Transcript Folie 1 - Springer Static Content Server

Posterior Scleritis
associated with
Orbital Pseudotumor
Nikolas London, MD
Retina Consultants San Diego
Ocular History
 34-year-old man with 2 months of headache, progressive
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proptosis, pain, redness, and decreased vision in his right eye
HPI: Pred Forte and scopolamine for NGAU x 4 weeks
POHx: none
PMH:
Mitral valve prolapse
Mental illness: self-described “not right in head”
Jaw surgery 1994
ALL: mushrooms, mayonnaise, anabolic steroids
SH: NC
ROS: pan-negative
First Presentation
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VA: bare CF OD, 20/25 OS
Pupil: + RAPD OS by reverse
IOP: 15 OU
Hertel: 5mm proptosis OD
SLE OD: 2+ conjunctival injection, 1+ AC and
anterior vitreous cell
First Presentation
 Funduscopy
 large amelanonic mass superior to the
optic nerve head causing
 retinal folds and
 obscuration of the optic nerve head.
First Presentation
 Fluoresceineangiography
 Early widefield angiogram of the right eye
 retinal distortion and folds.
 later frames: progressive stippled
hyperfluorescence of the mass
 prominent leakage from the optic nerve head
First Presentation
 Fluoresceineangiography
 Late frame widefield angiogram of the right
eye
 leakage from the mass and optic nerve head
 inferior peripheral nonperfusion and
adjacent vascular leakage.
First Presentation
 US
 vertical axial B-scan ultrasound
 thickening of the posterior wall complex
with sub-Tenon’s fluid (T-sign)
 shallow inferior retinal detachment
First Presentation
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periorbital edema
erythema
mild exotropia and
proptosis.
First Presentation
 Imaging
of the right orbit
 2.7 x 1.8 x 3.3 cm soft tissue mass
 involving the sclera with deformation
of the posterior globe.
 Pseudotumor orbitae (?)
Diagnosis
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Posterior scleritis
Associated to pseudotumor orbitae
workup for infectious and inflammatory etiologies
sent to Oculoplastics for evaluation to consider
biopsy and rule out lymphoma.
 biopsy was refused because quite risky
Laboratory Data
Quantiferon gold
FTA-ABS
RPR
ACE
C-ANCA
P-ANCA
X-ANCA
ANA
CXR
ESR
CRP
Chem-7
CBCD
Hgb/Hct
negative
NR
NR
21
negative
negative
negative
negative
wnl
25
1.10
wnl
mild anemia
12/36
Treatment and Follow-Up
 after infectious etiologies were ruled out he was
started on prednisone (60mg/day, 2 weeks)
 then reduction by 20 mg/week for 3 weeks,
staying on 10 mg/day for several weeks
 rapid improvement of his symptoms and
examination in between 1 week
 dramatic reduction in periorbital edema,
erythema, propsosis, head tilt, and exotropia
Follow-Up
1 week after begin of treatment
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dramatic reduction in size of the subretinal mass
residual RPE changes
mild horizontal retinal striae in the superior macula.
SD OCT: mild inner retinal distortion and subretinal
fluid.
Imaging: substantially smaller scleral mass with less
distortion of the posterior wall of the globe.
Final Diagnosis
 Posterior scleritis
 associated with idiopathic orbital
pseudotumor
 rapid resolution with oral
corticosteroids
Conclusion
 Posterior scleritis is a rare manifestation
of orbital pseudotumor
 Other diagnoses, including tuberculosis,
lymphoma, systemic lupus
erythematosus, syphilis, and sarcoidosis
should be considered