Binder’s syndrome is a rare congenital disorder affecting the face and cervical spine, often seen with other congenital conditions. It causes a flat nose bridge, an undeveloped upper jaw, and a protruding lower jaw line. The cause is unknown, but there may be a possible genetic link. Treatment options include nose graft surgery.
Also known as maxillary-nasal dysplasia, Binder’s syndrome is a rare congenital disorder affecting the face and cervical spine. It is often seen in conjunction with other congenital conditions, such as Down syndrome and cleft palate, although no direct link has been found to explain the correlation between the diseases. A person with Binder’s syndrome has a flat nose 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’s syndrome is not yet known as of 2011, although research has suggested there may be a possible genetic link. Case studies of individuals with the condition have shown a family history of the syndrome. While there is evidence of a familial correlation, no solid evidence has been found to prove or disprove a genetic cause. No evidence has yet been found showing that environmental causes are a contributing factor in the development of Binder’s syndrome.
Physically, the main physical feature of Binder’s syndrome is an extremely sunken nasal cavity, resulting in a flat nose. This abnormality is caused by the absence of the structure that supports the nose, called the nasal spine. Other physical characteristics attributed to the condition also include missing small incisors and molars.
When a patient’s skull is examined on X-rays, two key maxillofacial structures are often missing or deformed. The cartilage that divides the mouth from the nasal cavity is usually absent or severely malformed. The frontal sinuses are also sometimes known as misshapen or smaller than the average individual.
Complications associated with Binder’s syndrome are varied, depending on the individual patient. Many people show an increase in sinus infections. These infections are often due to blockage caused by a malformed nasal septum, which divides the two nostrils. Difficulty eating is sometimes noted, as the palate is often missing or deformed. Low intelligence has also been noted in people with Binder’s syndrome, although the exact cause of this has not yet been discovered.
When the complications that result from Binder’s syndrome become too bothersome, a nose graft is often a treatment option. During this surgery, the nose and nasal cavity are reconstructed from cartilage taken from an on-patient donor site or from an outside donor. The missing nasal spine is reconstructed and the deviations of the septum are corrected. This not only allows the patient to look more normal, but also corrects the associated breathing difficulties.
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