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Journal of Cranio-maxillo-facial... Dec 2016There is widespread lack of consensus regarding treatment of airway obstruction in children with Robin Sequence. This study aimed to systematically summarize outcomes of... (Review)
Review
There is widespread lack of consensus regarding treatment of airway obstruction in children with Robin Sequence. This study aimed to systematically summarize outcomes of non-surgical and surgical options to treat airway obstruction in children with Robin Sequence. The authors searched the Medline, EMBASE and CENTRAL databases. Studies primarily on mandibular distraction were excluded. Study quality was appraised with the Methodological Index for Non-Randomized Studies (MINORS) score. Forty-eight studies were included, of which 45 studies had a retrospective non-comparative set up, two studies had a prospective design and one study was a clinical trial. The mean MINORS score was 7.3 (range 3-10). The rates of successful relief of the airway obstruction (SRoAO) were: not available for orthodontic appliance (2 studies, n = 24), 67-100% for nasopharyngeal airway (6 studies, n = 126); 100 % for non-invasive respiratory support (2 studies, n = 12); 70-96% for tongue-lip adhesion (11 studies, n = 277); 50-84% for subperiosteal release of the floor of the mouth (2 studies, n = 47); 100% for mandibular traction (3 studies, n = 133); 100% for tracheostomy (1 study, n = 25). The complication rate ranged from zero to 55%. Although SRoAO rates seemed comparable, high-level evidence remains scarce. Future research should include description of the definition, treatment indication, and objective outcomes.
Topics: Airway Obstruction; Child; Humans; Nasopharynx; Orthodontic Appliances; Pierre Robin Syndrome; Respiration, Artificial; Treatment Outcome
PubMed: 27876376
DOI: 10.1016/j.jcms.2016.06.021 -
Seminars in Fetal & Neonatal Medicine Dec 2021There are a wide range of surgical and maxillofacial options to alleviate upper airway obstruction in children with craniofacial disorders. The nasopharyngeal prong... (Review)
Review
There are a wide range of surgical and maxillofacial options to alleviate upper airway obstruction in children with craniofacial disorders. The nasopharyngeal prong (NPP) is a simple idea where the airway obstruction arising from the posteriorly placed tongue secondary to a small mandible can be overcome quickly and without resorting to more invasive surgical procedures. The role of the NPP is of particular interest in Robin sequence (RS). RS describes a congenital anomaly with retrognathia (often with associated U-shaped cleft palate) where in some children the tongue and small jaw can significantly impact on airway patency with upper airway obstruction. The NPP is a modified endotracheal tube, of ideal diameter and cut to a desired length that can bypass the airway obstruction and regain patency to the upper airway. RS has a natural history of mandibular growth and resolution of the symptoms with time and thus the NPP presents a medium term solution precluding the need in selected children of more permanent and potentially unnecessary surgical procedures. The role of the NPP in other craniofacial disorders (either as a temporary solution or longer term option) requires further research.
Topics: Airway Obstruction; Child; Cleft Palate; Humans; Mandible; Pierre Robin Syndrome; Tongue
PubMed: 34602353
DOI: 10.1016/j.siny.2021.101285 -
The Cleft Palate-craniofacial Journal :... Aug 2020Mandibular distraction osteogenesis (MDO) is the primary surgical intervention to treat airway obstruction in Pierre Robin sequence (PRS). Current morphologic studies of...
BACKGROUND
Mandibular distraction osteogenesis (MDO) is the primary surgical intervention to treat airway obstruction in Pierre Robin sequence (PRS). Current morphologic studies of PRS mandibles do not translate into providing airway management decisions. We compare mandibles of infants with nonsyndromic PRS to controls characterizing morphological variances relevant to distraction. We also examine how morphologic measurements and airway grades correlate with airway management.
METHODS
Patients with PRS under 2 months old were age and sex matched to controls. Demographic and perioperative data, and Cormack-Lehane airway grades were recorded. Computed tomography scans were used to generate mandibular models. Bilateral condylions, gonions, and the menton were identified. Linear and angular measurements were made. Wilcoxon rank sum and 2-sample tests were performed.
RESULTS
Twenty-four patients with PRS and 24 controls were included. Seventeen patients with PRS required MDO. PRS patients had shorter ramus heights (16.7 vs 17.3 mm; = .346) and mandibular body lengths (35.3 vs 39.3 mm; < .001), more acute gonial angles (125.3° vs 131.3°; < .001), and more obtuse intergonial angles (94.2° vs 80.4°; < .001) compared to controls. No significant differences were found among patients requiring MDO versus conservative management nor among distracted patients with high versus low airway grades.
