Binder Syndrome

What is Binder syndrome? Binder syndrome is a rare disorder that affects your face. It’s congenital, meaning it’s present at birth. People with Binder syndrome have underdeveloped facial bones, usually in their nose and upper jaw. Binder syndrome may affect your child’s breathing or eating. Usually, people with Binder syndrome have maxillofacial surgery in adolescence to reconstruct facial bones.

What is Binder phenotype? Binder phenotype is another name for Binder syndrome. Other names for Binder syndrome include: Binder type nasomaxillary dysplasia. Maxillonasal dysplasia. Nasomaxillary hypoplasia.

How common is Binder syndrome? Binder syndrome is rare. By some estimates, it occurs in less than 1 per 10,000 babies. It’s equally common in men and people assigned male at birth and women and people assigned female at birth.

What are the symptoms of Binder syndrome?

The most common symptom of Binder syndrome is underdevelopment in the central part of your face. People with Binder syndrome often have:

- Flattened nose and upper lips.

- Lower jaw that sticks out.

- Misaligned upper and lower teeth (malocclusion).

- Triangular or half-moon-shaped nostrils.

Less commonly, they may also have:

- Cleft palate.

- Congenital heart defects.

- Hearing problems.

- Intellectual disability.

- Spinal malformations.

- Strabismus (crossed eyes).

What causes Binder syndrome?

Experts don’t know exactly what causes Binder syndrome. Most of the time, babies develop the condition for no known reason.

Some families have more than one child with Binder syndrome. This could mean genetics play a role in developing Binder syndrome, but researchers don’t know for sure.

Researchers think some environmental factors could increase your risk of having a baby with Binder syndrome. These factors include:

- Alcohol use during pregnancy.

- In utero exposure to certain drugs, including phenytoin (Dilantin®, Phenytek®) or warfarin (Coumadin®, Jantoven®).

- Vitamin K deficiency during pregnancy.

- Trauma to your infant during birth.

How is Binder syndrome diagnosed?

Healthcare providers may initially diagnose Binder syndrome based on your baby’s facial appearance. They usually take CT scans, MRIs or ultrasounds to view your baby’s bone structure and confirm or rule out the diagnosis.

How is Binder syndrome treated?

Binder syndrome treatment varies based on symptoms and may include:

Orthodontic care: Braces help realign your child’s jaw and straighten their teeth. In minor cases, orthodontic care may be the only treatment a child needs. Other times, children wear braces for a period before or after surgery.

Surgery: A craniofacial surgeon may use grafts of bone, cartilage or synthetic material to reshape your child’s nose (rhinoplasty) and may also perform a Le Fort I or II osteotomy, which are procedures to reposition the jaw. Usually, surgeons recommend waiting to have surgery until your child’s facial bones have stopped developing, usually between ages 15 and 19.

How can I prevent Binder syndrome?

Because experts don’t know exactly what causes Binder syndrome, there’s no guaranteed way to prevent it. People who are pregnant may lower their risk of having a child with the condition by limiting exposure to certain environmental factors. You might speak with your healthcare provider about:

- Medication safety during pregnancy, especially concerning phenytoin and warfarin.

- Vitamin deficiencies, including vitamin K deficiency.