July 2006


Editorial
Meet the Team

Acute Exacerbations of Chronic Obstructive Pulmonary Disease (COPD): Evidence-Based Approach

Acceptance of self-treatment in Hemophilic Patient: A Training Method

A Study of Depression Prevalence of  (in) Nurses and It’s Effective Factors in Shiraz Namazi Hospital


Home Health Care Team Members

Call for a Middle East Center of Disease Control

Skilled Health Workers - A Solution to Primary Health Problems in Pakistan

The Blind School Project - An activity from School Health Program

Scleromalacia Associated with Marfan’s Syndrome

Reference values of hematological parameters of healthy Anatolian males aged 18-45 years old

Aspiration and Death from Amitraz-Xylene Poisoning

Childhood Orbital Cellulitis Complicating Sinusitis in Tafila

 

 


Dr Abdulrazak Abyad
MD,MPH, AGSF
Editorial office:
Abyad Medical Center & Middle East Longevity Institute
Azmi Street, Abdo Center,
PO BOX 618
Tripoli, Lebanon

Phone: (961) 6-443684
Fax:     (961) 6-443685
Email:
aabyad@cyberia.net.lb

 
 

Lesley Pocock
medi+WORLD International
572 Burwood Road,
Hawthorn 3122
AUSTRALIA
Emai
l
: lesleypocock

 


Scleromalacia Associated with Marfan's Syndrome

 
Authors:


Suha M.Ajeilat, MD.
Reham I. Shaban,MD.
Ayman S. Madanat,DO,FRCS,FRCO phth.
K.H.M.C

 


ABSTRACT

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).


 
COMMENTS

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

 

 

REFERENCES
1. A Berry_Brincat, H Von Lany and N Evans.Scleromalacia as a complication of herpes zoster ophthalmicus.Eye (2003) 17,449-51.doi:10.1038/sj.eye.6700336.
2. Marsh RJ, Cooper M. Ophthalmicus herpes zoster.
Eye1993;7:350-370.
3. Modrzejewska M, Przerwa DM. A case of necrotic scleromalacia during the course of Rheumatoid arthritis.Klin Oczna. 1995 Jan-Feb;97(1-2):31-3.
4. Tabbara KF. Scleromalacia associated with Vogt Koyanagi Harada syndrome .
Am J Ophthalmol.1988 Jun 15;105(6) :694-5
5. Tesar PJ, Burgess JA,GoyJA,Lazell RW. Scleromalacia perforans in ulcerative colitis.Gastroenterology. 1981 Jul;81(1):153-5
6. Chechelnitsky M, Mannis MJ, chu TG. Scleromalacia after retinal detachment surgery Am J Ophthalmol. 1995 Jun;119(6):803-4
7. Robbins. Pathologic Basis of Disease. Ramzi S.Cotran, MD. Vinay Kumar MD,FRCPath. TuckerCollins, MD, Ph.D. Sixth edition. W.B.Saunders company.
Chapter 6.page 148.
8. Opthalmic Pathology. An Atlas and Textbook.
Hogan and Zimmerman.
Second edition. W.B.Saunders company.
Chapter 6(The Cornea and Sclera).page 335_336.