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Control #: 1737 Title: Mimics of Nontraumatic Solid Orbital Tumors: More Than Meets the Eye eEdE#: eEdE-119 (Shared Display) No Disclosures Mimics of Nontraumatic Solid Orbital Tumors: More Than Meets the Eye Rajput, Anuj MD, Ahluwalia, T. MD, Rajput, Aikta, Bangiyev, L. DO Stony Brook University Hospital, NY Purpose To review the incidence, clinical presentation, and significance of several non-traumatic orbital pathologies with our main focus on the thrombosed orbital varix. Familiarity with appearance of the various orbital lesions will help the radiologist to correctly diagnose a thrombosed orbital varix, exclude other differential diagnosis, and provide appropriate recommendations. Orbit Anatomy (Superior to inferior) Superior Ophthalmic Vein Superior Rectus Muscle Orbit Anatomy Extraconal Space Intraconal Space Lamina Papyracea Orbit Anatomy Medial Rectus Muscle Lateral Rectus Muscle Globe Optic Nerve Superior Orbital Fissure Orbit Anatomy Anterior Chamber Posterior Chamber Lens Sclera Inferior Rectus Muscle Orbit Anatomy Ophthalmic Artery Superior Rectus Muscle Superior Oblique Muscle Optic Nerve Inferior Rectus Muscle Lateral Rectus Muscle Medial Rectus Muscle CASE History of Present Illness • 69 year old female presents to the ED with chief complaint of bilateral eye swelling and vomiting, worse on the left. Patient states she woke up with bilateral eye pain, worse with lateral and medial gaze. She had intermittent episodes of blurry vision and multiple episodes of vomiting. Patient stated that when looking straight ahead, she has no pain or vision abnormality. She denies prior episodes. There were no relieving factors. • Otherwise, review of systems was noncontributory Medical History • • • • • PMHx: HTN, HLD, hypothyroidism FMHx: None SHx: Denied alcohol, drugs, tobacco SurgHx: Hysterectomy Allergies: NKA Physical Exam Vitals: Temp: 38.6*C,HR: 85, BP: 132/89, Pulse Ox: 94% General: Alert, no acute distress. Skin: Warm, intact. Head: Normocephalic, atraumatic. Neck: Supple, trachea midline. Eye: PERRLA, EOM intact, Visual acuity: OU 20/ 20; Slit-lamp exam: Viewed with fluorescein, questionable circular stromal lesion/infiltrate at approximately 6 o'clock which does not uptake fluorescein, similar lesion on right, not clearly ulcerative and appear almost crystalline. Ant. Chamber quiet. No photophobia IOP: OD: 15 OS: 18, ENT: Within normal limits Neurological: A&O X3, normal sensory, motor 5/5, normal speech, cranial nerves intact. Laboratory Values • General Chemistry Panel – unremarkable • TSH – within normal limits • General Hematology Panel – unremarkable • Coagulation – Antithrombin III and Protein C were elevated – Otherwise within normal limits CT Orbits C- Following CT C-, an MRI with contrast of orbit and brain were performed Differential Diagnosis? Axial CT C- of the orbits demonstrates a left intraorbital high density circumscribed mass (arrow) with Hounsfield units compatible with blood products. Coronal CT C- of the orbits shows left orbital intraconal mass with associated mass effect and displacement of the left optic nerve (blue arrow). Axial T2 MR image demonstrates low signal intensity mass in the left orbit (arrow). Axial T1 precontast MR image demonstrates iso to low signal intensity of the mass in the left orbit. Axial T1 post contrast MR image demonstrates thin rim enhancement around the left orbital lesion (arrow). A smaller homogeneously enhancing lesion is also present within the right orbit (yellow arrow) Coronal T1 post contrast MR image again demonstrates nonehancing left intracornal mass with mass effect on the optic nerve (red arrow). The smaller right intraconal mass is homogeneously enhancing without significant mass effect (yellow arrow). Differential Diagnosis of Solid Orbital Mass • Neoplastic - lymphoma, multiple myeloma, meningioma, metastasis, optic glioma • Inflammatory – Pseudotumor, thyroid ophthalmopathy • Granulomatous – Sarcoidosis, Wegner’s granulomatosis • Congenital - infantile hemangioma • Vascular - orbital varix, venolympathic malformation • Infectious Diagnosis Thrombosed Orbital Varix Background • Rare < 1.3% of all orbital tumors • Most common cause of spontaneous orbital hemorrhage. • Abnormally enlarged vein - single vessel with saccular or segmental dilatation or tangled plexus of venous channels. • 2nd and 3rd decade of life M=F • Primary or secondary • Intermittent diplopia or proptosis during of straining or prone. • Hemorrhage or thrombosis are possible complications can cause acute orbital pain and decreased visual acuity. Background Primary Form • Congenital venous malformation. • May manifest itself during infancy or delayed until young adulthood. • Transient exophthalmos induced by alsalva due to communication with the