Do patients with isolated Pierre Robin Sequence have worse outcomes after cleft palate repair: A systematic review

Wan, Teng; Chen, Yang; Wang, Guomin

doi:10.1016/j.bjps.2015.04.015

Cited by 19 publications

(14 citation statements)

References 18 publications

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“…All 5 of our patients with Stickler syndrome presented with features of PRS, and 3 of these developed VPI. Although not statistically significant in our cohort, PRS has been associated with higher levels of pharyngeal incompetence, worse speech scores, and increased cases of correction surgery, which are proposed to result from a short palate, wide cleft, and underlying muscular abnormalities (Robin et al, 1993; Stransky et al, 2013; Wan et al, 2015; Hardwicke et al, 2016). Whether the other genetic mutations seen in our cohort (IRF6, p63, BMP2, PTCH1) cause anatomic changes leading to VPI remains to be seen.…”

Section: Discussioncontrasting

confidence: 62%

Predictive Factors for Velopharyngeal Insufficiency Following Primary Cleft Palate Repair

Anderson

Fallah

Lignieres

et al. 2021

The Cleft Palate Craniofacial Journal

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Objective: Velopharyngeal insufficiency (VPI) remains a known complication of primary palatoplasty. We sought to identify factors associated with the incidence of VPI and create a predictive model for VPI development in our population. Design: A single-institution, retrospective review. Setting: Multidisciplinary clinic in a tertiary academic institution. Patients: A total of 453 consecutive patients undergoing primary palatoplasty from 1999 to 2016 were reviewed. Inclusion required follow-up past age 5. Patients who were non-verbal, and thus unable to undergo speech evaluation, were excluded. Main Outcome Measures: Primary outcome was VPI, defined as revision palatoplasty or recommendation by speech-language pathology. Results: Of 318 patients included, 179 (56%) were male. Median age at primary repair was 1.0 years (0.9-1.1) with a median age of 8.8 years at last follow-up. One hundred nineteen (37%) patients developed VPI at a median age of 5.0 years (3.8-6.5). Higher rates were seen with posterior fistula (65% vs 14%, P <.01) and straight-line repair (41% vs 9%, P <.01), with lower rates in patients with Veau I clefts (22% vs 39%, P <.05). Patients with VPI were older at last follow-up. Following multivariate regression, factors remaining significant were posterior fistula (odds ratio [OR]: 11.3, 95% CI: 6.1-22.0), primary Furlow repair (OR: 0.18, 95% CI: 0.03-0.68), genetic diagnoses (OR: 2.92, 95% CI: 1.1-7.9), and age at last follow-up (OR: 1.11, 95% CI: 1.01-1.2). Conclusions: Length of follow-up, posterior fistulae, and genetic diagnoses are associated with VPI formation. Furlow repair may protect against formation of VPI. Use of allograft, Veau class, birth type, birth weight, and race are not independently associated with VPI formation.

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Section: Discussioncontrasting

confidence: 62%

Predictive Factors for Velopharyngeal Insufficiency Following Primary Cleft Palate Repair

Anderson

Fallah

Lignieres

et al. 2021

The Cleft Palate Craniofacial Journal

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“…Our RS cohort demonstrated higher rates of cleft‐related speech errors and reduced intelligibility, but similar VPI and secondary surgery rates compared to the non‐Robin cohort. One systematic review has concluded there is insufficient evidence to determine if RS is an independent risk factor for poorer speech outcomes, consistent with our findings 19 . The importance of systematic ascertainment of clinical characteristics, speech production, and longitudinal outcomes is essential to improving cleft care.…”

Section: Discussionsupporting

confidence: 70%

Predictors of Success After Furlow Palatoplasty for Repair of Cleft Palate: Does Syndrome Matter?

Evans

Bly

Paulsgrove

et al. 2021

Otolaryngol.--head neck surg.

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Objective To determine predictors of success following Veau 1 and 2 cleft palate repair in patients with and without syndromes. Study Design Retrospective review of prospectively collected data. Setting Tertiary care children’s hospital. Methods All children <18 months of age undergoing Furlow palatoplasty for Veau 1 and 2 cleft repair between 2000 and 2014 with postoperative perceptual speech assessment (PSA). Results In total, 368 consecutive patients were identified; 95 were excluded, resulting in 273 patients. Median age at surgery was 13.0 months (interquartile range [IQR], 11-15 months) with postoperative PSA at a median of 32.3 months (IQR, 26.3-44.5 months). Fifty patients (18.3%) had syndrome diagnosis; 59 patients (21.6%) had nonsyndromic Robin sequence. Velopharyngeal insufficiency (VPI) occurred in 27 patients (10.5%); 13 underwent secondary speech surgery. Cleft-related speech errors occurred in 46 patients (17.6%). Non-cleft-related speech errors occurred in 155 patients (59.6%) and reduced intelligibility in 127 patients (47.9%). Oronasal fistula occurred in 23 patients (8.8%) and was exclusive to Veau 2 clefts. In multivariate analysis, age >13 months at palatoplasty demonstrated a 6-fold higher rate of VPI (hazard ratio [HR], 6.64; P < .01), worse speech outcomes (HR, 6.04; P < .01; HR, 1.60; P < .01; HR, 1.57; P = .02), and greater speech therapy utilization (HR, 2.18; P < .01). Conclusion VPI occurred in 10% of patients undergoing Furlow palatoplasty repair of Veau 1 or 2 clefts. Age <13 months at palatoplasty was associated with improved speech outcomes and lower VPI incidence (2.8% vs 16.2%). Syndromic diagnosis was associated with noncleft speech errors and reduced intelligibility on univariate analysis but not velopharyngeal function after palatoplasty.

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“…The rate of fistula and impaired speech are within the same range as that for a non-RS child with cleft palate 63. This is not true in all centers, with others reporting a higher rate of velopharyngeal insufficiency, but these are likely due to the heterogeneous populations of RS patients as well as RS experience of surgeon 61,63,64…”

Section: Treatment – Surgical/invasive Management (Cleft/craniofacialmentioning

confidence: 84%

Robin sequence: what the multidisciplinary approach can do

Cohen¹,

Greathouse²,

Rabbani³

et al. 2017

JMDH

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Robin sequence (RS) is a commonly encountered triad of micrognathia, glossoptosis, and airway obstruction, with or without a cleft palate. The management of airway obstruction is of paramount importance, and multiple reviews and retrospective series outline the diagnosis and treatment of RS. This article focuses on the multidisciplinary nature of RS and the specialists’ contributions and thought processes regarding the management of the RS child from birth to skeletal maturity. This review demonstrates that the care of these children extends far beyond the acute airway obstruction and that thorough monitoring and appropriate intervention are required to help them achieve optimal outcomes.

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Do patients with isolated Pierre Robin Sequence have worse outcomes after cleft palate repair: A systematic review

Cited by 19 publications

References 18 publications

Predictive Factors for Velopharyngeal Insufficiency Following Primary Cleft Palate Repair

Predictive Factors for Velopharyngeal Insufficiency Following Primary Cleft Palate Repair

Predictors of Success After Furlow Palatoplasty for Repair of Cleft Palate: Does Syndrome Matter?

Robin sequence: what the multidisciplinary approach can do

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