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Orbital Pseudotumor Micah Kinney, OD, FAAO 655 Bluff Park Rd Hoover, AL 35226 Tel: (205)739-1541 [email protected] I. Case History A. Patient demographics: 31- year old Caucasian Male B. Chief complaint: Bump on left lower eyelid for the past four months. The patient was seen by his flight surgeon four months prior and started a tenday course of Septra DS (160 mg trimethoprim/800 mg sulfamethoxazole) without any improvement. There was no follow-up by the patient; so further investigation did not take place at that time. Mild pain and tenderness upon palpation, warm compresses used at least BID. Diplopia in left gaze. C. Ocular, medical history: Negative Ocular History. History of Kidney stones x 8 years ago D. Medications: None E. Other salient information: None II. Pertinent findings A. Preliminary Testing: DVA sc 20/20 OD/OS EOMs: FROM, diplopia in left gaze Red Lens test: 5ΔBI OS Left Gaze CT: Ortho in Primary gaze (6m and 40cm) PERRL (-) APD IOP (app) OD: 10 mmHg OS: 11 mm Hg B. Physical: OD anterior segment unremarkable OS 1x1 cm palpable HARD mass superior to the inferior orbital rim. Not warm to touch, mild pain with palpation, no visible elevation with lower lid eversion. OU Posterior segment findings unremarkable C. Radiology studies: i. CT ordered with 1mm cuts revealed Left orbit inferiolateral aspect mass measuring 1.8 x 1.9 x 1.7 cm. Well defined. No significant infiltration of the intraorbital fat. No infiltration of the globe. Loss of clarity of the margins of the inferior rectus muscle. ii. MRI (with and without contrast) ordered per request of radiology. Enhancing mass within left orbit inferolateral aspect measuring 1.9 x 2 x 1.7 cm. Left intraorbital fat, bony orbit, extra ocular muscles, and optic nerve appear normal without infiltration III. Differential diagnosis A. Orbital Pesudotumor B. Dermoid C. Lymphoma D. Metastasis E. Bacterial Orbital Cellulitis IV. Diagnosis and discussion A. Orbital pseudotumor accounts for approximately 6-11% of all orbital lesions.5-6 It is the third most common orbital disorder.2 Diagnosis is based on clinical findings, imaging studies, response to steroids, and/or biopsy.16 Biopsy is indicated to aid in differentiation from neoplasia, provided the risk is minimal to surrounding structures. Unlike a true tumor or neoplasia, IOID does not indent the globe, cause tissue necrosis, or give rise to bone deformities.5 B. Symptoms of IOID1,3: Orbital Pain, Proptosis, Lid Swelling, Chemosis, Limited Ocular Motility, Pain on Palpation, Diplopia, Unilateral (although children are more likely to have bilateral symptoms) C. Types based on location1,2,4: i. Dacryoadenitis- inflammation of the lacrimal gland occurs in approximately 25% of IOID patients, making the most common form. Patients complaint of acute pain/discomfort in area of the lacrimal gland. ii. Orbital Myositis- inflammation of one or more extra ocular muscles. Symptoms include pain especially with eye movement, and diplopia. Signs include ptosis, lid edema, and chemosis. iii. Tolosa-Hunt Syndrome- a rare form of IOID in which the cavernous sinus, superior orbital fissure, and/or the orbital apex are inflamed. Patients present complaining of doubled vision with periorbial pain. Cranial nerve palsies including CN3, CN4, CN6, and a loss of sensation along the first and second divisions of CN5. iv. Optic Perineuritis- inflammation of the optic nerve causing decreased vision, occurring in less than 15% of IOID cases. MRI helps to differentiate from idiopathic optic neuritis as this can be an early sign of Multiple Sclerosis. v. Localized Inflammation- may not be defined to a specific tissue and may appear tumor-like, as is described in this case. V. Treatment, management A. Labs: CBC, Thyroid Panel, ACE with Chest X-ray, c-ANCA/p-ANCA B. Imaging: CT v/s MRI C. Oral/Injected Steroids D. NSAIDS, Cyclosporine A E. Surgery VI. Conclusion A. Non typical presentation and no response to antibiotic B. Management of orbital mass removal and post surgical diplopia References: 1. Kanski J. Clinical Ophthalmology a Systemic Approach. 6th ed. Edinburgh, England: Butterworth Heinemann Elsevier, 2007. 2. Weber A, Romo L, Sabates N. Pseudotumor of the orbit: Clinical, pathologic, and radiologic evaluation. Radiologic Clinics of North America. 1999; 1: 151168. 3. Espinoza G. Orbital inflammatory pseudotumors: etiology, differential diagnosis, and management. Curr Rheumatology Rep. 2010; 12:443-447. 4. Jacobs D, Galetta S. Diagnosis and management of orbital pseudotumor. Curr Opinion in Ophthal. 2002; 13:347-351. 5. Martin C. Orbital pseudotumor: case report and overview. JAOA. 2007; 12:775-781. 6. Mendenhall W, Lessner A. Orbital pseudotumor. Am J of Clinical Oncology. 2010; 33:304-306.