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Antral Polyp
Joseph Junewick, MD FACR
over 8 years ago
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Dural Angiomatosis - PHACES

Case Detail

Anatomy: Brain-Spine
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Benign
Created: over 8 years ago
Updated: over 6 years ago
Tags: PEDS
Modality/Study Types: MR
Activities:
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History

1 month old female being seen by pediatric ophthalmologist for rapidly enlarging orbital and facial hemangiomas.


Case Images


Diagnosis

Dural Angiomatosis – PHACES

Findings

MR – Extensive lobulated dural enhancement with infiltrative enhancing lesions in the orbit and face.

Discussion

PHACES association is a rare neurocutaneous syndrome characterized by posterior fossa brain malformations, large facial hemangiomas, arterial anomalies, cardiac anomalies and aortic coarctation, and eye abnormalities such as colobomas, optic nerve hypoplasia, increased retinal vascularity, and glaucoma. Recent studies suggest that 2% of children with facial hemangiomas and 20% of children with regional facial hemangiomas may have PHACES association.

Reference

Viswanathana V, Smith ER, Mulliken SJ et al. Infantile Hemangiomas Involving the Neuraxis: Clinical and Imaging Findings. AJNR (2009); 30:1005-1013.



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