Robin Sequence Patients Research Articles

Predictors of Respiratory Dysfunction at Diagnosis of Robin Sequence.

Robin sequence (RS) consists of associated micrognathia, glossoptosis, and respiratory dysfunction, with or without cleft palate. Studies on how different patient characteristics impact the severity of respiratory dysfunction are scarce and contradictory; this study investigates how different features affect respiratory obstruction severity at diagnosis of RS in controlled analysis. Retrospective cohort study that enrolled 71 RS patients under 90 days old who received care in our institution from 2009 to 2020. The primary outcome, respiratory dysfunction, was categorized into four severity groups and analyzed using a multinomial logistic regression model that considered age, sex, mandible length, cleft palate, syndromic diagnosis, other airway anomalies, and degree of glossoptosis. Mandible length, syndromic diagnosis, and Yellon grade 3 glossoptosis were related to poorer respiratory outcomes (need for respiratory support). In univariate analysis, for each additional 1 mm of mandible length at diagnosis, a mean reduction of 28% in the risk of needing respiratory support was observed (OR=0.72; 0.58-0.89); syndromic diagnosis and grade 3 glossoptosis also raised the risk (OR=6.50; 1.59-26.51 and OR=12.75; 1.03-157.14, respectively). In multivariate analysis, only mandible length significantly maintained its effects (OR=0.73; 0.56-0.96), a 27% reduction. Mandible length was an independent predictor for more severe respiratory dysfunction in RS patients, with larger mandibles showing protective effects. Syndromic diagnosis and Yellon grade 3 glossoptosis are also likely to be associated with poorer respiratory outcomes, although this was not demonstrated in multivariate analysis. 4 Laryngoscope, 131:2811-2816, 2021.

Respiratory sleep disorders, nasal obstruction and enuresis in children with non-syndromic Pierre Robin sequence

IntroductionObstructive sleep apnea is highly prevalent in non-syndromic Pierre Robin sequence patients. Studies have found a probable relationship between obstructive sleep apnea and nasal obstruction and between obstructive sleep apnea and enuresis. Assessment of the relationship between these variables in non-syndromic Pierre Robin sequence patients is scarce. ObjectiveThe present study aims to evaluate the relationship between symptoms of obstructive sleep apnea, nasal obstruction and enuresis, determining the prevalence of symptoms suggestive of these conditions, in schoolchildren with non-syndromic Pierre Robin sequence, and describe the prevalence of excessive daytime sleepiness habitual snoring and voiding dysfunction symptoms associated with enuresis. MethodsThis was a prospective analytical cross-sectional study developed at a reference center. Anthropometric measurements and a structured clinical interview were carried out in a sample of 48 patients. The instruments “sleep disorders scale in children” “nasal congestion index questionnaire” (CQ-5), and the “voiding dysfunction symptom score questionnaire” were used. Statistical analysis was performed for p < 0.05. ResultsPositive “sleep disorders scale in children” scores for obstructive sleep apnea and CQ-5 for nasal obstruction were observed in 38.78% and 16.33%, respectively. Enuresis was reported in 16.33% of children, being characterized as primary in 71.43% and polysymptomatic in 55.55%; according to the “voiding dysfunction symptom score questionnaire”. There was a significant relationship between nasal obstruction and obstructive sleep apnea symptoms (p < 0.05), but no significance was found between obstructive sleep apnea symptoms and enuresis, and between nasal obstruction and enuresis. The prevalence of excessive daytime sleepiness was 12.24% and of habitual snoring, 48.98%. A family history of enuresis, younger age in years and a positive “voiding dysfunction symptom score questionnaire” score were associated with a higher prevalence of enuresis (p < 0.05). ConclusionChildren with non-syndromic Pierre Robin sequence are at high risk for obstructive sleep apnea symptoms and habitual snoring, with a correlation being observed between nasal obstruction and obstructive sleep apnea symptoms. In addition, the study showed that non-syndromic Pierre Robin sequence, obstructive sleep apnea and nasal obstruction symptoms were not risk factors for enuresis in these patients.

A Comparison of Weight Gain Following Mandibular Distraction Osteogenesis and Tongue-Lip Adhesion in the Treatment of Robin Sequence.

