What is Binder Type Nasomaxillary Hypoplasia?

Binder Type Nasomaxillary Hypoplasia is an extremely rare congenital developmental disorder mainly characterized by an underdeveloped middle portion of the face to include the area around the nose and the upper jaw. The symptoms which an individual experiences due to Binder Type Nasomaxillary Hypoplasia are significantly variable and can range from mild to extremely severe deformities. The primary presenting features of it include an underdeveloped maxillary or the upper jaw bone and an underdeveloped or a flattened nose.

The root cause of Binder Type Nasomaxillary Hypoplasia is not yet known, but people with a family history of this disorder are known to have this condition. Thus a genetic link cannot be excluded in development of it. Surgery is the front line treatment for the deformities that are present in a child with Binder Type Nasomaxillary Hypoplasia.

What Causes Binder Type Nasomaxillary Hypoplasia?

As stated, the exact cause for Binder Type Nasomaxillary Hypoplasia is not yet known but there have been many cases where multiple family members of the affected child have had this condition which points towards a genetic link towards the development of it. Some studies also suggest that Binder Type Nasomaxillary Hypoplasia is inherited as an autosomal dominant or recessive manner although there is not confirmed evidence to prove this.

Some studies have also suggested a combination of genetic factors and environmental factors as a cause for the development of it. Some of the environmental factors that are believed to play a role in the development of Binder Type Nasomaxillary Hypoplasia are trauma at the time of the birth of the affected child, deficiency in vitamin K, or exposure of the fetus to anti-clotting agents like warfarin or antiseizure medications. However, there is not substantial proof to suggest this as being a confirmed cause of Binder Type Nasomaxillary Hypoplasia.

What Are The Symptoms of Binder Type Nasomaxillary Hypoplasia?

Because of the rarity of the disease condition and lack of enough studies and a very small number of patients being affected by this condition makes it difficult for physicians to form a comprehensive list of symptoms that a child tends to experience due to Binder Type Nasomaxillary Hypoplasia.

However, there are some symptoms which are quite characteristic of it and include underdevelopment of the central part of the face to include the nasal and the upper jaw region of the face. There is abnormal flattening of the central part of the face. The nasal bridge is flattened. There is abnormal positioning of the nasal bones. The columella which is a sheet of cartilage, which separates the right and the left nostrils is also abnormally short and is of the shape of a comma in children with Binder Type Nasomaxillary Hypoplasia.

There is also malocclusion or improper positioning of the upper teeth in comparison to the lower teeth in children with it. 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 Hypoplasia.

How Is Binder Type Nasomaxillary Hypoplasia Diagnosed?

The diagnosis of Binder Type Nasomaxillary Hypoplasia is typically made based on the characteristic symptoms displayed by the affected child. The physician will take a detailed history and inquire as so any other family members who may have similar symptoms as that of the child. The physician may order advanced imaging studies in the form of x-rays, CT and MRI scans of the facial area, which will clearly show an underdevelopment of the maxillofacial area and the areas of the nasal region which will confirm the diagnosis of Binder Type Nasomaxillary Hypoplasia.

How Is Binder Type Nasomaxillary Hypoplasia Treated?

Due to the rarity of the disease and extremely limited number of patients actually affected by this condition, there are as of yet no specialized treatments for Binder Type Nasomaxillary Hypoplasia. 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 Hypoplasia.

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 Hypoplasia, 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 Hypoplasia.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: November 20, 2017

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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