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Objective: To compare growth trends among infants with Pierre Robin sequence (PRS) to normal World Health Organization (WHO) growth standards.Study Design: Case series with chart review.Subjects and Methods: Twenty-four infants with syndromic and nonsyndromic PRS (54% male) treated at an urban academic medical center between 2009 and 2017 were included. Infants with symptomatic hypoventilation underwent mandibular distraction osteogenesis (71%). Weights were recorded at roughly 1- to 3-month intervals from birth to 12 months, with ages adjusted for prematurity. The 50th percentile (P50) for this cohort was calculated and compared to WHO standards.Results: In total, 135 weight entries for 24 subjects were included. The birth weight P50 was similar to the WHO standard (females: 0.09 kg above WHO [95% CI, -0.25 to +0.43; z score = 0.19]; males: 0.38 kg below WHO [95% CI, -0.77 to 0.00; z score = -0.79]). A slower growth rate was noted among female and male infants with PRS: in month 5, the PRS P50 among females was 1.42 kg below the WHO standard (95% CI, -1.77 to -1.07; z score = -1.64). Among males in month 3, the PRS P50 was 1.68 kg below the WHO standard (95% CI, -2.12 to -1.24; z score = -2.19). By month 12, weight deficiencies had resolved in both groups.Conclusion: Newborns with and without PRS may have similar birth weights, but the growth rate among male and female infants with PRS may lag behind that of unaffected infants, even when upper airway obstruction has been addressed in early infancy. [ABSTRACT FROM AUTHOR]

Objectives: First arch syndromes are congenital defects caused by failure of neural crest cells to migrate into the first branchial arch. First arch syndrome is classified into 2 main clinical manifestations: Treacher Collins syndrome, characterized by bilateral underdevelopment of the zygomatic bones; and Pierre Robin sequence. The aim of this study was to describe the feasibility of visualization of the fetal zygomatic bone and assess its application in cases referred for features suggestive of first arch syndrome. Methods: A prospective cohort study was conducted. The feasibility of visualization of the zygomatic bone was performed in 50 sequential fetuses with a normal anatomic scan between 12 and 24 weeks' gestation using 3‐dimensional sonography. Following this, cases referred for targeted scans for suspected first branchial arch syndrome were assessed for the presence or absence of the zygomatic bones. Results: Visualization of the fetal zygomatic bone was feasible in all low‐risk cases. Cases referred for targeted scans included 11 isolated cases of micrognathia or retrognathia, 3 cases of microtia, and 3 cases of auricular or facial vestiges. Within this group, the zygomatic bones were visualized in all but 2 cases. No associated extrafacial malformations were detected; therefore, this phenotype was consistent with Treacher Collins syndrome. Conclusions: Prenatal imaging of the zygomatic bones offers a clinically based sonographic approach to cases referred for features suggestive of first arch syndrome and enables differentiation between the 2 main clinical manifestations: Treacher Collins syndrome and Pierre Robin sequence. [ABSTRACT FROM AUTHOR]

Pierre Robin sequence (PRS) is a craniofacial abnormality comprising micrognathia, glossoptosis and airway obstruction, which can impair the newborn's feeding and breathing. While there has been much research around the cause of PRS and most appropriate methods of care, understanding the psychosocial aspects of a PRS diagnosis from the parents' perspective is lacking. The aim of this study is to understand parental experiences of having a child diagnosed with PRS, as well as the role of genetic counselling in PRS. Fourteen semi-structured interviews were conducted with parents of children diagnosed with isolated PRS between 2 and 5 years prior. From these 14 interviews, eleven transcripts were analysed to find common themes and experiences. The diagnosis was confusing and overwhelming for participants during emotionally sensitive periods and little was understood about the cause of their child's PRS. Those participants who did recall experiences with genetic services reported that they were minimal and uninformative. According to participant recollection, genetic counselling was rarely offered, despite there being a potential for this service in PRS. Genetic counselling would be a valuable source of information and support for parents both at the time of antenatal diagnosis, and potentially 6 to 12 months later in the outpatient environment when these children are all routinely reviewed by their clinical care team. [ABSTRACT FROM AUTHOR]

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 t 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; P =.346) and mandibular body lengths (35.3 vs 39.3 mm; P <.001), more acute gonial angles (125.3° vs 131.3°; P <.001), and more obtuse intergonial angles (94.2° vs 80.4°; P <.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. [ABSTRACT FROM AUTHOR]

Objectives: (1) To assess the evolution of prepalatoplasty sleep oximetry (PRESO) and postpalatoplasty sleep oximetry (POSSO) in cleft patients and (2) to evaluate the impact of the size of the nasopharynx on PRESO and POSSO values. Study Design: Retrospective cohort study. Patients and Methods: In 81 patients with cleft palate and/or cleft lip, the following data were prospectively collected: patient demographics and prepalatoplasty cleft palate measurements. All the patients had at least 1 PRESO and POSSO. A Kaplan-Meier curve was obtained from all the sleep oximetry results. Transverse nasopharyngeal area (TNA) pre- and postvalues were compared for each group with paired t tests, while analysis of variance was used to compare TNA pre- and postscores between the groups with a Bonferroni correction for multiple comparisons. Results: POSSO results were normal or showed mild desaturations in most patients in the few weeks following palatoplasty. For the cohort, no statistically significant changes were found between PRESO and POSSO values. A 2-fold variation in the area of the TNA was found before palatoplasty within identical cleft malformation cases. No statistically significant association was found between the TNA or the a /30 − b 1 parameter values and the sleep study scores. Conclusions: The patients with the smaller nasopharyngeal areas presented identical PRESO and POSSO results when compared to those with larger nasopharyngeal sizes. Future studies should address the possible association between prepalatoplasty and postpalatoplasty TNAs and the occurrence of velopharyngeal deficiency later in life. [ABSTRACT FROM AUTHOR]

Objective: To investigate airway morphology changes in patients with Pierre Robin sequence (PRS) pre–/post–mandibular distraction osteogenesis (MDO) and to compare morphologic changes to age-matched controls. Design: Retrospective case–control study. Setting: Urban, academic, tertiary medical center. Patients, Participants: Fifteen patients with PRS after MDO to relieve upper airway obstruction (UAO) (2008-2018); age-matched controls for post-MDO patients. Interventions: Mandibular distraction osteogenesis, curvilinear internal mandibular distractors. Main Outcome Measures: (1) Physiologic improvement after MDO (apnea–hypopnea index; minimum oxygen saturation); (2) airway size (volume, surface area, length, mean/minimum cross-sectional area), shape (lateral:anterior–posterior ratio, cross-sectional area ratios, uniformity, sphericity), and changes with MDO; and (3) post-MDO airway size, shape versus age-matched controls. Results: Airway size increased after MDO (volume, P =.01; surface area, P =.02; length, P =.01), as did cross-sectional area (mean, P =.02; minimum, P =.02; minimum retropalatal, P =.05, mid-retroglossal, P =.02). Post-MDO PRS airways were larger than controls (volume, P <.01; surface area, P <.01; length, P <.01, cross-sectional area, P =.03). Airway shape remained nonuniform and flat post-MDO; control airways were round. Two syndromic patients required repeat MDO and had subphysiologic post-MDO airway cross-sectional area. Post-MDO PRS patients with supraphysiologic cross-sectional area along the entire airway had no UAO recurrence. Conclusions: In this small, heterogenous patient sample, MDO increases airway size, may preferentially affect the retropalatal airway, and often results in supraphysiologic airway dimensions. These retropalatal changes may be important in relieving severe UAO in patients with PRS. Generalizability of our results is limited by small cohort size and patient heterogeneity. [ABSTRACT FROM AUTHOR]

