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Grand Rounds

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Grand Rounds Scleromalacia Amir R. Hajrasouliha, M.D. University of Louisville Department of Ophthalmology and Visual Sciences Friday, January 17, 2014 – PowerPoint PPT presentation

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Title: Grand Rounds


1
Grand Rounds
  • Scleromalacia
  • Amir R. Hajrasouliha, M.D.
  • University of Louisville
  • Department of Ophthalmology and Visual Sciences
  • Friday, January 17, 2014

2
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.

3
Patient Presentation
  • POH
  • AMD dry
  • PMH
  • Rheumatoid arthritis
  • H/o bladder cancer
  • Meds
  • AREDS
  • Plaquenil 400mg for 16 years (2,304g total)
  • Allergies
  • NKDA
  • SH
  • No tobacco, no ETOH
  • ROS
  • Negative (No joint pain and swelling now)

4
Exam
20/40
  • VA cc P
    TTP

  • EOM Full OU CVF FULL OU

(-) RAPD OU
20/25
5
Anterior exam
OD OS Ext wnl wnl L/L wnl wnl Conj
sup and temp wnl scleral
thinning K clear clear AC formed form
ed Iris wnl wnl Lens 2 NS 2NS
6
Photos
7
Assessment
  • 79 year old male with rheumatoid arthritis and
    scleral thinning without inflammation OD
  • Differential Diagnosis
  • scleromalacia

Plan
  • Referral to rheumatologist

8
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.

9
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.

10
Scleromalacia
  • A bulging staphyloma develops if intraocular
    pressure is elevated
  • Spontaneous perforation
  • is rare, although these eye
  • may rupture with minimal
  • Trauma.

11
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-Sep61(3)212-5.Infliximab
treatment in a case of rheumatoid scleromalacia
perforans.
12
MEDICAL TREATMENT
  • In patients with simple diffuse or nodular
    scleritis systemic non-steroidal
    anti-inflammatory 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

13
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.

14
  • Thank You
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