Kanski signs - orbit Flashcards
Axial proptosis 2/2 what type of lesions?
Axial proptosis 2/2 lesions WITHIN the muscle cone (i.e. cavernous hemangioma*, ON tumors, TED)
*cavernous hemangioma: MC in 4th-5th decade with slowly progressive proptosis, Rx: excision
Eccentric proptosis 2/2 what type of lesions?
Eccentric proptosis 2/2 Extraconal lesions in which direction of proptosis 2/2 site of lesion
Causes of pseudo-proptosis
Ipsi/L large globe (buphthalmos or very high myopia)
Ipis/L lid retraction
Contra/L enophthalmos
Acute orbital myositis: pain/diplopia increased on which attempted gaze?
Increased pain/diplopia on attempted gaze INTO the field of the affected muscle
lid edema/ptosis/chemosis
CT: w/w/o tendon insertion involvement
involvement can last a few weeks
Rx: steroids
Bilateral orbital inflammation systemic dz DDx
Wegener granulomatosis (bilateral orbtial involvement 2/2 contiguous spread from the paranasal sinus/nasophraynx; signs = congestive proptosis/ophthalmoloplegia, dacryoadenitis and NLDO)
Polyarteritis nodosa
Sarcoidosis
TB
Waldenstrom macroglobulinemia*
*a rare clinicopathologic entity demonstrating 10 percent or greater infiltration of the bone marrow by clonal lymphoplasmacytic cells and a monoclonal IgM gammopathy in the blood. Patients usually present in their seventh decade with symptoms related to the infiltration of the hematopoietic tissues or the effects of monoclonal IgM in the blood.
Lymphangioma MC located in which orbit? Age range? What else to look for?
nonfx vascular malformation]
1-15 yo
MC located in the superior orbit (nasal)
slowly progressive proptosis but can have sudden painful proptossi 2/2 spontaneous hemorrhage (chocolate cyst = encysted blood form)
MC to have visual compromise and EOM impairment (vs capillary hemangioma)
Look for involvement of sinus and oropharynx
Rx: drain, CO2 laser to vaporize (??)
Rhabdomyosarcoma MC location? What does it look like on imaging?
MC location = retrobulbar, followed by superior and inferior
CT = moderately well-defined homogenous mass that is isodense with EOM.
Rhabdomyosarcoma is what type of tumor? Demographic?
aggressive tumor derived from undifferentiated mesenchymal cell
typically affects boys ~ 7 yo
Myeloblastoma (Granulocytic granuloma) - what is it? signs?
localized form of AML that has a predilection for the orbit, may occasionally precede systemic manifestations
Signs: rapid onset of proptosis, sometimes bilateral. eyelid ecchymosis and edema.
Anterior orbital encephalocele - pathogenesis and signs
pathogenesis: congenital fronto-ethmoidal orbital defect
signs: proptosis - slowly progressive, pulsatile.
Increases in size on crying/straining.