To determine weight gain during treatment with the modified palatal plate (MPP) in infants with isolated and syndromic Pierre Robin Sequence (PRS) suffering from micrognathia, upper airway obstruction (UAO), and failure to thrive (FTT), the authors conducted a retrospective study of infants treated with the MPP. The main outcome measure was infant weight (g) for up to three months after birth. Demographic and outcome data (associated syndromes, comorbidities, presence of cleft lip or palate, intubation attempts, tracheotomy and cleft repair) were collected. 14 children born January 2010 - December 2019 were included. The majority (86%) of infants showed highly significant weight gain (p < 0.001) within a 3-month period (mean pretreatment weight 3147 g with a SD of 425 g vs mean weight at three months 4435 g with a SD of 635 g). Syndromic PRS was found in 7% of infants. 43% of nonsyndromic PRS patients were found to have other congenital anomalies. Genetic testing showed normal karyotypes in 93% of infants and a microdeletion in 7% of infants. 21% of infants required tracheotomy, but no patients required mandibular distraction (MDO) or tongue-lip adhesion (TLA) to relieve UAO. PRS infants treated with the MPP showed highly significant weight gain within a 3-month period and did not require mandibular surgery for early airway management, but faster gain of weight might have implications for strategies to perform surgery at an earlier point in time. [ABSTRACT FROM AUTHOR]

Objective: To identify concepts and constructs important to parents of children with Pierre Robin Sequence (PRS). Design: Qualitative study. Setting: All children received some care at a tertiary hospital with additional care at outside facilities. Interviews were conducted in nonclinical locations, including remote locations. Participants: Parents of children <5 years old with a diagnosis of PRS. Prior treatments included observation, positioning, nasal trumpet, mandibular distraction osteogenesis, tracheostomy, and gastrostomy. Intervention: Semi-structured interviews with individuals (4) and with groups (focus groups, 4) were conducted using open-ended questions and non-leading prompts. Transcripts were analyzed with iterative open and axial coding. Concepts and constructs were identified and refined into codes and central themes. Interviews were conducted until thematic saturation was achieved. Results: Sixteen parents were interviewed. Their experiences were coded into 5 main themes, which can be summarized as: (1) child's symptoms/well-being, (2) parents' grief/isolation, (3) family stress, (4) relationships with providers, and (5) psychological and technical growth. Difficulty with feeding, weight gain, and breathing problems were core physical issues described by participants with associated intense fear. Participants described frustration from not only lack of care coordination, slow diagnoses, and poor communication but also gratitude for providers who served as advocates. Participants described gradual development of knowledge/competencies. Conclusions: Families of children with PRS have experiences that profoundly affect their lives. Child's physical symptoms/well-being and parents' psychosocial well-being provide content for a future PRS-specific quality-of-life instrument. Concepts that emerged also provide a framework to improve parents' experience and enhance their children's quality of care. [ABSTRACT FROM AUTHOR]

There are no standardized management algorithms for neonates with Pierre Robin sequence. Currently available literature is variable in terms of outcomes assessed across studies. In this paper, we have aimed to summarize the currently available literature on longitudinal sleep and respiratory outcomes in Pierre Robin sequence neonates with a focus on identifying gaps in literature and areas for future research development. [ABSTRACT FROM AUTHOR]

Objective: Methadone is a vital treatment for women with opioid use disorder in pregnancy. Previous reports suggested an association between methadone exposure and Pierre Robin sequence (PRS), a rare craniofacial anomaly. We assessed the association between gestational methadone exposure and PRS.Design/setting: This case-malformed control study used European Surveillance of Congenital Anomalies population-based registries in Ireland, the Netherlands, Italy, Switzerland, Croatia, Malta, Portugal, Germany, Wales, Norway and Spain, 1995-2011.Patients: Cases included PRS based on International Classification of Disease (ICD), Ninth Edition-British Paediatric Association (BPA) code 75 603 or ICD, Tenth Edition-BPA code Q8708. Malformed controls were all non-PRS anomalies, excluding genetic conditions, among live births, fetal deaths from 20 weeks' gestation and terminations of pregnancy for fetal anomalies. An exploratory analysis assessed the association between methadone exposure and other congenital anomalies (CAs) excluding PRS. Methadone exposure was ascertained from medical records and maternal interview.Results: Among 87 979 CA registrations, there were 127 methadone-exposed pregnancies and 336 PRS cases. There was an association between methadone exposure and PRS (OR adjusted for registry 12.3, 95% CI 5.7 to 26.8). In absolute terms, this association reflects a risk increase from approximately 1-12 cases per 10 000 births. A raised OR was found for cleft palate (adjusted OR 5.0, 95% CI 2.7 to 9.2).Conclusions: These findings suggest that gestational methadone exposure is associated with PRS. The association may be explained by unmeasured confounding factors. The small increased risk of PRS in itself does not alter the risk-benefit balance for gestational methadone use. The association with cleft palate, a more common CA, should be assessed with independent data. [ABSTRACT FROM AUTHOR]

Objective: Children with Pierre Robin sequence (PRS) and cleft palate have a high rate of velopharyngeal insufficiency (VPI) following primary palatoplasty. Our purpose was to determine the long-term incidence of speech-correcting surgeries (SCSs) and fistula rates in PRS after primary palatoplasty and the influence of possible causal factors. Design: A retrospective single-center, observational chart review study. Participants: After exclusion, the study cohort comprised 78 nonsyndromic PRS children (48 females) born between 1990 and 2009 and treated at the Cleft Palate and Craniofacial Center of Helsinki University Hospital, Finland. Causal factors included gender, surgeon, age at primary palatoplasty, surgical technique, airway obstruction in infancy, and cleft severity. We analyzed the outcome at age 8 years and at data retrieval, with a median follow-up of 14 years (range: 8-27 years). Results: Thirty-four (43.6%) children received SCS by age 8 years, and of the 19 (24.4%) postoperative fistulas, 6 (7.7%) underwent closure. At data retrieval, 37 (47.4%) children had undergone SCS and 8 (10.3%) had a fistula closure. Median age at SCS was 6 years. The results showed no significant association for gender, surgeon, age at primary palatoplasty, surgical technique, cleft severity, or airway obstruction in infancy regarding incidence of SCS, fistulas, or repaired fistulas. Conclusion: Pierre Robin sequence in children is associated with a high incidence of SCS and fistula formation, which necessitates accurate clinical follow-up and observation of speech development. The development of VPI in PRS is complex and most likely involving multiple factors. [ABSTRACT FROM AUTHOR]

