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Dysphagia Oct 2023Dysphagia in Robin Sequence can be present in varying degrees, requiring multidisciplinary management and specific swallowing assessment by a specialist. Most studies... (Meta-Analysis)
Meta-Analysis Review
Dysphagia in Robin Sequence can be present in varying degrees, requiring multidisciplinary management and specific swallowing assessment by a specialist. Most studies published to date have evaluated only respiratory outcomes, and the available evidence on the improvement of swallowing is questionable. To conduct a systematic review and meta-analysis of studies evaluating swallowing in children with Robin Sequence before and after airway clearance procedures. The research question was developed based on the PICO strategy. The literature search was performed in electronic databases and gray literature. Studies were selected by 3 independent reviewers. The risk of bias and level of evidence of the studies were assessed. A proportion meta-analysis was performed to calculate the prevalence of dysphagia after airway clearance procedures. The search identified 4938 studies, 5 of which were included. All studies had limitations in terms of design and sample size. The prevalence of dysphagia after airway clearance was obtained by analyzing treatment subgroups: mandibular distraction osteogenesis, mandibular distraction osteogenesis + tracheostomy tube, and nasopharyngeal tube. Clinical and/or instrumental assessment was assessed by a swallowing specialist. The meta-analysis was precluded by the limitations of the studies, especially regarding sample size, which affected the accuracy of the findings. Dysphagia remained unresolved in 55% of children (95% CI 1-99%). The methodological quality of the studies indicated a high risk of bias and very low level of evidence. It was not possible to confirm that airway clearance techniques used in Robin Sequence improve dysphagia.
Topics: Humans; Child; Infant; Treatment Outcome; Deglutition Disorders; Deglutition; Pierre Robin Syndrome; Osteogenesis, Distraction; Retrospective Studies
PubMed: 36763186
DOI: 10.1007/s00455-023-10561-6 -
Orthodontics & Craniofacial Research Nov 2017To evaluate prevalence rates and patterns of permanent tooth agenesis in individuals with non-syndromic Robin sequence (ns-RS). (Meta-Analysis)
Meta-Analysis Review
OBJECTIVES
To evaluate prevalence rates and patterns of permanent tooth agenesis in individuals with non-syndromic Robin sequence (ns-RS).
MATERIAL AND METHODS
Two investigators independently carried out a literature search, using PubMed, Web of Science, Ovid, EMBASE and the Cochrane Library, identifying articles relating to permanent tooth agenesis, diagnosed using panoramic radiographs, in individuals with ns-RS. The outcomes desired were prevalence rates and patterns of permanent tooth agenesis. The final search was carried out in January 2017.
RESULTS
Six articles were selected for inclusion, with a total of 448 individuals with ns-RS. Data available in each study varied which allowed conducting further specific meta-analyses only on sample sizes of 339 or 378 individuals from three or four of the six included articles. The overall estimated prevalence rate of permanent tooth agenesis was 42% (95% CI: 35%-48%), excluding third molars, with no sex predilection. In individuals with tooth agenesis, approximately 30% had one missing tooth while 40% had two missing teeth. Tooth agenesis was more commonly bilateral (Relative Risk 2) and found in the mandible (Relative Risk 1.6). The highest prevalence of permanent tooth agenesis was found for mandibular second premolars (26%) followed by maxillary second premolars (14%). The most common tooth agenesis patterns were the agenesis of both mandibular second premolars, followed by the agenesis of all second premolars.
CONCLUSION
Individuals with ns-RS have high prevalence rates of permanent tooth agenesis. The most prevalent tooth agenesis patterns are bilateral symmetric tooth agenesis, most commonly agenesis of both mandibular second premolars.
