Orbital Hypertelorism

What is orbital hypertelorism?

Orbital hypertelorism is the medical term for having widely spaced eyes. You might see it referred to as ocular hypertelorism.

Orbital hypertelorism itself isn’t a disease or a condition. It’s usually a symptom of a birth defect or genetic disorder. No matter what causes it, babies born with orbital hypertelorism have eye sockets that are further apart than they should be. They’ll also have extra bone between their eyes that fills in the additional space.

Most people with orbital hypertelorism don’t have any vision problems. How much orbital hypertelorism affects your child’s vision depends on how wide their eyes are and any other symptoms or conditions they have.

How is orbital hypertelorism diagnosed?

Your healthcare provider will identify orbital hypertelorism when your baby is born or on an ultrasound while they’re developing.

There’s no perfect amount of space for a person to have between their eyes. However, healthcare providers usually use two sets of measurements to help diagnose orbital hypertelorism:

• Inner canthal distance: The distance from the inside edge (the side closest to your nose) of one eye to the other.
• Pupillary distance: The distance between the centers of your pupils.

Children born with orbital hypertelorism have wider amounts of space on both of their inner canthal distance and pupillary distance.

What are the most common causes of orbital hypertelorism?

Experts think orbital hypertelorism happens between the fourth and eighth week of a fetus’s development. A fetus’s skull forms in a few steps, like a puzzle coming together. But instead of a flat jigsaw puzzle on a table, its skull forms like a 3D model. If something changes or interrupts one of those steps, there’s a chance the fetus could develop orbital hypertelorism.

The most common causes of orbital hypertelorism are birth defects and genetic disorders.

Birth defects

A birth defect, or congenital condition, is something visibly, internally or chemically unusual about your newborn baby’s body. It can affect how your child’s body physically works or looks — it can also cause issues you can’t see from the outside. Congenital conditions are caused by:

• Genetic and hereditary factors.
• Infections.
• Radiation exposure.
• Exposure to recreational drugs or alcohol.

Some congenital conditions have no known cause and happen randomly.

If something interrupts the fetus’s development while its skull is forming, the brain will fill in the gaps with bone. If this happens before the fetus’s eye sockets form and move to their proper positions, it can develop orbital hypertelorism.

Craniosynostosis is a congenital condition that can cause orbital hypertelorism. Craniosynostosis occurs when the fetus’s skull bones fuse too early.

Genetic disorders

Genetic disorders happen when a mutation (a harmful change to a gene) affects your genes, or when you have the wrong amount of genetic material. Genes are made of DNA (deoxyribonucleic acid), that contain instructions telling cells how to function and the characteristics that make you unique. You receive half your genes from each biological parent and may inherit a gene mutation from one or both parents.

Orbital hypertelorism can be caused by a variety of genetic conditions, including:

• Apert syndrome.
• DiGeorge syndrome.
• Edwards syndrome.
• Diastrophic dysplasia.
• Crouzon syndrome.
• Noonan syndrome.
• Neurofibromatosis type 1.

Your provider might recommend genetic counseling to help you understand your family’s risk for genetic disorders.

How is orbital hypertelorism treated?

Children with orbital hypertelorism can have surgery to move their eyes closer together. This surgery is usually performed when they’re between 5 and 7 years old. Orbital hypertelorism surgery is a type of reconstructive surgery that gives children a more typical face shape and space between their eyes.

Your child will need regular eye exams before and after their surgery to monitor any changes in their eyes and vision.

There are two techniques your surgeon might use to realign your child’s eyes:

• Box osteotomy: Your surgeon will remove extra bone and skin from above your child’s nose and reshape their eye sockets into that space. Picture a rectangle where the long sides run along your eyebrows and right above your nose and the short sides run along the outside edges of your eyes. Your surgeon will reshape your child’s bones to fit into a similar shape.
• Facial bipartition: Facial bipartition is a more complicated surgery than box osteotomy. It’s usually done if a child with orbital hypertelorism has structural issues with other facial bones too. Your surgeon will reshape your child’s eye sockets, nose and cheekbones to realign their eyes and correct any issues with their jaw and teeth. Picture a V shape that with the two upper ends at the outside top corners of your eyes and the bottom point ending right under your nose in the middle of your upper lip. That’s roughly the shape your surgeon will follow to restructure your child’s face.

What are complications of orbital hypertelorism surgery?

Potential complications after orbital hypertelorism include:

• Bleeding.
• Infections.
• Scarring.
• Ptosis (drooping eyelids).
• Diplopia (double vision).
• Decreased vision or blindness.

When should I see my healthcare provider?

See your healthcare provider as soon as you notice any changes in your child’s eyes or vision.

Go to the emergency room if your child has any of the following symptoms:

• A sudden loss of vision.
• Severe eye pain.
• Sees new flashes or floaters in their eyes.

A note from Cleveland Clinic

Being born with orbital hypertelorism doesn’t mean your baby won’t be healthy or develop like they should. Depending on what caused the orbital hypertelorism, they might need treatment for a while. Talk to your provider about what your child will need, and which changes or symptoms you should look out for. Talk to your surgeon about what to expect before and after surgery, if your child will need it.