Obstructive respiratory stress and feeding difficulties in infants with Robin sequence (RS) may result in poor weight gain or loss. Following failure of conservative treatment, surgical options include mandibular distraction osteogenesis (MDO) and tongue-lip adhesion (TLA). Whilst both techniques have demonstrated to improve airway patency and feeding behavior, an advantage of either in restoring weight growth remains unknown. This study aimed to improve procedural selection by examining weight gain following MDO and TLA. A retrospective chart review was performed for 17 RS patients that had undergone MDO and 25 that had received TLA. The mean body weight in both groups was below the 50th population percentile at birth and fell further in the period before surgery. A mixed model analysis demonstrated that postoperative weight gain depended on the progression of time and preoperative weight. Conversely, biological sex, congenital comorbidities, method of feeding, the respective cleft team, and the type of surgery did not significantly influence the evolution of postoperative body weight. In conclusion, both MDO and TLA were able to restore weight growth in infants affected by RS, though a clear superiority of either technique could not be established.

Severity of Airway Obstruction May Not Correlate With Weight Gain or Failure to Thrive in Infants With Robin Sequence: A Pilot Study.

Patients with Robin sequence (RS) can present with varying degrees of upper airway obstruction, difficulty maintaining adequate weight gain, and failure to thrive (FTT). Although inductive reasoning would suggest that these issues should be interrelated, the relationships between these factors have not been formally studied. This investigation explores the correlation between polysomnographic (PSG) findings, weight gain, and FTT in patients with RS. A prospective database for baseline PSG parameters and serial weight measurements in infants with RS who were admitted for airway obstruction was reviewed. The association between PSG variables and calorie intake with FTT was assessed using univariate and multivariable logistic regression. Categorical analysis of the PSG variables against FTT was explored with a Poisson regression, and linear regression was performed to evaluate the correlation between PSG parameters and percentage of weight gain. Univariate and multivariate logistic regression in RS patients with (n = 13) and without (n = 20) FTT showed no significant association between apnea-hypopnea index (adjusted odds ratio [aOR]: 0.99, P-value = 0.403), O2 nadir (aOR: 0.98, P-value = 0.577), time of O2 saturation below 90% (aOR: 1.03, P-value = 0.574), maximum end tidal carbon dioxide (aOR: 1.0, P-value = 0.977), and average calorie intake (OR:1.02; P-value = 0.984). Furthermore, no significant associations were identified between these variables and weight gain. This pilot study questions the widely held and intuitively logical belief that poor weight gain and/or FTT should correlate with the severity of upper airway obstruction in patients with RS. Large prospective investigations should be initiated to better explore the authors' findings. Our results also underscore the importance of individualized treatment for these challenging patients.

Robin Sequence: 5-Year Speech Outcomes-A Case-Control Study.

Pierre Robin sequence (Robin sequence) is defined as the triad of micrognathia, glossoptosis, and airway obstruction. It is frequently associated with palatal clefting. In recent years, increased interest in speech outcomes of cleft patients diagnosed with Robin sequence has been shown. Speech outcomes of cleft patients with Robin sequence were assessed at age 5 in comparison with a cleft palate-only cohort. Speech parameters were evaluated according to the Cleft Audit Protocol for Speech-Augmented and analyzed using the National Audit Standards for Speech (United Kingdom). All patients were treated in the same institution during the same period (2005 to 2012). Subjects who needed nasopharyngeal airway support and those whose airway was managed by positioning only were eligible. Fifty-one cleft patients diagnosed with Robin sequence were included in this study. Outcomes were compared to those of 128 nonsyndromic cleft palate-only patients.Patients with Robin sequence were shown to present with a significantly higher rate of cleft speech characteristics in comparison to the reference cohort (p = 0.001). Furthermore, it was shown that Robin sequence is associated with a significantly higher rate of secondary speech surgery for velopharyngeal dysfunction before the age of 5 (p = 0.016). Robin sequence patients with a nasopharyngeal airway presented with a higher rate of cleft speech characteristics compared to Robin sequence patients managed with positioning only. Cleft patients with Robin sequence are more likely to need further surgery to correct velopharyngeal dysfunction before the age of 5 and are more prone to present with cleft speech characteristics at the age of 5. Risk, II.

Spatial Analysis of the Temporomandibular Joint and Condyle Following Mandibular Distraction in Robin Sequence.

