Orbital Hypertelorism: Causes, Symptoms & Treatment
Understanding orbital hypertelorism: causes, symptoms, diagnosis, and treatment options for wide-set eyes.
What Is Orbital Hypertelorism?
Orbital hypertelorism is a medical term used to describe a condition in which the eyes are abnormally far apart. This occurs due to a developmental irregularity during the prenatal period, when the fetal face is forming. Normally, the eye sockets (orbits) develop laterally and then rotate to their typical midline position. In orbital hypertelorism, this rotation does not occur properly, resulting in wide-set eyes and extra bone between them.
Causes of Orbital Hypertelorism
Orbital hypertelorism is not a disease in itself but rather a symptom that can be associated with a variety of underlying conditions. The most common causes include:
- Genetic syndromes: Many genetic disorders, such as craniosynostosis syndromes, frontonasal dysplasia, craniofrontonasal dysplasia, and Weaver syndrome, can cause orbital hypertelorism.
- Congenital malformations: Midline clefts involving the orbit, encephaloceles, and other craniofacial anomalies may also lead to this condition.
- Tumors: Rarely, orbital hypertelorism can be caused by tumors that push the orbits apart.
- Other causes: In some cases, orbital hypertelorism may be isolated, meaning it occurs without any other associated conditions.
Because orbital hypertelorism can be a sign of an underlying genetic disorder, providers may recommend genetic counseling to help families understand their risk for other genetic conditions.
Symptoms of Orbital Hypertelorism
The primary symptom of orbital hypertelorism is wide-set eyes. However, the severity and associated symptoms can vary depending on the underlying cause and the extent of the condition. Common symptoms include:
- Abnormally wide distance between the eyes
- Visual disturbances, such as double vision or difficulty focusing
- Facial asymmetry
- Breathing and eating difficulties, especially if the eyes are pushed into the nasal cavity
- Headaches, changes in vision, or cognitive function (if caused by a tumor)
It is important to note that isolated orbital hypertelorism (without other underlying conditions) does not usually affect vision, breathing, or development. However, it can have cosmetic and psychological impacts, which may prompt families to seek treatment.
Diagnosis of Orbital Hypertelorism
Diagnosing orbital hypertelorism involves a thorough medical evaluation. The process typically includes:
- Physical examination: A healthcare provider will measure the distance between the eyes and compare it to the width of the eye from corner to corner. If the distance between the eyes is greater than the width of the eye, orbital hypertelorism is suspected.
- Ophthalmology assessment: A complete eye exam is performed to evaluate vision and eye alignment.
- Imaging tests: CT scans or MRI are used to confirm the diagnosis and assess the extent of the condition. These imaging tests can also help identify any underlying causes, such as tumors or craniofacial anomalies.
Treatment Options for Orbital Hypertelorism
The treatment of orbital hypertelorism depends on the underlying cause and the severity of the condition. In some cases, no treatment is necessary, especially if the condition is isolated and does not cause any functional problems. However, if orbital hypertelorism is associated with other conditions or causes significant cosmetic concerns, treatment may be recommended.
Surgical Correction
Surgical correction is the main treatment for orbital hypertelorism. The goal of surgery is to move the orbits closer together, giving the child a more typical face shape and improving the space between the eyes. Surgery is usually performed when the child is between 5 and 7 years old, to allow for as much dental development as possible and to minimize the risk of injury to developing teeth and structures.
There are two main surgical techniques used to correct orbital hypertelorism:
- Box osteotomy: This procedure is used for mild to moderate cases. The surgeon removes part of the nasal bone and moves the remaining areas between the orbits closer together.
- Facial bipartition: This technique is used for severe cases or when there are also issues with the mouth or teeth. The surgeon removes a triangular section of the bone above the nose and divides the roof of the mouth down the middle. The two large areas around the orbits are then rotated to correct the hypertelorism.
In some cases, additional procedures may be needed to address other facial irregularities, such as rhinoplasty to improve the projection of the nose or orthodontic treatment to correct jaw misalignment and bite problems.
Non-Surgical Treatments
For cases where surgery is not necessary or not possible, other treatments may be considered:
- Orthodontic treatment: May be needed to correct jaw misalignment and bite problems caused by orbital hypertelorism.
- Physical therapy: Can help improve muscle strength and range of motion in the face and limbs when necessary.
- Radiation therapy: High-frequency radiation may be used to shrink or eliminate tumors causing hypertelorism.
- Medications: May be used to manage symptoms such as headaches, changes in vision, or cognitive function caused by tumors or other underlying conditions.
Potential Complications After Orbital Hypertelorism Surgery
While orbital hypertelorism surgery is generally safe, all surgeries carry some risks. Potential complications include:
- Infection
- Bleeding
- Scarring
- Changes in vision
- Need for additional surgeries to address long-term challenges
Patients sometimes require secondary procedures to rebalance the eyes and lids and to improve tear drainage, which are relatively common issues associated with surgical treatment of orbital hypertelorism. As the child grows and develops, additional procedures may be needed 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.
Outlook for People with Orbital Hypertelorism
The outlook for people with orbital hypertelorism is generally very good, especially for isolated cases. With or without corrective surgery, most individuals with orbital hypertelorism can lead healthy, normal lives. When hypertelorism is associated with an underlying condition, the outlook depends on the severity and treatment of that condition.
Frequently Asked Questions (FAQs)
Q: Is orbital hypertelorism a disease?
A: No, orbital hypertelorism is not a disease but a symptom that can be associated with various underlying conditions, including genetic syndromes and congenital malformations.
Q: Can orbital hypertelorism affect vision?
A: In isolated cases, orbital hypertelorism does not usually affect vision. However, if it is associated with other conditions, visual disturbances such as double vision or difficulty focusing may occur.
Q: When is surgery recommended for orbital hypertelorism?
A: Surgery is usually recommended when the child is between 5 and 7 years old, to allow for as much dental development as possible and to minimize the risk of injury to developing teeth and structures.
Q: What are the risks of orbital hypertelorism surgery?
A: Risks include infection, bleeding, scarring, changes in vision, and the need for additional surgeries to address long-term challenges.
Q: Can orbital hypertelorism be treated without surgery?
A: Yes, in some cases, non-surgical treatments such as orthodontic treatment, physical therapy, radiation therapy, or medications may be used to manage symptoms or correct associated problems.
Takeaway
Orbital hypertelorism is a developmental irregularity in which the orbits are widely spaced. It is a clinical feature of many different conditions, but isolated cases do not usually lead to any other physical complications. Surgery can correct it, usually between the ages of 5 and 7. When hypertelorism results from an underlying condition, treatment of that condition is usually necessary first.
References
- Orbital Hypertelorism — Children’s Hospital of Philadelphia. 2023. https://www.chop.edu/conditions-diseases/orbital-hypertelorism
- What Syndromes Cause Wide Eyes? — MedicineNet. 2023. https://www.medicinenet.com/what_syndromes_cause_wide_eyes/article.htm
- Hypertelorism: Definition, Diagnosis, Causes, and Treatment — Healthline. 2023. https://www.healthline.com/health/hypertelorism
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