Binder syndrome type nasomaxillary dysplasia

Binder type nasomaxillary 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).

Binder type nasomaxillary dysplasia is a rare congenital condition that affects males and females in equal numbers. The exact incidence or prevalence is unknown. One estimate suggests that Binder syndrome occurs in less than 1 per 10,000 live births. However, individuals may go undiagnosed or misdiagnosed making it difficult to determine the true frequency in the general population. Please discuss with your doctor for further information.

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The exact, underlying cause of Binder type nasomaxillary dysplasia is not fully understood. In many cases, the disorder is believed to occur spontaneously, for no apparent reason (sporadically). However, there have been reports in the medical literature of families in which more than one family member was affected. This suggests that genetic factors play a role in some affected individuals.

Some researchers have suggested that Binder type nasomaxillary dysplasia is a genetic disorder inherited in either an autosomal dominant or recessive manner. Other researchers have suggested that the disorder is caused by complex genetic factors, specifically the interaction of many different genes, possibility in combination with environmental factors (multifactorial inheritance).

Please consult with your doctor for further information.

Because of the rarity of the disease condition, lack of enough studies and a very small number of patients being affected by this condition, it is difficult for physicians to form a comprehensive list of symptoms that a child tends to experience due to Binder type nasomaxillary dysplasia.

However, there are some symptoms which are quite characteristic, including:

  • Underdevelopment of the central part of the face, particularly the nasal and the upper jaw region
  • Abnormal flattening of the central part of the face
  • Flattenednasal bridge
  • Abnormal positioning of the nasal bones
  • Abnormallyshort columella-a sheet of cartilage that separates the right and the left nostrils. Also this columella is of the shape of a comma
  • Malocclusion or improper positioning of the upper teeth in comparison to the lower teeth

It is also believed that children with it are more at risk for developing abnormalities of the spine. There have been a few cases in which the child has displayed hearing impairment, cleft palate, strabismus of the eyes, and congenital heart defects but studies are inconclusive as to whether these abnormalities are related in any way to Binder type nasomaxillary dysplasia.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

A diagnosis of Binder syndrome is based upon identification of characteristic symptoms, a detailed patient history, and a thorough clinical evaluation. Certain specialized tests can be used to confirm the diagnosis.

Clinical testing and workup

Specialized imaging techniques may be used to help obtain a diagnosis of Binder syndrome. Such tests include computerized tomography (CT) scanning and magnetic resonance imaging (MRI). During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of certain tissue structures. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues.

Such exams may yield specific findings including underdevelopment or absence of the bony protrusion that projects from the base of the nasal septum to join with the middle part of the upper jaw (anterior nasal spine); thinness of a portion of the upper jaw known as the alveolar bone, which forms the dental arch over the upper incisors; underdevelopment or absence of the frontal sinuses; and/or certain abnormalities detected with cephalometric studies, which are scientific measurements of particular craniofacial dimensions.

There are as of yet no specialized treatments for Binder type nasomaxillary dysplasia. However, there are some forms of treatments that are recommended for the few cases of it. These treatment options include primarily surgery of the maxillofacial and nasal areas to correct the abnormalities of the nose and the jaw.

The type of surgery performed depends on the severity of the abnormality and other factors like the overall age and health of the patient. It is likely that the patient will require more than one surgery to correct the abnormality.

In most cases, the patient is treated in childhood but there have been some cases where the doctors prefer to wait it out till the child grows up a little bit in order to perform the complex and exhaustive surgeries that are required to treat Binder type nasomaxillary dysplasia.

In cases of malocclusion where the upper teeth are not completely aligned then use of braces will be required to correct the abnormality. Nasal reconstruction is yet another surgery that is usually performed in severe cases of deformities in the nasal region as a result of it.

For extremely severe cases of Binder type nasomaxillary dysplasia, surgical procedures known as Le Fort I and Le Fort II osteotomy are performed, in which the upper jaw is repositioned to correct the malocclusion and cleft palate if it is present and Le Fort II surgery involves repositioning of the upper jaw and nose to correct the deformities caused by Binder type nasomaxillary dysplasia.

If you have any questions, please consult with your doctor to better understand the best solution for you.

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