Orbital cavernous venous malformation
Updates to Synonym Attributes
Updates to Article Attributes
A cavernous haemangiomaCavernous venous malformations of the orbit is(aka cavernous haemangioma) are the most common vascular lesion of the orbit in adults.
It is important to note that according to newer nomenclature (ISSVA classification of vascular anomalies) these lesions are merely known as slow flow venous malformations. Having said that it is probably helpful in reports to include the word 'cavernous' as this term is ubiquitous in the literature and most familiar to many clinicians.
Cavernous malformations are found throughout the body. This article focuses on cerebral cavernous haemangiomas. For a general discussion please refer to the general article on cavernous venous malformation.
Epidemiology
Cavernous haemangiomas are the most common vascular lesions of the orbit in adults and accounting for 5 - 7% of all orbital tumours. However, debate exists about whether these lesions should actually be considered tumours 3. They usually present in middle age (30 - 50 years of age) and there appears to be a a female predilection 2-3.
Clinical presentation
Clinical presentation is usually with a slowly growing orbital mass resulting in proptosis. Diplopia and visual field defects (from optic nerve compression) may also occur 3.
Pathology
Cavernous haemangiomas are well circumscribed masses bounded by a fibrous pseudocapsule, without prominent arterial supply (accounting for the relatively slow enhancement). They are composed of dilated large vascular spaces (thus cavernous) lined by flattened and attenuated endothelial cells 1,3.
As flow is slow, and vascular spaces large, areas of thrombosis are common 3-4.
Unlike the name 'haemangioma' suggests, these lesions may not be tumours as there is no cellular proliferation 3, but rather gradually enlarging vascular malformations and as such some authors prefer the term cavernous malformation.
In some cases prominent fibrosis is present, and these lesions are referred to by some authors as sclerosing haemangiomas 1.
Radiographic features
Although cavernous haemangiomas can be located anywhere within the orbit (and for that matter pretty much anywhere in the body : see cavernous haemangiomavenous malformation article article) over 80% are located within the intraconal compartment, most commonly in the lateral aspect 1-3.
They are usually round or oval in cross section and although frequently abut the globe, they do no deform it, but rather are deformed by the globe, on account of their soft consistency 1,3.
Large lesions may be associated with expansion of the bony confines of the orbit 3.
Ultrasound
Ultrasound demonstrates a smoothly circumscribed retrobulbar lesion with regular moderate to high internal echogenicity 3-4. No flow can be demonstrated on doppler scanning 4.
CT
Cavernous haemangioma appears as a well circumscribed, rounded or oval soft tissue density mass, somewhat hypoattenuating compared to muscle, which gradually fills in following administration of contrast 1-2.
The orbital apex is usually spared 3.
Sclerosing haemangiomas sometimes demonstrate calcification 1.
MRI
Appearance on MRI is the same morphologically as on CT, with the following signal intensities:
-
T1
- iso-intense c.f muscle
- if areas of thrombosis are present, then hyperintense regions may be visible 3
-
T2
- hyper-intense c.f. muscle
- may have low intensity septation
- pseudocapsule is of low intensity
- T1 C+ (Gd) : slow gradual irregular enhancement with delayed wash out
DSA - angiography
Angiographically haemangiomas are occult as enhancement occurs only in a delayed fashion 2.
Treatment and prognosis
If the lesion is found incidentally and no exophthalmos or visual complications are present then conservative management is recommended, with periodic MRI imaging.
In cases where symptoms are present, or growth of the lesion is demonstrated on follow-up imaging, surgical removal is curative.
Differential diagnosis
The differential depends on the location, but is essentially that of orbital vascular lesions with the addition of a few non-vascular tumours.