The aim of this retrospective study is to determine the predictive factors of postoperative dyspnea in infants with Pierre Robin sequence (PRS). Forty children with PRS, who underwent general anesthesia, were retrospectively analyzed. The patient’s physiological status and anesthesiology data were collected accordingly, demographic characteristics including age, gender, height and weight at surgery, weight gain, preoperative airway status, tracheal intubation route, American Society of Anesthesiologists grading and airway Cormack–Lehane classification. Weight gain, dyspnea before the operation, Cormack–Lehane grade distribution showed a significant difference between patients with and without postoperative dyspnea (p = 0.0175, p = 0.0026, and p = 0.0038, respectively). Incompetent weight gain was identified as a predictor (p = 0.0371) of PRS postoperative dyspnea through the binary logistic regression model. In conclusion, this study established an early alerting model by monitoring the weight gain, dyspnea before the operation, Cormack–Lehane grade as potential combinations to predict the risk of postoperative dyspnea for PRS. [ABSTRACT FROM AUTHOR]

Infants diagnosed with Pierre Robin sequence frequently have airway obstruction. In severe cases of obstruction, mandibular distraction osteogenesis (MDO) can alleviate the airway blockage through elongation of the mandible and subsequent anterior placement of the tongue. However, there are several complications associated with MDO in the very young child. Among those are injuries to teeth that develop in the area of the MDO osteotomies. Such injuries include distalization and/or morphologic anomalies of primary and permanent molars. We describe a case of an unusual macro-dontia of the primary mandibular left second molar in a six-year-old male who underwent MDO as an infant. We believe that the mesial-distal elongation of the crown of the primary second molar occurred through distraction histogenesis of the tooth structures during the distraction of the mandible. We discuss the importance of preoperative planning to minimize such damages to the developing dentition. [ABSTRACT FROM AUTHOR]

Cleft lip and cleft palate (CP) are the most common facial malformations. Two-dimensional (2D) ultrasound (US) is the first-line examination in the prenatal diagnosis of CP. Three-dimensional, four-dimensional US and MRI provide a better detection of facial clefts. We present two fetuses with micrognathia and suspected secondary CP on 2D US: fetal tongue appeared in an unusual position (low tip and high dorsum position) and showed uncoordinated movements. MRI did not confirm the US suspicion, but at birth the two fetuses were affected by Pierre Robin sequence. [ABSTRACT FROM AUTHOR]

Abstract Background Interstitial microdeletion 14q22q23 is a rare chromosomal syndrome associated with variable defects: microphthalmia/anophthalmia, pituitary anomalies, polydactyly/syndactyly of hands and feet, micrognathia/retrognathia. The reports of the microdeletion 14q22q23 detected in the prenatal stages are limited and the range of clinical features reveals a quite high variability. Case presentation We report a detection of the microdeletion 14q22.1q23.1 spanning 7,7 Mb and involving the genes BMP4 and OTX2 in the foetus by multiplex ligation-dependent probe amplification (MLPA) and verified by microarray subsequently. The pregnancy was referred to the genetic counselling for abnormal facial profile observed in the first trimester ultrasound scan and micrognathia (suspicion of Pierre Robin sequence), hypoplasia nasal bone and polydactyly in the second trimester ultrasound scan. The pregnancy was terminated on request of the parents. Conclusion An abnormal facial profile detected on prenatal scan can provide a clue to the presence of rare chromosomal abnormalities in the first trimester of pregnancy despite the normal result of the first trimester screening test. The patients should be provided with genetic counselling. Usage of quick and sensitive methods (MLPA, microarray) is preferable for discovering a causal aberration because some of the CNVs cannot be detected with conventional karyotyping in these cases. To the best of our knowledge, this is the earliest detection of this microdeletion (occurred de novo), the first case detected by MLPA and confirmed by microarray. Literature review of the genotype-phenotype correlation in similar reports leads us to the conclusion that dosage imbalance of the chromosomal segment 14q22q23 (especially haploinsuffiency of the genes BMP4 and OTX2) contributes significantly to orofacial abnormalities. Association of the region with the Pierre Robin sequence appears to be plausible.

This is a brief clinical report describing an 18-month-old female with Robin sequence found to have an incidental mandibular cystic lesion on a head computed tomography scan in the preoperative workup before performing mandibular distraction. She underwent enucleation of the tumor, which was found to be a dentigerous cyst. One year following cyst enucleation, mandibular distraction was performed in order to alleviate her tongue-based obstruction. This case demonstrates the ability of the mandibular bone to successfully regenerate after undergoing cyst enucleation.

Gene trap mutagenesis is a powerful tool to create loss-of-function mutations in mice and other model organisms. Modifications of traditional gene trap cassettes, including addition of conditional features in the form of Flip-excision (FlEx) arrays to enable directional gene trap cassette inversions by Cre and Flpe site-specific recombinases, greatly enhanced their experimental potential. By taking advantage of these conditional gene trap cassettes, we developed a generic strategy for generating conditional mutations and validated this strategy in mice carrying a multipurpose allele of the Prdm16 transcription factor gene. We demonstrate that the gene trap insertion creates a null mutation replicating the Pierre Robin sequence-type cleft palate phenotype of other Prdm16 mutant mice. Consecutive breeding to Flpe and Emx1IREScre deleter mice spatially restricted Prdm16 loss to regions of the forebrain expressing the homeobox gene Emx1, demonstrating the utility of the technology for the analysis of tissue-specific gene functions.

