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Houston Cranio Facial

Frontonasal Dysplasia

Frontonasal dysplasia (FND) is a condition typically involving a midfacial cleft and one or more other signs. Children with FND often have wide-set eyes, a vertical groove down the middle of the face, a flat, broad nose, encephalocele, and/or other characteristics. Present at birth, some aspects of this disorder may worsen over time without treatment; however, advanced techniques make it possible to correct many of the concerns and achieve the discussed cosmetic and functional results. Dr. Eric Payne our experienced craniofacial and plastic surgeon, customize each treatment plan to best suit the needs of the child. Our skilled multispecialty medical team is extensively trained and highly knowledgeable, and we can provide information, support, and a compassionate environment while your family is in our care.

The Craniofacial and Plastic Surgery Center is committed to helping you and your family throughout the treatment process. To learn more about FND, or to schedule a consultation with our physician, please contact our practice today.

What is frontonasal dysplasia?

Also known as median cleft face syndrome, FND is a condition that occurs when there is abnormal neonatal (before birth) development of the head and face. Many characteristics are associated with this disorder, which primarily affect the forehead, orbits (eye sockets), nose, and upper lip. Patients may experience a few or several of these effects. Furthermore, FND may in some cases be a part of another condition. If this is a concern, Dr. Payne will take into consideration the additional disorder, which may affect the body as well as the face, when developing a treatment plan. While many children have a mild to moderate expression of FND, severity can range dramatically. Intellectual disability can occur in more significant instances of FND, although most children with this malformation experience no mental defects. There are many different classifications for FND. It has commonly been referred to as Tessier’s Facial cleft #0 and/or #14. But this lacks more description in the variation of the deformity.

How does frontonasal dysplasia occur?

As a fetus develops, the craniofacial tissues gradually integrate to form recognizable features. Should something disrupt this process, which occurs in the first trimester, facial clefts and other concerns can arise. FND is a rare condition that most commonly occurs as a random, or sporadic, event. As a result, the cause is unknown (idiopathic) and there are no blood tests that can be performed to aid in diagnosis. Most children will have the disorder identified by ultrasound or after birth by visual inspection. There is some evidence to suggest it can run in families; however, the gene or genes that may be responsible are currently unknown.

Can frontonasal dysplasia be passed on through the family?

While the gene or genes that may contribute to the condition have not been identified, FND may be heritable. It is unknown if one or both parents are required to have a link to the disorder, but individuals who have a family history of FND may wish to speak to a genetic counselor about their options. Some scientists believe that several genes as well as environmental factors (multifactorial inheritance) work together to cause FND. However, this is a very rare syndrome, and little is currently known about its causes. The majority of cases have developed as a sporadic event.

What are the signs and symptoms of frontonasal dysplasia?

FND can vary widely from patient to patient, including the type and number of signs and symptoms as well as their degree of severity. FND is also linked with a number of other disorders, which can affect the heart, skeletal structure, or other areas of the body. If your child is diagnosed with a subtype of FND, Dr. Payne will work with your pediatrician and the necessary medical specialists to provide optimal care. In general, an FND diagnosis will include two or more of the following signs and symptoms:

  • Hypertelorism (wide-set eyes)
  • Eyelid defects
  • Ocular abnormalities
  • Vertical cleft (facial groove) that can extend from the upper lip to the forehead (or any distance between the two)
  • V-shaped hairline that comes to a peak in the central forehead
  • Flat, broad, or divided nose
  • Slit-like nostrils
  • Notched or missing nasal tip
  • Cleft lip and/or palate
  • Encephalocele (protrusion of brain tissue through an opening in the base of the skull)
  • Abnormally short and wide head (brachycephaly)
  • Skin-covered gap on the front of the head (cranium bifidum occultum)
  • Ear skin tags
  • Hearing loss
  • Anomalies of the Central Nervous System (CNS) – Holoprosencephaly
  • Intellectual disability in severe cases

Children with a mild to moderate form of FND typically have normal intelligence and a normal lifespan. In the event hypertelorism is severe, or if FND is syndromic (occurring in association with other malformations), learning disabilities, cardiac abnormalities, and other serious issues may be a concern. Craniofacial surgery is often used to address the various signs affecting the head and face, and this can be very effective in correcting and normalizing the patient’s features.

What are the treatment options?

Treatment for FND begins with an accurate diagnosis and thorough evaluation of the patient’s unique expression of the condition. As a result, Dr. Payne utilize the most advanced craniofacial technology available to help obtain a precise understanding of the child’s needs. Typically radiology tests such as a CT scan is necessary to determine the extent of bone defect and any possible brain abnormalities. With this information in hand, the surgical approach can be customized to optimize care at every stage. Some of the most common procedures will include forehead and nasal reconstruction. Orbital reconstruction typically will be necessary in instances of significant hypertelorism, and other techniques may be recommended based on the patient’s anatomic indicators.

In severe cases of FND, some immediate treatment may be necessary to address problems with eating or breathing. If urgent medical care is unnecessary, most reconstructive procedures will begin when the patient is approximately six to eight years of age. In some cases, Dr. Payne may advise performing surgery earlier or later, depending on your child’s needs. If a cleft lip and/or palate are present, these concerns can be corrected as early as 3 to 12 months of age. If skull bone is missing (as in cranium bifida occultum), cranial vault reconstruction can be performed and a nasal bone graft can be used to build the nose. Depending on the severity of the signs, additional surgery may be necessary later in life to further improve the appearance and correct the position of certain features. Throughout this sensitive process, our extensively trained medical team will provide unparalleled care designed to support you and your child and keep you and your family well-informed.

What is the recovery process like?

Since the treatment plan will range from patient to patient depending on their unique signs and symptoms, the recovery process will similarly vary. Additionally, every child’s healing rate will be different, and Dr. Payne can provide more specific information about the timeline during your consultations. Our skilled medical team is committed to providing exceptional patient care and assisting your family at every stage of treatment.

Procedures involving cranial reconstruction typically require the child to remain in the Pediatric Intensive Care Unit (PICU) for at least a day, followed by several more days in the hospital. While rhinoplasty is often an outpatient procedure, if a bone graft is needed, an overnight stay in the hospital will be necessary. Activities during recovery will be limited for as long as Dr. Payne recommends, and the length of time will depend on the type of surgery and patient’s rate of healing. Ongoing care may be needed for children with associated syndromes, depending on the condition and symptoms. Our team will provide more information if this applies to you.

We understand that having a child with abnormalities can be very stressful and emotional. Our experienced and knowledgeable team’s highest priority is the care and well-being of your child, and we will do everything in our power to make the treatment process as quick, gentle, and safe as possible. For more information about FND, or to schedule a consultation with Dr. Payne, please contact us today.