Topics: Anodontia; Child; Child, Preschool; Female; Humans; Male; Pierre Robin Syndrome; Prevalence
PubMed: 29027749
DOI: 10.1111/ocr.12204 -
The Cleft Palate-craniofacial Journal :... Apr 2023Robin Sequence (RS), characterized by micrognathia, glossoptosis, and upper airway obstruction, is an increasingly recognized diagnosis. An effective surgical... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Robin Sequence (RS), characterized by micrognathia, glossoptosis, and upper airway obstruction, is an increasingly recognized diagnosis. An effective surgical intervention is mandibular distraction osteogenesis (MDO). This study analyzes published evidence regarding facial nerve dysfunction (FND) associated with MDO.
DESIGN AND SETTING
According to PRISMA guidelines, a systematic review was carried out with databases queried in June 2019 using MESH terms, or equivalent terms, as follows: "distraction osteogenesis" and "Robin Sequence". A review of original Spanish and English articles, were included. Outcome measures included the prevalence of FND; the affected branches; the rate of permanent vs. transient FND; the use of an internal vs. external device; the daily distraction rate; and finally, the overall distraction length. Subsequently, a meta-analysis was conducted to collate results regarding the prevalence of FND and the factors associated with it.
RESULTS
Of 239 unique studies identified, 19 studies with 729 patients met inclusion criteria; 52 patients developed FND after MDO. A random-effects meta-analysis yielded a pooled prevalence of FND of 6.40%, with moderately heterogeneous studies (I2 = 41%, τ2 = 0.006). Marginal mandibular nerve involvement was most commonly noted. Nine studies reported transient FND, six permanent, one both, and two unspecified. Internal distractors were used in 8 studies and external in 3 and both in 2. Distraction rate was 1.00 to 2.00 mm/day and total distraction length ranged from 13.00 to 22.3 mm. Sample size was the only parameter inversely associated with rate of FND (p = 0.04).
CONCLUSION
This analysis of FND associated with MDO for patients with RS demonstrates a lack of consistent documentation. MDO-associated FND does not appear to be uncommon, and permanent dysfunction can occur. This review underscores the importance of thorough documentation to elucidate the mechanism of FND.
Topics: Humans; Facial Nerve; Osteogenesis; Retrospective Studies; Treatment Outcome; Mandible; Pierre Robin Syndrome
PubMed: 35001639
DOI: 10.1177/10556656211070728 -
The Journal of Craniofacial Surgery Sep 2016Tongue-lip adhesion (TLA) involves surgically tethering the tongue forward to the lower lip and is a technique to relieve airway obstruction caused by glossoptosis and... (Review)
Review
BACKGROUND
Tongue-lip adhesion (TLA) involves surgically tethering the tongue forward to the lower lip and is a technique to relieve airway obstruction caused by glossoptosis and retrognathia. The purpose of the present study was to evaluate the effectiveness of TLA for the treatment of airway compromise in patients with Pierre Robin sequence (PRS).
METHODS
A comprehensive literature review was performed. Inclusion criteria consisted of patients having undergone isolated tongue lip adhesion and results that included airway outcome. Selected manuscripts were analyzed with regards to patient demographics, principle diagnosis, pre and postintervention airway status, and complications.
RESULTS
Thirteen manuscripts met inclusion criteria, yielding 268 patients with PRS who underwent TLA. The mean age at the time of procedure was 30.5 days. Tongue lip adhesion proved to be successful in relieving airway obstruction caused by PRS in 81.3% (n = 218) of patients. Nonsyndromic patients benefited from a higher success rate as compared with the syndromic cohort (91.5% and 79.8% respectively, P = 0.0361). Eight patients who were initially successfully managed with TLA required a repeat procedure due to dehiscence.
CONCLUSIONS
Tongue-lip adhesion is a safe and effective technique and is associated with lower morbidity and mortality as compared with mandibular distraction osteogenesis and tracheostomy and should be considered in patients with PRS who fail conservative management. The greater success and lower complication rates in nonsyndromic patients reinforce the importance of proper patient selection and consideration of other techniques such as mandibular distraction osteogenesis should be given in patients with associated syndromic diagnoses.
