Binder Type Maxillonasal Dysplasia
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Binder Type Maxillonasal Dysplasia is not the name you expected.
Binder type maxillonasal dysplasia is a rare developmental defect that is present at birth (congenital). The disorder is characterized by the underdevelopment (hypoplasia) of the central portion of the face, particularly the area including the nose and upper jaw (maxillonasal region). The specific symptoms and the severity of the disorder can vary from one person to another. Characteristic symptoms include an abnormally short, flattened nose and underdevelopment of the upper jaw bone (maxillary bone). The exact cause of Binder syndrome is not fully understood. Most cases appear to occur sporadically, but familial cases have been reported as well. Surgical and orthodontic treatment is recommended.
Binder type maxillonasal dysplasia was first described in the medical literature as far back 1882. Dr. Noyes described the essential features in a single patient in 1939. Dr. von Binder first identified the condition as a distinct clinical entity in 1962 in a comprehensive report of three children; the disorder now bears his name. There is some debate in the medical literature as to whether Binder type maxillonasal dysplasia is a syndrome or an association. A syndrome is typically a genetic disorder, in which a group of symptoms consistently occur together. An association is a nonrandom collection of birth defects that may have been caused by a number of factors, including genetic ones, and can potentially be associated with a variety of underlying conditions.
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Children's Craniofacial Association
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Craniofacial Foundation of America
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For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.
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This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
Last Updated: 10/9/2014
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