Orbital Hypertelorism

  • What is orbital hypertelorism

    Orbital hypertelorism happens during prenatal development when the fetal face is forming. In normal development, the eye sockets (orbits) develop laterally and rotate to their normal midline position. In orbital hypertelorism, the eye sockets fail to rotate into their normal position, resulting in wide-set eyes with extra bone between the eyes.

    Orbital hypertelorism is a symptom found in many conditions, most commonly frontonasal dysplasia, craniosynostosis syndromes, craniofrontonasal dysplasia, midline clefts involving the orbit, encephaloceles and others.

  • Symptoms

    Orbital hypertelorism is characterized by wide-set eyes with excess bone between the eyes.

  • Diagnosis

    Wide set eyes are identified when the distance between a child’s eyes is greater than the width of the eye from corner to corner. Orbital hypertelorism is diagnosed by a thorough medical evaluation that will include a complete ophthalmology assessment as well as imaging tests such as a CT scan or MRI to confirm the diagnosis.

  • Treatment

    It is important for children with orbital hypertelorism to be treated by a team with experience in the management of this condition due to the complex problems associated this craniofacial anomaly.

    Orbital hypertelorism can be treated surgically.

    For minor cases of orbital hypertelorism, your child’s surgeon may recommend an extracranial correction. In an extracranial correction, the bones of the inner portions of the nose and eye socket are brought closer together without going inside the skull.

    In more severe cases, your child may undergo a combined intracranial and extracranial procedure performed by a plastic surgeon and neurosurgeon. In this procedure, an ear-to-ear incision is made, the forehead bone is temporarily removed and the brain retracted, and the excess bone beneath the eyes is removed. The eye sockets are then cut and moved closer together and soft tissue is redraped. If other areas of the face such as the nose and upper jaw are displaced, those areas can be moved along with the orbit and brought together.

    Your child's surgeon will make an incision to move the entire eye socket, along with the eyes, towards the middle of the face, while also removing the excess bone between the eyes.

    An isolated osteotomy is the type of procedure most commonly used in this situation. An isolated osteotomy involves cutting and repositioning of the eye sockets. Some children with orbital hypertelorism also have a midface that is shortened and cleft. In these cases, surgeons will move the entire facial section inward, including the cranium, eye sockets, teeth and upper jaw.

    Surgical correction of orbital hypertelorism is generally performed at age 5 or older to allow for as much dental development as possible and prevent the risk of injury to developing teeth and structures. Earlier treatment may be needed if your child had eyelid abnormalities that cause eye exposure issues, lacrimal drainage problems such as blocked tear ducts, or strabismus, a condition in which the eyes don’t line up and do not look at the same object at the same time, causing depth perception and vision problems.

    Patients sometimes require secondary procedures to rebalance the eyes and lids and to improve tear drainage, relatively common issues associated with surgical treatment of orbital hypertelorism. To ensure the best outcomes for your child, you should seek treatment from a skilled team of surgeons and pediatric ophthalmologists who are experienced in treating children with this complex condition and the challenging complications associated with it.

    As your child grows and develops, secondary procedures may be required to elevate and tuck the corners of the eyes, remove excess soft tissue or nasal skin, or address other small irregularities of the skull and eye sockets. These secondary procedures may need to be repeated throughout your child’s life to address long-term challenges.

  • Why choose us

    At CHOP, children with orbital hypertelorism have access to the nation’s top pediatric specialists and coordinated care through our Craniofacial Program, one of the oldest, most experienced programs in the country dedicated to caring for children with craniofacial differences. Our experienced plastic and reconstructive surgeons, neurosurgeons and ophthalmologists are world-renowned for their skill and expertise treating rare conditions like orbital hypertelorism, working closely together to manage your child’s condition and connect him with any related services he may need, all within one institution.

    Reviewed by: Scott Bartlett, MD and Jesse Taylor, MD
    March 2014

Reviewed by Scott P. Bartlett, MD, Jesse A. Taylor, MD on March 31, 2014