CONCLUSION
Our study examines the largest and youngest PRS population to date regarding management of early airway obstruction with MDO. Our findings indicate that univector mandibular body distraction allows for normalization in nonsyndromic patients with PRS, and airway obstruction management decisions should remain clinical.
Topics: Airway Obstruction; Humans; Infant; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome; Retrospective Studies; Treatment Outcome
PubMed: 32253927
DOI: 10.1177/1055665620913780 -
The Journal of Laryngology and Otology May 2017To search for studies on tongue-lip adhesion and tongue repositioning used as isolated treatments for obstructive sleep apnoea in children with Pierre Robin sequence. (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To search for studies on tongue-lip adhesion and tongue repositioning used as isolated treatments for obstructive sleep apnoea in children with Pierre Robin sequence.
METHODS
A systematic literature search of PubMed/Medline and three additional databases, from inception through to 8 July 2016, was performed by two authors.
RESULTS
Seven studies with 90 patients (59 tongue-lip adhesion and 31 tongue repositioning patients) met the inclusion criteria. Tongue-lip adhesion reduced the mean (± standard deviation) apnoea/hypopnoea index from 30.8 ± 22.3 to 15.4 ± 18.9 events per hour (50 per cent reduction). The apnoea/hypopnoea index mean difference for tongue-lip adhesion was -15.28 events per hour (95 per cent confidence interval = -30.70 to 0.15; p = 0.05). Tongue-lip adhesion improved the lowest oxygen saturation from 75.8 ± 6.8 to 84.4 ± 7.3 per cent. Tongue repositioning reduced the apnoea/hypopnoea index from 46.5 to 17.4 events per hour (62.6 per cent reduction). Tongue repositioning improved the mean oxygen saturation from 90.8 ± 1.2 to 95.0 ± 0.5 per cent.
CONCLUSION
Tongue-lip adhesion and tongue repositioning can improve apnoea/hypopnoea index and oxygenation parameters in children with Pierre Robin sequence and obstructive sleep apnoea.
Topics: Child; Humans; Lip; Oxygen Consumption; Pierre Robin Syndrome; Severity of Illness Index; Sleep Apnea, Obstructive; Tissue Adhesions; Tongue; Treatment Outcome
PubMed: 28193305
DOI: 10.1017/S0022215117000056 -
The Laryngoscope Apr 2017Determine predictive patient characteristics that guide the decision to proceed with surgical management of tongue-based airway obstruction (TBAO) in Pierre Robin...
OBJECTIVES/HYPOTHESIS
Determine predictive patient characteristics that guide the decision to proceed with surgical management of tongue-based airway obstruction (TBAO) in Pierre Robin sequence (PRS) patients.
STUDY DESIGN
Retrospective review of PRS patients between 2005 and 2014 requiring observation in the neonatal intensive care unit (NICU).
METHODS
Patient charts were reviewed for prenatal diagnoses, clinical course, and need for surgical intervention (tracheotomy or mandibular distraction osteogenesis), and the nonsurgical and surgical group were compared with a logistic regression model.
RESULTS
Thirty-eight PRS patients who were identified with TBAO and required NICU observation had an average follow-up of 5.4 years. Associated anomalies identified in the PRS patients included neurologic disease (n = 6), renal abnormalities (n = 5), limb abnormalities (n = 4), and cardiac abnormalities (n = 12). Nonsurgical management of PRS TBAO included side (n = 12)/stomach positioning (n = 15), oral airway (n = 6), nasopharyngeal airway (NPA) (n = 14), and intubation (n = 12). Surgical intervention occurred in 13 patients, with tracheotomy in eight due to unstable airway, and mandible distraction in five due to NPA dependence. Factors with significant uncontrolled correlations with the need for surgical airways included presence of cardiac disease (P = .03), cardiac disease severity (P = .03), neurologic disease (P = .01), and continuous positive airway pressure (CPAP)/bilevel positive airway pressure (BiPAP) use (P = .006). Further, stepwise regression showed strong predictive value for CPAP/BiPAP use (odds ratio [OR]: 10.43) and presence of neurological disease (OR: 9.16). PRS TBAO patients required multiple modalities of noninvasive surgical intervention to stabilize their airway.
CONCLUSIONS
This study identified patient characteristics predictive of progression to a surgical airway to address TBAO, which may decrease healthcare utilization and improve quality of life for these families.
