Grand Rounds
Scleromalacia
Amir R. Hajrasouliha, M.D.
University of Louisville
Department of Ophthalmology and Visual Sciences
Friday, January 17, 2014
Patient Presentation

HPI: 79 y/o white male present for annual
work up. He has a h/o dry AMD with no new
complaint. He has no pain or discomfort.
Patient Presentation

POH:






AMD dry
PMH:


Rheumatoid arthritis
H/o bladder cancer


AREDS
Plaquenil 400mg for 16 years (2,304g total)

NKDA

No tobacco, no ETOH

Negative (No joint pain and swelling now)
Meds:
Allergies:
SH:
ROS:
Exam
VA cc
20/40
20/25
EOM: Full OU
P
32mm
(-) RAPD OU
32mm
CVF: FULL OU
TTP
13
12
Anterior exam
Ext
L/L
Conj
K
AC
Iris
Lens
OD
OS
wnl
wnl
sup and temp
scleral thinning
clear
formed
wnl
2+ NS
wnl
wnl
wnl
clear
formed
wnl
2+NS
Photos
Assessment
79 year old male with rheumatoid arthritis and scleral
thinning without inflammation OD

•Differential Diagnosis
•scleromalacia
Plan

Referral to rheumatologist
Scleromalacia


Also known as necrotizing scleritis without
inflammation.
It is clinically distinct from other forms of
anterior scleritis in which typical signs (redness,
edema) and symptoms (pain) of inflammation
are not apparent.
Scleromalacia


Typically occurs in patient with long standing
rheumatoid arthritis.
It has been also reported to have association
with Wegener's granulomatosis, SLE, JRA,
PAN, Relapsing Polychondritis, psoriasis, gout,
TB, syphilis, HSV, HZV.
Scleromalacia
A bulging staphyloma develops if intraocular
pressure is elevated;
 Spontaneous perforation
is rare, although these eye
may rupture with minimal
Trauma.

Case report


A clinical case of scleromalacia perforans in a 56-year-old woman with 20 years of
seropositive rheumatoid arthritis. She developed rapidly progressed to scleromalacia
perforans OS and became perforated. It was surgically enucleated, and the patient was
maintained with steroidal therapy.
2 months later she developed new-onset scleromalacia OD. She was first evaluated by
a rheumatologist and treated with 200 mg/dose of infliximab, which was administered
monthly for the following four months. The biological treatment was accompanied by
methotrexate and prednisone. With this therapy, the ocular lesion dramatically
improved, and complete remission of rheumatoid arthritis and scleritis was archived
four months later. In conclusion, tumur necrosis factor (TNF) blockers are effective
therapeutic agents in ocular complications of rheumatoid arthritis.
Reumatismo. 2009 Jul-Sep;61(3):212-5.Infliximab treatment in a case of
rheumatoid scleromalacia perforans.
MEDICAL TREATMENT


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In patients with simple diffuse or nodular scleritis systemic non-steroidal antiinflammatory drug therapy is almost invariably effective
For unresponsive cases and posterior scleritis, the mainstay of treatment is
systemic steroids in a dose of 1 mg/kg/day. As soon as the patient responds,
the dose should be tapered once 20 mg/day is reached, alternate day therapy
can be started. Topical steroids can be applied for symptom relief.
Immunosuppressive therapy is mandatory for definitively diagnosed systemic
vasculitic disease and/or progressive destructive ocular lesions. If the
necrotizing scleritis is not severe, not rapidly progressing, the first choice of
therapy is methotrexate 7.5 mg once a week as a starting dose
Scleromalacia

Extreme corneal thinning or perforation
requires reinforcement. Donor sclera, fascia lata,
periosteum or artificial materials can be used. To
maintain its integrity the material must be
covered by conjunctiva.
Thank You