Keutel syndrome is characterized by calcification and/or ossification of the cartilage in the external ears, nose, larynx, epiglottis, thyroid, trachea, ala nasi, and ribs this syndrome also presents with peripheral pulmonary stenosis, sensory hearing loss, and borderline-to-mild mental retardation. The syndrome involves hypoplasia of variable severity of cartilaginous nasal septum and premaxilla. The characteristics of the syndrome are typically visible. It is a rare disorder and the causes are unclear. Rudiger syndrome presents with short digits and talipes. Binders Syndrome/Binder Syndrome (Maxillo-Nasal Dysplasia) is a developmental disorder primarily affecting the anterior part of the maxilla and nasal complex (nose and jaw). Apert syndrome is characterized by irregular craniosynostosis, short occipital-frontal diameter, flat occiput, ventriculomegaly, and syndactyly. Am J Orthod Dentofacial Orthop 2007 132:704-9). Crouzon syndrome is associated with craniosynostosis and short occipital-frontal diameter. A different look: 3-dimensional facial imaging of a child with Binder syndrome. Aarskog syndrome is associated with brachycephaly and clinodactyly of the 5th finger. Robinow syndrome is associated with short forearms, clinodactyly, and macrocephaly. Chondrodysplasia punctata is also associated with scoliosis and asymmetrical shortening of the limbs. The characteristic findings are a failure of development in the premaxillary area with associated deformities of the nasal skeleton and the overlying soft tissues.
Binder syndrome should be suspected when prenatal ultrasound identifies a low flat nasal bridge. Binder syndrome or maxillonasal dysplasia is an uncommon developmental anomaly affecting primarily the anterior part of the maxilla and nasal complex. The differential diagnosis of Binder syndrome includes other facial disorders, most of which are usually associated with genetic disorders (Table 12.1).
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