Kanski signs - orbit

Question 1

Axial proptosis 2/2 what type of lesions?

Answer 1

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

Question 2

Eccentric proptosis 2/2 what type of lesions?

Answer 2

Eccentric proptosis 2/2 Extraconal lesions in which direction of proptosis 2/2 site of lesion

Question 3

Causes of pseudo-proptosis

Answer 3

Ipsi/L large globe (buphthalmos or very high myopia)
Ipis/L lid retraction
Contra/L enophthalmos

Question 4

Acute orbital myositis: pain/diplopia increased on which attempted gaze?

Answer 4

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

Question 5

Bilateral orbital inflammation systemic dz DDx

Answer 5

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.

Question 6

Lymphangioma MC located in which orbit? Age range? What else to look for?

Answer 6

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 (??)

Question 7

Rhabdomyosarcoma MC location? What does it look like on imaging?

Answer 7

MC location = retrobulbar, followed by superior and inferior

CT = moderately well-defined homogenous mass that is isodense with EOM.

Question 8

Rhabdomyosarcoma is what type of tumor? Demographic?

Answer 8

aggressive tumor derived from undifferentiated mesenchymal cell
typically affects boys ~ 7 yo

Question 9

Myeloblastoma (Granulocytic granuloma) - what is it? signs?

Answer 9

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.

Question 10

Anterior orbital encephalocele - pathogenesis and signs

Answer 10

pathogenesis: congenital fronto-ethmoidal orbital defect
signs: proptosis - slowly progressive, pulsatile.
Increases in size on crying/straining.
Assoc/w/lateral displacement of the globe

Question 11

Posterior orbital encephalocele - pathogenesis and signs

Answer 11

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

Question 12

Causes of rapid proptosis in children - nonmalignant

Answer 12

orbital cellulitis
ruptured deep dermoid cyst
chocolate cyst with lymphangioma
idiopathic orbital inflammatory disease

Question 13

Causes of rapid proptosis in children - malignant

Answer 13

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)

Question 14

Carotid-cavernous fistula - cause, signs, look for

Answer 14

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

Question 15

cavernous sinus thrombosis - cause/signs

Answer 15

cause: skin/paranasal sinus infection
signs: similar to c-c fistula except that the patient is usually more ill 2/2 systemic infection

Question 16

Inflammatory orbital disease in children

Answer 16

preseptal/orbital cellulitis
pseudotumor (orbit)
TED

Question 17

Benign tumors in children

Answer 17

capillary hemangioma
lymphangioma
plexiform neurofibroma (non-axial proptosis, may see pulsatile proptosis if assoc/defect in sphenoid bone)

Question 18

Malignant tumors in children

Answer 18

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.

Question 19

Congestive proptosis in adults

Answer 19

TED
C-c fistula
cavernous sinus thrombosis
mtz tumor (breast carcinoma)

Question 20

Chronic non-congestive axial proptosis

Answer 20

TED
cavernous hemangioma
optic nerve sheath meningioma
orbital varices

Question 21

Chronic non-congestive non-axial proptosis: 2/2 anterior orbital disorders which displace the globe AWAY from the lesion

Answer 21

lymphoid tumors
sphenoid ridge meningioma
mucocele

Question 22

Lymphoid tumors

Answer 22

usually present in old age, may involve any part of one or both orbits.

Question 23

sphenoidal ridge meningioma - presentation/signs

Answer 23

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)

Question 24

sinus mucocele - frontal

Answer 24

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.

Question 25

sinus mucocele - ethmoidal

Answer 25

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.

Question 26

Dynamic proptosis - intermittent

Answer 26

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)

Question 27

Pulsatile proptosis (dynamic) - assoc/w/ or not/assoc/w/bruit

Answer 27

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

Question 28

Dacryoadenitis - infectious or inflam

Answer 28

Upper lid = shows an “S” 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

Question 29

Pleomorphic adenoma = presentation/signs

Answer 29

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

Question 30

Lacrimal gland carcinoma (Adenoid cystic carcinoma) = presentation/signs

Answer 30

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

Question 31

dacryops

Answer 31

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

Question 32

B/L lacrimal gland enalrgement

Answer 32

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 (+/-

Question 33

orbital rim lesions

Answer 33

superficial dermoid cyst (superotemporal usually, well-circumscribed, heterogenous on CT)
subperiosteal hemotoma
subperiosteal abscess

Question 34

Orbital cellulitis (bacteria) - MCC from which sinus? Rx?

Answer 34

Usually ethmoidal
Rx: Vancomycin + one of the following:

• Ceftriaxone or
• Cefotaxime or
• Ampicillin-sulbactam (unasyn) or
• Piperacillin-tazobactam (zosyn)

Question 35

Fungal orbital cellulitis complications

Answer 35

retinal vascular occlusion
cranial nerve palsies
cerebrovascular occlusion

Question 36

pseudotumor (idiopathic orbital inflammatory disease) = definition/presentation/CT/course

Answer 36

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)

Question 37

pseudotumor (idiopathic orbital inflammatory disease) Rx

Answer 37

NSAIDS/Oral therapy
radiotherapy = if no improvement s/p 2 weeks of steroids
antimetabolites or systemic infilixmab = if resistant to steroids and radiotherapy

Question 38

Tolosa-Hunt syndrome -

Answer 38

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

Question 39

Indirect c-c fistula (dural shunt)

Answer 39

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

Question 40

Orbital invasion by sinus tumors

Answer 40

maxillary carcinoma
ethmoidal carcinoma
nasophrayngeal carcinoma (through inferior orbital fissure)