Case 21: What is the most likely diagnosis?
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Case 21: What is the most likely diagnosis?
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DDx: Parasellar and Right Orbital AVF-Congestive Case 21: What is the most likely diagnosis? Orbitopathy 1. 2. 3. 4.
Anterior Cranial Fossa Dural Fistulas Cirsoid Aneurysms Traumatic Carotid-Cavernous Fistulas Wyburn-Mason syndrome
Parasellar and Right Orbital Dural AVF- Congestive Orbitopathy Arteriovenous malformations (AVMs) are developmental anomalies that occur when the embryonic vascular network fails to differentiate. Intraorbital AVMs are rare. Unilateral exophthalmos can be caused by both intraorbital and extraorbital vascular malformations. With the exception of bruit, the most common presenting symptoms of orbital AVMs are related to venous drainage involving the orbit. The congestion is reflected by dilation of the veins on the surface of the sclera (red arrows), producing chemosis. Severity of visual symptoms correlates with the adequacy of the venous drainage pathways more than with the speed or volume of the arteriovenous shunting.
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DDx
Case 22: What is the most likely diagnosis?
1-22-10
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DDx: Disappearing Orbital Varix 1. 2. 3. 4.
Case 22: What is the most likely diagnosis?
Cavernous sinus thrombosis Orbital lymphangioma Orbital hemangioma Carotid caveronous fistula
Disappearing Orbital Varix Orbital Varices are uncommon lesions (red arrows) that classically manifest in the 2nd or 3rd decades of life. They result from a most likely congenital weakness in the postcapillary venous wall, which leads to the proliferation of venous elements and massive dilatation of the valveless orbital veins. Most varices have a large communication with the venous system and they distend during maneuvers that increase venous pressure. Patients with orbital varices usually manifest stress proptosis. 1-22-10At MRI, varices have hypo- to hyperintense signal on T1-weighted images, and have hyperintense signal on T2-weighted images. They usually enhance intensely after contrast administration. CT is also valuable to detect bone anomalies related to orbital varices. CT or MR can be performed both at rest and with a Valsalva maneuver, as in some cases no abnormallity is visible on routine imaging- the change in size is helpful in distinguishing the entitiy from other vascular lesions of the orbit.
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DDx
Case 23: What is the most likely diagnosis?
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Case 23: What is the most likely diagnosis?
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DDx: Lacrimal Sac Region Dermoid-Choristoma 1. 2. 3. 4.
Case 23: What is the most likely diagnosis?
Teratoma Hamartoma Colobomatous Cyst Lipodermoid
Lacrimal Sac Region Dermoid-Choristoma Choristomas are congenital lesions representing normal tissues in an abnormal location (red arrows). They are the most common epibulbar and orbital tumors in children. Single tissue choristomas consist of dermis-like tissue or ectopic tissues of mesectodermal origin. The association of choristomas with various craniofacial and systemic defects is well documented in the literature. The finding of a choristoma may signal a simultaneous occurrence of other lesions of an analogous origin in other localities than the orbit. MR imaging is the modality of choice in patients with congenital midface masses.
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DDx
Case 24: What is the most likely diagnosis?
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Case 24: What is the most likely diagnosis?
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DDx: Pleomorphic Adenoma 1. 2. 3. 4.
Case 24: What is the most likely diagnosis?
Dacryoadenitis Lymphoma Adenoid cystic CA Pseudotumor
Pleomorphic Adenoma The pleomorphic adenoma is also referred to as benign mixed tumor. All components of the tumor are of epithelial origin. Pleomorphic adenomas are found after the 2 nd decade of life with a peak incidence between the ages of 25 and 40. A usually painless tumor develops over the course of many years, and the tumor (red arrows) is surrounded by a pseudocapsule formed by compressed adjacent tissue and fibrosis. There is a high recurrence rate with up to 20% malignant degeneration. On CT, it is round and smooth in outline, displacing and deforming the globe. The contrast enhancement is moderate to marked. The pleomorphic adenoma is mostly located in the orbital and rarely in the palpebral portion of the lacrimal gland. Pleomorphic adenoma is one of the most frequent tumors of the lacrimal gland and constitutes 50% of tumors of epithelial origin. Treatment is usually surgical resection due to malignant potential.
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DDx
DDx: Optic Sheath Meningioma 1. 2. 3. 4.
Case 25: What is the most likely diagnosis?
Optic Neuritis, Adult Sarcoidosis Optic Nerve Glioma Pseudotumor
Optic Sheath Meningioma Optic nerve sheath meningioma (ONSM) is a benign, slow growing neoplasm of the optic nerve sheath. It is a distinct entity from an intracranial meningioma that has intraorbital extension. ~90% of meningiomas that involve the orbit are secondary lesions, however. ONSM tend to present early due to symptoms of slowly progressive, painless vision loss and proptosis. Presents typically 4 th-5th decade, but broad range. Juvenile presentation tends to be more aggressive and likely a/w NF2. 2-4:1 Female:Male. Observation if vision remains stable and functional. Stereotactic radiosurgery first line treatment as longer preservation of vision. Surgey used with intracranial extension or if vision preservation not possible. Key imaging is seeing peripheral mass distinct from more central optic nerve. “Tram tracking” of mass/ calcium along periphery of ONS.
