Subependymal hamartoma

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Subependymal hamartomas are seen in patients with tuberous sclerosis ~~within the first six month of age~~ ². They ~~appear as subependymal nodules in~~are located along the ventricles and are mostly asymptomatic. As with other hamartomas, they grow at the same rate as the surrounding tissues.

On imaging, they appear as small intraventricular mass, smaller than 1 cm, and demonstrates variable signal in MRI, calcification and contrast enhancements.

Epidemiology

Subependymal hamartomas are a well-known manifestation of tuberous sclerosis, affecting 80% of patients with the condition ¹. They are visible within the first six months of age².

Clinical presentation

Subependymal hamartomas are often asymptomatic. When symptoms occur, they are usually a result of obstructive hydrocephalus from the mass effect to the ventricular system.

Radiographic features

Subependymal hamartomas are small irregular ~~subependymal nodule~~nodules, measuring less than 1 cm, with their long axis perpendicular to the ventricular surface. They grow in proportion to the surrounding tissues and may calcify with increasing age.

CT

appear as small irregular intraventricular mass.
smaller than 1 cm
calcification is common (88%)
accompanying hydrocephalus may be present
variable contrast enhancement

MRI

T1: variable signal, frequently hyperintense to grey matter
T2: variable signal, frequently iso to hyperintense to grey matter
T1 C+ (Gd): also shows variable enhancement
Marked hypointense areas are in keeping with calcification

Treatment and prognosis

Subependymal hamartomas are mostly asymptomatic. However, they may progress to subependymal giant cell ~~astrocytomas and further~~astrocytoma which may lead to obstructive hydrocephalus, causing in morbidity or mortality. Therefore, surveillance is offered to patients with tuberous sclerosis.

Should these tumours become symptomatic or ~~enlarging~~large, surgical treatment is required.

Differential diagnosis

subependymal giant cell ~~astrocytomas~~astrocytoma
- larger than ~~1cm~~1 cm
- increases in size in serial imaging^3,4
~~Subependymal~~subependymal grey matter heterotopia
- smooth and avoid
- long axis parallel to the ventricular surface

-<p><strong>Subependymal hamartomas </strong>are seen in patients with <a href="/articles/tuberous-sclerosis">tuberous sclerosis</a> within the first six month of age <sup>2</sup>. They appear as subependymal nodules in the ventricles and are mostly asymptomatic. As with other hamartomas, they grow at the same rate as the surrounding tissues.</p><p>On imaging, they appear as small intraventricular mass, smaller than 1 cm, and demonstrates variable signal in MRI, calcification and enhancements.</p><h4><strong>Epidemiology</strong></h4><p>Subependymal hamartomas are a well-known manifestation of tuberous sclerosis, affecting 80% of patients with the condition <sup>1</sup>. They are visible within the first six months of age<sup>2</sup>.</p><h4><strong>Clinical presentation</strong></h4><p>Subependymal hamartomas are often asymptomatic. When symptoms occur, they are usually a result of <a href="/articles/obstructive-hydrocephalus">obstructive hydrocephalus</a> from the mass effect to the ventricular system.</p><h4><strong>Radiographic features</strong></h4><p>Subependymal hamartomas are small irregular subependymal nodule, measuring less than 1 cm, with their long axis perpendicular to the ventricular surface. They grow in proportion to the surrounding tissues and may calcify with increasing age.</p><h5><strong>CT</strong></h5><ul>
+<p><strong>Subependymal hamartomas </strong>are seen in patients with <a href="/articles/tuberous-sclerosis">tuberous sclerosis</a>. They are located along the ventricles and are mostly asymptomatic. As with other hamartomas, they grow at the same rate as the surrounding tissues.</p><p>On imaging, they appear as small intraventricular mass, smaller than 1 cm, and demonstrates variable signal in MRI, calcification and contrast enhancements.</p><h4>Epidemiology</h4><p>Subependymal hamartomas are a well-known manifestation of tuberous sclerosis, affecting 80% of patients with the condition <sup>1</sup>. They are visible within the first six months of age <sup>2</sup>.</p><h4>Clinical presentation</h4><p>Subependymal hamartomas are often asymptomatic. When symptoms occur, they are usually a result of <a href="/articles/obstructive-hydrocephalus">obstructive hydrocephalus</a> from the mass effect to the ventricular system.</p><h4>Radiographic features</h4><p>Subependymal hamartomas are small irregular nodules, measuring less than 1 cm, with their long axis perpendicular to the ventricular surface. They grow in proportion to the surrounding tissues and may calcify with increasing age.</p><h5>CT</h5><ul>
~~-</ul><h5><strong>MRI</strong></h5><ul>~~
+</ul><h5>MRI</h5><ul>
-</ul><h4><strong>Treatment and prognosis</strong></h4><p>Subependymal hamartomas are mostly asymptomatic. However, they may progress to subependymal giant cell astrocytomas and further obstructive hydrocephalus, causing in morbidity or mortality. Therefore, surveillance is offered to patients with tuberous sclerosis.</p><p>Should these tumours become symptomatic or enlarging, surgical treatment is required.</p><h4><strong>Differential diagnosis</strong></h4><ul>
~~-<li>subependymal giant cell astrocytomas<ul>~~
~~-<li>larger than 1cm</li>~~
~~-<li>increases in size in serial imaging<sup>3,4</sup>~~
+</ul><h4>Treatment and prognosis</h4><p>Subependymal hamartomas are mostly asymptomatic. However, they may progress to <a title="Subependymal giant cell astrocytoma" href="/articles/subependymal-giant-cell-astrocytoma">subependymal giant cell astrocytoma</a> which may lead to obstructive hydrocephalus, causing morbidity or mortality. Therefore, surveillance is offered to patients with tuberous sclerosis.</p><p>Should these tumours become symptomatic or large, surgical treatment is required.</p><h4>Differential diagnosis</h4><ul>
+<li>
+<a href="/articles/subependymal-giant-cell-astrocytoma">subependymal giant cell astrocytoma</a> <ul>
+<li>larger than 1 cm</li>
+<li>increases in size in serial imaging <sup>3,4</sup>
~~-<li>Subependymal grey matter heterotopia<ul>~~
+<li>
+<a href="/articles/subependymal-grey-matter-heterotopia">subependymal grey matter heterotopia</a><ul>

References changed:

1. Portocarrero L, Quental K, Samorano L, Oliveira Z, Rivitti-Machado M. Tuberous Sclerosis Complex: Review Based on New Diagnostic Criteria. An Bras Dermatol. 2018;93(3):323-31. <a href="https://doi.org/10.1590/abd1806-4841.20186972">doi:10.1590/abd1806-4841.20186972</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29924239">Pubmed</a>
2. Atlas S. Magnetic Resonance Imaging of the Brain and Spine. (2009) ISBN: 9780781769853 - <a href="http://books.google.com/books?vid=ISBN9780781769853">Google Books</a>
3. Kwiatkowski D, Whittemore V, Thiele E. Tuberous Sclerosis Complex. (2010) ISBN: 9783527322015 - <a href="http://books.google.com/books?vid=ISBN9783527322015">Google Books</a>
4. Barkovich A. Pediatric Neuroimaging. (2005) ISBN: 9780781757669 - <a href="http://books.google.com/books?vid=ISBN9780781757669">Google Books</a>

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