For the more common intra-conal variety the differential includes 1-3:
- optic nerve meningioma
- orbital schwannoma
- haemangiopericytoma
- sclerosing haemangioma (a variant rather than a distinct entity)
- orbital metastases
- orbital fibrous histiocytoma
- orbital lymphoma
- orbital venous varix
- capillary haemangioma of orbit
If extra-conal the differential also includes 1-3:
-<p>A <strong>cavernous haemangioma of the orbit </strong>is the most common <a style="COLOR: rgb(63,117,216); TEXT-DECORATION: none" title="Orbital vascular lesions" href="/articles/orbital-vascular-lesions">vascular lesion of the orbit </a>in adults.</p><h4>Epidemiology</h4><p>Cavernous haemangiomas are the most common vascular lesions of the orbit in adults and accounting for 5 - 7% of all <a title="orbital tumours" href="/articles/orbital-tumours">orbital tumours</a>. However, debate exists about whether these lesions should actually be considered tumours <sup>3</sup>. They usually present in middle age (30 - 50 years of age) and there appears to be a a female predilection <sup>2-3</sup>. </p><h4>Clinical presentation</h4><p>Clinical presentation is usually with a slowly growing orbital mass resulting in proptosis. Diplopia and visual field defects (from optic nerve compression) may also occur <sup>3</sup>. </p><h4>Pathology</h4><p>Cavernous haemangiomas are well circumscribed masses bounded by a fibrous pseudocapsule, without prominent arterial supply (accounting for the relatively slow enhancement). They are composed of dilated large vascular spaces (thus <em>cavernous</em>) lined by flattened and attenuated endothelial cells <sup>1,3</sup>. </p><p>As flow is slow, and vascular spaces large, areas of thrombosis are common <sup>3-4</sup>. </p><p>Unlike the name 'haemangioma' suggests, these lesions may not be tumours as there is no cellular proliferation <sup>3</sup>, but rather gradually enlarging vascular malformations and as such some authors prefer the term cavernous malformation. </p><p>In some cases prominent fibrosis is present, and these lesions are referred to by some authors as <a title="sclerosing haemangioma" href="/articles/sclerosing-haemangioma">sclerosing haemangiomas</a> <sup>1</sup>. </p><h4>Radiographic features</h4><p>Although cavernous haemangiomas can be located anywhere within the orbit (and for that matter pretty much anywhere in the body : see <a title="cavernous haemangioma" href="/articles/cavernous-haemangioma">cavernous haemangioma</a> article) over 80% are located within the <a title="intraconal orbital compartment" href="/articles/intraconal-orbital-compartment">intraconal compartment</a>, most commonly in the lateral aspect <sup>1-3</sup>. </p><p>They are usually round or oval in cross section and although frequently abut the globe, they do no deform it, but rather are deformed by the globe, on account of their soft consistency <sup>1,3</sup>. </p><p>Large lesions may be associated with expansion of the bony confines of the orbit <sup>3</sup>. </p><h5>Ultrasound</h5><p>Ultrasound demonstrates a smoothly circumscribed retrobulbar lesion with regular moderate to high internal echogenicity <sup>3-4</sup>. No flow can be demonstrated on doppler scanning <sup>4</sup>. </p><h5>CT</h5><p>Cavernous haemangioma appears as a well circumscribed, rounded or oval soft tissue density mass, somewhat hypoattenuating compared to muscle, which gradually fills in following administration of contrast <sup>1-2</sup>. </p><p>The orbital apex is usually spared <sup>3</sup>. </p><p>Sclerosing haemangiomas sometimes demonstrate calcification <sup>1</sup>. </p><h5>MRI</h5><p>Appearance on MRI is the same morphologically as on CT, with the following signal intensities:</p><ul>- +<p><strong>Cavernous venous malformations of the orbit </strong>(aka <strong>cavernous haemangioma</strong>) are the most common <a href="/articles/orbital-vascular-lesions">vascular lesion of the orbit </a>in adults.