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Hypoplastic Internal Carotid Artery Co-Presenting with Neurofibromatosis and Intracranial Masses

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ABSTRACT

Neurofibromatosis type 1 (NF1) is associated with systemic vascular disease, and it can also affect intracranial vasculature in a small percentage of patients. Very rarely, NF1 may co-present with hypoplasia of the internal carotid artery (ICA). Prior reports have documented NF1 with bilateral optic gliomas and a unilateral hypoplastic internal carotid artery; however, we report a case with the aforementioned findings in addition to a right-sided lentiform mass. This case report further suggests a common congenital pathway related to neurofibromin loss of function resulting in both nerve sheath tumors and cerebrovascular anomalies.

No MeSH data available.


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Posterior anterior (PA) angiogram of the anterior circulationLeft ICA demonstrates absence of the intracranial left ICA distal to ophthalmic branch consistent with hypoplasia of the left ICA.
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FIG4: Posterior anterior (PA) angiogram of the anterior circulationLeft ICA demonstrates absence of the intracranial left ICA distal to ophthalmic branch consistent with hypoplasia of the left ICA.

Mentions: The patient then underwent a diagnostic six-vessel cerebral angiogram (DCA) that demonstrated a complete left internal carotid occlusion distal to the branch of the ophthalmic artery. In addition, the entire left middle cerebral artery (MCA) and anterior cerebral artery (ACA) vascular territories were supplied by the right internal carotid via a robust anterior communicating artery (Figures 4-5).


Hypoplastic Internal Carotid Artery Co-Presenting with Neurofibromatosis and Intracranial Masses
Posterior anterior (PA) angiogram of the anterior circulationLeft ICA demonstrates absence of the intracranial left ICA distal to ophthalmic branch consistent with hypoplasia of the left ICA.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5037063&req=5

FIG4: Posterior anterior (PA) angiogram of the anterior circulationLeft ICA demonstrates absence of the intracranial left ICA distal to ophthalmic branch consistent with hypoplasia of the left ICA.
Mentions: The patient then underwent a diagnostic six-vessel cerebral angiogram (DCA) that demonstrated a complete left internal carotid occlusion distal to the branch of the ophthalmic artery. In addition, the entire left middle cerebral artery (MCA) and anterior cerebral artery (ACA) vascular territories were supplied by the right internal carotid via a robust anterior communicating artery (Figures 4-5).

View Article: PubMed Central - HTML - PubMed

ABSTRACT

Neurofibromatosis type 1 (NF1) is associated with systemic vascular disease, and it can also affect intracranial vasculature in a small percentage of patients. Very rarely, NF1 may co-present with hypoplasia of the internal carotid artery (ICA). Prior reports have documented NF1 with bilateral optic gliomas and a unilateral hypoplastic internal carotid artery; however, we report a case with the aforementioned findings in addition to a right-sided lentiform mass. This case report further suggests a common congenital pathway related to neurofibromin loss of function resulting in both nerve sheath tumors and cerebrovascular anomalies.

No MeSH data available.


Related in: MedlinePlus