INTRODUCTION: Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and upper airway obstruction. It is commonly associated with the secondary cleft palate. Infants with PRS commonly have sleep-disordered breathing (SDB); including obstructive sleep apnea (OSA) as well as central sleep breathing abnormalities that are present from infancy. AIM OF THE STUDY: Evaluate the prevalence and severity of SDB in infants with PRS using polysomnography (PSG). SETTINGS AND DESIGN: We retrospectively reviewed the sleep laboratory database at The Hospital for Sick Children, Toronto, during the period of May 2007 to March 2016. STATISTICAL ANALYSIS: Comparisons of PSG data were made between the OSA and non-OSA group using the Student′s t-test for age and body mass index, Wilcoxon signed ranks test for the continuous PSG data and Chi-squared test for the categorical variables. METHODS: Patients with PRS were identified and their initial PSG was selected for this study. The main indication for referral was ongoing concerns regarding OSA symptoms. RESULTS: A total of 46 patients (28 females) were included with a mean age (±standard deviation) of 0.8 (±0.3) year. Twenty-two out of 46 (47%) had evidence of OSA of which 10 had mild, 3 had moderate, and 9 had severe OSA. The PRS infants with OSA were younger than the non-OSA group. Significant correlations were found between desaturation and arousal indices with obstructive apnea-hypopnea index. CONCLUSION: This retrospective chart review confirms a high prevalence of OSA in this population. Prospective longitudinal studies are needed to evaluate the outcomes of OSA in PRS population.

Objectives: Compare the feeding management practices in infants with cleft palate with and without Pierre Robin sequence (PRS) and determine if specific feeding difficulties or interventions predict delayed palate repair., Design: Retrospective cross-sectional study., Setting: Seventeen cleft palate teams contributed data., Patients: 414 infants were included in this study: 268 infants with cleft palate only and 146 infants with cleft palate and PRS., Procedures: Data were collected via parent interview and electronic health records., Main Outcome Measures: Outcomes for the primary objective included categorical data for: history of poor growth, feeding therapy, milk fortification, use of enteral feeding, and feeding difficulties. The outcome for the secondary objective was age in months at primary palate repair., Results: Infants with PRS had a significantly higher prevalence of feeding difficulties (81% versus 61%) and poor growth (29% versus 15%) compared to infants with cleft palate only. Infants with PRS received all feeding interventions-including feeding therapy, milk fortification, and enteral feeding-at a significantly higher frequency. Infants with PRS underwent primary palate repair at a mean age of 13.55 months (SD = 3.29) which was significantly ( P  < .00001) later than infants with cleft palate only who underwent palate repair at a mean age of 12.05 months (SD = 2.36). Predictors of delayed palate repair included diagnosis of PRS as well as Hispanic ethnicity and a history of poor growth., Conclusions: These findings can be used to establish clinical directives focused on providing early, multimodal feeding interventions to promote optimal growth and timely palate repair for infants with PRS., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: Infants with significant retrognathia often have difficulty forming a latch adequate to establish exclusive breastfeeding. This article describes the use of at-breast supplementers (ABSs) to facilitate extended breastfeeding relationships, even when supplementation is necessary for growth. Methods: Two cases are described where infants with severe retrognathia initially struggled with weight gain necessitating supplementation but were able to ultimately exclusively feed at-breast with the use of ABSs. Results: While the two cases differed in the form of supplemental milk used and duration of ABS use, both dyads breastfed for beyond 2 years. Conclusions: Feeding solely at the breast and subsequent extended breastfeeding may be possible even for infants who require supplementation, including those with anatomical or functional challenges such as retrognathia. The ABS is a relatively simple system that may be beneficial for difficulties such as poor latch and low milk supply. More awareness and education is needed so that clinicians consider supporting dyads with this approach.

Objectives: 1. To assess the skeletal class occlusion and lateral cephalometry in children with isolated cleft palates (non-Robin sequence) and 2. to identify associations between these findings and pre-palatoplasty cleft palate measurements., Study Design: Retrospective cohort study., Setting: North American Institutional Tertiary Paediatric Center., Patients: Our cleft database was reviewed, and patients were included if they had an isolated cleft palate without a Robin Sequence diagnosis, had a Furlow palatoplasty and had available per operative cleft palate measurements and available lateral cephalogram between 6 and 8 years old. Thirty-two patients matched to inclusion criteria., Intervention: Furlow's Palatoplasty., Main Outcome and Measures: Cleft size at palatoplasty, cephalometric measurements and skeletal occlusal classes were analysed. ANOVA was used to test the association between cephalometric measurements and occlusal classes. Results are presented as means with a 95% confidence interval. The association between cleft measurements and cephalometric parameters was tested with Spearman Correlation (r s )., Results: The skeletal occlusal outcome at 7 years old for this series of patients was: Class I: 19%; Class II: 59% and Class III: 22%. No single cleft measurement at palatoplasty was predictive of the skeletal occlusal outcome. A larger hard palate cleft was associated with a shorter antero-posterior maxilla., Conclusions: The skeletal occlusal class outcomes were similar to those found in a previous study in the literature. The occlusal prognosis appears to be better than in patients with Robin Sequence or with an associated cleft lip. No preoperative measurement was found to be associated with the occlusal outcome., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: To identify weight gain trends of infants with Robin sequence (RS) treated by the Stanford Orthodontic Airway Plate treatment (SOAP)., Design: Retrospective longitudinal cohort study., Setting: Single tertiary referral hospital., Patients: Eleven infants with RS treated with SOAP., Interventions: Nonsurgical SOAP., Main Outcome Measures: Body weight, Weight-for-age (WFA) Z-scores, and WFA percentiles at birth (T0), SOAP delivery (T1), SOAP graduation (T2), and 12-months old (T3)., Results: Between T0 and T1, the weight increased but the WFA percentile decreased from 36.5% to 15.1%, and the Z-score worsened from -0.43 to -1.44. From T1 to T2, the percentile improved to 22.55% and the Z-score to -0.94. From T2 to T3, the percentile and the Z-scores further improved to 36.59% and -0.48, respectively., Conclusions: SOAP provided infants experiencing severe respiratory distress and oral feeding difficulty with an opportunity to gain weight commensurate with the WHO healthy norms without surgical intervention., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: The main clinical manifestations of patients with Pierre Robin sequence (PRS) include micrognathia, the glossoptosis and dyspnoea. The difficulty of tracheal intubation (TI) in such patients is increased. Objective: The purpose of the study was to evaluate the reliability and efficacy of the PRS simulator. Methods: A PRS simulator was developed by using 3-dimensional (3D) printing technology under computer-aided design. A total of 12 anaesthesiologists each trained 5 times for TI on the PRS Training Simulator-1 and recorded the simulation time. After the training, they were randomly divided into three groups with a total of 12 nontrained anaesthesiologists, and the simulation was completed on PRS Simulator-2, 3 and 4. The simulation time was recorded, and the performance was evaluated by three chief anaesthesiologists. Then, all 24 anaesthesiologists completed the questionnaire. Results: A PRS simulator developed by 3D printing was used to simulate the important aspects of TI. The average number of years worked was 6.3 ± 3.1 years, and 66.7% were female. The time for the 12 anaesthesiologists to complete the training gradually decreased ( p < 0.01). Compared with the trained anaesthesiologists, the simulation time of TI in the nontrained anaesthesiologists was much longer (all p < 0.01). In addition, the simulation performance of the trained anaesthesiologists was relatively better (all p < 0.01). Conclusion: The reliability and efficacy of the PRS simulator is herein preliminarily validated, and it has potential to become a teaching and training tool for anaesthesiologists., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Mao, Liu, Zhong, Qin, Ma, Zuo, Zhang and Yang.)

