08/11/2020 Pseudotumor ‐ entity Pseudotumor cerebri Cerebral edema and raised Pseudo‐tumors (orbit, sinus intracranial pressure without focal cavernosus, meningeal) neurological signs Inflammatory pseudotumor A tumorlike mass resulting from an Jasmina Boban, MD, PhD inflammatory reaction University of Novi Sad, Department of Radiology Malta, November 2020. 12 Orbit Idiopathic orbital inflammation • „a window to systemic disease“ • Rate of onset? –acute ‐subacute NON‐INFECTIVE ORBITAL INFLAMMATION ‐chronic • Type of inflammation? • Anatomical location? SPECIFIC CAUSES IDIOPATHIC ORBITAL INFLAMMATION („pseudotumor“) 34 1 08/11/2020 Idiopathic orbital inflammation – type Idiopathic orbital inflammation – of inflammation anatomic location • Usually dense lymphocytic infiltrate (T‐cells, Extraconal fat Eyeball Globe plasma cells, neutrophils, eosinophils) w Zygomatic phlebitis bone Intraconal space • Rarely scelorosing inflammation – chronic Lateral rectus cases Intraconal fat Muscle cone Medial rectus Optic nerve • Granulomatous inflammation (hystiocytes, Extraconal space non‐caseating granulomas) Annulus of Zinn (Wichmann & Muller-Forell, Eur J Radiol. 2004) 56 Idiopathic orbital inflammation – Orbital Imaging ‐Techniques anatomic location ◉ Ultrasound: • Anterior ◉ CT • Periscleritis • good for lesions within globe or • Diffuse ◉ MRI foreign bodies in orbit • Perineuritis ◉ Ultrasound • poor penetration • Posterior (apical) • Focal mass ◉ Plain film ◉ Plain film: • Myositis ◉ Angiography • mainly for screening for metallic foreign bodies before MRI • Dacryoadenitis • useless for soft tissue details ◉ Angiography: • good for vascular malformations and vascularized tumors • invasive and time-consuming 78 2 08/11/2020 Orbital Imaging Orbital Imaging CT protocol MRI protocol 910 Orbital myositis • W>M, Y>O • Acute onset • Unilateral/bilateral painful eye movement • Medial, superior, lateral and inferior rectus – fusiform enlargement w CE w/wo tendon involvement • Responsive to oral steroids, recurrence often • Associated w systemic disease = autoimmune process, inflammatory/ infectious disease, Fat‐saturation improves ability to visualize paraneoplastic (breast, lung, seminoma) optic nerve due to retro‐orbital fat 11 12 3 08/11/2020 Dacryoadenitis • Most common (50% of all IOI) • Acute onset: painful, firm, erythematous mass w edema in the lateral upper eyelid • Unilateral (80%) • Enlargement of the lacrimal gland w ill‐ defined margins • Dif dg: lymphoma (DWI!) 13 14 Periscleritis • Rare • Usually acute/ subacute: orbital pain, exophtalmos, eyelid edema, vision decrease w/wo uveitis • Sclera, uvea, Tenon’s capsule • Sarcoidosis! • Heterogenous thickening of sclera and/or uvea,a ssociated w edema („ring sign“) 15 16 4 08/11/2020 Perineuritis • Perioptic neuritis • Rare • Acute/ subacute • Pain, swollen optic disc, arcuate7 pericentral visual field defects • Great responsiveness to steroids! • Circumferential enhancement around optic nerve sheath („doughnut sign“, „tram‐track sign“) 17 18 Doughnut sign Focal mass • Rare • Variable clinical presenatation • Subacute/ chronic • Symptoms dependent on anatomic location • Symptoms related to mass effect or inflammation: proptosis, motility disturbancw, optic nerve compression; eema, hyperemia 19 20 5 08/11/2020 Need for biopsy? Empirical treatment w steroids! Yesiltas et al. Middle east Afr J Ophtalmol 2018. 21 22 Graves orbitopathy (GO) Graves orbitopathy • Associated with Graves’ thyreotoxicosis BUT • Exophtalmos (distance from anterior surface up to 10% euthyroid (30‐50% of Graves’ of the globe to the interzygomatic line diseases pts develop clinical sings, while 60‐ >23mm) 90% have imaging signs of orbitopathy) • Extraocular muscle enlargement w/wo fatty • Main cause of imaging presentation of orbital infitlration (I’M SLOw), bilateral and myositis (>50%) symmetrical • Increase in retrobulbar fat component 23 24 6 08/11/2020 GO Inflammatory bowel disease Coca‐Cola bottle sign • Multiple extraocular muslces involved bilaterally • Sometimes precedes IBD • Often recurrent • Resistant to steroids? 