</p><p>It is important to note that according to newer nomenclature (<a href="/articles/issva-classification-of-vascular-anomalies">ISSVA classification of vascular anomalies</a>) these lesions are merely known as slow flow venous malformations. Having said that it is probably helpful in reports to include the word 'cavernous' as this term is ubiquitous in the literature and most familiar to many clinicians. </p><p>Cavernous malformations are found throughout the body. This article focuses on cerebral cavernous haemangiomas. For a general discussion please refer to the general article on <a href="/articles/cavernous-venous-malformation-1">cavernous venous malformation</a>. </p><h4>Epidemiology</h4><p>Cavernous haemangiomas are the most common vascular lesions of the orbit in adults and accounting for 5 - 7% of all <a href="/articles/orbital-tumours">orbital tumours</a>. However, debate exists about whether these lesions should actually be considered tumours <sup>3</sup>. They usually present in middle age (30 - 50 years of age) and there appears to be a a female predilection <sup>2-3</sup>.</p><h4>Clinical presentation</h4><p>Clinical presentation is usually with a slowly growing orbital mass resulting in proptosis. Diplopia and visual field defects (from optic nerve compression) may also occur <sup>3</sup>. </p><h4>Pathology</h4><p>Cavernous haemangiomas are well circumscribed masses bounded by a fibrous pseudocapsule, without prominent arterial supply (accounting for the relatively slow enhancement). They are composed of dilated large vascular spaces (thus <em>cavernous</em>) lined by flattened and attenuated endothelial cells <sup>1,3</sup>. </p><p>As flow is slow, and vascular spaces large, areas of thrombosis are common <sup>3-4</sup>.</p><p>Unlike the name 'haemangioma' suggests, these lesions may not be tumours as there is no cellular proliferation <sup>3</sup>, but rather gradually enlarging vascular malformations and as such some authors prefer the term cavernous malformation.</p><p>In some cases prominent fibrosis is present, and these lesions are referred to by some authors as <a href="/articles/sclerosing-haemangioma">sclerosing haemangiomas</a> <sup>1</sup>.</p><h4>Radiographic features</h4><p>Although cavernous haemangiomas can be located anywhere within the orbit (and for that matter pretty much anywhere in the body : see <a href="/articles/cavernous-haemangioma">cavernous venous malformation</a> article) over 80% are located within the <a href="/articles/intraconal-orbital-compartment">intraconal compartment</a>, most commonly in the lateral aspect <sup>1-3</sup>.</p><p>They are usually round or oval in cross section and although frequently abut the globe, they do no deform it, but rather are deformed by the globe, on account of their soft consistency <sup>1,3</sup>.</p><p>Large lesions may be associated with expansion of the bony confines of the orbit <sup>3</sup>.</p><h5>Ultrasound</h5><p>Ultrasound demonstrates a smoothly circumscribed retrobulbar lesion with regular moderate to high internal echogenicity <sup>3-4</sup>. No flow can be demonstrated on doppler scanning <sup>4</sup>.</p><h5>CT</h5><p>Cavernous haemangioma appears as a well circumscribed, rounded or oval soft tissue density mass, somewhat hypoattenuating compared to muscle, which gradually fills in following administration of contrast <sup>1-2</sup>.</p><p>The orbital apex is usually spared <sup>3</sup>.</p><p>Sclerosing haemangiomas sometimes demonstrate calcification <sup>1</sup>.</p><h5>MRI</h5><p>Appearance on MRI is the same morphologically as on CT, with the following signal intensities:</p><ul>
