Week 3 - Cases 31-40

Case 31: What is the most likely diagnosis?

Pneumatic Retinopexy- Scleral Banding In pneumatic retinoplexy, gas is injected into the eye (red arrow) and the patient is positioned to “put the bubble on the trouble,” thereby flattening the retina. Cryopexy or laser is applied around the tear to cause permanent attachment. Pneumatic retinopexy is limited to very specific instances, particularly when the retinal hole is either a single hole or several holes close together and are in the superior portion of the eye. In other instances, scleral buckling/banding is necessary by placing a silicone band around the eye (blue arrow) and is sutured onto the eye. The buckle is tightened to give pressure from the outside toward the inside, effectively decreasing the diameter of the eye in the area of the sceral buckle. The retinal holes on the inside of the eye are closed, allowing the retina to reattach. Scleral banding is many times accompanied by subretinal fluid drainage and/or gas bubble injection to aid in reattachment. Retinal reattachment has a 90-95% success rate.

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Case 32: What is the most likely diagnosis?

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DDx: Optic Neuritis 1.

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Case 32: What is the most likely diagnosis?

Glioma Pseudotumor Sarcoidosis Meningioma Devic’s disease

Idiopathic Optic Neuritis A triad of symptoms often heralds optic neuritis: loss of vision, ipsilateral eye pain, and dyschromatopsia. It is unilateral in 70% of cases. Optic neuritis is predominantly a disease of young adults. The disease is rare in children unless accompanied by infection, sometimes bilateral, and generally characterized by a good visual prognosis. In patients over 50 years of age, optic neuritis can be a misleading diagnosis as other disorders commonly cause acute or subacute visual loss. Acute optic neuritis usually presents with obscuration of vision in bright light, and patients with chronic optic neuritis see better in dim light. CT can show transient enlargement and contrast enhancement of the affected nerve (red arrows). MRI is more sensitive to the multifocal plaques in the white matter. Optic neuritis is highly associated with conversion to multiple sclerosis.

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DDx

DDx: Ocular Melanoma 1.

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Case 33: What is the most likely diagnosis?

Choroidal metastsis (lung/breast) Choroidal hemangioma Retinal detachment Pseudotumor Lymphoma

Ocular Melanoma Choroidal melanoma is the most common primary malignant intraocular tumor and the second most common type of primary malignant melanoma in the body. Intraocular melanomas simultaneously can involve more than one uveal structure. Arising from melanocytes within the choroid, they are typically dome shaped (red arrows).

If they break through Bruch’s membrane they can assume a mushroom configuration. Growth of choroidal melanomas can occur silently until it produces enough visual loss through consequent ischemia. The retina overlying the tumor can separate into cystoid spaces and larger schisis cavities. Ocular melanomas can prove fatal due to a high rate of metastasis, but fortunately most choroidal melanomas are confined to the globe.

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DDx

Case 34: What is the most likely diagnosis?

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DDx: Optic Neuritis 1.

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Case 34: What is the most likely diagnosis?

Glioma Meningioma Sarcoidosis Pseudotumor

Post-viral Optic Neuritis Most cases of optic neuritis are due to an immune-mediated process associated with a viral or other infection or with immunization. Patients may present with persistent visual loss and pain on eye movements. They may also complain of dyschromatopsia that is exacerbated by heat or exercise, and typically present as otherwise healthy young adults. In adults, optic neuritis is most often unilateral and retrobulbar with a high probability of recurrent inflammatory demyelinating events in the CNS. Optic neuritis is most often seen in adults aged 20-45 and affects caucasian women twice as often as men. MRI helps rule out structural lesions, and utilization of fat saturation techniques helps to visualize gadolinium enhancement of the optic nerve (red arrows) and is the best imaging technique to visualize inflammation of the optic nerve.

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DDx

Case 35: What is the most likely diagnosis?

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Central Nervous System Neurosarcoidosis

Case 35: What is the most likely diagnosis?

Sarcoidosis is a multisystem granulomatous disease of unknown etiology and the nervous system is a relatively uncommon site for the disease to manifest. The range of abnormalities include: white matter lesions, hydrocephalus, mass lesions in the brain parenhyma, and lesions of the parenchyma of the optic nerves (red arrows) and spinal cord. MRI is often used to monitor treatment of the disease and can contribute to the criteria for starting and tapering corticosteroid therapy. The development of neurosarcoidosis is primarily leptomeningeal and vascular in nature- most likely resulting in disruption of the leptomeningeal blood-brain barrier, permitting the granulomatosis to infiltrate the CNS. Type 1 lesions exhibit gadolinium enhancement that reflect damage to the blood-brain barrier. Abnormal hyperintense foci at the corticomedullary junction on T2weighted images are often nonspecific.

DDx: CNS Neurosarcoidosis 1. Lyme Disease 2. TB/Fungal ds 3. Meningeal Carcinomatosis

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DDx

DDx: Idiopathic Perineuritis 1.

