Suha M.Ajeilat, MD.
Reham I. Shaban,MD.
Ayman S. Madanat,DO,FRCS,FRCO phth.
Scleromalacia means sclero-malakia
(Thinning), scleromalacia occurs as a complication
of Herpes Zoster Ophthalmicus, Rheumatoid
arthritis, Vogt Koyanagi Harada syndrome
and following retinal detachment surgery
but it never has been reported as a complication
of Marfan's syndrome.
49 year old female patient presented to
the emergency room with bilateral anterior
chamber dislocated lenses with high intraocular
pressure. She was medically treated for
the high intraocular pressure, then she
underwent left pars plana lensectomy and
vitrectomy, during surgery it was noted
that she had blue discoloration of the sclera
in her left eye. She was lost to follow
up to present eight months later with a
protruding mass in her right eye near the
limbus with deterioration of visual aquity,
normal intraocular pressure. She was diagnosed
as a case of scleromalacia perforans. History
and general examination revealed family
history of Marfan's syndrome (one brother),
the patient was tall and thin, long limbs,
fingers and hands are long, slender and
have spider like appearance, high arched
With this patient we were faced with scleromalacia
without any history of any known cause other
than association with Marfan's syndrome.
49 year old female patient
presented to the emergency department at
King Hussein Medical Center with severe
pain in both eyes. Examination revealed
visual acuity of 6/36 in the right eye,
6/60 in the left eye, dislocated lenses
in the anterior chamber in both eyes, intra
ocular pressure 45 mm Hg in each eye with
no corneal edema.The patient was managed
medicalyto decrease intraocular pressure,
followed by lensectomy_vitrectomy for the
left eye. During surgery the sclera was
found to be very thin. The patient was given
appointment for surgery to the right eye,but
the patient was lost for follow-up. She
presented eight months later with a black
mass in the right eye with deterioration
of visual acuity, she gave no history of
recent pain nor inflammation of the eye.
On examination: Right eye, a well defined
superonasal dark coloured bulge of the sclera
encroaching on the limbus was found. It
could be brightly transilluminated. The
visual acuity unaided was counting fingers
4 meters improving to 6/24 with -1.00,+5.00x180°
(irregular retinoscopy reflex). Intra ocular
pressure was 10 mm Hg. Supero_temporal sublaxated
lens. Cup_disc ratio was 0.3 with no evidence
of any retinal nor choroidal pathology.
Left eye was aphakic with dark discoloration
of the sclera superiorly, visual acuity
unaided was 6/60 improving to 6/12 with
+10, posterior segment was normal and visual
field was not informative. Coloured photography
was taken to document the extent of the
General examination revealed tall thin patient
with long limbs and high arched palate compared
to other family members. The fingers and
hands were long, slender and have spider
like appearance, joint mobility was normal.
There was no evidence of cardiovascular
disease and the echocardiogram was normal.
Family history was positive for Marfan's
syndrome (one brother).
Scleromalacia means sclero-malakia
(thinning) on presentation it is characterized
by thinning and discoloration of the sclera
which can be confused for extrascleral extension
of uveal melanoma(1). All previous reported
cases of scleromalacia occur as a complication
of herpes zoster ophthalmicus(2), rheumatoid
arthritis(3), Vogt Koyanagi Harada syndrome(4),
ulceraltive colitis(5), and following retinal
Regarding our patient, we were faced with
a patient who presented with high intra
ocular pressure due to acute, anterior dislocation
of the crystalline lens which was complicated
months later by scleromalacia without any
known cause of scleromalacia other than
the characteristics Marfanoid features including
ectopia lentis, tall thin patient, long
limbs, slender and spider shaped, hands
and fingers, high arched palate and positive
family history of Marfan's syndrome. Marfan's
syndrome results from an inhereted defect
in the extracellular glycoprotein called
fibrillin, these fibrils form a scafolding
on which tropo_elastin is deposited to form
elastic fibers(7).The sclera is a dense
tough fibrous structure consisting mainly
of collagen and elasic fibers embedded in
mucopolysaccharide matrix(8). Under the
effect of attack of high intraocular pressure
the defective scleral tissue has yielded
resulting in scleromalacia. Based on that
it was concluded that it was a case of scleromalacia
associated with Marfan's syndrome.
Figure 1 - Both eyes
Figure 2 - Right eye
Berry_Brincat, H Von Lany and N Evans.Scleromalacia
as a complication of herpes zoster ophthalmicus.Eye
RJ, Cooper M. Ophthalmicus herpes zoster.
Modrzejewska M, Przerwa DM. A case of
necrotic scleromalacia during the course
of Rheumatoid arthritis.Klin Oczna.
KF. Scleromalacia associated with Vogt
Koyanagi Harada syndrome .
Am J Ophthalmol.1988 Jun 15;105(6) :694-5
PJ, Burgess JA,GoyJA,Lazell RW. Scleromalacia
perforans in ulcerative colitis.Gastroenterology.
M, Mannis MJ, chu TG. Scleromalacia
after retinal detachment surgery Am
J Ophthalmol. 1995 Jun;119(6):803-4
Pathologic Basis of Disease. Ramzi S.Cotran,
MD. Vinay Kumar MD,FRCPath. TuckerCollins,
MD, Ph.D. Sixth edition. W.B.Saunders
Chapter 6.page 148.
Pathology. An Atlas and Textbook.
Hogan and Zimmerman.
Second edition. W.B.Saunders company.
Chapter 6(The Cornea and Sclera).page