pathogenic variants in TP63 were the most frequent finding in the 11 patients who had genetic testing. Aberrations from a typical clinical course included failure of presurgical dentofacial orthopedics, dehiscence of nasolabial adhesion, and total palatal absence requiring free-flap construction. Two patients had prolonged postoperative admission for respiratory infection. High fistula (8%) and velopharyngeal insufficiency (33%) rates reflected the predominance of bilateral complete forms. Conclusions: As in other types of syndromic CL/P, cleft phenotypic expression in ED is more severe than the general cleft population. Further studies are needed to correlate genotype and phenotype for the distinct syndromes included in the ED spectrum.

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Anatomic Severity, Midfacial Growth, and Speech Outcomes in Van der Woude/Popliteal Pterygium Syndromes Compared to Nonsyndromic Cleft Lip/Palate

Abstract: Patients with VWS/PPS have more severe forms of labiopalatal clefting and higher incidences of midfacial retrusion, palatal fistula, and velopharyngeal insufficiency following primary repair as compared with nonsyndromic CL/P.

Cited by 10 publications

References 24 publications

Benchmarking Speech, Velopharyngeal Function Outcomes and Surgical Characteristics Following the Sommerlad Protocol and Palate Repair Technique

Benchmarking Speech, Velopharyngeal Function Outcomes and Surgical Characteristics Following the Sommerlad Protocol and Palate Repair Technique

Surgical, Speech, and Audiologic Outcomes in Patients With Orofacial Cleft and Van der Woude Syndrome

Cleft Lip and Palate in Ectodermal Dysplasia

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