Assoc/w/lateral displacement of the globe
Posterior orbital encephalocele - pathogenesis and signs
pathogenesis: dysplasia of the sphenoid bone that may be associated with NF1
Signs: similar to the anterior variety except that the globe is displaced downwards
Causes of rapid proptosis in children - nonmalignant
orbital cellulitis
ruptured deep dermoid cyst
chocolate cyst with lymphangioma
idiopathic orbital inflammatory disease
Causes of rapid proptosis in children - malignant
rhabomyosarcoma
acute leukemia
metastases (like neuroblastoma - bilateral in 40% of cases)
Langerhans-cell histocytosis (bilateral or u/L bony lysis and soft tissue proliferation which typically involves the superotemporal orbit)
Carotid-cavernous fistula - cause, signs, look for
Cause: head trauma/spontaneous rupture of intracavernous aneurysm leads to high flow shunt in which carotid artery passes directly into the cavernous sinus through defect in internal carotid artery
signs: unilateral, painful, pulsatile proptosis assoc/w/bruit and severe chemosis
Look for:
-grossly dilated epibulbar vessels, may be present in absence of chemosis
-ophthalmoplegia
-retinopathy (ON swelling, venous dilation, intraretinal hemorrhages)
-pulsatile proptosis assoc/w/ bruit, abolished by carotid compression
Triad: pulsatile protposis, conjunctival chemosis, whoosing noise in the head
Rx: interventional radiology - occulde the fistula
cavernous sinus thrombosis - cause/signs
cause: skin/paranasal sinus infection
signs: similar to c-c fistula except that the patient is usually more ill 2/2 systemic infection
Inflammatory orbital disease in children
preseptal/orbital cellulitis
pseudotumor (orbit)
TED
Benign tumors in children
capillary hemangioma
lymphangioma
plexiform neurofibroma (non-axial proptosis, may see pulsatile proptosis if assoc/defect in sphenoid bone)
Malignant tumors in children
Rhabdomyosarcoma
Optic glioma*
Mtz neuroblastoma
myelobastoma
*Optic nerve gliomas that are confined to the orbit can be observed, particularly if vision is good. Radiation is used if the tumor is unresectable because of chiasm or tract involvement and symptoms are progressing. Chemotherapy is effective in progressive chiasmal or hypothalamic gliomas and may avoid complications of radiation. Surgery may be used for progressive lesions expanding toward the chiasm.
Congestive proptosis in adults
TED
C-c fistula
cavernous sinus thrombosis
mtz tumor (breast carcinoma)
Chronic non-congestive axial proptosis
TED
cavernous hemangioma
optic nerve sheath meningioma
orbital varices
Chronic non-congestive non-axial proptosis: 2/2 anterior orbital disorders which displace the globe AWAY from the lesion
lymphoid tumors
sphenoid ridge meningioma
mucocele
Lymphoid tumors
usually present in old age, may involve any part of one or both orbits.
sphenoidal ridge meningioma - presentation/signs
presentation - in middle age with slowly progressive, painless proptosis
signs - lateral globe displacement and periocular swelling
fullness of the temporal fossa
hyperostosis (excessive bone growth)
sinus mucocele - frontal
mucoceles - combo of ptosis, proptosis, globe displacement
Proptosis - may fluctuate when the walls of the mucocele become inflamed
Frontal - displaces globe downward and may cause impairment of upgaze. CT shows bony dehiscence in the orbital roof.
sinus mucocele - ethmoidal
mucoceles - combo of ptosis, proptosis, globe displacement
Proptosis - may fluctuate when the walls of the mucocele become inflamed
Ethmoidal - causes lateral displacement which my be assoc/w/proptosis. CT shows loss of ethmoidal septae and erosion into the orbit.
Dynamic proptosis - intermittent
orbital varices (Valsalva) = weakened segments of the orbital venous system which enlarged with increased venous maneuver. Present early childhood to late middle-age. Nonpulsatile/no bruit. can see atrophy of the surrounding fat and enophthalmos
mucocele (inflammed walls)
capillary hemangioma (crying)
lymphangioma (URI)
Pulsatile proptosis (dynamic) - assoc/w/ or not/assoc/w/bruit
assoc/w/bruit 2/2 arterial pulsation (c-c fistula or AV communication like Wyburn-Mason)
not assoc/w/bruit: 2/2 transmitted CSF pulsation. may be 2/2 encephalocele* in which pulsation transmitted ot the orbit through a defect in the sphenoid bone or an orbital roof fracture.