LEVEL OF EVIDENCE
4 Laryngoscope, 127:945-949, 2017.
Topics: Airway Management; Airway Obstruction; Clinical Decision-Making; Cohort Studies; Disease Progression; Female; Follow-Up Studies; Humans; Infant, Newborn; Intensive Care Units, Neonatal; Intubation, Intratracheal; Length of Stay; Logistic Models; Male; Monitoring, Physiologic; Odds Ratio; Patient Selection; Pierre Robin Syndrome; Predictive Value of Tests; Retrospective Studies; Risk Assessment; Severity of Illness Index
PubMed: 27378721
DOI: 10.1002/lary.26143 -
Archives of Disease in Childhood Feb 2024
Topics: Humans; Ireland; Pierre Robin Syndrome; United Kingdom
PubMed: 38199816
DOI: 10.1136/archdischild-2023-326079 -
The Cleft Palate-craniofacial Journal :... May 2022To assess the prevalence and patterns of hypodontia in nonsyndromic Pierre Robin sequence (PRS) and compare it with hypodontia in nonsyndromic isolated cleft palates and...
OBJECTIVE
To assess the prevalence and patterns of hypodontia in nonsyndromic Pierre Robin sequence (PRS) and compare it with hypodontia in nonsyndromic isolated cleft palates and isolated cleft lips.
DESIGN
Retrospective cohort study.
SETTING
Alder Hey Children's Hospital, United Kingdom.
PATIENTS
Patients with nonsyndromic PRS (group 1), isolated cleft palate (group 2), and isolated cleft lip (group 3).
MAIN OUTCOME MEASURES
Hypodontia in the permanent dentition assessed from orthopantomographs.
RESULTS
A total of 154 patients were included. Group 1 had the highest incidence of hypodontia with 47% having at least one tooth congenitally absent. Groups 2 and 3 had reduced rates of hypodontia with 27% and 19% of the groups missing teeth, respectively; 93% of cases of hypodontia in group 1 involved the absence of at least one second premolar. Of these patients, there was found to be bilateral agenesis of second premolars in 50% of cases.
CONCLUSIONS
Patients with PRS and cleft palates are more likely to have hypodontia than those with isolated cleft palates or unilateral cleft lips. Patients with PRS have more severe hypodontia than those with isolated cleft palates or unilateral cleft lips. Bilateral agenesis of lower second premolars is a commonly seen pattern among patients with PRS. In this large UK study, a similar prevalence and pattern of hypodontia to other nonsyndromic PRS populations worldwide has been demonstrated.
Topics: Anodontia; Bicuspid; Child; Cleft Lip; Cleft Palate; Humans; Pierre Robin Syndrome; Retrospective Studies
PubMed: 34018409
DOI: 10.1177/10556656211017778 -
The Laryngoscope Jul 2021The anatomy of children with severe Pierre Robin sequence can present a challenge for direct laryngoscopy and intubation. Advanced techniques including flexible...
OBJECTIVES/HYPOTHESIS
The anatomy of children with severe Pierre Robin sequence can present a challenge for direct laryngoscopy and intubation. Advanced techniques including flexible fiberoptic laryngoscopic intubation have been described but require highly specialized skill and equipment. Rigid video laryngoscopy is more accessible but has not been described in this population.
STUDY DESIGN
Retrospective cohort study.
METHODS
A retrospective review was completed at a tertiary care center of all children between January 2016 and March 2020 with Pierre Robin sequence who underwent a mandibular distraction osteogenesis procedure. Intubation events were collected, and a descriptive analysis was performed. A univariate logistic regression model was applied to direct laryngoscopy and flexible fiberoptic laryngoscopy with rigid video laryngoscopy as a reference.
RESULTS
Twenty-five patients were identified with a total of 56 endotracheal events. All patients were successfully intubated. Direct laryngoscopy was successful at first intubation attempt in 47.3% (9/19) of events. Six direct laryngoscopy events required switching to another device. Rigid video laryngoscopy was successful at first intubation attempt in 80.5% (29/36) of events. Two cases required switching to another device. Flexible fiberoptic laryngoscopy was found successful at first intubation attempt in 88.9% (8/9) of events. Direct laryngoscopy was 4 times more likely to fail first intubation attempt when compared to rigid video laryngoscopy (P < .05). There was no significant difference between rigid video laryngoscopy and flexible fiberoptic laryngoscopy for intubation.
CONCLUSIONS
For children with Pierre Robin sequence rigid video laryngoscopy should be considered as a first attempt intubation device both in the operating room and for emergent situations.
