What Are the Characteristics of the Upper Airway in Patients With Craniofacial Microsomia?

Abstract

IgAdef, B-cell defects were mainly restricted to surface membrane (sm)IgA 1 PCs and MBCs, with 2 clear subgroups showing strongly decreased numbers of smIgA 1 PCs with mild versus severe smIgA 1 MBC defects and higher frequencies of nonrespiratory tract infections, autoimmunity, and affected family members. Patients with IgG subclass deficiency with IgA deficiency and those with CVID showed defects in both smIgA 1 and smIgG 1 MBCs and PCs. Reduced numbers of switched PCs were systematically found in patients with CVID (absent in 98%), with 6 different defective MBC (and clinical) profiles: (1) profound decrease in MBC numbers; (2) defective CD27 1 MBCs with almost normal IgG 3 1 MBCs; (3) absence of switched MBCs; and (4) presence of both unswitched and switched MBCs without and; (5) with IgG 2 1 MBCs; and (6) with IgA 1 1 MBCs. Conclusion: Distinct PAD defective B-cell patterns were identified that are associated with unique clinical profiles. (J

Postoperative Changes in the Upper Airway Following Mandibular Distraction Osteogenesis in Pediatric Hemifacial Microsomia

“…26 Additionally, the literature demonstrates that incidences of breathing complications in HFM are related to severity of mandibular malformation. 4,6,26 A distinction is often made between Kaban-Pruzansky grades I-IIa and IIb-III in the literature, with the latter being considered severe. Sixty percent of the patients in our study had Kaban-Pruzansky scores considered less severe, and of our 40% with severe phenotypes, none had grade III classification.…”

A decade of clinical research on clinical characteristics, medical treatments, and surgical treatments for individuals with craniofacial microsomia: What have we learned?

“…Severe OSA was observed in patients with severe mandibular hypoplasia (50). In 2019, a crosssectional study by Klazen et al showed by studying CT scans of 79 patients with CFM and 88 controls that patients with CFM have a smaller oropharynx volume and retropalatal area compared to controls (51). Patients with CFM and OSA showed, compared to other CFM patients, a smaller retroglossal area and different uniformity and sphericity.…”

“…Patients with CFM and OSA showed, compared to other CFM patients, a smaller retroglossal area and different uniformity and sphericity. Multivariate regression analysis showed sphericity is the main predicting variable for OSA in patients with CFM (51). (50,52)Retrospective data showed that placement of a tracheostomy is often considered a first choice of treatment for patients with CFM and severe OSA (50).…”

Intra-individual variation of upper airway measurements based on computed tomography

“…Airway shape may contribute to the development of OSA [1,10]. Recently, a derived variable, that is sphericity of the upper airway, was suggested and investigated [10,30]. Klazen et al found that less sphericity was the main predictor for OSA in patients with craniofacial macrosomia [30].…”

“…Recently, a derived variable, that is sphericity of the upper airway, was suggested and investigated [10,30]. Klazen et al found that less sphericity was the main predictor for OSA in patients with craniofacial macrosomia [30]. It is interesting to note that sphericity had low ICC values for intra-individual repeatability; however, it also showed low variation between T0 and T1 in both the total airway and each segment, all the relative differences being below 15%.…”