SYMPTOMS Ocular pain, Diplopia, Decrease in vision
SIGNS Unilateral
APD, Rapidly developing proptosis (most common sign), Globe displacement (down and out), Ptosis, EOM restriction, Eyelid swelling, Conjunctival chemosis, Choroidal folds, Tortuous retinal vessels, Optic nerve edema or pallor
WORK-UP Pupils, EOMs, Cover test, Ptosis evaluation, Exophthalmometry, Full eye exam with dilation
TREATMENT Refer to a Pediatric ophthalmologist or Ocular oncologist STAT for treatment which could include surgical removal, radiation, and/or chemotherapy
FOLLOW-UP Once the rhabdomyosarcoma is treated by an ocular oncologist/pediatric ophthalmologist and the eye is stable, the patient should be seen back in 3-4 months for the first visit followed by every 4-6 months
ADDITIONAL LAB | TESTS CT or MRI of the orbit (most likely will be ordered by the ocular oncologist or pediatric ophthalmologist)
ETIOLOGY Malignant sarcoma that develops in the orbital soft tissue
DIFFERENTIAL DX Neuroblastoma, Lymphangioma, Hemangioma, Orbital cellulitis
NOTES An orbital rhabdomyosarcoma is the most common primary orbital malignancy in children
The majority of rhabdomyosarcomas involve the superior or superior nasal aspect of the orbit
If left to grow, an orbital rhabdomyosarcoma can cause a neoplastic meningitis