25 26 IgG4‐related disease • Second to GO • Dense lymphoplasmatic infiltrate with fibrosis (w/o obliterative phlebitis) • Combined diagnostic criteria: clinical features, serum IgG4 levels (over 40% ratio to IgG+ plasma cells) • Lacrimal gland – sclerosing dacryoadenitis • Extraocular muscle disease w trigeminal nerve enlargement • Paranasal sinuses • Fibrosing inflammation of retrobulbar fat tissue 27 28 7 08/11/2020 Tolosa‐Hunt syndrome Tolosa‐Hunt syndrome • No age or sex predilection; usually unilateral but • Pathological process located in the cavernous ocasionally bilateral symultaneous sinus/ superior orbital fissure • Self‐limiting condition • Initial study: CE MRI • Acute in onset, clinical course unpredictable • Soft‐tissue component involving the cavernous sinus on the painful side • PAIN – main symptom (periorbital) • High‐resolution CT less sensitive • Ocular motor cranial nerve palsies may coincide • Differential: lymphoma, meningioma, sarcoidosis • Optic nerve involvement not common (lack of specificity!) • Follow up studies after corticosteriod treatment – diagnosis of exclusion! 29 30 Tolosa‐Hunt syndrome • Non‐specific, chronic inflammation with proliferation of fibroblasts and infiltration of the wall and septa of cavernous sinus with lymphocytes and plasma cells • Granulomatous inflammation with epitheloid cells and giant cells • Necrosis rare but possible • Etiology unknown – idiopathic orbital inflammation 31 32 8 08/11/2020 Idiopathic intracranial hypertension Idiopathic intracranial hypertension • Headache (90%), vision loss (86%), pulsatile • Dandy criteria: papilledema, elevated opening tinnitus, transient visual obscurations, diplopia pressure normal neurological examination (except VI nerve palsy), normal neuroimaging • Obese women in chilbearing age • Revised criteria: normal opening pressure in • Umbrella term pseudotumor cerebri children, MRI suggestive of increased ICP syndrome: PRIMARY (IIH) and SECONDARY • IIH without papilledema: elevated ICP, VI nerve (identifiable cause: cerebral venous disorder, palsy, 3 of MRI findings suggestive of elevated ICP medications, clinical conditions) (empty sella, posterior globe flattening, perioptic subarachnoid space dilatation and transverse sinus narrowing) 33 34 Idiopathic hypertrophic meningitis • Dural thickening and enhancement • Detailed clinical exploration usually reveals the etiology: inflammatory (neurosarcoidosis, Wegener granulomatosis, Sjogren’s syndrome, rheumatoid arthritis, IgG4‐related disease), infectious (TB, syphilis, Lyme disease), neoplastic (dural metastasis, en plaque meningioma), iatrogenic‐ posttraumatic (intracranial hypotension) • If no underlying disease is identified – idiopathic (over 50% of „idiopathic“ in fact IgG4‐related!!!)! 35 36 9 08/11/2020 Clinical features IgG4‐related meningitis • Headache • Most commonly pachymeningitis • Loss of cranial • Focal (due to neural and vascular compression) or nerve function diffuse (headache, neck stiffness, seizures) (idiopathic, symptoms neurosarcoidosis) • Rarely: seizures, • Diagnosis: serology (elevated IgG4 serum level in ataxia, sensory loss 90% pts), CSF (lymphocytic pleocytosis ‐ exclusion of other causes), pathology (storiform fibrosis, dense lymphocytic infiltrate, obliterative phlebitis) • Radiology: linear dural thickening/ bulky mass 37 38 IgG4‐related leptomeningits Boban J, Ardali S, Thurnher MM. Neuroradiology 2018 . 39 40 10 08/11/2020 Post‐contrast SPACE and FLAIR Imaging recommendations • 3D‐T1 SPACE superior for dural and sulcal • T1‐mVISTA („black‐blood imaging“) increases abnormalities the sensivity for detection of infectious or • 3D‐FLAIR superior for basal cisterns neoplastic meningitis, compared to 3D –T1‐ • CE 3D‐T1‐SPACE and CE 3D‐FLAIR superior for MPRAGE evaluation of meningeal abnormalities (NPV • Earlier diagnostic clue! ca. 100%), than 3D‐T1‐MPRAGE Sommer NN, Pons Lucas R, Coppenrath E, er al. Eur Radiol 2020. Jeevanandham B, Kalyanpur T, Gupta P, Cherian M. Br J Radiol 2017. 41 42 Thank you for attention! [email protected] 43 11.