Topics: Airway Obstruction; Child; Humans; Lip; Osteogenesis, Distraction; Pierre Robin Syndrome; Tongue; Treatment Outcome
PubMed: 27548826
DOI: 10.1097/SCS.0000000000002721 -
The Journal of Craniofacial Surgery Sep 2022Mandibular distraction osteogenesis (MDO) is becoming the procedure of choice for patients with Robin Sequence (RS) as it offers superior long-term respiratory outcomes...
INTRODUCTION
Mandibular distraction osteogenesis (MDO) is becoming the procedure of choice for patients with Robin Sequence (RS) as it offers superior long-term respiratory outcomes in avoidance of tracheostomy. Lacking, is an analysis of the short- and long-term complications. To that end, we have conducted a comprehensive review focusing on complications of MDO.
MATERIALS AND METHODS
A systematic review of primary clinical studies reporting outcomes and complications of MDO in RS patients. Outcomes included tracheostomy avoidance and decannulation rate. Complications included dental trauma, nerve injury, surgical site infection and hardware failure. Complications were stratified according to distractor type (internal versus external) and age (>2 months versus <2months).
RESULTS
A total of 49 studies yielded 1209 patients with a mean follow-up of 43.78 months. The tracheostomy avoidance rate was 94% (n = 817/870) and the mortality rate was 0.99% (n = 12/1209). The complication rate was 28.9% (n = 349/1209) with surgical site infections (10.5%) being most common. Dental and nerve injuries occurred in 7.9% and 3.2% of patients, respectively. Hardware replacement occurred in 1.2% of patients. internal distractors had higher rates of dental injury whereas external distractors had higher technical failure rates. There were no differences in complication rates ( P= 0.200), mortality ( P = 0.94) or tracheostomy avoidance ( P = 0.058) between patients >2months or <2months of age.
CONCLUSION
Mandibular distraction osteogenesis is highly reliable and effective with a low mortality and high tracheostomy avoidance rate. There are important complications including nerve and dental injuries which require long-term follow-up. Neonatal patients do not appear to be at higher risk of complications reinforcing the safety of MDO in this population.
Topics: Airway Obstruction; Humans; Infant; Infant, Newborn; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome; Retrospective Studies; Treatment Outcome
PubMed: 35258012
DOI: 10.1097/SCS.0000000000008611 -
The Cleft Palate-craniofacial Journal :... Mar 2022Mandibular distraction osteogenesis (MDO) is frequently performed to address airway obstruction in patients with Pierre Robin sequence (PRS), though more recently the...
OBJECTIVE
Mandibular distraction osteogenesis (MDO) is frequently performed to address airway obstruction in patients with Pierre Robin sequence (PRS), though more recently the technique of orthodontic airway plating (OAP) has gained traction. We aimed to evaluate OAP compared to MDO for airway obstruction in PRS.
DESIGN
A systematic literature search across PubMed, Embase, and Google Scholar identified all studies published in English, which involved MDO or any form of OAP as treatments for PRS. All relevant articles were reviewed in detail and reported on, adhering to PRISMA guidelines.
MAIN OUTCOME MEASURES
Airway (tracheostomy avoidance, decannulation rate), feeding (full oral feeding tolerance).
RESULTS
Literature search identified 970 articles, of which 42 MDO studies and 9 OAP studies met criteria for review. A total of 1159 individuals were treated with MDO, and 322 individuals were treated with OAP. Primary outcomes appear similar for MDO and OAP at face value; however, this must be interpreted with different pretreatment contexts in mind.
CONCLUSIONS
Orthodontic airway plating may be considered for airway obstruction in PRS, as some airway-related and feeding-related outcomes appear similar with MDO, per existing evidence in the literature. However, since PRS severity differed between studies, OAP cannot be uniformly considered a replacement for MDO. Further research is required to more comprehensively assess these treatment modalities inclusive of metrics that allow for direct comparison.
