What Is Binder Syndrome?

Also referred to as Maxilla-Nasal Dysplasia, Binder Syndrome is a rare congenital disease affecting the face and cervical spine. It is often seen in conjunction with other congenital conditions, such as Down Syndrome and cleft palate, though no direct link has been found to explain the correlation between the diseases. A person with Binder Syndrome has a flat nasal bridge and an unusually short nose. Other noticeable disfigurements are an abnormally undeveloped upper jaw and a protruding lower jaw line.

The cause of Binder Syndrome is still not known as of 2011, although research has suggested there could be a possible genetic link. Case studies of individuals with the condition have shown a family history of the syndrome. Although there is evidence of a familial correlation, sound evidence has not been found to prove or disprove a genetic cause. No evidence has yet to be found showing environmental causes to be a contributing factor to the development of Binder Syndrome.

Physically, the chief physical characteristic of Binder Syndrome is an extremely recessed nasal cavity, resulting in a flat nose. This abnormality is caused by the absence of the structure supporting the nose, referred to as the nasal spine. Other physical characteristics attributed to the condition also include small incisors and missing molars.

When a patient's skull is examined under x-ray, two key maxillofacial structures are often found to be missing or deformed. The cartilage which divides the mouth from the nasal cavity is usually absent or severely malformed. The frontal sinuses are at times also noted to be either misshapen or smaller than the average individual.

Complications associated with Binder Syndrome are varied, depending on the individual patient. Many individuals show an increase in sinus infections. These infections are often due to the occlusion caused by a malformed nasal septum, which divides the two nostrils. Difficulty in eating is sometime noted, since the palate is often either missing or deformed. Poor intelligence has also been noted in persons with Binder Syndrome, although the exact cause of this has yet to be discovered.

When the complications which arise from Binder Syndrome become too troublesome, nasal grafting is often a treatment option. During this surgery, the nose and nasal cavity are reconstructed from cartilage taken from either a donor site on the patient or from an outside donor. The missing nasal spine is rebuilt and the septum's deviations are corrected. This not only allows the patient to have a more normal appearance, but it also corrects the associated breathing difficulties.