Pierre Robin Sequence is characterised by a small lower jaw, tongue displacement and, often, a U-shaped cleft palate, leading to breathing and feeding problems. Orthodontic airway plates have been developed as a non-invasive treatment option. A systematic review was conducted to evaluate the outcomes of orthodontic airway plates in children with Pierre Robin Sequence. Databases were searched for studies published up to December 2024, in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Ten clinical studies with a total of 598 patients were included, among which 483 had isolated Pierre Robin Sequence, and 115 had syndromic Pierre Robin Sequence. Orthodontic airway plates improved airway obstruction and helped avoid tracheostomy in most patients. A small proportion of syndromic cases still required surgery. Feeding outcomes improved, with fewer children needing tube feeding. Speech development was good, though hypernasality persisted in some. Orthodontic airway plates are a minimally invasive yet effective way to manage airway complications in Pierre Robin Sequence patients.

A common intervention to address aspiration and reflux in infants is thickening milk. However, thickening milk may further complicate feeding management for infants with cleft palate using adaptive feeding methods. The purpose of this scoping review was to identify and describe the types of milk thickener used for infants with cleft palate (with or without cleft lip) and the feeding modalities through which thickened milk is administered. A comprehensive scoping review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews guidelines. Five databases were searched: PubMed, Scopus, CINAHL, Google Scholar, and EBSCO Open Dissertations, in addition to citation searching. Records were limited to English-language publications with no date restrictions. Both peer-reviewed and gray-literature sources were included. After removing 868 duplicates, 2,282 records were screened by title and abstract. Sixty-four full-text articles were assessed for eligibility. One study met the inclusion criteria. Milk thickening was used as an intervention for infants with isolated Pierre Robin sequence and cleft palate. The thickener used was a modified corn-based flour at approximately 3% concentration to achieve a thicker liquid consistency. The thickened milk was offered via a bottle with latex nipples of varying hole sizes (original, intermediate, and 1-mm) depending on the infant's needs. Despite only one study meeting the inclusion criteria by specifying thickener type and/or feeding modality, 11 tutorial and seminar articles that discussed milk thickening as an intervention strategy in this population were identified. However, these articles lacked any information on the type of thickener and feeding modality, and none implemented the intervention with infants. Although milk thickening is described as a strategy in several tutorial and seminar articles, there is a paucity of empirical evidence to support milk thickening as a feeding intervention strategy for infants using cleft-adapted feeding methods.

Effective airway management is critical to Pierre Robin Sequence treatment. The goal of this study is to assess the three-dimensional changes in airway size and shape in 117 newborns with isolated Pierre Robin sequence who underwent mandibular distraction osteogenesis. During the study period (11/29/2016 to 11/26/2019), 117 newborns affected by isolated Pierre Robin sequence met the inclusion criteria for the present study. All 117 included patients underwent linear distraction. Demographic variables were recorded and analyzed. Cone-beam computed tomography were performed before and after mandibular distraction osteogenesis. A systemic quantitative three-dimensional analysis of size and shape of upper airway was performed. The mean age was 71 day (range 12 to 213). The mean weight was 3.9 kg (range 2.3-6.8). A total of 53 patients are female and 64 are male. When the distraction device was removed, the upper and lower jaws were symmetrically aligned. Pre- and post-distraction comparison clearly showed osteogenesis. For the size of the upper airway, airway volume, anteroposterior dimension of the retroglossal airway, lateral dimension of retroglossal airway, minimum retropalatal area, minimum retroglossal area, average cross-sectional area and minimum cross-sectional area increased significantly after mandibular distraction osteogenesis (P < 0.001). However, the airway length did not change significantly (P > 0.05). For the shape of the upper airway, the lateral/anteroposterior ratio in the retroglossal region and the ratio of the retropalatal airway diameter to the retroglossal airway diameter significantly decreased after mandibular distraction osteogenesis (P < 0.001). The airway uniformity significantly increased after mandibular distraction osteogenesis (P < 0.001). Mandibular distraction osteogenesis for isolated Pierre Robin sequence improved size and shape of the upper airway, further confirming mandibular osteogenesis distraction as an effective surgical modality to address the airway obstruction in newborns affected by isolated Pierre Robin sequence. Cone-beam computed tomography scanning and analysis can serve as a safe and effective examination modality for upper airway applications of PRS newborns.

There is a lack of uniformity in the diagnostic criteria and system for the morphologic classification of micrognathia in infants with isolated Pierre Robin sequence (IPRS). Therefore, the aim of this study was to create a morphologic classification system for IPRS-affected mandibles that may guide surgical management and osteotomy design. We designed and implemented a retrospective cross-sectional study. The study sample included infants with IPRS. The predictor variables included shape variables of the IPRS-affected mandibles. The outcome of interest was morphologic differences among the IPRS-affected mandibles in the infancy stage. The original coordinate data of the mandibular images were analyzed by a generalized Procrustes analysis and 2-block partial least squares analysis to identify the focal and nonfocal areas in the IPRS-affected mandibles. The original feature points were modified according to the results of 2-block partial least squares analysis. The modified feature points were further analyzed by principal component analysis, K-means cluster analysis, and canonical variate analysis to obtain a morphologic classification of the IPRS-affected mandibles. One hundred fifty infants with IPRS were enrolled in this study. Principal component analysis showed that the variations among IPRS-affected mandibles were mostly in terms of the shapes of the mandibular ramus, mandibular body, and angle of the mandible. On the basis of the results of K-means cluster analysis and canonical variate analysis, the mandibles in group A3 showed characteristics such as a simply shorter mandibular body. Group B3 was adjusted to show characteristics such as a shorter mandibular body with a more obtuse mandibular angle, whereas group C3 showed characteristics such as a shorter mandibular body with a shorter mandibular ramus. Our study confirmed the hypothesis that there are quantifiable morphologic differences among the IPRS-affected mandibles, and it provided a morphologic classification of the IPRS-affected mandibles that will help to promote the clinical diagnosis and treatment.

To compare arch widths of patients with isolated Robin sequence (IRS) operated using modified von Langenbeck technique and modified Furlow double-opposing z-plasty. Retrospective, transversal study. Hospital of Rehabilitation of Craniofacial Anomalies, University of São Paulo, Bauru, Brazil. Three groups of patients were analyzed. Group VL comprised 30 patients with IRS operated by von Langenbeck technique (mean age of 8.1 years); group FL included 30 patients with IRS operated by Furlow technique (7.6 years); and the control group included 30 noncleft patients with class I occlusion (7.4 years). The palate repair was performed between 1.0 and 1.7 years of age in both study groups. Transversal measurements of maxillary and mandibular arches were performed digitally on 3D digital models. The intergroup comparison was performed using analysis of variance and Tukey test. An independent t test was used to compare the complete and incomplete types of cleft in both study groups. The level of significance was 5%. No statistically significant difference was found between the VL and FL groups for maxillary and mandibular arches. However, both groups showed decreased transversal dimensions compared with the control group (P < .01) for both dental arches. No differences for arch widths were observed for complete or incomplete palatal clefts. No influence of palate repair techniques was observed in the transversal arch widths in patients with IRS. Children with IRS operated for palate repair showed constriction of the maxillary and mandibular dental arches compared with noncleft children regardless the palatal cleft extension.