Hypertelorism

Hypertelorism

Hypertelorism is an increase in interobital distance. This article details causes, classifications and treatment options

Summary Card


Definition
An increase in the interorbital distance between the dacryons.

Classification
Tessier classification is based on interobital distance. Normal is 20-30mm.

Causes
Syndromic (Apert's, Crouzens, Edwards) or non-syndromic (facial clefts, midline tumours).

Management
An MDT to restore orbital dystopia and cosmesis through box osteotomy, facial bipartition, and medial canthopexy.



Definition of Hypertelorism

Hypertelorism is an increase in the interorbital distance as measured between the dacryons. It results in a true lateralization of the orbit where the inner canthal distance (ICD), the outer canthal distance (OCD) and interpupillary distance (IPD) are all increased.

It is a congenital condition caused by overexpansion of ethmoid sinuses.

Definition of Hypertelorism


Classification of Hypertelorism

Hypertelorism is a measurement from the dacryon. This is the junction between the maxillary, frontal and lacrimal bones. It is a radiological, not a clinical measurement.

averagermal adult interorbital distance can range from 20-30mm.  Tessier provides a classification sythis, as detailed below in the illustration.

Classification of Hypertelorism

Itessentialtant to be aware of telecanthus. This increases in the intercanthal distance with the normal distance between the bony orbits.

  • Creates an appearance of pseudo-hypertelorism in patients with a flat nasal bridge or prominent epicanthal folds.
  • Linked to Down Syndrome, Fetal Alcohol Syndrome, Klinefelter Syndrome.


Causes of Hypertelorism

Hypertelorism can be syndromic or non-syndromic:

  • Syndromic: Apert's, Craniofrontonasal dysplasia, Crouzens, Edwards
  • Non-syndromic: Sincipital encephaloceles, facial clefts, midline tumours

An example of hypertelorism is seen in the illustration below of a patient with craniofrontonasal dysplasia.

Causes of Hypertelorism

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Tip: Hypertelorism can prevent the development of binocular vision and result in cosmetic concerns for the patient. 


Management of Hypertelorism

The management of hypertelorism is centred on a multi-disciplinary team. The goal of treatment is to restore function, normal orbital dystopia, cosmesis and normal anatomical alignment.

Surgical techniques that can be considered are:

  1. Box Osteotomy: normal midface width and hypertelorism
  2. Facial Bipartition: an inverted V deformity of maxillary occlusion.
  3. Medial Canthopexy: reattach the medial canthal ligament after osteotomies

Surgery is often indicated between the ages of 5 - 7. This is to reduce the risk of damage to unerupted teeth or maxillary growth.

These surgical treatments for hypertelorism are visualised below.  

Treatment of Hypertelorism

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Tip: perform a CT and ophthalmic assessment prior to any surgical intervention. 


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