Objective: The only findings consistent among infants with Robin sequence (RS) are the presence of micrognathia, glossoptosis, and upper airway obstruction (UAO). Feeding and growth dysfunction are typical. The etiopathogenesis of these findings, however, is highly variable, ranging from sporadic to syndromic causes, with widely disparate levels of severity. This heterogeneity has created inconsistency within RS literature and debate about appropriate workup and treatment. Despite several attempts at stratification, no system has been broadly adopted., Design: We recently presented a novel classification that is summarized by the acronym MicroNAPS . Each of 5 elements is scored: Micro gnathia, N utrition, A irway, P alate, S yndrome/comorbidities, and element scores are summarized into a "stage"., Results: Testing of this system in a sample of 100 infants from our center found it to be clinically relevant and to predict important management decisions and outcomes., Conclusions: We herein present an interactive website (www.prscalculator.com) and printable reference card for simple application of MicroNAPS, and we advocate for this classification system to be adopted for clinical care and research., Competing Interests: Declaration of Conflicting InterestsCory M. Resnick: Consultant for AbbVie Pharmaceuticals without relevant relationship to the subject matter contained within this manuscript.

Objective: Stickler Syndrome (SS) is an inherited collagenopathy characterized by heterogenous orofacial, ocular, auditory, and skeletal abnormalities. The orofacial manifestations are variable and some patients present with cleft palate and velopharyngeal insufficiency (VPI). The incidence of VPI in SS is poorly studied and no studies have compared the incidence of VPI between Type I (COL2A1) and Type II (COL11A1) SS. The objective of this study is to compare the incidence of VPI between SS subtypes and discuss the surgical techniques used to treat them., Design: Single-institution, retrospective chart review., Setting: Tertiary pediatric hospital., Patients/participants: Forty-three children were diagnosed with SS between January 2003 and December 2018. Genetic testing results, genetics notes, craniofacial clinic notes, and operative reports were reviewed. Patients without genetic testing or craniofacial/otolaryngologic evaluation were excluded. Thirty-one patients met criteria and were included., Main Outcome Measure: Primary outcome was VPI incidence., Results: There were 18 patients with Type I SS and 13 with Type II SS. Five (16%) patients had VPI, 2 (11%) with Type I SS compared to 3 (23%) with Type II SS ( P  > .05). All patients with VPI underwent surgery with either sphincter pharyngoplasty (3) or pharyngeal flap (2). Two patients with Type II SS underwent revision sphincter pharyngoplasty, with one conversion to pharyngeal flap., Conclusion: VPI is common for patients with SS. In this study, there was no significant difference in the incidence of VPI between SS subtypes. Future studies are needed to confirm these findings, which could be important for patient counseling and treatment planning., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

To describe perioperative feeding performance in infants with Robin sequence (RS) who underwent mandibular distraction osteogenesis (MDO)., A retrospective study of infants that underwent MDO from May 2010 to December 2019., Tertiary pediatric hospital., A total of 40 patients underwent MDO and 20 met inclusion criteria. Of the included infants, 6 had an associated syndrome and 80% were male., Time to full oral feeds, rate of G-tube placement, and change in weight percentile following MDO., Average oral intake prior to MDO was 22.1% of individual goal feeds. Among the 15 (75%) children that did not require G-tube placement, mean time to full oral feeds after MDO was 11 days ± 5.7 days, with 80% of infants reaching full oral feeds within 2 weeks after extubation. The proportion of G-tube placement in patients with a syndrome was higher than in isolated RS (-0.6; 95% CI: -1.0, -0.2). Mean percentages of weight-for-age percentile decreased during the first 3 months after the procedure. This was followed by a mean upturn in weight starting after the third month after MDO with a recovery to preoperative mean weight-for-age percentiles by 6 months after surgery., This study suggests that infants with RS may achieve full oral feeds despite poor feeding performance before MDO. Infants with syndromic RS are more likely to require G-tube. These findings may be used to inform G-tube discussion and offer a timeline to work toward goal oral feeds for infants with RS after MDO., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: Describe the intelligence quotient (IQ) of children with Pierre Robin sequence (PRS)., Design: Prospective cohort study., Setting: Neurodevelopmental follow-up clinic within a hospital., Patients: Children with PRS (n = 45) who had been in the Neonatal Intensive Care Unit (NICU) were classified by a geneticist into 3 subgroups of isolated PRS (n = 20), PRS-plus additional medical features (n = 8), and syndromic PRS (n = 17) based on medical record review and genetic testing., Main Outcome Measure: Children with PRS completed IQ testing at 5 or 8 years of age with the Wechsler Preschool and Primary Scale of Intelligence, Third Edition (WPPSI-III) or Fourth Edition (WPPSI-IV) or the Wechsler Intelligence Scale for Children, Fourth Edition (WISC-IV) or Fifth Edition (WISC-V)., Results: IQ scores were more than 1 to 2 standard deviations below the mean for 36% of the overall sample, which was significantly greater compared to test norms (binomial test P  = .001). There was a significant association between PRS subtype and IQ (Fisher's exact P  = .026). While only 20% of children with isolated PRS were within 1 standard deviation below average and 35% of children with syndromic PRS were below 1 to 2 standard deviations, 75% of PRS-plus children scored lower than 1 to 2 standard deviations below the mean., Conclusion: PRS subgroups can help identify children at risk for cognitive delay. The majority of children with PRS-plus had low intellectual functioning, in contrast to the third of children with syndromic PRS who had low IQ and the majority of children with isolated PRS who had average or higher IQ., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

To present the application of the pre-epiglottic baton plate (PEBP) in infants with Pierre Robin sequence (PRS) in the Southern Chinese population (Hong Kong) and to present the diagnosis and management protocol of these infants in our centre. Retrospective case series of three patients with PRS. Neonatal Intensive Care Unit in Kwong Wah Hospital and Craniofacial Orthodontic Centre in United Christian Hospital, Hong Kong. Three new-born infants (two girls, one boy) with PRS and upper airway obstruction due to glossoptosis. A protocol for the diagnosis and management of these infants in the Southern Chinese population (Hong Kong) was presented. The three patients received nasal high-flow oxygen and/or continuous positive airway pressure (CPAP) as first-line respiratory support, followed by PEBP for 3–5 months. A two-stage approach was undertaken to ensure accurate positioning of the PEBP. All three infants had improvement in clinical signs, symptoms and polysomnography upon discharge. PEBP and other respiratory aids were weaned off at 3–6 months. The PEBP, combined with other respiratory support, is a useful modality in the treatment of obstructive sleep apnoea in infants with PRS. [ABSTRACT FROM AUTHOR]

