Hypertelorism
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Hypertelorism refers to an abnormal increase in distance between any two organs although some authors use the term synonymously with orbital hypertelorism meaning an abnormal increase in distance between the two eyes. The article mainly focuses on the latter. The abnormality is similar to telecanthus which means an increased distance between the medial canthi of the eyelids.
PathologyEpidemiology
Over development of the lesser wings of the sphenoid bone may be an underlying factor.
Associations
While it can asbe an isolated anomaly, there are numerous syndromic as well as non syndromic-syndromic associations with orbital hypertelorism which include:
- aneuploidic syndromic
-
non
aneuploidic-aneuploidic syndromic- Aarskog syndrome
- Apert syndrome 1
-
Baraitser
–Winter-Winter syndrome8 - Crouzon syndrome 1
- DiGeorge syndrome
- Ehlers-Danlos syndrome
- frontonasal dysplasia
- Greig syndrome
- Gorlin syndrome
- LEOPARD syndrome
- Loeys-Dietz syndrome (LDS)2
- mucopolysaccharidoses
- Neu-Laxova syndrome (NLS)
- Noonan syndrome 5
- Saethre-Chotzen syndrome6
- Sotos syndrome
- Weaver syndrome
- Wolf-Hirschhorn syndrome
-
non
syndromic-syndromic
There are many more associations other than those mentioned on this list. Please add and/or reference as appropriate.
Pathology
Over development of the lesser wings of the sphenoid bone may be an underlying factor.
Radiographic assessment
Antenatal ultrasound
It is canHypertelorism may be assessed by measuring the distance between the lateral orbital walls or the medial orbital walls. The interocular distance/diameter (IOD) and the biocular diameter (BOD) are both typically increased with hypertelorism.
Due to its associations with other conditions, detailed sonographic surveillance for other fetal anomalies is recommended if hypertelorism is seen on ultrasound.
Some indices used include:
-
orbital angle index: a value
greater than 42>42 is suggestive of orbital hypertelorism -
interorbital-orbital index: a value
greater than 8>8 is suggestive of orbital hypertelorism
See also
-<p><strong>Hypertelorism</strong> refers to an abnormal increase in distance between any two organs although some authors use the term synonymously with orbital hypertelorism meaning an abnormal increase in distance between the two eyes. The article mainly focuses on the latter. The abnormality is similar to <a href="/articles/telecanthus">telecanthus</a> which means an increased distance between the medial canthi of the eyelids.</p><h4>Pathology</h4><p>Over development of the lesser wings of the sphenoid bone may be an underlying factor.</p><h5>Associations</h5><p>While it can as an isolated anomaly, there are numerous syndromic as well as non syndromic associations with orbital hypertelorism which include:</p><ul>- +<p><strong>Hypertelorism</strong> refers to an abnormal increase in distance between any two organs although some authors use the term synonymously with <strong>orbital hypertelorism</strong> meaning an abnormal increase in distance between the two eyes. The article mainly focuses on the latter. The abnormality is similar to <a href="/articles/telecanthus">telecanthus</a> which means an increased distance between the medial canthi of the <a href="/articles/eyelid">eyelids</a>.</p><h4>Epidemiology</h4><h5>Associations</h5><p>While it can be an isolated anomaly, there are numerous syndromic as well as non-syndromic associations with orbital hypertelorism which include:</p><ul>
-<strong>non aneuploidic syndromic</strong><ul>- +<strong>non-aneuploidic syndromic</strong><ul>
-<li>Baraitser–Winter syndrome<sup>8</sup>- +<li>
- +<a href="/articles/baraitser-winter-syndrome">Baraitser-Winter syndrome</a> <sup>8</sup>
-<li><a href="/articles/22q112-deletion-syndrome-1">DiGeorge syndrome </a></li>- +<li><a href="/articles/22q112-deletion-syndrome-1">DiGeorge syndrome</a></li>
-<a href="/articles/loeys-dietz-syndrome-lds">Loeys-Dietz syndrome (LDS)</a> <sup>2</sup>- +<a href="/articles/loeys-dietz-syndrome-lds">Loeys-Dietz syndrome (LDS)</a> <sup>2</sup>
-<a href="/articles/saethre-chotzen-syndrome-3">Saethre-Chotzen syndrome</a> <sup>6</sup>- +<a href="/articles/saethre-chotzen-syndrome-3">Saethre-Chotzen syndrome</a> <sup>6</sup>
-<strong>non syndromic</strong><ul>- +<strong>non-syndromic</strong><ul>
-<a href="/articles/dysgenesis-of-the-corpus-callosum">agenesis of the corpus callosum</a> <sup>ref</sup>- +<a href="/articles/dysgenesis-of-the-corpus-callosum">agenesis of the corpus callosum</a> <sup>ref</sup>
-<a href="/articles/congenital-dacrocystocoeles">congenital dacrocystocoeles </a><sup>4</sup>- +<a href="/articles/congenital-dacrocystocoeles">congenital dacrocystocele</a> <sup>4</sup>
-<a href="/articles/hypospadias">hypospadia(s) </a><sup>3</sup>- +<a href="/articles/hypospadias">hypospadia(s)</a> <sup>3</sup>
-<a href="/articles/naso-ethmoidal-encephalocoele">naso ethmoidal encephalocoele</a>/<a href="/articles/frontal-encephalocoele">frontal encephalocoele</a>- +<a href="/articles/naso-ethmoidal-encephalocoele">nasoethmoidal encephalocele</a>/<a href="/articles/frontal-encephalocoele">frontal encephalocele</a>
-</ul><p>There are many more associations other than those mentioned on this list. Please add and/or reference as appropriate.</p><h4>Radiographic assessment</h4><h5>Antenatal ultrasound</h5><p>It is can be assessed by measuring the distance between the lateral orbital walls or the medial orbital walls. The <a href="/articles/interocular-distance-1">interocular distance/diameter (IOD) </a>and the <a href="/articles/biocular-diameter-bod">biocular diameter (BOD)</a> are both typically increased with hypertelorism.</p><p>Due to its associations with other conditions, detailed sonographic surveillance for other fetal anomalies is recommended if hypertelorism is seen on ultrasound.</p><p>Some indices used include:</p><ul>- +</ul><p>There are many more associations other than those mentioned on this list. Please add and/or reference as appropriate.</p><h4>Pathology</h4><p>Over development of the <a href="/articles/lesser-wing-of-sphenoid">lesser wings of the sphenoid bone</a> may be an underlying factor.</p><h4>Radiographic assessment</h4><h5>Antenatal ultrasound</h5><p>Hypertelorism may be assessed by measuring the distance between the lateral orbital walls or the medial orbital walls. The <a href="/articles/interocular-distance-1">interocular distance/diameter (IOD) </a>and the <a href="/articles/biocular-diameter-bod">biocular diameter (BOD)</a> are both typically increased with hypertelorism.</p><p>Due to its associations with other conditions, detailed sonographic surveillance for other fetal anomalies is recommended if hypertelorism is seen on ultrasound.</p><p>Some indices used include:</p><ul>
-<a href="/articles/orbital-angle-index">orbital angle index</a>: a value greater than 42 is suggestive of orbital hypertelorism</li>- +<a href="/articles/orbital-angle-index">orbital angle index</a>: a value >42 is suggestive of orbital hypertelorism</li>
-<a href="/articles/interorbital-orbital-index">interorbital-orbital index</a>: a value greater than 8 is suggestive of orbital hypertelorism</li>- +<a href="/articles/interorbital-orbital-index">interorbital-orbital index</a>: a value >8 is suggestive of orbital hypertelorism</li>
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