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 distance between the medial canthi of the eyelids.
Pathology
Over development of the lesser wings of the sphenoid bone may be an undelying factor.
Associations
While it can as an isolated anomaly, there are numerous syndromic as well as non syndromic associations with orbital hypertelorism which include:
- aneuploidic syndromic:
-
non aneuploidic syndromic:
- Aarskog syndrome
- Apert syndrome 1
- 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 syndrome 6
- Sotos syndrome
- Weaver syndrome
- Wolf-Hirschhorn syndrome
- non syndromic:
There are many more associations other than those mentioned on this list. Please add and / or/or reference as appropriate :).
Radiographic assessment
Antenatal ultrasound
It is can be assesssed by measuring the distance between the lateral orbital walls or the medial orbital walls. The interocular distance / diameter/diameter (IOD) and the biocular diameter (BOD) are both typically increased with hypertelorism.
Due to its associations with other conditions, a detailed sonographic surveillence for other fetal anomalies are recommended if hypertelorism is seen on ultrasound.
Some indices used include:
- orbital angle index: a value greater than 42 is suggestive of orbital hypertelorism
-
interorbital
- orbital-orbital index: a value greater than 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 <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" title="telecanthus">telecanthus</a> which means an increased distance 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 undelying 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 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 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 undelying 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>
-<strong>aneuploidic syndromic</strong><ul>-<li><a href="/articles/trisomy-21" title="trisomy 21">trisomy 21</a></li>-<li><a href="/articles/patau-syndrome" title="Trisomy 13">trisomy 13</a></li>- +<strong>aneuploidic syndromic:</strong><ul>
- +<li><a href="/articles/trisomy-21">trisomy 21</a></li>
- +<li><a href="/articles/patau-syndrome">trisomy 13</a></li>
-<a href="/articles/turner-syndrome" title="Monosomy X">monosomy X</a> - <a href="/articles/turner-syndrome" title="Turner syndrome">Turner syndrome</a>- +<a href="/articles/turner-syndrome">monosomy X</a>-<a href="/articles/turner-syndrome">Turner syndrome</a>
-<strong>non aneuploidic syndromic</strong><ul>-<li><a href="/articles/aarskog-syndrome" title="Aarskog syndrome">Aarskog syndrome</a></li>- +<strong>non aneuploidic syndromic:</strong><ul>
- +<li><a href="/articles/aarskog-syndrome">Aarskog syndrome</a></li>
-<a href="/articles/apert-syndrome" title="Apert syndrome">Apert syndrome</a> <sup>1</sup>- +<a href="/articles/apert-syndrome">Apert syndrome</a> <sup>1</sup>
-<a href="/articles/crouzon-syndrome" title="Crouzon syndrome">Crouzon syndrome</a> <sup>1</sup>- +<a href="/articles/crouzon-syndrome">Crouzon syndrome</a> <sup>1</sup>
-<li><a href="/articles/22q112-deletion-syndrome-1" title="DiGeorge syndrome">DiGeorge syndrome </a></li>-<li><a href="/articles/ehlers-danlos-syndrome-2" title="Ehlers-Danlos disease">Ehlers-Danlos syndrome</a></li>-<li><a href="/articles/frontonasal-dysplasia" title="Frontonasal dysplasia">frontonasal dysplasia</a></li>-<li><a href="/articles/greig-syndrome" title="Greig syndrome">Greig syndrome</a></li>-<li><a href="/articles/gorlin-goltz-syndrome-1" title="Gorlin syndrome">Gorlin syndrome</a></li>-<li><a href="/articles/leopard-syndrome" title="LEOPARD syndrome">LEOPARD syndrome</a></li>- +<li><a href="/articles/22q112-deletion-syndrome-1">DiGeorge syndrome </a></li>
- +<li><a href="/articles/ehlers-danlos-syndrome-2">Ehlers-Danlos syndrome</a></li>
- +<li><a href="/articles/frontonasal-dysplasia">frontonasal dysplasia</a></li>
- +<li><a href="/articles/greig-syndrome">Greig syndrome</a></li>
- +<li><a href="/articles/gorlin-goltz-syndrome-1">Gorlin syndrome</a></li>
- +<li><a href="/articles/leopard-syndrome">LEOPARD syndrome</a></li>
-<a href="/articles/loeys-dietz-syndrome-lds" title="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/mucoploysaccharidoses" title="Mucopolysaccharidoses">mucopolysaccharidoses</a><ul><li><a href="/articles/morquio-syndrome" title="Morquio syndrome">Morquio syndrome</a></li></ul>- +<a href="/articles/mucopolysaccharidoses-2">mucopolysaccharidoses</a>:<ul><li><a href="/articles/morquio-syndrome">Morquio syndrome</a></li></ul>