systemic venous system. Secondary Form • Acquired due to increased blood flow as a result of intracranial AVM, CC fistula or dural AVF which drain vial the orbit. • Associated with vein of Galen malformation and occasionally with venous angiomas. Clinical Presentation • Presenting symptoms include intermittent diplopia or proptosis during episodes of straining or prone positioning. • Hemorrhage or thrombosis are possible complications that can cause an acute onset of retro- orbital pain and decreased visual acuity. Work up & Treatment • Difficult to diagnose without provocative examination with valsalva • Large may present as a hyperdense orbital mass on CT, especially if thrombosed • Thrombosed orbital varix show complex MR signal of blood products and/or fluid levels • Treatment ranges from observation with mild symptoms to embolization or surgery with intractable pain or threatened vision. Case Review of Relevant Differential Diagnosis Ocular Lymphoma • B-cell mucosa-associated lymphoid tissue (MALT) are the most common14,15 • 5-10% of orbital masses • Associated with systemic collagen vascular diseases and immunocomprised patients. • Indolent course and painless mass • Key imaging features - intermediate or low T2, homogeneous enhancement, ↑ DWI and ↓ ADC24 • Favorable prognosis. • Sensitive to radiation16,17 • High grade lesions may require systemic chemotherapy. Ocular Lymphoma A B C D FLAIR (A), T1 Post-Gd (B), B1000 (C), ADC (D) demonstrate a mildly enhancing extraconal mass in the superior medial right orbit with a small central focus of irregular, spiculated T2 and FLAIR hyperintensity and more intense enhancement. It restricts diffusion. This is displacing the medial rectus as well as the optic nerve laterally. There is remodeling medial deviation of the inner wall of the right orbit suggesting a benign or long-standing lesion. Multiple Myeloma • Clonal proliferation of malignant plasma cells in the bone marrow leading to multiorgan failure13 • Hypercalcemia, renal dysfunction and bone lesions13 • Rarely presents as orbital lesion9 • Solitary extramedullary orbital plasmacytomas have been reported as the initial presention 11 • Treatment include systemic chemotherapy and local12 Multiple Myeloma Figure 1: Prominent soft tissue mass associated with bone destruction located in the left superior orbital fissure region. (arrow). The left optic canal is fully destroyed and occupied by tumor. Figure 2: Numerous lytic lesions in the calvarium. (blue arrows) Figure 1 Figure 2 Meningioma • • • • Benign mass, presents in 4th or 5th decade of life. Painless eye mass, vision loss, and proptosis 24 Unilateral. Bilateral in neurofibromatosis type 2. 24 Primary and Secondary: – Primary arises from capillary cells of the arachnoid around the intraorbital or intraconal portions of the optic nerve.25-26 – Secondary arises intracranially and invades the optic canal and orbit by extension25-26 • Imaging – mass surrounding and constricting the optic nerve; calcification on CT; ‘‘tram track’’ or ‘‘target’’ like enhancement.24 • Fractionated stereotactic radiotherapy is first line therapy Meningioma A B C Axial T1 C+ (A) demonstrates an enhancing mass surrounding the optic nerve in a “tram-track” pattern. Coronal T2 (B) and Coronal C+ (C) MR images demonstrate T2 hyperintensity and contrast enhancement surrounding the optic nerve creating a target-like pattern. Ocular Metastasis • Carcinomatous origin in adults. Sarcomatous or neural embryonal tumors in children.27 • Account for 1–13% of all orbital tumors.28 • Typically occur in the anterior orbit.29 • Most frequent: breast, lung, prostate, melanoma, carcinoid, GI, renal cell, neuroblastomas and rhabdomyosarcomas.28 • Imaging findings vary widely. Globe retraction is highly suspicious for scirrhous breast cancer metastasis.24 Ocular Metastasis A B C Bilateral lobular metastasis with heterogeneous signal on T1WI (A) and T2WI (B) with diffuse enhancement (C). More advanced on the right demonstrated by right globe retraction. Findings consistent with schirrous breast metastasis. Optic Glioma • Indolent and low grade tumors • Typically associated with NF-1 and found in mostly in children. • Key features: Smooth, homogeneous spherical mass with hypointense T1 signal, and T2 hyperintense signal with or without enhancement. Optic Glioma A C B T1 hypointense (B,E), T2 hyperintense (C), heterogenously enhancing left intraconal mass (A,D) associated with the left optic nerve. D E Thyroid Ophthalmopathy • Most common cause of proptosis in adults.35 • Optic nerve dysfunction affects ~5% of patients.34 • Enlargement of the ocular muscles by edema and infiltration of inflammatory cells.35 • Imaging features enlargement of the extra ocular bellies (T1 Isointense, T2 Hyperintense in acute and iso-hypointense in chronic