-</ul><h5>DSA - angiography</h5><p>Angiographically haemangiomas are occult as enhancement occurs only in a delayed fashion <sup>2</sup>. </p><h4>Treatment and prognosis</h4><p>If the lesion is found incidentally and no exophthalmos or visual complications are present then conservative management is recommended, with periodic MRI imaging. </p><p>In cases where symptoms are present, or growth of the lesion is demonstrated on follow-up imaging, surgical removal is curative. </p><h4>Differential diagnosis</h4><p>The differential depends on the location, but is essentially that of <a title="Orbital vascular lesions" href="/articles/orbital-vascular-lesions">orbital vascular lesions</a> with the addition of a few non-vascular tumours. </p><p>For the more common <strong>intra-conal </strong>variety the differential includes<sup> 1-3</sup>: </p><ul>-<li><a title="optic nerve meningioma" href="/articles/optic-nerve-sheath-meningioma">optic nerve meningioma</a></li>-<li><a title="orbital schwannoma" href="/articles/orbital-schwannoma">orbital schwannoma</a></li>-<li><a title="Haemangiopericytoma" href="/articles/haemangiopericytoma-1">haemangiopericytoma</a></li>- +</ul><h5>DSA - angiography</h5><p>Angiographically haemangiomas are occult as enhancement occurs only in a delayed fashion <sup>2</sup>.</p><h4>Treatment and prognosis</h4><p>If the lesion is found incidentally and no exophthalmos or visual complications are present then conservative management is recommended, with periodic MRI imaging.</p><p>In cases where symptoms are present, or growth of the lesion is demonstrated on follow-up imaging, surgical removal is curative.</p><h4>Differential diagnosis</h4><p>The differential depends on the location, but is essentially that of <a href="/articles/orbital-vascular-lesions">orbital vascular lesions</a> with the addition of a few non-vascular tumours.</p><p>For the more common <strong>intra-conal </strong>variety the differential includes<sup> 1-3</sup>:</p><ul>
- +<li><a href="/articles/optic-nerve-sheath-meningioma">optic nerve meningioma</a></li>
- +<li><a href="/articles/orbital-schwannoma">orbital schwannoma</a></li>
- +<li><a href="/articles/haemangiopericytoma-1">haemangiopericytoma</a></li>
-<li><a title="orbital metastasis" href="/articles/orbital-metastasis">orbital metastases</a></li>-<li><a title="orbital fibrous histiocytoma" href="/articles/orbital-fibrous-histiocytoma">orbital fibrous histiocytoma</a></li>-<li><a title="Primary orbital lymphoma" href="/articles/orbital-lymphoma">orbital lymphoma</a></li>-<li><a title="Orbital venous varix" href="/articles/orbital-venous-varix">orbital venous varix</a></li>-<li><a title="Capillary haemangioma of the orbit" href="/articles/capillary-haemangioma-of-the-orbit">capillary haemangioma of orbit</a></li>-</ul><p>If <strong>extra-conal </strong>the differential also includes<sup> 1-3</sup>: </p><ul>-<li><a title="lacrimal gland tumours" href="/articles/lacrimal-gland-tumours">lacrimal gland tumours</a></li>- +<li><a href="/articles/orbital-metastasis">orbital metastases</a></li>
- +<li><a href="/articles/orbital-fibrous-histiocytoma">orbital fibrous histiocytoma</a></li>
- +<li><a href="/articles/orbital-lymphoma">orbital lymphoma</a></li>
- +<li><a href="/articles/orbital-venous-varix">orbital venous varix</a></li>
- +<li><a href="/articles/capillary-haemangioma-of-the-orbit">capillary haemangioma of orbit</a></li>
- +</ul><p>If <strong>extra-conal </strong>the differential also includes<sup> 1-3</sup>:</p><ul>
- +<li><a href="/articles/lacrimal-gland-tumours">lacrimal gland tumours</a></li>
-<li><a title="orbital metastases" href="/articles/orbital-metastases">orbital metastases</a></li>-<li><a title="orbital fibrous histiocytoma" href="/articles/orbital-fibrous-histiocytoma">orbital fibrous histiocytoma</a></li>-<li><a title="Primary orbital lymphoma" href="/articles/orbital-lymphoma">orbital lymphoma</a></li>-<li><a title="Orbital venous varix" href="/articles/orbital-venous-varix">orbital venous varix</a></li>- +<li><a href="/articles/orbital-metastases">orbital metastases</a></li>
- +<li><a href="/articles/orbital-fibrous-histiocytoma">orbital fibrous histiocytoma</a></li>
- +<li><a href="/articles/orbital-lymphoma">orbital lymphoma</a></li>
- +<li><a href="/articles/orbital-venous-varix">orbital venous varix</a></li>
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