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DDx
Case 26: What is the most likely diagnosis? Patient Placed on Steroids after 1995
1-11-95
7-5-01
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DDx: Orbital Lipomatosis
1. 2. 3. 4.
Case Dermoid 26b: What is the most likely diagnosis? Pseudotumor Grave’s disease Leiomyoma
Orbital Lipomatosis-Patient on Steroids Elevated serum levels of corticosteroids have been associated with lipomatosis or excessive deposition of adipose tissue. Such accumulation of fat in the orbit (red arrows) can cause proptosis in patients with Cushing’s syndrome. Periorbital steroid injections have been reported to result in subcutaneous lipatrophy. The frequency of lipomas varies from 0-9% of orbital tumors. A lipoma by definition should exist as an independent mass within the orbit, and growth would displace rather than insinuate other soft tissues. Clinically, lipomas develop slowly without infiltration, and have been reported with proptosis with disseminated benign lipomas of lipomatosis. They may be hyperintense on T1-weighted MRI studies.
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DDx
Case 27: What is the most likely diagnosis?
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Case 27: What is the most likely diagnosis?
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Case 27: What is the most likely diagnosis?
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V1 Schwannoma
Case 27: What is the most likely diagnosis?
Schwannoma are slow growing, benign nerve sheath tumors of the Schwann cells of the neural sheath (red arrows). These are usually solitary lesions. Multiple lesions are rare and may occur as multiple localized tumors or in schwannomatosis, a non-hereditary disease characterized by multiple subcutaneous and intradermal schwannomas together with tumors of internal organs. The tumor is surrounded by a true capsule consisting of the epineurium. Tumors arising from a small nerve resembling a neurofibroma often obliterate the nerve of origin. Schwannoma are seldom painful, and it can reach considerable size. There is little potential for it to become cancerous unless the patient has many nerve tumors. Note the smooth remodeling of the superior orbital fissure in this case.
DDx: V1 Schwannoma 1. 2. 3. 4.
Varix Glioma Meningioma Carotid cavernous fistula
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DDx
Case 28: What is the most likely diagnosis?
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DDx: Drusens
Case 28: What is the most likely diagnosis?
1. Choroidal osteoma 2. Choroidal hemangioma 3. Retinoblastoma
Drusen Optic disc drusens are globules of mucoproteins and mucopolysaccharides that progressively calcify in the optic disc (red arrows). They are thought to be remants of the axonal transport system of degenerated retinal ganglion cells. Drusen is found clinically in about 1% of the population, and most cases are bilateral. Caucasians are the most susceptible ethnic group. May occur as early as 12 yo. With age, the overlying axons become atrophied and the drusen become exposed and more visible. In most patient, drusens are an incidental finding. However, drusens can compress and eventually compromise the vasculature and retinal fibers. Optic nerve damage can rarely be progressive and insidious. Most patients (70%) will develop some peripheral field defects. Central vision loss is a rare complication of bleeding from perpapillar choroidal neovascular membranes which can develop adjacent to the optic disc; early detection and treatment can help prevent serious complcations.
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DDx
DDx: Posterior Scleritis 1. 2. 3. 4. 5.
Case 29: What is the most likely diagnosis?
Allergic Conjunctivitise Episcleritis Pseudotumor Lymphoma Sarcoid
Posterior Scleritis-Idiopathic Orbital Inflammatory Disease Scleritis is a chronic, painful, and potentially blinding inflammatory disease that is characterized by edema and cellular infiltration of the sceral and episcleral tissues (red arrows). Scleritis is commonly associated with systemic autoimmune disorders. Correct and rapid diagnosis and appropriate systemic therapy can halt the relentless progression of both ocular and systemic processes preventing destruction of the globe. Scleritis may be classified into anterior or posterior. Posterior scleritis is characterized by flattening of the posterior aspect of the globe, thickening of the posterior coats of the eye, and retrobulbar edema. An autoimmune dysregulation in a genetically predisposed host is presumed to cause scleritis. Scleritis is most common in the 4 th to 6th decades of life, and is slightly more common in women. Symptoms include pain, tearing, photophobia, tenderness, decreased visual acuity. The primary sign is redness.
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DDx
Case 30: What is the most likely diagnosis?
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Case 30: What is the most likely diagnosis?
Bilateral Foreign Bodies (Glass) This patient has multiple foreign glass fragments in both orbits resulting from a car accident. Bilateral globe ruptures.
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