To comprehensively assess changes in temporomandibular joint and mandibular condyle morphology in Robin sequence (RS) patients undergoing mandibular distraction osteogenesis (MDO). A retrospective analysis of pediatric patients with RS and functional temporomandibular joints who underwent MDO by a single surgeon was performed. Preoperative and postoperative computed tomography (CT) scans were morphometrically analyzed three-dimensional in 3-matic and Mimics (Materialise). Comparative analysis was performed using Wilcoxon signed-rank tests. Fourteen RS patients were included (28 condyles, 56 CT scans), 78% male and 22% females. The mean age at surgery was 9.4 weeks (range 1.6-46.7 weeks). The average age at initial CT was 5.3 days (range 0-11 days). The mean time interval for CT scan before MDO and after hardware removal were 8.8 ± 6.4 days and 11.2 ± 25.8 days, respectively. Rotatory changes of the condyle revealed a significantly decreased horizontal angle following MDO (-7.55°, 95% confidence interval -11.13° to -3.41°; P < 0.001). Anteroposteriorly, the angle between condylar process and ramus (incline) increased significantly (14.14°, 95% confidence interval 10.71°-19.59°; P < 0.001). Intragroup analysis revealed no difference between left versus right condylar position measurements. The condyle itself increased in size and length and the intercondylion distance increased post-MDO. There were no complications and all patients achieved relief of airway obstruction without tracheostomy. Morphologic and positional changes of the mandibular condyle following MDO in RS patients parallel changes that occur during normal development in non-RS patients. As a result, MDO may facilitate normal condylar morphology and function in RS patients.

Perioperative complications in cleft palate repair with Robin sequence following Tuebingen palatal plate treatment

Our study aimed to evaluate perioperative complications following our institutional pre- and intraoperative management in cleft palate repair with Robin sequence (RS).RS patients who underwent cleft palate repair between 2000 and 2020 were retrospectively analysed. RS children with complete documentation and whose initial treatment involved the Tuebingen palatal plate (TPP) were included. Clinical records and operative charts were reviewed with regard to clinical characteristics as well as the neonatal and perioperative course. Results before and after adjustment of the anesthesiology protocol in 2014 were compared.143 RS patients (41% male, 59% female) were included. Median pretherapeutic mixed-obstructive apnea index (MOAI) after birth was 9.4/hour (IQR 20.0). TPP treatment was associated with normalisation of the MOAI and adequate weight gain until surgery. At surgery, median age was 10 months (IQR 3), MOAI 0.1/h (IQR 0.5), and weight 8.7 kg (IQR 1.7). In 93% of cases (n = 133), the postoperative course was uneventful. Refinement of the anesthesiology protocol showed positive effects on the perioperative course and led to a reduction in perioperative events (10.7% vs. 2.9%). No severe perioperative complications occurred.We recommend the adoption of TPP treatment in the therapy of RS children. Our favourable results show that early TPP treatment minimizes perioperative complications in cleft palate repair by effectively and sustainably correcting upper airway obstruction.

Long-term Speech Outcomes of Cleft Palate Repair in Robin Sequence versus Isolated Cleft Palate.

This retrospective case series of consecutive syndromic and isolated RS- and ICP-patients (1990-2016) includes indications and outcomes of straight-line repair with intravelar veloplasty (SLIV) or Furlow repair depending on cleft and airway characteristics. Seventy-five RS and 83 ICP patients underwent CP repair. Velopharyngeal insufficiency (VPI) occurred in 41% of RS versus 17% of ICP patients (P = 0.012), and in 60% of patients with syndromic RS versus 16% with isolated RS (P = 0.005). In multivariable logistic regression analysis, wider and more severe CP anatomy was the only factor independently associated with VPI (P = 0.028), in contrast to age at repair, syndromic RS compared with isolated RS, and isolated RS compared with ICP and initial tongue-lip adhesion. Secondary Furlow after primary SLIV was used to treat VPI in all groups, and more frequently in syndromic versus isolated RS patients (P = 0.025). Variability of RS anatomy and airway compromise necessitates individualized treatment protocols. Despite differing CP etiology and other variables, our findings demonstrate cleft anatomy as the only independent variable predictive of VPI comparing RS and ICP patients. Patients with isolated RS should ultimately attain similar VPI outcomes compared with ICP patients. Obstructive speech operations in RS patients can be avoided without compromising speech outcome by reserving the prsocedure for secondary cases.

