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Houston Cranio Facial

Maxillonasal Dysplasia – Binder Syndrome

Also known as Binder syndrome, maxillonasal dysplasia is an uncommon congenital birth defect that affects the midface, nose, and upper jaw. “Maxillo” is a medical term that refers to the upper jaw, “nasal” refers to the nose, and dysplasia refers to a developmental disorder. In Binder syndrome, the maxillonasal region is underdeveloped, resulting in a short, broad, and flattened nose and a recessed or sunken upper jaw. Signs can range from mild to severe, and treatment should be tailored to suit the unique needs of the individual. Dr. Eric Payne, our experienced craniofacial surgeon, and his skilled multispecialty team offer the latest diagnostic tools and reconstructive techniques available.

At The Craniofacial and Plastic Surgery Center, we are committed to the compassionate care of our patients and their families. If you would like to know more about maxillonasal dysplasia, or if you would like to schedule a consultation with Dr. Payne, please contact our office today.

What is Binder syndrome?

Occurring in about one in every 10,000 births, Binder syndrome is a congenital (present at birth) defect that is primarily associated with facial bone deformities of the midface, nose, and jaw. Also known as maxillonasal dysplasia, this condition is not well understood. While there is some evidence for genetic and/or environmental influence, most cases appear to be spontaneous. Medical professionals have yet to identify the gene or genes that may play a role in this abnormality. Binder syndrome can cause a variety of signs and symptoms, and the type and severity of each will determine the treatment plan. This birth defect can be mild enough to go unnoticed and undiagnosed. However, recognizable characteristics include a sunken-in midface, short, flattened nose, recessed upper jaw, and protruding lower jaw. In some cases the child may have a cleft palate. Occasionally, spinal defects, hearing impairment, and heart problems can be present. The sense of smell typically will be unaffected.

How does Binder syndrome occur?

The cause of maxillonasal dysplasia is unknown. The small number of cases means limited research has been performed. The condition may be more common than realized due to the fact that mild instances may go undiagnosed. One possibility is that Binder syndrome is caused by the interaction of multiple genes, potentially in combination with environmental factors such as birth trauma, vitamin K deficiency, Phenytoin (an anti-seizure drug), or Warfarin (an anticoagulant drug). However, no environmental factor has been conclusively linked to the disorder. There have been individuals who had other affected family members, suggesting a genetic connection. Generally, however, Binder syndrome will develop as a result of random chance.

Can Binder syndrome be passed on through the family?

Binder syndrome may be a heritable condition, and there have been siblings or other family members of patients with maxillonasal dysplasia that showed signs. The anomaly seems to occur more frequently as a sporadic event. If you have a history of this deformity in your family and you’re planning on having children, you may wish to consult with a geneticist to determine what the risks, if any, may be.

What are the signs and symptoms of Binder syndrome?

In addition to the malformation of the midfacial area, nose, and jaw, maxillonasal dysplasia can include a wide variety of signs, depending on the severity of the disorder. Furthermore, this condition shares traits with some other syndromes, including chondrodysplasia, fetal Warfarin syndrome, Stickler syndrome, Crouzon syndrome, Apert syndrome, and Robinow syndrome. Proper diagnosis can enhance the treatment process, and Dr. Payne utilizes the most advanced technology available. Binder syndrome signs can include:

  • Flattened midface (midface hypoplasia)
  • Underdeveloped nose (nasal hypoplasia)
  • Short, flattened nasal bridge
  • Recessed upper jaw
  • Protruding lower jaw
  • Cleft palate
  • Backward-slanting upper lip
  • Malocclusion (bite misalignment); underbite
  • Cervical spine deformities (rare)
  • Misalignment of the eyes (rare)
  • Congenital heart defects (rare)
  • Hearing impairment (rare)
  • Mild intellectual disability (uncommon)

A child with mild Binder syndrome might have a slightly underdeveloped midface and nose, which could require a single operation (rhinoplasty, most commonly) to address. Other patients may require more significant facial reconstruction. The first step should be to determine if there are airway obstructions that can make breathing difficult. Dr. Payne will provide additional details about treatment options during the initial consultation.

What are the treatment options?

Each treatment plan will be tailored to the unique needs of the patient. Since Binder syndrome can include a broad array of signs and symptoms with varying degrees of severity, each child will have singular requirements. Evaluating and addressing any airway blockages is the primary consideration. If Dr. Payne and his team determine there is a significant concern, jaw advancement surgery, also called jaw distraction osteogenesis, may be performed to open up the airway to enhance breathing. For some children, jaw surgery will be the only recommended operation, while others may require initial and revision rhinoplasty, and some others may require both jaw and nasal surgery. Utilizing 3D virtual medical modeling, our skilled multispecialty team can develop a precise, customized treatment plan that optimizes your child’s outcome.

In the event the child can breathe relatively comfortably without surgical intervention, surgery may be delayed until the facial structures have matured. Between the ages of seven and 10, a rib cartilage bone graft may be placed to provide structural support to the nose and make breathing easier as the child grows. Once the patient is about 16 to 18 years old, a secondary rhinoplasty may be performed to improve the function and aesthetics of the nasal area. Prior to this, Dr. Payne will assess the structures of the upper and lower jaw to determine if reconstructive surgery will be needed. He may recommend using a specific technique, called Le Fort osteotomy, which is designed to address jaw malocclusion (misalignment). Orthodontics (or braces) is utilized to help position the teeth in a more ideal location in advance of the procedure. Binder syndrome can require several stages of treatment, and Dr. Payne is committed to helping parents understand each step of the process. Some patients may need bone grafts or facial implants to augment the maxillary midface around the nose, cheek, and orbital rims.

What is the recovery process like?

As with the treatment plan, the recovery process will be unique to the patient. Each surgical technique will have its own approximate recovery time, and each child will heal differently. Our experienced team is dedicated to the well-being of your child and your family. We can provide information and support as needed to help make this experience as gentle, fast, and safe as possible. If jaw advancement surgery is performed early in life, your child will need to spend a one to two days in the PICU (Pediatric Intensive Care Unit), followed by a few more days in the hospital. After jaw surgery at any age, the patient will first be given intravenous nutrition, and then a liquid and soft-food diet will have to be adopted until the jaw has sufficiently healed. Rhinoplasty typically will be an outpatient procedure, and your child should be able to return home the same day. In cases where a rib cartilage graft is used, an overnight stay in the hospital is necessary. We recommend the patient rest comfortably and avoid normal activities for one to two weeks, or as long as directed by Dr. Payne, before returning to school. Strenuous exercise and recreational events that could result in trauma to the nose should be avoided for at least eight weeks. We will provide more detailed instructions during your pre- and post-operative appointments.

Helping your child through treatment is our primary consideration. If you have additional questions about Binder syndrome, or if you would like to schedule a consultation with Dr. Payne, please contact our office today.