Topics: Airway Obstruction; Humans; Infant; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome; Retrospective Studies; Treatment Outcome
PubMed: 34075816
DOI: 10.1177/10556656211011886 -
The Cleft Palate-craniofacial Journal :... Jan 2017Tongue-lip adhesion (TLA), mandibular distraction osteogenesis (MDO), and subperiosteal release of the floor of the mouth (SPRFM) are the most commonly performed... (Review)
Review
BACKGROUND
Tongue-lip adhesion (TLA), mandibular distraction osteogenesis (MDO), and subperiosteal release of the floor of the mouth (SPRFM) are the most commonly performed surgical procedures to treat severe airway obstruction in infants born with Pierre Robin sequence (PRS).
OBJECTIVES
To determine the rate of failure of each type of procedure, in terms of mortality and the need for tracheostomy, and to determine what proportion of infants have significant airway obstruction postoperatively as determined by polysomnography (PSG) and compare the data by procedure type.
METHOD
A comprehensive literature search (1981 through June 2015) was done of the National Library of Medicine database using PubMed. Extracted data included diagnosis, type of surgery, and outcome including mortality, need for postoperative tracheostomy and details of PSG. Persistence of significant airway obstruction was defined as an apnea-hypopnea index >15 events/h on PSG.
RESULTS
Both mortality rate and need for tracheostomy were low for all procedures. Many studies lacked sufficient detail to identify significant airway obstruction postoperatively. In studies with sufficient data, MDO was associated with the lowest percentage of significant airway obstruction postprocedure (3.6%) compared to 50% for infants who underwent TLA. Insufficient PSG data was available for patients who were treated with SPRFM.
CONCLUSIONS
There is a paucity of objective PSG data to definitively assess postoperative airway outcomes for PRS. MDO appears to be the most effective technique based on the available PSG data. Standardized use of PSG may lead to better identification and treatment of patients at risk for suboptimal airway outcomes postoperatively.
Topics: Airway Obstruction; Humans; Infant; Micrognathism; Osteogenesis, Distraction; Pierre Robin Syndrome; Polysomnography; Tracheostomy; Treatment Outcome
PubMed: 27414091
DOI: 10.1597/15-282 -
Clinical Oral Investigations Nov 2013Mandibular distraction osteogenesis (MDO) has been successfully applied in infants suffering Robin sequence (RS) with severe upper airway obstruction, but no comparative... (Review)
Review
OBJECTIVES
Mandibular distraction osteogenesis (MDO) has been successfully applied in infants suffering Robin sequence (RS) with severe upper airway obstruction, but no comparative studies for the different types of MDO exist to date. The objective of the current study was to systematically review the published data considering this matter, providing a fundament for protocols and a more conscious treatment strategy for infants with RS in the near future.
MATERIAL AND METHODS
For the period from January 1966 to January 2012, the Pubmed, EMBASE, and Cochrane Library databases were searched. Abstracts were screened based on predetermined selection criteria. Relevant full-text articles were retrieved. The articles were analyzed on the type of MDO used, preoperative workup, patient characteristics, postoperative outcome, and complications.
RESULTS
The search yielded 109 articles. After checking abstracts and full texts on predetermined inclusion and exclusion criteria, 12 studies (four describing external MDO, five internal MDO, and three both types) were extracted for further analyses.
CONCLUSION
Internal MDO seems very feasible in infants suffering RS, minimizing side effects such as hypertrophic scarring, nerve damage, and extensive care needs, although the indications for usage are more limited compared to the external device. Corresponding protocols and long-term outcome studies are needed to make a better comparison and the use and indication of the different types of distraction even more distinct.
CLINICAL RELEVANCE
A base for a guideline to support the choice of a designated operative management for neonates with RS is provided, hereby obviating possible complications of the different types of MDO in the future.
Topics: Humans; Infant; Infant, Newborn; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome
PubMed: 23722462
DOI: 10.1007/s00784-013-0998-z -
Journal of Clinical Sleep Medicine :... Aug 2021Identifying optimal treatment for infants with Robin sequence (RS) is challenging due to substantial variability in the presentation of upper airway obstruction (UAO) in...