Fetal micrognathia can be detected early in pregnancy. Prognosis of micrognathia depends on the risk of respiratory distress at birth and on the long‐term risk of intellectual disability. The purpose of this study was to evaluate the long‐term prognosis of fetuses with prenatal diagnosis of micrognathia by estimating the prevalence and the severity of confirmed genetic diagnosis in our cohort. Our retrospective study included 41 fetuses with prenatal diagnosis of micrognathia referred to the multidisciplinary centers for prenatal diagnosis in Nice and Marseille, France, between 2006 and 2016. Fetal micrognathia was associated with cleft palate in 27 cases. A genetic cause was confirmed in 21 cases (67%). A chromosomal abnormality was present in 12 cases, including three copy‐number variations diagnosed by array CGH. Monogenic disorders were identified in nine cases, most often after birth. Fetuses with family history of micrognathia or Pierre Robin sequence had a favorable outcome. Prognosis was good for the fetuses without associated findings and normal chromosomal analysis, with the exception of one case with a postnatal diagnosis of mandibulofacial dysostosis with microcephaly. Prognostic was poor for the fetuses with additional ultrasound anomalies, as only 5 out of 28 children had a good outcome. Prenatal diagnosis of micrognathia is an indicator of a possible fetal pathology justifying multidisciplinary management. Our study confirms the necessity of performing prenatal array CGH. Use of high‐throughput gene sequencing in prenatal period could improve diagnostic performance, prenatal counseling, and adequate postnatal care. [ABSTRACT FROM AUTHOR]

Various treatments, many of them considerably invasive, are currently applied to infants with Robin sequence (RS) and accompanying upper airway obstruction (UAO). We present a narrative review of our data on the Tübingen palatal plate (TPP) which show the following: a) in a randomized trial, the TPP was superior to a sham procedure in alleviating UAO; b) children treated with the TPP in infancy showed an intellectual development within the reference range; c) prone positioning is no alternative, as it is ineffective and associated with an increased risk of sudden death; d) the TPP reduces the mixed-obstructive apnea index to near-normal values, both in isolated and most (83%) syndromic RS, e) of 443 infants (129 syndromic) treated with the TPP in our center, 23 (5%) ultimately received a tracheostomy (all with syndromic RS), f) recent data suggest that the TPP may induce mandibular catch-up growth, g) the TPP may also help to reduce respiratory complications following cleft closure in RS, and h) TPP treatment is applied by various centers around the world, although it is unclear if its effectiveness is invariably controlled by endoscopy and sleep studies, although both are necessary. Given these data from peer-reviewed studies, it may be questioned whether the "First do no harm" principle is always adhered to when subjecting RS infants to more invasive procedures such as mandibular distraction osteogenesis or tongue-lip adhesion. [ABSTRACT FROM AUTHOR]

Objectives: To assess for differences in postoperative care following mandibular distraction osteogenesis (MDO) in infants before and after implementation of a standardized protocol.Study Design: Retrospective chart review.Setting: Urban tertiary pediatric hospital.Subjects and Methods: The inpatient charts of infants who underwent MDO before 90 days of age were assessed for metrics such as postoperative length of stay (LOS), duration of mechanical ventilation, and the choice and duration of sedating medications.Results: Over a 6-year period, 16 patients met inclusion criteria. The first 4 consecutive patients were managed at the discretion of the critical care staff. The remaining 12 infants were managed with a planned 4- to 6-day period of postoperative intubation, during which a standard protocol determined the choice, dosage, and duration of sedating medications. The mean age was similar between groups (preprotocol: mean, 26.5 days; protocol: mean, 20.3 days; P = .51). The mean postoperative LOS was 13.3 days less among infants managed with the protocol (P = .06), and the mean number of midazolam boluses was fewer among protocol patients (P < .01). A more consistent postoperative LOS, duration of mechanical ventilation, and exposure to sedating medications was observed among protocol subjects (P < .01). The LOS for 2 patients in the preprotocol group was extended due to iatrogenic withdrawal syndrome. There were no instances of accidental extubation or anoxia in either group.Conclusions: Among infants undergoing MDO, standardizing postoperative airway and sedation practices may offer a more predictable postoperative course as compared with a case-by-case management philosophy. [ABSTRACT FROM AUTHOR]

We describe a case of the combined use of acellular dermal matrix and pedicled buccal fat pad (BFP) in a wide U-shaped cleft palate repair. Acellular dermal matrix was used as a "patch" repair for the nasal mucosa defect as opposed to the conventional inlay graft. The advantages include reduced cost and a smaller avascular graft load. Lateral relaxing incisions were made to ensure tension-free closure of oromucosa at midline. Lateral oromucosa defect closure with well-vascularized pedicled BFP ensures enhanced healing, less palatal contracture and shortening, and reduced infection. The palate healed with mucosalization at 2 weeks, and no complications were noted at 6 months follow-up. [ABSTRACT FROM AUTHOR]

Objective: Pierre Robin Sequence (PRS) is a heterogeneous pathological condition characterized by the coexistence of micrognathia, glossoptosis, and cleft palate, resulting in upper airway tract obstruction. Among the treatment modalities, the orthodontic approach is one part of the comprehensive care of those patients and will be present in the treatment modalities during all the growth period of the child. Methods: All patients with PRS observed in the period 2013-2017 were treated with a definite functional approach. The results were retrospectively analyzed with regard to functional outcome, total treatment time, and number of plates provided for a single patient. Results: In all the patients, the indicated treatment protocol has been applied as early impression and plate supply, stimulation of bottle feeding with the use of the plate, eventual substitution of the plate if no more adequate to the transverse and sagittal growth of the palate, and continuing the use until the surgical closure of the cleft. All the patients showed a positive outcome to the proposed treatment approach, evaluated with regard to the incidence of feeding improvement and weight gain, to the limit for the surgical phase, in the absence of adverse effects. Conclusion: The use of a functional obturator plate, removing functional alterations to mandibular growth, reduces and, in some cases, eliminates the need for surgical intervention. As also stated in the literature, if despite the presence of the plate nutritional problems persist, immediate different surgical approaches, mainly mandibular osteodistraction, become necessary. [ABSTRACT FROM AUTHOR]

