What causes orbital hypertelorism?

            Have you ever pondered how a perfect human is tailored with certain measurements of organs, the complex body functions and numbers of body parts which makes a human able to live their daily life? An alteration even in the molecular level or gene will cause disturbance to the normal body function. In this article, we will be talking more about orbital hypertelorism, a condition making a person’s eye look different to others. In medicine, this condition usually does not pose problems for a person to function well but it may cause wariness especially for parents.

            Orbital hypertelorism is a condition describing the distance between the eyes is larger than normal. It is considered a birth defect. In a normal condition, the eye sockets develop laterally (a direction further away from the face’s middle) and rotate to their normal middle line position of the face. This is in contrast with orbital hypertelorism where the eye socket fails to rotate into the normal position, leading to the wide distance between the eyes. It is thought to be due to an alteration in the development of the face during its embryo phase during the 4 to 8 weeks of pregnancy. 

            Orbital hypertelorism can be caused by many different causes and sometimes is unknown. These causes are difficult to identify since it usually involves changes during the pregnancy and to determine the exact causes may need many additional tests such as genetic testing and imagine tests such as CT and MRI scan. Below are common causes related to the hypertelorism:

  • Abnormal development of the forehead bones and base of skulls
  • A cleft between the eyes, the bones of the nose or upper face
  • Craniosynostosis syndromes, premature fusion of the bony plates in the baby’s skull too early that is way before the baby’s brain is fully formed
  • Edwards syndromes
  • Apert syndrome
  • Crouzon syndrome
  • Aarskog syndrome
  • Waardenburg syndrome
  • Down syndrome
  • Cri-du-Chat syndrome

       Orbital hypertelorism usually does not cause significant symptoms but some would actually face vision problems. Aside from the widened distance between the eyes, a flat nasal bridge may be seen. Dystopia of the eye, a condition of the eyes that is not on the same horizontal line on the face may occur. This led to one eye positioned lower than the other eye. Typically, other symptoms are associated with the causes underlying the hypertelorism. For instance, if the causes are an encephalocele, a defect of the skull over the nose and forehead can be found. A child with orbital hypertelorism may show psychological distress such as being irritable due to the environment such as from getting judged or bullied by others.

       Treating orbital hypertelorism may not be urgent as it is usually for cosmetic purposes. However, this is a necessary step to take as it affects the child’s life both physically and mentally. It can help a child to avoid visual issues and to be more confident interacting with other people. Surgery is the mainstay for orbital hypertelorism. Timing of surgery is usually between the ages 5 and 7 years old to avoid injury of the yet erupted tooth or maxillary growth problem that could lead to dental malformation. This timing could change depending on the causes such as craniosynostosis that need to be corrected before the age of one. Patients sometimes need secondary procedures repeatedly throughout their life to help improve the eyes position and to improve tear drainage. Patients with hypertelorism may be associated with intellectual disability, other anatomical abnormalities that could affect their daily life function and risk for other diseases. These patients will need to be treated with the help and support of other medical specialties such as paediatrician and rehabilitative team. Get Vaccinated.