Download An Orbital Tumor - University of Louisville Ophthalmology

Grand Rounds Conference
Tala Kassm DO
June 3rd, 2016
University of Louisville
Department of Ophthalmology and Visual Sciences
Subjective

CC: “My right eyelid has been swollen for a few
weeks.”

HPI: 55 year old African American woman presents
to ER with progressively worsening right upper
eyelid swelling. Initially thought it was a bug bite
that wouldn’t get better. No history of trauma or
recent surgery. Denies vision loss, discharge, eye
pain, watering or diplopia.

Review of Systems: negative for fever, weight loss,
headaches, night sweats.
History
POH: none
PMH: hypertension
Family Hx: noncontributory
Meds: hydrochlorothiazide
Allergies: NKDA
Clinical Exam
VA(cc, N):
OD
20/25
OS
20/25
+2.50 OU
Pupils:
IOP:
EOM:
4->2 no rAPD 4->2
17
16
-3
Full
-1
-2
-1
CVF:
Full
Full
Clinical Exam
PLE:
External/Lids
Cornea
Ant Chamber
Iris
Lens
OD
OS: unremarkable
Ptosis
Hypotropic and proptotic eye
Firm mass superonasal orbit, nontender
Areas of subconj heme temporally, chemosis
no salmon patch
clear
formed
WNL
1-2+ NS
DFE:
Unremarkable OU
Conj/Sclera
Clinical photo
Clinical
Photos
5 days
later
CT Scan
MRI
T1
MRI – T2
Lab results

CBC, CMP and TSH all within normal limits
Assessment

55 year old African American woman with 3
weeks of progressive, painless eyelid swelling,
proptosis and diplopia.

Differential includes: lymphoma, orbital
metastasis, sarcoid
Plan


Started on Prednisone 40 mg
Right anterior orbitotomy for diagnostic biopsy
of orbital lesion
Follow Up

1 week post op


Persistent chemosis and restriction of EOM’s
Pathology report:
Non necrotizing granulomatous inflammation
 GMS and AFB stains negative for fungal and
mycobacterial microorganisms.
 “In the absence of infection as would be confirmed
by culture studies, the findings are consistent with
sarcoidosis.”

Plan




Increase Prednisone to 60 mg daily
Refer to Rheumatology
Angiotensin-converting enzyme ordered as well
as chest CT, both of which are pending
Patient has missed two follow up appointments
Sarcoidosis





Defined as multisystem granulomatous disease
of unknown origin
Most common manifestations are intrathoracic
(90%)
Other sites include lymph nodes, skin, eyes,
CNS, bones and joints, liver and heart
Worldwide distribution
In the United States, it is 20 times more
prevalent in African Americans than whites
Sarcoidosis

Histology reveals a noncaseating granuloma of
epithelioid histiocytes
Without evidence of infection or foreign body
 Minimal cuff of lymphocytes and plasma cells

Pathogenesis is unclear
 No single etiologic
agent or genetic locus has
been clearly identified

Ocular Involvement



Occurs in roughly 50% of patients with systemic
sarcoidosis
Any part of the eye may be involved – orbit and its
adnexa, cutaneous, lacrimal, intraocular, neurologic
Uveitis is the most frequent


Of all ocular involvement, anterior uveitis makes up
two thirds
Within the orbit, the lacrimal gland is most
frequently affected, inflammation is typically
bilateral
Diagnosis of Sarcoid

Gold standard – tissue biopsy
Lung, lymph node, skin, conjunctiva,
lacrimal gland, orbital tissue



Imaging – chest X-ray or CT scan
Gallium Scintigraphy


Angiotensin-converting enzyme


Not specific
Elevated in 52 to 90% of cases
Lysozyme
Treatment


Corticosteroids remain the mainstay of
treatment
Other immunomodulators may be used in
patients who are intolerant or unresponsive to
corticosteroid treatment, or adjunctive therapy
The






Multicenter retrospective study including patients with
biopsy-proven noncaseating granuloma involving the
orbit or adnexa as well as evidence of systemic
sarcoidosis
26 patients total, 19 female, 7 male, mean age of 52 years
Lacrimal gland involvement in 11 patients
Extralacrimal orbit involvement in 10 patients
Eyelid in three and lacrimal sac in two patients
Most common complaint was slowly progressing mass
The


Orbital involvement is more common in the fifth to
seventh decades and more frequent in women.
Occurs in two forms – diffuse and discrete



Diffuse involvement more commonly occurred in patients
with active systemic sarcoidosis
Discrete lesions had a predilection for the anterior and
inferior quadrants of the orbit
Orbital disease tends to respond well to systemic
steroids

Methotrexate was second line
References









BCSC: Orbit, Eyelids and Lacrimal system. 63
Thomas KW, Hunninghake GW. Sarcoidosis. JAMA. 2003;289(24):3300-3303.
BCSC: Intraocular Inflammation and Uveitis. 166-171.
Baughman RP, Lower EE. Ingledue R. Kaufman AH. Management of ocular
sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2012;29(1):26-33.
Chen ES, Moller DR. Etiology of sarcoidosis. Clin Chest Med. 2008;29(3):365-377.
BCSC: Ophthalmic Pathology and Intraocular Tumors. 52,212.
Dana MR, Merayo-Lloves J, Schaumberg DA, Foster CS. Prognosticators for visual
outcome in sarcoid uveitis. Ophthalmology. 1996; 103(11):1846-1853.
BCSC: Neuro-Ophthalmology. 315-316
Herbort CP, Rao NA, Mochizuku M; members of Scientific Committee of First
International Workshop on Ocular Sarcoidosis. International criteria for the diagnosis
of ocular sarcoidosis: results of the first International Workshop on Ocular Sarcoidosis
(IWOS). Ocul Immunol Inflamm. 2009;17(3):160-169.
Thank you for listening
Ocular Manifestations

Clinical findings suggestive of sarcoidosis
Nodular infiltration of the angle
 Nodular iridocyclitis, Keoppe nodules, and large
mutton-fat keratic precipitates
 Vitreous string of pearls and snowball opacities
 Chorioretinal granulomas
 Retinal perivasculitis
 Candlewax drippings and yellow ‘waxy’ spots