STUDY OBJECTIVES
Identifying optimal treatment for infants with Robin sequence (RS) is challenging due to substantial variability in the presentation of upper airway obstruction (UAO) in this population. Objective assessments of UAO and treatments are not standardized. A systematic review of objective measures of UAO was conducted as a step toward evidence-based clinical decision-making for RS.
METHODS
A literature search was performed in the PubMed and Embase databases (1990-2020) following PRISMA guidelines. Articles reporting on RS and UAO treatment were included if the following objective measures were studied: oximetry, polysomnography, and blood gas. Quality was appraised by the methodological index for nonrandomized studies (range: 0-24).
RESULTS
A total of 91 articles met the inclusion criteria. The mean methodological index for nonrandomized studies score was 7.1 (range: 3-14). Polysomnography was most frequently used (76%) followed by oximetry (20%) and blood gas (11%). Sleep position of the infant was reported in 35% of studies, with supine position most frequently, and monitoring time in 42%, including overnight recordings, in more than half. Of 71 studies that evaluated UAO interventions, the majority used polysomnography (90%), of which 61% did not specify the polysomnography technique. Reported polysomnography metrics included oxygen saturation (61%), apnea-hypopnea index (52%), carbon dioxide levels (31%), obstructive apnea-hypopnea index (27%), and oxygen desaturation index (16%). Only 42 studies reported indications for UAO intervention, with oximetry and polysomnography thresholds used equally (both 40%). In total, 34 distinct indications for treatment were identified.
CONCLUSIONS
This systematic review demonstrates a lack of standardization, interpretation, and reporting of assessment and treatment indications for UAO in RS. An international, multidisciplinary consensus protocol is needed to guide clinicians on optimal UAO assessment in RS.
CITATION
Logjes RJH, MacLean JE, de Cort NW, et al. Objective measurements for upper airway obstruction in infants with Robin sequence: what are we measuring? A systematic review. . 2021;17(8):1717-1729.
Topics: Airway Obstruction; Humans; Infant; Oxygen Saturation; Pierre Robin Syndrome; Polysomnography; Sleep
PubMed: 33960296
DOI: 10.5664/jcsm.9394 -
The Cleft Palate-craniofacial Journal :... Mar 2016Objective To gather data from relevant experimental and observational studies to determine the relationship between micrognathia and cleft palate. The goal is to raise... (Review)
Review
Objective To gather data from relevant experimental and observational studies to determine the relationship between micrognathia and cleft palate. The goal is to raise awareness and motivate clinicians to consider the cause and effect relationship when confronted with patients with cleft palate, even if there is no clearly noticeable mandibular abnormality. Design Several electronic databases were systematically examined to find articles for this review, using search terms including "cleft palate," "micrognathia," "tongue," and "airway obstruction." PubMed was the source of all the articles chosen to be included. Exclusion criteria included case reports, articles focused on treatment options, and articles only tangentially related to cleft palate and/or micrognathia. Results A total of 930 articles were screened for relevance, and 82 articles were chosen for further analysis. Evidence gathered in this review includes a variety of etiological factors that are causative or associated with both micrognathia and cleft palate. Observational studies relating the two abnormalities are also included. Much of the included literature recognizes a cause-and-effect relationship between micrognathia and cleft palate. Conclusion On the basis of the published data, we suggest that micrognathia does induce cleft palate in humans and animals. With knowledge of this causative relationship, clinicians should consider the importance of gathering cephalometric data on the mandibles and tongues of patients presenting with isolated cleft palate to determine whether they have micrognathia as well. With more data, patterns may emerge that could give insight into the complex etiology of nonsyndromic cleft palate.
Topics: Animals; Cephalometry; Cleft Palate; Humans; Micrognathism
PubMed: 25658963
DOI: 10.1597/14-238