Objective: To determine whether nonsupine sleep improves obstructive sleep apnea (OSA) in infants with cleft palate undergoing polysomnography (PSG). Design: Retrospective chart review. Setting: Tertiary care pediatric hospital. Patients: Twenty-seven infants (1 month to 1 year) with cleft palate with or without cleft lip (CP ± L) undergoing PSG testing for suspected OSA were included. Main Outcome Measures: Polysomnography measures included obstructive apnea–hypopnea index (OAHI), central apnea–hypopnea index (CAHI), oxygen saturation (SpO2) nadir, SpO2, and end-tidal carbon dioxide (ETCO2). Results: Twenty-three PSGs with at least 20 minutes of sleep in both the supine and the nonsupine positions were analyzed. The supine OAHI (mean: 16.8 events/hour; standard deviation [SD]: 18.5) and nonsupine OAHI (mean: 12.6 events/hour; SD: 12.6) did not differ significantly (P =.10). The supine CAHI (mean: 1.9 events/hour; SD: 2.7) and nonsupine CAHI (mean: 3.1 events/hour; SD: 3.7; P =.15), the supine SpO2 nadir (mean: 81.2%; SD: 6.3) and nonsupine SpO2 nadir (mean: 81.8%; SD: 5.3; P =.70), the supine mean SpO2 (mean: 95.5%; SD: 1.9) and nonsupine mean SpO2 saturation (mean: 95.3%; SD: 2.4; P =.34), and the supine ETCO2 (mean: 45.4 mm Hg; SD: 5.3) and nonsupine ETCO2 (mean: 42.5 mm Hg; SD: 10.1; P =.24) were also similar. Conclusions: There were no significant improvements in OSA metrics during nonsupine sleep in infants with CP ± L. Prior to recommending nonsupine positioning which increases infant's exposure to sudden infant death syndrome risk, we advocate obtaining a PSG to verify an objective improvement in OSA. [ABSTRACT FROM AUTHOR]

Objective: To describe the impact of timing of tympanostomy tube insertion on the number of tubes received and complications in children with routine tube placement. Design: Retrospective case series. Setting: Tertiary care children's hospital. Participants: Records from a consecutive sample of 401 children with cleft palate were reviewed. Sixty-five patients with isolated cleft palate and 82 patients with cleft lip and palate had follow-up until 5 years of age and were included. Interventions: Tympanostomy tubes. Main Outcome Measure(s): Number of tubes received and tube-related complications. The hypothesis was formulated prior to data collection. Results: Males comprised 55.8% of included patients, and tubes were placed in 98.6% of patients at a median age of 6.5 months. Effusion was documented at first tube placement for 96.5% of patients. Most (67.4%) patients required replacement of tubes, and 10.6% required long-term tubes. Complications included otorrhea (71.0%), myringosclerosis (35.2%), granulation (22.8%), perforation (17.9%), retained tubes (5.5%), and cholesteatoma (1.4%). Cleft lip and palate (P <.001) and otorrhea (P =.023) were associated with tube placement before palatoplasty. Patients with tube placement before palatoplasty (P =.033), genetic disorders (P =.007), failed newborn hearing screen (P =.012), otorrhea (P <.001), and granulation (P <.001) received more tubes. Conclusions: Nearly universal effusion in patients with cleft palate supports the need for routine tube placement. The potential for otorrhea and requiring more tubes should be weighed against the risks associated with prolonged effusion when considering tube placement before palatoplasty. [ABSTRACT FROM AUTHOR]

Bone morphogenetic protein (BMP) signaling plays a crucial role in the development of craniofacial organs. Mutations in numerous members of the BMP signaling pathway lead to several severe human syndromes, including Pierre Robin sequence (PRS) caused by heterozygous loss of BMP2. In this study, we generate mice carrying Bmp2-specific deletion in cranial neural crest cells using floxed Bmp2 and Wnt1-Cre alleles to mimic PRS in humans. Mutant mice exhibit severe PRS with a significantly reduced size of craniofacial bones, cleft palate, malformed tongue and micrognathia. Palate clefting is caused by the undescended tongue that prevents palatal shelf elevation. However, the tongue in Wnt1-Cre;Bmp2f/f mice does not exhibit altered rates of cell proliferation and apoptosis, suggesting contribution of extrinsic defects to the failure of tongue descent. Further studies revealed obvious reduction in cell proliferation and differentiation of osteogenic progenitors in the mandible of the mutants, attributing to the micrognathia phenotype. Our study illustrates the pathogenesis of PRS caused by Bmp2 mutation, highlights the crucial role of BMP2 in the development of craniofacial bones and emphasizes precise coordination in the morphogenesis of palate, tongue and mandible during embryonic development. [ABSTRACT FROM AUTHOR]

Abstract Objectives To assess the impact of feeding practices on growth in infants with Pierre Robin Sequence (PRS) during their inpatient stay in a neonatal intensive care unit in a large tertiary paediatric hospital setting. Methods A retrospective review of feeding practices in infants with PRS was conducted between January 2006 and September 2017. Baseline demographics, nutrition-related and general outcomes were collected. Feeding difficulties, length of stay (LOS) and malnutrition were the primary outcome measures. Feeding difficulties included absence or poor-quality suck, episodes of aspiration, use of proton pump inhibitors or multiple episodes of vomiting. Malnutrition was classified as a weight-for-age Z score of < -1. Results Analysis was conducted on 49 infants with PRS that met eligibility criteria. Feeding difficulties correlated with a longer LOS (24.1 vs 6.8 days (p = 0.001)) Z-scores differed significantly between birth and discharge (0.21(1.84) vs −1.27(2.14)) (p < 0.001*) with malnutrition being evident in 26 infants of which only 17 infants were seen by a dietitian. Presence of intrauterine growth restriction (IUGR) increased the likelihood of malnutrition (OR 1.40(CI-1.11–1.77)). Conclusion Infants with PRS are highly likely to have feeding difficulties and malnutrition. Early intervention by a dietitian is recommended to reduce the impacts of feeding difficulties, meet elevated energy requirements and facilitate growth. Infants with a longer inpatient stay or presence of IUGR should have their growth and feeding routinely monitored. [ABSTRACT FROM AUTHOR]

Objective: The workup of patients with Pierre Robin sequence (PRS) consists of a physical examination, O2 saturation, and polysomnography to determine the severity of respiratory obstruction and need for surgery. We suggest that capillary blood gas (CBG) may be a better physiologic representation of airway obstruction and should be routinely used in the management of patients with PRS. Design: This is a multicenter study based on a retrospective review of medical records. Setting: The study was performed at tertiary care centers. Interventions: Patients with PRS <1 year old underwent mandibular distraction osteogenesis. Main Outcome Measure: Using successful treatment outcome as a reference standard, receiver operating characteristic (ROC) curve was used to determine the accuracy of the diagnostic test and values for the best sensitivity and specificity to determine the need for surgical intervention. Results: Of 73 patients, 48 had sporadic PRS, 23 had syndromes, 2 had micrognathia, not otherwise specified. Mandibular distraction osteogenesis was performed in 62 patients at a mean age of 39 days. The mean initial Apnea-Hypopnea Index (AHI) in nonsurgical versus surgical groups was 10 versus 31 (P = .063), pH 7.41 versus 7.34 (P = .003), pCO2 43 versus 56 (P < .001), and HCO3 27 versus 30 (P = .022). The ROC curve showed that pCO2 of 49.5 has the best specificity (100%) and sensitivity (72.6%) profile in terms of need for definitive airway. Conclusion: A simple CBG heel stick may better predict the physiologic effects of obstructive apnea; therefore, it should be added to the algorithm of PRS workup. [ABSTRACT FROM AUTHOR]

