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Case 1

Enhancing nodule in the Pons, DDx:

Neoplasm

Low-grade neoplasm

Lymphoma

Mets

Inflammatory

Demyelinating disease

Vascular

Deep Venous Anomaly (DVA)

Capillary Telangiectasia

Cavernous Malformation

Diagnosis

Neoplasm

Low-grade neoplasm

Lymphoma

Mets

Inflammatory

Demyelinating Disease

Vascular

Deep Venous Anomaly (DVA)

Capillary Telangiectasia

Cavernous Malformation

Imaging Features

T1 WI

Isointense

No abnormal signal

T2 WI

50%: Isointense

50%: Stippled Hyperintensity

Imaging Features, cont.

T1 WI, post-Gd

Faint stippled or “brush-like” enhancement

Poorly demarcated

If mixed with a DVA:

Linear/branching vessels +/- collecting vein

Imaging Features, cont

T2* GRE

Moderately hypointense

Thought due to intravascular deoxyhemoglobin from stagnant blood flow

Capillary Telangiectasia

Cluster of capillaries with normal intervening brain parenchyma

Capillaries are dilated but are histologically normal.

Location:

Most common

Pons

Medulla

Spinal Cord

1/3: other, such as subcortical white matter

Capillary Telangiectasia

Incidence

15-20% of all intracranial vascular malformations

Clinical

Typically clinically silent.

Incidentally discovered on MRI.

Age: any; 30-40 y.o. is most common

Etiology

Most: sporadic with unknown etiology

Children with Cranial XRT: 20% develop Capillary Telangiectasias

Capillary Telangiectasia

Association with Osler-Weber-Rendu disease / Hereditary Hemorrhagic Telangiectasia (HHT)

AVM’s

*The most common intracranial vascular malformation with HHT is an AVM (not capillary telangiectasia).

Multiple pulmonary AVM’s (AVF’s) or cerebral AVM’s in a patient with recurrent epistaxis (median age of onset: 11 y.o.) and family hx of HHT (autosomal dominant)..

HHT, cont.

Most cerebral AVM’s are low-grade (Spetzler-Martin I or II).

Pulmonary AVF’s result in right-to-left shunts and can present with an embolic CVA or brain abscess.

Associations:

Multiple pAVM’s: 70% have HHT.

Multiple cAVM’s: 50% have HHT.

HHT: 5-15% have pAVM’s.

HHT: 5-13% have cAVM’s

Hepatic AVM’s

GI (stomach, duodenum) AVM’s: GI bleeding usually begins after age 50.

Osler-Weber-Rendu, cont.

Capillary Telangiectasias

Most commonly EXTRA-axial

Face, lips, scalp

Nasopharynx

Oral Cavity

Orbit

Rarely intra-axial