Go Back ENT/22. Orbit/Neoplasm, Neoplasm-like condition/22.3 - Orbital Venolymphatic Malformation/ Go to Index
Case #1
History:
7 y.o. male with proptosis (painless)
Findings
Intraconal (primarily) and Extraconal
Lobulated margins
T1: hypointense
T2:
Very hyperintense fluid
Fluid-fluid levels
T1 post-Gd: enhancing septae
(No flow voids)
DDx
Irregular enhancing orbital mass in a child:
Capillary Hemangioma
Cavernous Hemangioma
Lymphangioma
Plexiform Neurofibroma
Orbital Pseudotumor
Rhabdomyosarcoma
Hematologic malignancy
Leukemia, Lymphoma
Langerhans cell histiocytosis
Diagnosis
Irregular enhancing orbital mass in a child:
Capillary Hemangioma
Cavernous Hemangioma
Lymphangioma
Plexiform Neurofibroma
Orbital Pseudotumor
Painful proptosis
Rhabdomyosarcoma
Hematologic malignancy
Leukemia, Lymphoma
Langerhans cell histiocytosis
Orbital Lymphangioma
Also known as:
Cystic hygroma
Orbital lymphatic-venous malformation
Infants, young adults
Usually presents in 1st decade
Slight female predominance
Orbital Lymphangioma
Pathology
Congenital, benign vascular tumor consisting of dilated vascular channels surrounded by lymphoid tissue
Etiology
Hamartoma from vascular mesenchymal anlage that develops with no systemic vascular or lymphatic connection
Orbital Lymphangioma
Epidemiology
3 in 100, 000
5% or childhood orbital tumors
8% of expanding orbital tumors
Less common than orbital
Capillary Hemangioma
Cavernous Hemangioma
Orbital Lymphangioma
Clinical
Progressive painless proptosis
Periorbital ecchymosis
Diplopia, ptosis, restricted EOM movement
Orbital Lymphangioma
Natural History, Prognosis
Continues to expand
Does not involute (like capillary hemangioma)
May intermittently increase in size with upper respiratory infection (lymphatic tissue)
May rapidly increase in size due to hemorrhage
Increased recurrence
Refractory visual problems and disfigurement are common
Poor visual acuity assoc. with multiple surgical resections
Orbital Lymphangioma
Treatment
Conservative
If vision not threatened
Systemic steroids
May decrease pain, swelling, and proptosis.
Surgery
50% recurrence
Subsequent visual impairment
May result in additional mass effect due to hemorrhage