Palatal Re-Repair With Z-Plasty in Treatment of Velopharyngeal Insufficiency of Syndromic and Nonsyndromic Patients With Cleft Palate.

Velopharyngeal insufficiency (VPI) often results from palatal shortening or insufficient levator function after cleft palate repair. To assess the efficacy of palatal re-repair with Z-plasty in treatment of VPI for patients with isolated cleft palate (ICP). This retrospective analysis comprised 130 consecutive patients who had ICP with VPI that required Z-plasty as secondary surgery between 2008 and 2017. Pre- and post-operative evaluation of velopharyngeal function was done perceptually and instrumentally by Nasometer. Median patient age at Z-plasty was 6.8 years (range 3.0-20.1). Of the 130 patients, preoperatively VPI was severe in 73 (56%), mild-to-moderate in 55 (42%), and borderline in 2 (2%). Postoperatively, 105 (81%) of patients achieved adequate (normal or borderline) velopharyngeal competence and 16 (12%) required second operation for residual VPI. The success rate was 84% in nonsyndromic patients, 79% in nonsyndromic Pierre Robin sequence patients, and 58% in syndromic patients. In syndromic children, the speech outcome was significantly worse than in nonsyndromic children (P = 0.014). Complications included wound healing problems in 3 patients (2%), mild infection in 1 patient (1%), postoperative bleeding in 1 (1%), and postoperative fistula in 2 (2%). Palatal re-repair with Z-plasty is a safe operation for VPI correction in patients with ICP with a success rate of 81%. In syndromic patients, the procedure did not seem to work as well as in nonsyndromic patients.

Long-Term Orthognathic Considerations in the Pierre Robin Sequence Patient.

One of the arguments against early intervention for micrognathia in Pierre Robin sequence is the concept that the growth of the mandible will eventually "catch up." Long-term growth of the mandible and occlusal relationships of conservatively managed Pierre Robin sequence patients remain unknown. In this study, the authors evaluated the orthognathic surgery requirements for Pierre Robin sequence patients at skeletal maturity. Orthognathic surgical requirements of conservatively managed Pierre Robin sequence and isolated cleft patients (aged ≥13 years) at two institutions were reviewed and analyzed using t test, chi-square test, and Fisher's exact test. Values of p < 0.05 were considered statistically significant. Of the Pierre Robin sequence patients (n = 64; mean age ± SD, 17.9 ± 2.9 years), 65.6 percent were syndromic (primarily Stickler and velocardiofacial syndrome), 96.9 percent had a cleft palate, and 39.1 percent required orthognathic surgery at skeletal maturity. Nonsyndromic and syndromic Pierre Robin sequence patients demonstrated no differences in occlusal relationships or mandibular surgery frequency. The majority of Pierre Robin sequence patients requiring mandibular advancement had a class II occlusion. Comparison of Pierre Robin sequence patients to isolated cleft palate patients (n = 17) revealed a comparable frequency of orthognathic surgery between the two; however, Pierre Robin sequence patients did require mandibular advancement surgery at a greater frequency than cleft palate patients (p = 0.006). The present study found that 39.1 percent of conservatively managed Pierre Robin sequence patients required orthognathic surgery at skeletal maturity, of which the vast majority required mandibular advancement for class II malocclusion. These data suggest that mandibular micrognathia in conservatively managed Pierre Robin sequence patients may not resolve over time and may require surgical intervention. Risk, II.

Imaging characteristics of the mandible and upper airway in children with Robin sequence and relationship to the treatment strategy

Some patients with Robin sequence (RS) may respond to conservative therapy, while others require surgery; however, there are currently no objective anatomical criteria to gauge surgical indication. The purpose of this study was to explore the imaging characteristics of the mandible and upper respiratory tract in children with RS and examine how individual morphometric parameters influence the treatment strategy. Cone beam computed tomography (CBCT) images were obtained from 57 children with RS. Twenty parameters were measured as potential surgical indicators. The children were divided into two groups according to surgical requirement: a non-surgical group (26 children) and a surgical group (31 children who underwent mandibular osteodistraction). Differences in the imaging parameters were compared between the groups. The mandibular ramus was shorter bilaterally (left, P = 0.047; right, P = 0.027) and the mandibular body was longer bilaterally (left, P = 0.030; right, P = 0.025) in the surgical group when compared to the non-surgical group. In addition, the mandibular angle bilaterally (left, P = 0.023; right, P = 0.003) and the cross-sectional area at the epiglottis tip (P = 0.010) were smaller in the surgical group. Compared to RS patients receiving conservative treatment, children requiring surgery have specific anatomical features of the mandible and upper airway that can be used as surgical indications.