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Case 36: What is the most likely diagnosis?

Optic Nerve Sheath Meningioma Sarcoidosis Pseudotumor Tuberculosis

Idiopathic Perineuritis Optic neuritis is a form of idiopathic orbital inflammatory disease targeting the optic nerve sheath. Optic perineuritis occasionally occurs as a manifestation of a specific infectious or inflammatory disorder. Patients present with acute monocular visual loss, pain with ocular movement, and either a normal or swollen optic disk.

Neuroimaging typically shows a characteristic pattern of enhancement around the optic nerve (“tramtrack” on axial views and “doughnut” on coronal views [red arrows]). In addition, MRI scans can show streaky enhancement of orbital fat; which is not found in patients with typical demyelinating ON. In some patients, close inspection may reveal subtle enhancement of extraocular muscles and sclera with changes in the optic nerve sheath.

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DDx

Case 37: What is the most likely diagnosis?

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Case 37: What is the most likely diagnosis?

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DDx: Optic Glioma-Neurofibromatosis

Case 37: What is the most likely diagnosis?

1. Lymphoma 2. Meningioma 3. Metastasis

Bilateral Optic Glioma-Neurofibromatosis Type 1 Optic nerve glioma is the most common primary neoplasm of the optic nerve. In children, it is often a low-grade benign optic glioma and in another form, is an aggressive and potentially fatal glioma in adults. MR imaging of the orbit with T1-weighted images with fat saturation can define the extent of aggressive glioma. T2-weighted images demonstrate a mixed appearance that is isointense to hyperintense relative to white matter and the cortex. A diagnosis of NF-1 may be supported by findings of bilateral optic nerve gliomas (fusiform enlargement of the optic nerve), and spongiform changes in the CNS (red arrows). Adult lesions may involve the orbital, intracanalicular, or prechiasmal portions of the optic nerve, resulting in enlargement and retrochiasmatic extension. The diagnosis may be made with a high level of confidence when the lesion involves the optic chiasm and retrochiasmatic optic pathway.

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DDx

Case 38: What is the most likely diagnosis?

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DDx: Optic Sheath Meningioma

Case 38: What is the most likely diagnosis?

1. Optic Neuritis/ Perineuritis 2. Neurosarcoidosis 3. Pseudotumor

Optic Sheath Meningioma Meningiomas present with a profile characteristic of optic neuropathy and rarely present in children. Optic sheath meningiomas are usually painless, with chronic visual loss and may be accompanied by proptosis. CT with contrast evaluate ONSMs well with thin slices and demonstrate a distinct, fusiform thickening of the optic nerve most commonly originating from the orbital apex. ONSMs usually enhance homogeneously with patchy calcifications. Meningiomas surround the optic nerve (red arrows) and the diameter of the nerve is attenuated within the surrounding mass. Exact knowledge of bony involvement in meningiomas is imperative because unresected meningiomatosis bone is one the important factors that contribute to recurrence.

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DDx

Case 39: What is the most likely diagnosis?

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Case 39: What is the most likely diagnosis?

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DDx: Optic Sheath Meningioma 1.

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Case 39: What is the most likely diagnosis?

Optic Neuritis Neurosarcoidosis Glioma Pseudotumor

Optic Sheath Meningioma Meningiomas present with a profile characteristic of optic neuropathy and rarely present in children. Optic sheath meningiomas are usually painless, with chronic visual loss and may be accompanied by proptosis. CT with contrast evaluate ONSMs well with thin slices and demonstrate a distinct, fusiform thickening of the optic nerve most commonly originating from the orbital apex. ONSMs usually enhance homogeneously with patchy calcifications. Meningiomas surround the optic nerve (red arrows) and the diameter of the nerve is attenuated within the surrounding mass. MRI allows visualization of meningiomas as localized or tubular enlargement with significant contrast enhancement.

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DDx

Case 40: What is the most likely diagnosis?

DDx: Preseptal Orbital Cellulitis 1. Edema- allergic, post trauma 2. MALT lymphoma 3. Mycosis fungoides

Preseptal Orbital Cellulitis Preseptal cellulitis is a common infection of the eyelid and periorbital soft tissues characterized by acute eyelid erythema and edema. Patients may present with temperature elevation, painful and limited eye movement, conjunctivitis, epiphoria, and blurred vision. A CT scan of the orbit is necessary for all cases of preseptal cellulitis. CT findings often include swelling of the eyelid and adjacent preseptal soft tissues (red arrows), obliteration of the fat planes, and absence of orbital inflammation. CT will also delineate the extent of orbital involvement. Preseptal cellulitis is a less severe disease than orbital cellulitis, and is usually caused by bacterial infection secondary to upper respiratory or external ocular infection, and is also caused by eyelid trauma.

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DDx