LEVEL OF EVIDENCE
4 Laryngoscope, 131:1647-1651, 2021.
Topics: Adolescent; Airway Obstruction; Child; Child, Preschool; Equipment Failure; Female; Humans; Infant; Infant, Newborn; Intubation, Intratracheal; Laryngoscopes; Laryngoscopy; Male; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome; Retrospective Studies; Severity of Illness Index; Treatment Outcome
PubMed: 33300625
DOI: 10.1002/lary.29262 -
Plastic and Reconstructive Surgery Aug 2018There is a paucity of literature directly comparing tongue-lip adhesion versus mandibular distraction osteogenesis in surgical treatment of patients with Pierre Robin... (Comparative Study)
Comparative Study Review
There is a paucity of literature directly comparing tongue-lip adhesion versus mandibular distraction osteogenesis in surgical treatment of patients with Pierre Robin sequence. This study comprehensively reviews the literature for evaluating airway and feeding outcomes following mandibular distraction osteogenesis and tongue-lip adhesion. A search was performed using the MEDLINE and Embase databases for publications between 1960 and June of 2017. English-language, original studies subjects were included. Extracted data included prevention of tracheostomy (primary airway outcome) and ability to feed exclusively by mouth (primary feeding outcome). A total of 67 studies were included. Ninety-five percent of subjects (657 of 693) treated with mandibular distraction osteogenesis avoided tracheostomy, compared to 89% of subjects (289 of 323) treated with tongue-lip adhesion. Eighty-seven percent of subjects (323 of 370) treated with mandibular distraction osteogenesis achieved full oral feeds at latest follow-up. Seventy percent of subjects (110 of 157) treated with tongue-lip adhesion achieved full oral feeds at latest follow-up. The incidence of second intervention for recurrent obstruction ranged from 4 to 6 percent in mandibular distraction osteogenesis studies, compared to a range of 22 to 45 percent in tongue-lip adhesion studies. Variability of patient selection, surgical techniques, outcomes measurement methods, and follow-up length across studies precluded meta-analysis of the data. Both mandibular distraction osteogenesis and tongue-lip adhesion are effective alternatives to tracheostomy for patients who fail conservative management and improve feeding. Mandibular distraction osteogenesis may be superior to tongue-lip adhesion in long-term resolution of airway obstruction and avoidance of gastrostomy, but is associated with notable complications.
Topics: Humans; Lip; Mandible; Osteogenesis, Distraction; Pierre Robin Syndrome; Plastic Surgery Procedures; Tongue; Treatment Outcome
PubMed: 29870511
DOI: 10.1097/PRS.0000000000004581 -
Otolaryngology--head and Neck Surgery :... Apr 2022To evaluate the performance of 4-dimensional computed tomography (4D-CT) in assessing upper airway obstruction (UAO) in patients with Robin sequence (RS) and compare the...
OBJECTIVES
To evaluate the performance of 4-dimensional computed tomography (4D-CT) in assessing upper airway obstruction (UAO) in patients with Robin sequence (RS) and compare the accuracy and reliability of 4D-CT and flexible fiber-optic laryngoscopy (FFL).
STUDY DESIGN
Prospective survey of retrospective clinical data.
SETTING
Single, tertiary care pediatric hospital.
METHODS
At initial and 30-day time points, a multidisciplinary group of 11 clinicians who treat RS rated UAO severity in 32 sets of 4D-CT visualizations and FFL videos (dynamic modalities) and static CT images. Raters assessed UAO at the velopharynx and oropharynx (1 = to 5 = complete) and noted confidence levels of each rating. Intraclass correlation and Krippendorff alpha were used to assess intra- and interrater reliability, respectively. Accuracy was assessed by comparing clinician ratings with quantitative percentage constriction (QPC) ratings, calculated based on 4D-CT airway cross-sectional area. Results were compared using Wilcoxon rank-sum and signed-rank tests.
RESULTS
There was similar intrarater agreement (moderate to substantial) with 4D-CT and FFL, and both demonstrated fair interrater agreement. Both modalities underestimated UAO severity, although 4D-CT ratings were significantly more accurate, as determined by QPC similarity, than FFL (-1.06 and -1.46 vs QPC ratings, = .004). Overall confidence levels were similar for 4D-CT and FFL, but other specialists were significantly less confident in FFL ratings than were otolaryngologists (2.25 and 3.92, < .0001).
CONCLUSION
Although 4D-CT may be more accurate in assessing the degree of UAO in patients with RS, 4D-CT and FFL assessments demonstrate similar reliability. Additionally, 4D-CT may be interpreted with greater confidence by nonotolaryngologists who care for these patients.
Topics: Child; Four-Dimensional Computed Tomography; Humans; Laryngoscopy; Pierre Robin Syndrome; Prospective Studies; Reproducibility of Results; Retrospective Studies
PubMed: 34253111
DOI: 10.1177/01945998211027353