OMENS+ Classification Correlations Analysis of Craniofacial Microsomia in China: The Relationship Between Macrostomia and Mandibular Hypoplasia.

ABSTRACT: Macrostomia is arare congenital craniofacial deformity that influences the appearance and function of patients. In most cases, it coexists with craniomaxillofacial deformities such as craniofacial microsomia (CFM). This study aimed to analyze the relationship between macrostomia and mandibular hypoplasia so as to facilitate the early detection and diagnosis of children with CFM. It included 236 patients diagnosed with CFM. All underwent facial expression analysis, multi-angle photography, computed tomography, and three-dimensional reconstruction of soft and hard tissues. The clinical classification was performed according to OMENS+. Spearman (rank) correlation analysis was used to analyze the relationship between the severity of macrostomia (C1 and C2) and the degree of mandibular involvement (M1, M2a, M2b, and M3), and the correlation among the components of OMENS+. Of the 80 cases of macrostomia (34%) reported, 72 cases (90%) were C1 and 8 (10%) were C2. The analysis of OMENS+ revealed significant correlations among OMENS+ components. Also, a high correlation was observed between macrostomia (C) and hypoplasia of the mandible (M) ( P  = 0.002). Macrostomia was closely related to mandibular hypoplasia among children diagnosed with CFM. These results suggested that patients with macrostomia, who might also have craniofacial malformations caused by other first branchial arch anomalies, should be comprehensively physically examined for other syndromes.

[Experience of midfacial distraction osteogenesis in upper airway stenosis].

OBJECTIVE: To investigate the therapeutic effects of upper airway stenosis after Le Fort III osteotomy and midfacial distraction osteogenesis (DO). METHODS: Eleven cases (age, 5-16 yrs) with severe midface dysostosis complicated with exophthalmos, anterior crossbite and upper airway stenosis were treated by using Le Fort III osteotomy and midfacial DO from August 2000 to February 2007. The 3D reconstruction of the upper-airway from CT data was used to evaluate the upper airway volume before and after the operation. And meanwhile polysomnography was carried out to demonstrate the upper airway functional changes. RESULTS: There was a 64.3% mean increase [mean, (9.13 +/- 6.94) ml, P < 0.05] in upper airway volume in the 11 cases after the operations. It showed that there was significant improvements in the indexes of polysomnography after the operations, such as apnea and hypopnea index, average SaO2, minimum oxygen saturation and snore index. CONCLUSIONS: The Le Fort III osteotomy and midfacial distraction osteogenesis can efficiently relieve the symptoms of upper-airway stenosis in severe midfacial dysostosis.

[Surgical correction of craniofacial dysostosis with midface distraction osteogenesis].

OBJECTIVE: To investigate the effect of distraction osteogenesis on correction of craniofacial dysostosis. METHODS: Le Fort III osteotomy was applied through coronal route on patients with craniofacial dysostosis such as Crouzon and Apert syndrome. The procedures included disconnecting the skeletal midface from base of cranium, setting up a RED II distraction device, and directing the device bars. The distraction was started 5 days after the surgery, with a rate of 1 mm forward per day. When midface approaching the right position, i.e. a slightly over correction of occlusion was reached, stopped distraction and kept the device for 2 - 4 months. RESULTS: Eight cases completed all the therapy. The average blood lose was 300 ml and the average operation time was 3.5 hours. The midface had been moved averagely 9 mm forwardly and 1.5 mm downwards. The features had been improved obviously and the occlusion reached nearly normal. No serious complications occurred except for 1 case of seroma and 1 case of infection around pin on scalp. No recurrence was found in the 5 months of follow-up. CONCLUSIONS: Midface distraction osteogenesis is propitious to teenage or severe cases of craniofacial dysostosis.

[Changes of different section area at different parts of upper-airway after Le Fort III osteotomy].

OBJECTIVE: To observe the therapeutic effects of Le Fort III osteotomy and midface distraction osteogenesis (DO) on the upper-airway narrow. METHODS: Since 2000, 11 cases (10 cases of Crouzon syndrome and 1 case of Apert syndrome) with severe midface deficiency were treated with Le Fort III osteotomy and midface DO. The section area of different parts of upper-airway were tested by computer assistants image measurement preoperatively and postoperatively. Some patients received sleep function monitoring. RESULTS: The face appearance and the function of upper-airway improved significantly after Le Fort III osteotomy and Midface DO. The section area at the level of posterior nasal spine and uvula increased obviously after treatment (P < 0.05), however the section area at the level of epiglottis and separation between airway and esophagus were not obviously enlarged (P > 0.05). CONCLUSIONS: Midface DO after Le Fort III osteotomy can effectively improve the upper-airway narrow, especially the upper part from uvula.