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Case #2

History: one month old male

Findings

Infiltrative mass with both Intraconal and Extraconal components

Iso- to Hypointense T1

Hyperintense T2

Some internal heterogeneity

Intensely enhancing

No change with fat saturation

DDx:

Trans-spatial (Intraconal, Extraconal) Orbital mass in an infant

Lymphangioma

Capillary Hemangioma

Rhabdomyosarcoma

Neuroblastoma

Orbital Cellulitis

Hematopoietic malignancy

Leukemia, Lymphoma

Langerhans cell histiocytosis

Diagnosis

Trans-spatial (Intra/Extraconal) Orbital mass in an infant

Lymphangioma

Multilocular; blood-fluid levels

Capillary Hemangioma

Rhabdomyosarcoma

Invasive, bone destruction

Neuroblastoma

Rapidly progressive, skull base involvement

Orbital Cellulitis

Inflammatory changes, may have abscess

Hematopoietic malignancy

Leukemia, Lymphoma

Langerhans cell histiocytosis

Capillary Hemangioma

Also known as:

Infantile periocular hemangioma

Benign hemangioendothelioma

Most common benign orbital tumor of infants (1% of neonates)

30% present at birth; most present within first 6 months

50 % occur in head & neck

Capillary Hemangioma

Etiology

Hamartomatous proliferation of vascular endothelium

Thin-walled capillary-sized vascular spaces in lobules with thin, fibrous septae

Genetics

Most sporadic

Some assoc. with pleiotropic genetic syndromes

Small % Autosomal Dominant (5q35.3, 5q31-q33)

Capillary Hemangioma

Location

Most common: superomedial extraconal

May extend intraconal and into superior orbital fissure

Exclusively retrobulbar: only 10%

Also predilection for superior orbit, eyelids, supranasal periorbita

Morphology

Margins vary from infiltrative to well-circumscribed

Lobulated, irregular contours

Capillary Hemangioma

Clinical

Unilateral (eyelid, brow, nasal)

Bluish discoloration of skin or conjunctive in 80%; blanches with pressure

Enlarge with crying/Valsalva in 50%

May see proptosis, ptosis

Female: Male 2-3: 1

Two phases

Growth phase: first 1-2 years

Involutional phase: following 1st year

Capillary Hemangioma

Treatment

Observation unless complications

Corticosteroid treatment very effective

Large: intratumoral laser therapy

Recalcitrant:

Interferon

Surgical ligation

Laser ablation

Advanced lesion: radiation