Presentation
Acute and painful left side proptosis.
Patient Data
There is solid appearing well-defined and lobulated margin intraconal mass lesion within the left orbital cavity which has completely encased the optic nerve sheath complex and also has an impression on adjacent extraocular muscles and it caused left side proptosis.
There is also remodeling of the left orbit medial wall due to the impression of the mass on the adjacent medial rectus muscle and medial orbital wall.
Microscopic findings: There are dilated lymphatic channels within loose stroma which are lined up by flat endothelial cells. Red blood cells are seen in the vascular channel's lumen due to hemorrhage and there is also some lymphocytic infiltration.
The report has been prepared by Dr. Nozarian Zohre. pathologist, molecular and cellular fellowship.
Case Discussion
Orbital lymphangioma or lymphatic malformation is congenital or acquired for example can be due to previous trauma, radiation, or surgery and it usually occurs in early childhood and rarely occurs in adulthood.
The usual presentation of orbital lymphangioma is sudden unilateral painful proptosis due to an episode of acute hemorrhage within the mass.
The lymphangioma has minimal internal blood flow and has no obvious vascular connection and usually presents as a multiloculated cystic mass lesion.
The imaging modality of choice for primary evaluation of proptosis is non-contrast orbital MDCT but for further characterization and the relation of the mass to the optic nerve and pretreatment mapping, MRI of the orbit with and without gadolinium is mandatory.
Orbital lymphangioma can encase the optic nerve sheath complex and lead to compressive optic neuropathy.
The main treatment of orbital lymphangioma is complete surgical resection of the mass but sclerotherapy, sildenafil, or sirolimus therapy can also be considered based on the severity of the patient's visual impairment and proptosis.
Unable to process the form. Check for errors and try again.