A 14-Year-Old Boy With Painful Periorbital Edema

Alexis R. Pascoe, BS; Amit R. Bhatt, MD; Kimberly G. Yen, MD; Eric J. Kim, MD; Madhuri Chilakapati, MD


July 10, 2019

Case Diagnosis

Idiopathic orbital inflammation is most consistent with this presentation, with diffuse enlargement of the involved area evident on imaging. Idiopathic orbital inflammation typically localizes in five various locations or patterns: lacrimal gland (as in this case), extraocular muscles, the anterior orbit, the orbital apex, or diffuse inflammation through the orbit. Symptoms associated with idiopathic orbital inflammation generally include, but are not limited to, proptosis, motility restriction, periorbital edema, and periorbital erythema; the five most common signs are palpable mass, motility restriction, swollen eyelid, proptosis, and increased orbital pressure.[1,2,3,4]

Orbital cellulitis presents with periorbital edema and erythema, but typically is associated with constitutional symptoms such as fever, which this patient did not have. In addition, the hyperintensity of the lacrimal gland on imaging makes this diagnosis less likely. MRI findings consistent with orbital cellulitis typically demonstrate an isodense signal as well as mass effect leading to some degree of globe distortion. In addition, if this were a case of orbital cellulitis, there would be evidence of sinus disease or another possible external source of infection, as well as postseptal abscess or phlegmon.

Neurofibromas have a progressive onset of symptoms instead of the acute onset, as in this case. They also cause globe displacement, decreased visual acuity, impaired extraocular motility, and proptosis. Neurofibromas are associated with neurofibromatosis type 1. An ocular finding associated with neurofibromatosis type 1 is Lisch nodules.

Allergic blepharitis is inflammation of the eyelids secondary to exposure to certain irritating antigens. This interaction results in erythema, swollen eyelids, and tearing resulting from the irritation. Although this patient experienced swollen eyelids, the presence of pain, the increase in intraocular pressure, and the restriction in extraocular muscle motility suggests another diagnosis.

Rhabdomyosarcoma is a malignant neoplasm that requires prompt diagnosis to save the life of the patient. Typical presentation consists of an acute onset of globe displacement, eyelid swelling, pain, ptosis, and palpable mass. On CT scan, a homogenous, well-circumscribed, round mass will be present within the orbital soft tissues and be isodense to muscle.

Clinical Course

With a presumed diagnosis of cellulitis initially, the patient was treated with intravenous antibiotics but did not improve.

A biopsy confirmed the diagnosis of idiopathic orbital inflammation, and the patient responded well to steroid therapy. Once discharged, his care continued on an outpatient basis to taper therapy and monitor for any steroidal adverse effects (eg, glaucoma, cataract, ocular rebound inflammation, opportunistic infections).


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