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 palate.
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 lesion.
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 detachment surgery(6).
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