Hypertelorism is the excessively increased distance between the paired organs of the human body. The most visible and noticeable for the environment is ocular hypertelorism, i.e. wide spacing of eye sockets. It is believed in many circles that ocular hypertelorism in women increases their sexual attractiveness.

Ocular hypertelorismwas first described by David Greig in 1924. It is assessed on the basis of many measurements, especially the distance between the pupils. Ocular hypertelorism often occurs with other conditions such as, for example :

  • Loeys-Dietz syndrome
  • Edwards syndrome
  • Turner syndrome
  • Zellweger syndrome

Ocular hypertelorism - causes

The change is caused by a defective structure of the skull. There are several theories explaining the development of ocular hypertelorism.

The first one emphasizes the role of inhibiting the growth of larger wings of the sphenoid bone at the stage of embryonic development. As a consequence, the smaller wings are spaced wider, so the walls of the eye sockets are further apart.

Another theory takes into account clefts within the eye socket and skull.

Yet another approach identifies the cause of this congenital defect as premature fusion (ossification) of the cranial sutures, which leads to midfacial underdevelopment (hypoplasia) and narrowing of the skull (craniostenosis).

Ocular hypertelorism can vary in severity. A change occurs when the distance between the pupils is greater than the average value characteristic for the representatives of a given age and ethnic group by 2 standard deviations.

For example: the width of the eyelid gap and the distance between its outer angles in white babies is smaller compared to black babies, but the distance between the inner angles of the eyelid gap is similar.

Ocular hypertelorism - symptoms

Widely spaced eye sockets are the main symptom of hypertelorism. This condition may also be accompanied by other symptoms, which include:

  • shallower eye sockets
  • forward shifting of the eyeballs (proptosis)
  • drying of the conjunctiva and the cornea
  • regurgitation of the eyelids
  • optic disc swelling
  • divergent squint
  • disorderseye mobility

Ocular hypertelorism can be an isolated congenital anomaly or develop, rather rarely, as a result of facial trauma. This form of the condition is known assecondary hypertelorism .

Widely spaced eye sockets may occur in other craniofacial syndromes. Ocular hypertelorism can be part of the clinical picture of such entities as :

  • Down syndrome
  • Turner syndrome
  • Crouzon's syndrome, the so-called craniofacial dysostosis
  • Aperta team
  • Edwards syndrome
  • triploidy (presence of an additional set of chromosomes in the cell)
  • Wolf-Hirschhorn syndrome

Ocular hypertelorism - diagnosis

The diagnosis of ocular hypertelorism is based primarily on the following measurements:

  • the width of the eyelid gap
  • interpupillary distance
  • distances between the inner angles of the eyelid gap
  • distances between the outer angles of the eyelid gap

and the calculation of the Pharkas index, where a value greater than 42 indicates hypertelorism.

Other abnormal facial dysmorphic features may contribute to the incorrect diagnosis of ocular hypertelorism, including:

  • flat nose
  • divergent squint
  • diagonal wrinkles
  • prominent eyebrows
  • telecanthus (increased distance between the inner angles of the eyes)
  • narrow eyelid gaps

Ocular hypertelorism - treatment

Surgical treatment is the most effective therapeutic solution in the case of ocular hypertelorism.

One of the most frequently undertaken methods is the Tessier operation. During the procedure, excess tissues located in the orbital area are cut out.

The treatment significantly improves the appearance of the face.

Isolated, slight ocular hypertelorism is relatively common, and in the case of women it is considered a trait that increases sexual attractiveness.

About the authorAnna Jarosz A journalist who has been involved in popularizing he alth education for over 40 years. Winner of many competitions for journalists dealing with medicine and he alth. She received, among others The "Golden OTIS" Trust Award in the "Media and He alth" category, St. Kamil awarded on the occasion of the World Day of the Sick, twice the "Crystal Pen" in the national competition for journalists promoting he alth, and many awards and distinctions in competitions for the "Medical Journalist of the Year" organized by the Polish Association of Journalists for He alth.

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Category:

Genetic diseases