Objective/background: Obstructive sleep apnea syndrome (OSA) is frequent in Pierre Robin sequence (PRS) infants. Prone positioning (PP) is commonly recommended but has never been studied by polysomnography (PSG). This study aimed to evaluate the impact of the PP on sleep and breathing outcomes measured by PSG.Patients/methods: Retrospective study conducted between 2015 and 2017 in a tertiary hospital. A PSG with pulse oximetry and transcutaneous carbon dioxide was performed in PRS infants in the supine position (SP) and the PP. Sleep and breathing outcome measures were compared between SP and PP.Results: Among the 18 PRS (mean ± SD age: 44 ± 26 days at evaluation), 11 had clinical manifestations of OSA. All had severe OSA diagnosed on PSG. In the PP, infants had a significantly higher sleep efficiency (median [IQR]: 83% [69-90]) than in the SP (70% [55-77], p = 0.04). During REM, there was a trend towards lower OAHI in the PP (50/h [28-82] versus 61/h [40-103], p = 0.05). For 13, the PP was the best sleep position (72%), and for four the SP was the best sleep position (22%; p < 0.01). The PP was sufficient alone to decrease OSA index <10 events/hour in three infants.Conclusion: Positioning infants in the PP led to an improvement of sleep quality and an incomplete correction of OSAS in the vast majority of PRS infants. A nocturnal sleep recording seems to be indicated systematically in the early evaluation of these young patients to choose the best therapeutic option for OSAS.Clinical Trial Registration: Not applicable. [ABSTRACT FROM AUTHOR]

Objective: To assess the dental class occlusion and lateral cephalometry of children with conservatively treated Pierre Robin sequence (PRS) and to identify associations between these findings and prepalatoplasty cleft palate measurements.Study Design: Retrospective cohort study.Subjects and Methods: Among 22 patients with PRS, the following data were prospectively collected: demographics and preoperative cleft palate measurements. After patients reached age 6 years, an orthodontist assessed dental occlusion class and performed a lateral cephalometric analysis. PRS cephalometric data were compared with reference population values. Bivariate logistic regression was used to test the association with malocclusion class. Results are presented as odds ratios with 95% profile likelihood confidence intervals. The association between cleft measurements and cephalometric parameters was tested with Spearman's correlation ( rs).Results: All 22 patients had bimaxillary hypoplasia and were prone to hyperdivergency, with a 41% rate of dental class III malocclusion. An increased anterior growth of the still retrusive mandible mostly accounts for the occurrence of the class III malocclusion in PRS (class II SNB = 74.3° vs class III SNB = 77.6°, P = .04). A larger cleft at the time of the cleft repair (mean, 11 months) was associated with increased mandibular retrusion (smaller SNB angle, rs = -0.5, P = .02).Conclusions: The 41% rate of class III malocclusion among these conservatively treated patients needs to be considered in the choice of the initial airway approach. The future impact of early mandibular advancement will have to be determined. [ABSTRACT FROM AUTHOR]

Objective: The concept of mandibular catch-up growth is often quoted in the literature regarding Pierre Robin sequence (PRS). We endeavored to perform a systematic review of whether the literature supports this concept. Design: Systematic review. Interventions: A PubMed-based systematic review of the English literature was performed of articles objectively measuring mandibular growth or position after nonoperative management of PRS. Main Outcome Measures: Rate and end point of mandibular length, ramus length, gonial angle, and maxillomandibular discrepancy. Results: The initial search delivered 607 English-language abstracts. Of these, 16 met inclusion criteria. Eight articles evaluating 143 patients followed longitudinal patient data and therefore allowed comparison of growth rates to controls. Ten articles evaluating 228 patients presented cross-sectional data and therefore could only evaluate a single time point. Two of the 8 longitudinal studies reported faster than normal growth of mandibular length in a significant portion of their cohort. Five of 8 reported equal growth rates. One of 16 studies reported that mandibular length of patients with PRS normalized compared to controls. Two of 16 studies reported no difference in maxillomandibular discrepancy between PRS and controls, whereas 10 reported a posteriorly displaced mandible relative to the maxilla in PRS. Significant differences in control groups, patients, and age existed between studies. Conclusions: While the concept of catch-up growth in PRS is often quoted, a minority of objective studies suggest increased mandibular growth rates in isolated PRS. Even fewer studies suggest that the maxillomandibular discrepancy in PRS completely resolves. [ABSTRACT FROM AUTHOR]

Highlights • The high prevalence of taurodontism and tooth agenesis in the population with ns-PRS. • The taurodontism is a new dental phenotype not yet described in the Pierre Robin sequence. • To know the most prevalent anomalies in subjects with ns-PRS for the planning of the rehabilitation odontological treatment. Abstract Objective The objective was to investigate dental phenotypes in individuals with non-syndromic Pierre Robin Sequence (ns-PRS) and compare the prevalence of these phenotypes with subjects with non-syndromic cleft palate (ns-CP) and a control group with subjects without any craniofacial anomalies. Methods A total of 760 panoramic radiographs of 330 individuals (110 with ns-PRS; 110 with ns-CP and 110 without any malformations) were digitized and evaluated regarding the diagnosis of taurodontism, tooth agenesis, root dilaceration and tooth transposition. Chi-square test was applied to compare the occurrence of dental phenotypes between groups. A P value of less than 0.05 was considered statistically significant. Results Total prevalence of dental phenotypes was 94.5% of ns-PRS; 54.5% of ns-CP and 59.1% of the control group subjects with a statistically significant difference for the ns-PRS when compared to the other two groups. Two dental phenotypes, taurodontism and dental agenesis were identified with statistically significant higher prevalences in subjects with ns-PRS when compared with the ns-CP group and the control group (p < 0.001). Taurodontism was the most prevalent dental phenotype, with 92.73% in the ns-PRS group, 40.91% for ns-CP and 44.55% in the control group. Tooth agenesis had a prevalence of 22.7% for ns-PRS, 4.5% for ns-CP and no case in the control group. For the prevalence of root dilaceration and tooth transposition, no statistically significant differences were observed between the three groups. Conclusions Due to the high frequency of taurodontism in individuals with ns-PRS, we suggested that this novel phenotype would be important in the phenotypic screening of ns-PRS and could be considered as a phenotype associated with ns-PRS. [ABSTRACT FROM AUTHOR]