Maxillonasal dysplasia (Binder's syndrome): a critical review and case study.
Binder's syndrome (maxillonasal dysplasia) is a disorder of unknown etiology characterized by nasomaxillary hypoplasia and a 40-50% association of an underdeveloped frontal sinus and cervicospinal abnormalities. The midfacial retrusion is similar to that in chondrodysplasia punctata, resulting in confusion regarding diagnosis. This paper outlines the distinguishing features of Binder's syndrome, the treatment considerations, and presents 24 patients seen and treated. The facial and skeletal characteristics and developmental findings are emphasized to affirm the diagnosis of Binder's syndrome. A familial finding of Binder's features in five patients raises the possibility of a genetic mechanism, a previously undisclosed finding.
Horswell
B B
BB
Holmes
A D
AD
Barnett
J S
JS
Levant
B A
BA
D000015:Abnormalities, Multiple; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008437:Maxilla / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D009295:Nasal Bone / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D010375:Pedigree; D011859:Radiography