-<li><a href="/articles/neu-laxova-syndrome" title="Neu-Laxova syndrome (NLS)">Neu-Laxova syndrome (NLS)</a></li>- +<li><a href="/articles/neu-laxova-syndrome">Neu-Laxova syndrome (NLS)</a></li>
-<a href="/articles/noonan-syndrome" title="Noonan syndrome">Noonan syndrome</a> <sup>5</sup>- +<a href="/articles/noonan-syndrome">Noonan syndrome</a> <sup>5</sup>
-<a href="/articles/saethre-chotzen-syndrome" title="Saethre-Chotzen syndrome">Saethre-Chotzen syndrome</a> <sup>6</sup>- +<a href="/articles/saethre-chotzen-syndrome">Saethre-Chotzen syndrome</a> <sup>6</sup>
-<li><a href="/articles/sotos-syndrome-2" title="Sotos syndrome">Sotos syndrome</a></li>-<li><a href="/articles/weaver-syndrome" title="Weaver syndrome">Weaver syndrome</a></li>-<li><a href="/articles/wolf-hirschhorn-syndrome" title="Wolf-Hirschhorn syndrome">Wolf-Hirschhorn syndrome</a></li>- +<li><a href="/articles/sotos-syndrome-2">Sotos syndrome</a></li>
- +<li><a href="/articles/weaver-syndrome">Weaver syndrome</a></li>
- +<li><a href="/articles/wolf-hirschhorn-syndrome">Wolf-Hirschhorn syndrome</a></li>
-<strong>non syndromic</strong><ul>- +<strong>non syndromic:</strong><ul>
-<a href="/articles/dysgenesis-of-the-corpus-callosum" title="Agenesis 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/autosomal-recessive-osteopetrosis-1" title="Autosomal recessive osteopetrosis">autosomal recessive osteopetrosis</a> <sup>7</sup>- +<a href="/articles/autosomal-recessive-osteopetrosis-1">autosomal recessive osteopetrosis</a> <sup>7</sup>
-<a href="/articles/congenital-dacrocystocoeles" title="congenital dacrocystocoeles ">congenital dacrocystocoeles </a><sup>4</sup>- +<a href="/articles/congenital-dacrocystocoeles">congenital dacrocystocoeles </a><sup>4</sup>
-<a href="/articles/hypospadias" title="hypospadias">hypospadia(s) </a><sup>3</sup>- +<a href="/articles/hypospadias">hypospadia(s) </a><sup>3</sup>
-<a href="/articles/naso-ethmoidal-encephalocoele" title="naso ethmoidal encephalocoele">naso ethmoidal encephalocoele</a> / <a href="/articles/frontal-encephalocoele" title="frontal encephalocoele">frontal encephalocoele</a>- +<a href="/articles/naso-ethmoidal-encephalocoele">naso ethmoidal encephalocoele</a> / <a href="/articles/frontal-encephalocoele">frontal encephalocoele</a>
-</ul><p><em>There are many more associations other than those mentioned on this list. Please add and / or reference as appropriate :)</em></p><h4>Radiographic assessment</h4><h5>Antenatal ultrasound</h5><p>It is can be assesssed by measuring the distance between the lateral orbital walls or the medial orbital walls. The <a href="/articles/inter-ocular-distance-1" title="Inter ocular distance">interocular distance / diameter (IOD) </a>and the <a href="/articles/missing?article%5Btitle%5D=biocular%20diameter%20(BOD)" title="biocular diameter (BOD)">biocular diameter (BOD)</a> are both typically increased with hypertelorism.</p><p>Due to its associations with other conditions, a detailed sonographic surveillence for other fetal anomalies are 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>Radiographic assessment</h4><h5>Antenatal ultrasound</h5><p>It is can be assesssed by measuring the distance between the lateral orbital walls or the medial orbital walls. The <a href="/articles/inter-ocular-distance-1">interocular distance/diameter (IOD) </a>and the <a href="/articles/missing?article%5Btitle%5D=biocular%20diameter%20(BOD)">biocular diameter (BOD)</a> are both typically increased with hypertelorism.</p><p>Due to its associations with other conditions, a detailed sonographic surveillence for other fetal anomalies are recommended if hypertelorism is seen on ultrasound.</p><p>Some indices used include:</p><ul>
-<a href="/articles/orbital-angle-index" title="orbital angle index">orbital angle index</a> : a value greater than <strong>42</strong> is suggestive of orbital hypertelorism</li>- +<a href="/articles/orbital-angle-index">orbital angle index</a>: a value greater than 42 is suggestive of orbital hypertelorism</li>
-<a href="/articles/interorbital-orbital-index" title="interorbital - orbital index ">interorbital - orbital index </a>: a value greater than <strong>8 </strong>is suggestive of orbital hypertelorism</li>-</ul><h4>See also</h4><ul><li><a href="/articles/hypotelorism" title="hypotelorism">hypotelorism</a></li></ul>- +<a href="/articles/interorbital-orbital-index">interorbital-orbital index</a>: a value greater than 8 is suggestive of orbital hypertelorism</li>
- +</ul><h4>See also</h4><ul><li><a href="/articles/hypotelorism">hypotelorism</a></li></ul>
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