Mid-Term Outcome of Mandibular Distraction Osteogenesis in Pierre Robin Sequence.

The aim of the present study was to present a single center's experience with mandibular distraction osteogenesis (MDO) in Pierre Robin sequence (PRS) patients. A retrospective chart review was performed to identify patients with PRS that underwent MDO at our institution from 2003 to 2012. Inclusion criteria were as follows:Evaluation included demographic information, postoperative complications, and surgical outcomes. Twenty-four patients met the inclusion criteria. No complications related to our distraction technique were reported. Most of the patients who had a tracheostomy were successfully decannulated and the rest were able to avoid a tracheostomy. Two patients had superficial infections that were treated conservatively with topical antibiotics. One patient, who was our first case in the series, required 3 episodes of distraction osteogenesis. Another patient demonstrated recurrent symptoms of obstructive sleep apnea after MDO and was treated with continuous positive airway pressure. Over-correction during MDO in PRS is an efficient method for preventing future airway problems. Patients who required a tracheotomy pre-distraction and cases in whom distraction was performed at older age (>2 months of age), had a lower success rate in achieving de-cannulation and a higher rate of complications. Laryngomalacia, gastro-esophageal reflux disease, cardiac, and GI anomalies are not associated with increased failure rates of MDO in PRS.

Case report of neonate Pierre Robin sequence with severe upper airway obstruction who was rescued by finger guide intubation

BackgroundPierre Robin Sequence (PRS) patients are known for their triad of micrognathia, glossoptosis, and airway obstruction. Their airway can be a challenge even for the most experienced pediatric anesthesiologist.Case presentationWe report the case of a 9 day old 3.5 kg boy diagnosed with PRS, cleft palate, and a vallecular cyst with severe upper airway obstruction. The combination of PRS, cleft palate and the presence of vallecular cyst made this a cascade reaction of difficult airway. Due to his unique anatomy, we didn’t appreciate how difficult his airway was until multiple attempts with high-tech equipment failed. Ultimately it was the finger guide intubation, this old technique without any equipment, that rescued this patient from lose of airway.ConclusionsThe boy was successfully rescued by finger guided intubation. Finger guide intubation should be added to the anesthesiologist’s newborn rescue intubation training.

Tongue Lip Adhesion in the Treatment of Robin Sequence: Respiratory, Feeding, and Surgical Outcomes.

Objective evaluation of the efficacy of tongue lip adhesion (TLA) in the management of Robin sequence (RS). Retrospective cohort study. Tertiary referral hospital. The craniofacial database of Amsterdam UMC, Vrije Universiteit Amsterdam was searched to identify infants with RS who underwent tong lip adhesion (TLA). Forty-one RS infants who underwent TLA from 1993 to 2016 were identified. TLA. The outcome measures were pre- and postoperative polysomnography results, nutritional status, weight gain, age at operation, hospital stay length, extubation time after TLA, and complications. Forty-one RS patients were included who had TLA at an average age of 26.6 days. In 16 cases a pre- and postoperative polysomnography was performed. In 13 of these cases (81.3%) improvement was observed, in 2 (12.5%) the results were inconclusive, and in 1 (6.3%) no improvement was seen. Patients were extubated after a mean of 2.2 days.The mean hospital stay was 40.2 days. Reintervention was needed in 7 patients because of a wound dehiscence. The mean age of TLA release was 9.7 months. At discharge, 9 (22%) children still needed total nutritional support for persistent feeding difficulties. The average growth from birth to adhesion release was 4.6 kg. This cohort demonstrates that TLA is a successful procedure in children with RS in terms of respiratory, feeding, and growth outcome. Only minor complications were seen in our cohort.

