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Upskill in high growth, advanced imaging areas.
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Emergency Call Prep
Prepare trainees to be on call for the emergency department with this specialized training series.
22 topics, 1 hr. 12 min.
Introduction to Neurocutaneous Syndromes Part 1 (NF1)
1 m.Chiari Malformation Type 1 in a Patient with NF1
3 m.NF1, With Waxing and Waning Cystic Lesion
5 m.NF1 with Developing Myelin Vacuolization, and Optic Glioma
4 m.NF1 with Suspicious Lesions and Tortuous Optic Nerve
7 m.NF1 with Bilateral Thalamic Lesions and Differential Diagnosis
5 m.Normal Brain MRI in Patients with NF1
5 m.Mild NF1 Phenotype with Sphenoid Wing Dysplasia
4 m.NF1 with High Grade Glioma
3 m.NF1 with Bilateral Optic Nerve Glioma
3 m.Focal Optic Pathway Glioma in NF1
4 m.NF1 with Fusiform Optic Pathway Glioma
3 m.NF1 with Optic Chiasmatic Glioma
3 m.NF1 with Evolution of Optic Nerve Glioma
5 m.NF1 with Optic Pathway Glioma and Moyamoya Disease
6 m.Developing Right Fusiform Optic Glioma
3 m.Glaucoma, an Orbital Manifestation of NF1
3 m.NF1 with a Brain Stem Lesion and Optic Nerve Glioma
4 m.NF1 with Buphthalmos and Orbital Plexiform Neurofibroma
4 m.MRI Appearance of Sphenoid Wing Dysplasia in NF1
2 m.NF1 with Moyamoya Vasculopathy
4 m.Neurocutaneous Syndromes Part 1 (NF1) Summary
2 m.0:01
This is an axial T2-weighted image
0:02
in a 14-year-old child
0:04
with neurofibromatosis type 1.
0:06
And we can see some cystic changes in the
0:09
region of the right lentiform nucleus.
0:13
If we go down,
0:14
we see they're originating inferiorly
0:16
and projecting superiorly.
0:18
And we see a similar finding on the left side.
0:24
If we look on this coronal image,
0:28
we can see a more solid component
0:30
of a lesion here and here, medially.
0:35
And anteriorly,
0:36
It all connects to this more
0:40
discrete solid lesion.
0:44
On this sagittal T1 post-contrast image,
0:46
we see post-contrast enhancement within
0:49
this supercellular lesion.
0:51
We're seeing a focal indentation along
0:53
the superior aspect of the lesion,
0:55
which is likely due to it being bounded
0:58
by the anterior communicating artery.
1:00
Here are the A2 segments of
1:02
the anterior cerebral artery,
1:03
this part of the lesion is bowing up superiorly here.
1:06
And anterior to the intercerebral arteries,
1:09
this is bowing up superiorly posterior
1:11
to the anterior cerebral arteries.
1:13
And this notch is where it is unable to grow
1:17
superiorly because of the anterior
1:19
communicating artery.
1:21
So,
1:22
we see a separate pituitary gland,
1:27
which can be confirmed
1:31
on other imaging.
1:33
But more than anything,
1:36
we also do not see a discrete optic chiasm.
1:40
And one of the things that helps us identify
1:43
what the origin of this lesion is is knowing
1:47
that the patient has neurofibromatosis type 1,
1:49
where we know they have a propensity
1:51
for optic pathway gliomas.
1:53
And the fact that we do not
1:55
see the optic chiasm,
1:56
that this is a chiasmic glioma in the setting
1:59
of neurofibromatosis type 1.
2:01
Now, there's also involvement along
2:04
the optic tracts bilaterally,
2:06
and the portion along the optic tracts
2:08
actually has this cystic enlargement that
2:11
extends pushes superiorly to the inferior
2:13
aspect of the lentiform nuclei bilaterally.
2:16
So if we look at the orbital segment
2:20
of the optic nerves,
2:22
we're actually not seeing any
2:24
definite involvement.
2:25
So, this is almost purely chiasmatic
2:29
and optic tract involvement.
2:31
So while optic pathway gliomas,
2:34
we often think of the optic nerve involvement,
2:36
in particular the orbital segment
2:38
of the optic nerve.
2:39
It can be anywhere along this optic pathway,
2:42
including, as seen in this case,
2:43
the optic chiasm and optic tracts.
Interactive Transcript
0:01
This is an axial T2-weighted image
0:02
in a 14-year-old child
0:04
with neurofibromatosis type 1.
0:06
And we can see some cystic changes in the
0:09
region of the right lentiform nucleus.
0:13
If we go down,
0:14
we see they're originating inferiorly
0:16
and projecting superiorly.
0:18
And we see a similar finding on the left side.
0:24
If we look on this coronal image,
0:28
we can see a more solid component
0:30
of a lesion here and here, medially.
0:35
And anteriorly,
0:36
It all connects to this more
0:40
discrete solid lesion.
0:44
On this sagittal T1 post-contrast image,
0:46
we see post-contrast enhancement within
0:49
this supercellular lesion.
0:51
We're seeing a focal indentation along
0:53
the superior aspect of the lesion,
0:55
which is likely due to it being bounded
0:58
by the anterior communicating artery.
1:00
Here are the A2 segments of
1:02
the anterior cerebral artery,
1:03
this part of the lesion is bowing up superiorly here.
1:06
And anterior to the intercerebral arteries,
1:09
this is bowing up superiorly posterior
1:11
to the anterior cerebral arteries.
1:13
And this notch is where it is unable to grow
1:17
superiorly because of the anterior
1:19
communicating artery.
1:21
So,
1:22
we see a separate pituitary gland,
1:27
which can be confirmed
1:31
on other imaging.
1:33
But more than anything,
1:36
we also do not see a discrete optic chiasm.
1:40
And one of the things that helps us identify
1:43
what the origin of this lesion is is knowing
1:47
that the patient has neurofibromatosis type 1,
1:49
where we know they have a propensity
1:51
for optic pathway gliomas.
1:53
And the fact that we do not
1:55
see the optic chiasm,
1:56
that this is a chiasmic glioma in the setting
1:59
of neurofibromatosis type 1.
2:01
Now, there's also involvement along
2:04
the optic tracts bilaterally,
2:06
and the portion along the optic tracts
2:08
actually has this cystic enlargement that
2:11
extends pushes superiorly to the inferior
2:13
aspect of the lentiform nuclei bilaterally.
2:16
So if we look at the orbital segment
2:20
of the optic nerves,
2:22
we're actually not seeing any
2:24
definite involvement.
2:25
So, this is almost purely chiasmatic
2:29
and optic tract involvement.
2:31
So while optic pathway gliomas,
2:34
we often think of the optic nerve involvement,
2:36
in particular the orbital segment
2:38
of the optic nerve.
2:39
It can be anywhere along this optic pathway,
2:42
including, as seen in this case,
2:43
the optic chiasm and optic tracts.
Report
Description
Faculty
Asim F Choudhri, MD
Chief, Pediatric Neuroradiology
Le Bonheur Children's Hospital
Tags
Syndromes
Pediatrics
Neuroradiology
Neuro
Neoplastic
MRI
Brain
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