*encephalocele = herniation of intracranial contents into orbit through a congenital skull defect (usually presents in infancy)
Anterior encealocele = forward/lateral displacement
Posterior encephalocele = forward/downward displacement
cyst can enlarge in size on straining/crying, reduced by manual pressure
Dacryoadenitis - infectious or inflam
Upper lid = shows an āSā shaped curve with erythema of the skin and globe displaced down and in
lacrimal secretion may be reduced
Gland feels gritty and there is injection of the gland and adjacent conjunctiva (acute discomfort over region)
CT = shows opacification that extends beyond the gland itself
Rx: usually not required 2/2 spontaneous resolution
Pleomorphic adenoma = presentation/signs
Presentation = middle age (20s-50s) with long PAINLESS history
Signs = nontender firm fullness of the upper eyelid and globe displacement down and in
Tumor of palpebral lobe may be visible to inspection and does not displace the globe
CT = shows oval mass with smooth outline that may indent the globe and lacrimal fossa
Lacrimal gland carcinoma (Adenoid cystic carcinoma) = presentation/signs
presentation = 40s-50s, shorter history than an adenoma and is associated with pain +/- hyposthesia in the region supplied by lacrimal nerve
CT = shows globular mass with IRREGULAR SERRATED BORDER, often with contiguous erosion/invasion of bone and occasionally spotty calcification
dacryops
epithelial cyst of the lacrimal gland (2/2 obstruction and dilation of major lacrimal ducts)
On lid eversion - superior fornix - bluish dome-shaped lesion is present (orginates from palpebral portion of lacrimal gland)
Rx: aspiration
B/L lacrimal gland enalrgement
physiologic enlargement - assoc/w/shallow orbits
late TED
sarcoidosis = bilateral firm enlarged lacrimal glands
lymphomas = typically cause a firm or rubbery enlargement which may be u/L or b/L
sjogren syndrome: bilateral lacrimal gland enlargement assoc/w/dry eyes and xerostomia
Mikulicz syndrome = lacrimal and salivary gland hypertrophy assoc/w/dry eyes and xerostomia (+/-
orbital rim lesions
superficial dermoid cyst (superotemporal usually, well-circumscribed, heterogenous on CT)
subperiosteal hemotoma
subperiosteal abscess
Orbital cellulitis (bacteria) - MCC from which sinus? Rx?
Usually ethmoidal
Rx: Vancomycin + one of the following:
- Ceftriaxone or
- Cefotaxime or
- Ampicillin-sulbactam (unasyn) or
- Piperacillin-tazobactam (zosyn)
Fungal orbital cellulitis complications
retinal vascular occlusion
cranial nerve palsies
cerebrovascular occlusion
pseudotumor (idiopathic orbital inflammatory disease) = definition/presentation/CT/course
definition: may involve any or all of orbital soft tissue
presentation: 3rd-6th decade with usually u/L acute periorbital redness, swelling and pain (congestive protpsosis)
CT: ill-defined orbital opacification and loss of definition of contents
Course: spontaneous remission s/p few weeks, prolonged intermittent activity with eventual remission
severe prolonged activity resulting in fibrosis of orbital tissues (frozen orbit)
pseudotumor (idiopathic orbital inflammatory disease) Rx
NSAIDS/Oral therapy
radiotherapy = if no improvement s/p 2 weeks of steroids
antimetabolites or systemic infilixmab = if resistant to steroids and radiotherapy
Tolosa-Hunt syndrome -
Pathogenesis: nonspecific granulomatous inflammation of the cavernous sinus, superior orbital fissure, and/or orbital apex
Presentation - diplopia with ipsilateral periorbital or hemicranial pain
Signs: ocular motor nerve palsies often with pupil involvement (Cardinal features include retroorbital pain and ophthalmoplegia affecting the third, fourth, and/or sixth cranial nerves. All age groups may be affected.)
sensory loss along the distribution of the V1 and V2
Rx: systemic steroids
Indirect c-c fistula (dural shunt)
definition = slow-flow shunt in which arterial blood flows throught he meningeal branches of the external/internal carotid artery INDIRECTLY into the cavernous sinus
Cause: congenital malformation, spontaneous rupture which may be 2/2 minor trauma
signs: dilated epibulbar vessels,
exaggerated ocular pulsation on applanation tonometry
elevation of IOP
mild ptoptosis occasionally asosoc/w/ soft bruit
ophthalmoloplegia 2/2 CN6 palsy or swelling of EOM
fundus - normal/moderate venous dilation
Rx: interventional radiology
Orbital invasion by sinus tumors
maxillary carcinoma
ethmoidal carcinoma
nasophrayngeal carcinoma (through inferior orbital fissure)
