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Binder Type Maxillonasal Dysplasia

Abstract

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NORD gratefully acknowledges Scott P. Bartlett, MD, Chief, Division of Plastic and Reconstructive Surgery, Director, Craniofacial Program, Professor of Plastic Surgery, Perelman School of Medicine at The University of Pennsylvania, for assistance in the preparation of this report.

Synonyms of Binder Type Maxillonasal Dysplasia

  • Binder Syndrome
  • Maxillonasal Dysplasia
  • Nasomaxillary Hypoplasia

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Summary
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.

Introduction
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.

Binder Type Maxillonasal Dysplasia Resources

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