stages), with sparing of the myotendinous junction. • Lateral rectus muscle is the last extra ocular muscle affected. Thyroid Ophthalmopathy A B C D T1WI (A), T2WI (B), T1WI C+ (C), and Axial T1WI C+ images demonstrate enlargement of the ocular muscles with sparing of the lateral rectus muscle and myotendinous junction. There is slight proptosis of the left orbit. Pseudotumour (Idiopathic Orbital Inflammation) •Unknown etiology of orbital inflammation that mimics a tumor mass •Histology demonstrates lymphocitic infiltration.36 •Affects adjacent structures such as the lacrimal gland, mimicing a mass. •Rapid onset and produce symptoms similar to thyroid ophthalmopathy •Involve the myotendinous junction, unlike thyroid ophthalmopathy. •Key findings include enlargement of the bellies of the extraocular muscles including the myotendinous junction, T1 iso/hypointense, T2 hypointense, and diffuse enhancement with C+ imaging. Pseudotumour (Idiopathic Orbital Inflammation) A B C Coronal T1WI (A), T1WI C+ (B), T2WI (C), and Axial T2WI (D) demonstrate enhancing T1 hypointense, T2 hyperintense signal adjacent to the right superior rectus muscle with inferior displacement of the muscle and the optic nerve with proptosis in the patient with eye pain and visual disturbance compatable with orbital pseudotumor. D Sarcoidosis • Multisystem granulomatous disease. • Ocular involvement usually includes uveitis and conjunctival granulomas. • Orbital tissue involvement is rarely reported, varying from lacrimal gland infiltration to optic nerve sheath involvement.5 • Pathology reveals non-caseating granulomas that infiltrate orbital fat. • Laboratory values demonstrate elevated blood and CSF ACE levels. • Additional imaging may reveal hilar lymphadenopathy on a chest x-ray or chest CT. • Responds well to systemic steroids. Sarcoidosis A D B C E FIGURES A-C: Multiple coronal MR images demonstrate a T1 hypointense (A), T2 hyperintense (B), and homogenously enhancing mass of the R orbit (C), with medial displacement of the optic nerve. FIGURES D&E: Bilateral hilar lymphadenopathy; typical findings of Sarcoidosis Infantile Hemangioma • The most common tumor in infancy and appear within the first 6 months of life.18-20 • A proliferative phase occurs up to 10 months, followed by a slow involution phase for up to 10 years.21-23 • Diagnosis is established clinically, however, contrast enhanced MRI is performed to evaluate the extent of the lesion and possible mass effect. • Key distinguishing imaging features include bright T2 signal, lobular borders, fine internal flow voids, extraconal location, and intense homogeneous enhancement.24 Infantile Hemangioma A B C Multiple MR images demonstrate a lobulated mass within the right orbit with FLAIR hyperintense signal (B), T1 isointense signal (A,D) with intense homogeneous enhancement (E), and internal flow voids (C). D E Venolympathic Malformation • Benign vascular malformations commonly affecting children and rarely involving the orbit. • Unencapsulated and composed of fibrous material including endothelial lacunae filled with blood or serious fluid.30 • Symptoms include swelling, intraorbital hemorrhage, ocular proptosis, blepharoptosis and cellulitis. • MR demonstrates vascular components, and fluid filled levels or menisci can be seen.31 • T1 isointense, T2 hyperintense relative to the brain with internal septations.31 • Venous component of a lesion will enhance, whereas the lymphatic component will only show fine enhancement of septations. 24 Venolympathic Malformation A B C T2 MR images (A,D) demonstrates a heterogeneous mass in the left orbit with T2 hyperintense components and fluid-filled levels. T1 C- (B,C) and C+ (E) demonstrate a hypointense mass with homogenous enhancement, encasement of the optic nerve, and proptosis of the left eye. D E Conclusion • Orbital varix is a rare – 1% of all orbital masses. • Presents with intermittent diplopia or proptosis during episodes of straining or prone positioning. • Hemorrhage or thrombosis can cause an acute onset of pain and decreased visual acuity. • Review of this educational exhibit will enabled a radiologist to be familiar with this entity, consider associated complications, narrow relevant differential diagnoses, and offer appropriate recommendations. References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30. 31. 32. 33. 34. 35. 36. Karcioglu ZA. Orbital Tumors, Diagnosis and Treatment: Springer, New York; ISBN 978-0-387-21321-7; 2005. Smoker WR, Gentry LR, Yee NK, Reede DL, Nerad JA. Vascular lesions of the orbit: more than meets the eye. Radiographics : a review publication of the Radiological Society of North America, Inc 2008;28:185-204; quiz 325 Ulus OS, Kararaslan E. Orbital varix: CT and MRI findings. 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