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Hypoplasia of the upper jaw may cause the lower jaw (mandible) to appear to protrude or stick out (relative prognathism). The maxillae also contain the sockets of the upper teeth. The maxillae are the large bones of that form the upper jaw and assist in the formation of the nasal cavities, the bony cavities of the eyes (orbits), and the roof of the mouth (palate). Underdevelopment (hypoplasia) upper jaw (maxillary bone) is another key feature of Binder type nasomaxillary dysplasia. Despite the various nasal abnormalities, the sense of smell is unaffected. In cases where the columella is severely short, the nostrils may appear triangular. The columella is abnormally short and the nostrils have a half-moon or comma-shaped appearance. The bottom of the sheet of cartilage and bone (nasal septum) that separates the right and left nostrils is known as the columella. The nasal bones may be underdeveloped or abnormally positioned.
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Affected individuals have a short nose and flattened bridge of the nose. In some patients the frontal sinuses may be underdeveloped or absent. The midface appears abnormally flattened. The characteristic finding of the disorder is the abnormal development (dysplasia) of the central or mid portion of the face. Several factors including the small number of identified affected individuals, the lack of large clinical studies, and the possibility of other genes influencing the disorder prevent physicians from developing an accurate picture of associated symptoms and prognosis. Characteristic symptoms include an abnormally short, flattened nose and underdevelopment of the upper jaw bone (maxillary bone).Īlthough researchers have been able to establish characteristic or “core” symptoms, much about Binder type nasomaxillary dysplasia is not fully understood.
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The specific symptoms and the severity of the disorder can vary from one person to another. Your child may appear to have an underdeveloped upper jaw and facial imbalance. The primary physical characteristic of Binder syndrome is a flat, underdevelopment of the central portion of the face (midfacial hypoplasia) particularly the area including the nose and upper jaw (maxillonasal region) and flattened nose associated with the absence of the anterior nasal spine that supports the nose in normal development. Binder syndrome results in undergrowth of the central face and may include elements of the nose and upper jaw. Binder syndrome also called nasomaxillary hypoplasia or maxilla-facial dysplasia or Binder’s syndrome, is a rare present at birth (congenital) disease affecting the face 1).