Treacher Collins Syndrome and Tracheostomy: Decannulation Using Mandibular Distraction Osteogenesis.

Treacher Collins syndrome (TC) and Pierre Robin sequence (RS) are associated with hypoplastic mandible, glossoptosis, and consequent airway obstruction. Although TC and RS are often grouped together, airway outcomes of bilateral mandibular distraction osteogenesis (MDO) have not been specifically studied in TC. The purpose of this study is to report on the clinical outcomes of MDO in the TC patient population. A twenty-year single-institution retrospective review of all patients with TC who underwent bilateral MDO was performed. Twenty-four patients were identified after exclusion due to different diagnoses or insufficient medical records. Data on comorbidities, airway status, MDO operations, and complications were collected. Data were compared with published clinical outcomes in RS and data for 13 RS patients from our institution. Surgical success, defined as prevention of imminent tracheostomy or successful decannulation within 1 year after primary distraction, was observed in 21% of TC patients and 65% of RS patients (P = 0.01). Repeat distraction was necessary for 11 TC patients (46%) and 1 RS patient. Complications were divided into minor, moderate, and major based on need for invasive management. Overall, 67% of TC patients had complications, 20% of which were major. Clinical outcomes to airway function after MDO are significantly inferior in patients with TC compared with patients with RS. Repeat MDO and longer course to decannulation are more prevalent in patients affected by TC.

Male individuals with Robin Sequence: emerging significant association with ABO and RhD blood group phenotypes

BackgroundThis study investigated the association of Robin Sequence with ABO and RhD blood group phenotypes. MethodsA retrospective cross-sectional study was performed of a cohort of Robin Sequence patients of the Hospital de Reabilitação de Anomalias Craniofaciais – Universidade de São Paulo (USP), Brazil. The study group was composed of 339 individuals of both genders with Robin Sequence referred for specific treatment. A control group was composed of 1780 individuals without syndromes. The groups were compared using the Pearson’ chi-square test (χ2) with statistical significance being defined for an alpha error of 5% (p-value<0.05). ResultsA comparison of gender found a significant difference for the AB phenotype between groups (p-value=0.007). Comparing blood type by gender there was no significant difference within the same group (p-value=0.117 and 0.388 respectively, for Robin Sequence and the control group). When comparing the AB blood type between groups, there was no difference for females (p-value=0.577), but there was a significant difference for males (p-value=0.0029). ConclusionsThis study showed that the population with Robin Sequence had different patterns related to gender concerning the phenotypic distribution of ABO and RhD blood group phenotypes. Robin Sequence is more common among females. The AB phenotype was significantly higher in males with Robin Sequence than in males of the Control Group. The prevalence of the RhD-negative phenotype is higher in individuals with Robin Sequence. This result suggests a possible association of ABO and RhD phenotypes with Robin Sequence that should be better investigated by molecular studies, as it deserves greater attention.

Association of polysomnographic parameters with clinical symptoms severity grading in Robin sequence patients: a cohort nested cross-sectional study

ObjectivesTo evaluate the association of polysomnographic parameters with clinical symptom severity in Robin sequence (RS) patients. MethodsAll patients diagnosed as presenting with RS at Hospital de Clínicas de Porto Alegre from October 2012 to June 2016 were enrolled. They were classified as isolated RS, RS-plus, and syndromic RS. Polysomnography (PSG) was performed, except for those patients in need of respiratory support. Symptom severity was evaluated as defined by the Cole et al. classification. Ordinal OR (for the chance of increase in one grade on the clinical severity scale) and R2 (determination coefficient from ordinal logistic regression) were computed from data analysis. ResultsA total of 80 participants were enrolled in the study. Fifty-five of these were able to undergo polysomnography. Worsening of the studied PSG parameters was associated with increase in clinical severity grading, as follows: desaturation index (OR 1.27; 95% CI; 1.07–1.51; R2 = 19.8%; p = 0.006); apnea/hypopnea Index (OR 1.13; 95% CI; 1.01–1.26; R2 = 12.5%; p = 0.02); sleep mean oxygen saturation (OR 0.16; 95% CI; 0.05–0.52; R2 = 22.6%; p = 0.002); oxygen saturation nadir (OR 0.73; 95% CI; 0.56–0.96; R2 = 10.0%; p = 0.02); percentage of time with oxygen saturation <90% (OR 9.49; 95% CI; 1.63–55.31, R2 = 37.6%; p = 0.012); and percentage of time presenting with obstruction (OR 2.5; 95% CI; 1.31–4.76; R2 = 25.1%; p = 0.006). ConclusionsPolysomnography parameters were associated with severity of clinical manifestations in patients with RS. Oxyhemoglobin saturation-based parameters had surprisingly significant R2 values. Therefore, those parameters, which have traditionally been undervalued in other clinical settings, should also be assessed in the polysomnographic evaluation of RS patients.

The Pierre Robin Mandible is Hypoplastic and Morphologically Abnormal.

For Pierre Robin sequence (PRS) patients, there is incomplete characterization of 3D differences and effects of mandibular distraction osteogenesis (MDO) on the mandible compared to normal controls. PRS infants who underwent MDO at 2 craniofacial referral centerals with pre- and postoperative computed tomography (CT) scans were identified. A group of age-matched control patients with CTs were identified in the PACS database. Demographic and perioperative data were recorded. Mandibular lengths, angles, and volumes were measured. Morphologic and outcomes data were analyzed in a case-control comparison. Sixty-three CT scans were analyzed. Fifteen pre-op PRS patient and 15 control CTs were well matched in terms of age and sex. Mandibular volume (78%), ramus length (87%), and body length (95%) were all decreased in the PRS patients. Anterior symphyseal angle (84%) was significantly reduced in PRS patients while mandibular angle (102%) was maintained. Eighteen post-op PRS patient and 15 control CTs were well matched in terms of age and gender. Mandibular volumes (106%) were normalized following distraction with shorter mandibular rami (88%) and longer mandibular bodies (109%). Postoperatively, mandibular angle (100%) and anterior symphyseal angle (99%) were ultimately indistinguishable from controls. The mandible in PRS is dysmorphic compared to age-matched controls. Overall, they have a smaller volume, shorter ramus, and an obtuse symphyseal angle. MDO improves mandibular volume and normalizes the symphyseal angle, but results in a longer mandibular body and shorter mandibular ramus.

Diagnostic accuracy of current glossoptosis classification systems: A nested cohort cross-sectional study.

To assess the performance of endoscopic grading systems of glossoptosis in identifying severe clinical manifestations in children with Robin sequence (RS). Nested cohort cross-sectional study. All RS patients diagnosed at Hospital Clinics of Porto Alegre from October 2012 to June 2016 were enrolled in this cohort. Patients underwent sleep endoscopy and were classified according to Yellon (Y) and de Sousa et al. (S) scales. Symptom severity evaluation was performed as defined by Cole et al. The outcome of interest was Cole's clinical classification grade 3. Eighty patients were eligible for analysis. Sensitivity (Y: 56.2%, S: 28.1%, P < .001) and specificity (Y: 85.4%, S: 93.8%, P = .038) in identifying severe clinical symptoms patients (i.e., Cole grade 3) were statistically different between Y and S classifications. A low but significant overall correlation was observed for both Y (rho = 0.372, P < .001) and S (rho = 0.439, P < .001) classifications when compared with Cole classification. Diagnostic odds ratio (DOR) for Y (DOR: 7.53, 95% confidence interval [CI]: 4.15-10.90) and S (DOR: 5.87, 95% CI: 1.86-9.87) were equivalent (P = .92). Also, receiver operating characteristic curves area under the curve were not significantly different between them. The positive likelihood ratio was 3.86 (95% CI: 1.82-8.16) and 4.50 (95% CI: 1.32-15.36) for Y and S, respectively. Y and S grading systems showed a low sensitivity and moderate to high specificity in detecting patients with severe clinical manifestations. Correlation between Y/S and Cole et al. grading were also considered low. Development of a more discriminative anatomic grading system is still needed for this specific disorder. 2b. Laryngoscope, 128:502-508, 2018.

Abstract 63. Long Term Assessment of Pierre Robin Sequence Patients Treated with a Vertically-Oriented Mandibular Distraction Vector

Abstract 63. Long Term Assessment of Pierre Robin Sequence Patients Treated with a Vertically-Oriented Mandibular Distraction Vector