VAKA TAKDiMi

TERRIEN'S MARGINAL DEGENERATION OF THE : A case report Terrien'in marjinal kornea dejenerasyonu: Vaka takdimi

Sarper Karak.iiJ;ukl, G Ertugrul Mirza 2, Nurullah c;agrll , A Kerim Durukan1

Summary: A rare case of Terrien's marginal Ozet: Terrien' in marjinal dejenerasyonu tan lSI degeneration is reported. The case had the konulan ve ender rastlanan bir vaka takdim edil­ characteristic features of the disease including mi§tir. Vakada hastaltgm karakteristik ozellikleri progressive caused by ectasia and arasmda bulunan periferik korneamn incelmesi, thinning of the peripheral cornea. The literature ektazisi ve buna baglt ilerleyici astigmatizma relevant to this disorder is reviewed. bulunmaktadtr. Bu hastaltkla ilgili literatur gdzden ge~irilmi§tir.

Key Words: Corneal diseases, Astigmatism, Cor­ Anahtar Kelimeler: Korneal hastaltklar, Astig­ neal opacity, Corneal stroma matizma, Korneal opasite, Korneal stroma

Terrien's marginal degeneration is an uncommon superior limbus, along with stromal deposits disease of the peripheral cornea, characterized by having some vascularization approaching from the gradual thinning of the stroma which in turn leads peripheral (Figure).No inflammatory to corneal ectasia and gutter formation. It is signs were accompanying the picture. usually bilateral, although it may be asymmetric. One eye may be involved many years before the History revealed that the condition was present other. About 75% of patients are males. The since 1981, the astigmatism showed progression disease can be seen at any age between 10 and 75 and topical steroids were occasionally prescribed. years with the highest incidence in the 20 to 30 age No systemic abnormalities were found on group. We report a patient with Terrien's marginal examination. A diagnosis of Terrien's marginal degeneration with the classical features of the degeneration was made; she was informed about disease. the condition and instructed to come should any symptoms persist. CASE REPORT DISCUSSION A 20 year-old-female was referred to the Clinic of Erciyes University Terrien's marginal degeneration has been called Faculty of Medicine complaining of burning with many other names including peripheral furrow sensation on both eyes in September, 1992. The , ectasic marginal dystrophy, peripheral refraction showed -0.75 -1.00x15o<' RE and -l.00- corneal ectasia, and senile marginal atrophy. Fine, 0.50x700 LE; her corrected vision was 9/10 on both yellow-white punctate peripheral opacities which eyes. The fundus examination was bilaterally resemble arcus senilis are among the early clinical normal. features. Pseudopterygia may occasionally develop, occurring at unusual or oblique meridians(8, 5). Biomicroscopy of the right eye showed arcuate thinning at the inferior cornea near the limbus. On The corneal thinning process in Terrien's marginal the left, areas of marginal degeneration with degeneration begins by an indentation in the clear gradual and arcuate thinning were seen at the cornea between marginal opacification and the upper limbus. Gradually a gutter-like furrow forms Erciyes University Medical Faculty 38039 Kayseri, TURKIYE here. As the guttering progresses, its central wall Department of Ophthalmology. Resident!, Associate Prof2 makes a sharp inclination while the peripheral side

102 ------Erciyes T1p Dergisi 15 (I) 102-104, 1993 Karakiit;iik, Mirza, C,:agtl ve ark.

shelves gradually. The sharp central edge is Terrien's marginal degeneration affects primarily demarcated by a grayish-white line. Irregular the corneal stroma.Pathological examination may deposits of lipid can be seen within the deep layers show that the majority of stroma undergoes of the furrow. The floor of the gutter gradually fibrillary degeneration along with lipid infiltration becomes thin and vascularized. Since the epithelial of collagen fibrils. Stromal thinning is always layer remains intact , it does not stain with present, In advanced cases. the stroma may be fluorescein. Interpalpebral periphery is usually extremely thin. In the epithelium overlying ectatic spared. As a result of continued thinning, a furrow areas, multiple basal mitoses and hypercellularity is in the lower periphery is seen along with an ectasia noted. Local defects in Bowman's membrane, and in the upper cornea (8, 10). Clinically in some cases healed ruptures of Descemet's progressive thinning and ectasia causes high membrane can be seen. astigmatism since the cornea flattens in the vertical meridian; gradual deterioration of vision occurs. The etiology of Terrien's marginal degeneration The ectatic areas may eventually perforate, either remains unknown. Various histopathologic and spontaneously or with mild trauma. Patients are ultrastructural studies of affected have usually asymptomatic unless there is severe demonstrated evidence of degeneration, irregular astigmatism. Our patient also had high particularly of the basal epithelium- basement astigmatism gradually increasing since 1981. membrane -anterior stroma complex (6, 11). Occasionally, intermittent, mild ocular initation may be seen , however, Austin and Brown (2), and must be made between lrkec;: (7) have described recurrent episodes of Mo01·en's ulcer, which is usually painful, painful ocular inflammation in Terrien's marginal accompanied with inflammatory signs and can degeneration, which they termed as inflammatory cause severe melting (3). However, epithelium in Terrien's Disease. Binder and associates described Mooren 's ulcer is non-intact, the lesion spreads a similar case with moderately severe ocular centrally and circumferentially; 1apid progression is int1ammation (3). sometimes seen and no lipid is associated with the lesions ( 1).

Disease progression in Terrien's marginal degeneration is classically slow. Usually the patient does not present until progressive inegular astigmatism deteriorates visual acuity. At this I \ \ stage. spectacle correction of astigmatic enor is '\j sufficient. Rigid contact lenses or piggy-back (combined rigid-soft) contact lenses can be prescribed at advanced stages. In advanced cases, corneal irregularity and astigmatic error increases too much as to make contact fitting impossible. Surgical repair is then indicated both to decrease high astigmatism and to prevent perforation of thinned ectatic corneal regions . Excision of the ectatic tissue followed by suturing of the walls of the furrow, annular full thickness or crescentic lamellar keratoplasty or eccentric penetrating graft fitting to the shape of the defect Figure. Arcuate thinning at the superior limbus (small is sufficient to solve the problem (4. 9). arrows) and stromal vascularization approaching from the peripheral conjunctiva (hu·ge arrow) Terrien's marginal degeneration must be

Erciyes T1p Dergisi 15 (1) 102-104,1993------103 Terrien's marginal degeneration. of the cornea

contemplated in any patient with progressive be informed about their disease and carefully astigmatism. Once the diagnosis is made, the followed-up. This can help better supporting them patients with Ten-ien's marginal degeneration must and solve disease oriented problems.

REFERENCES 6. lvamato T: Electron microscopy in cases of 1. Arffa RC: Degenerations. fn Arffa RC (ed): marginal degeneration of the cornea . Invest Grayson's Diseases of the Cornea. Mosby Ophthalmol Vis Sci 11 :241 -258, 1972. Year Book fnc. , St. Louis 1991 pp 333-363. 7. lrker; M, Atabay C: Terrien' in mwjinal 2. Austin ? , Brown Sf: lnflammatory Terrien's komea dejenerasyonu. Tiirk Ojtalmoloji marginal corneal disease. Am J Ophtlzalmol Ga:zetesi 13: 129-132, 1983. 92:189-192,1981. 8. Kanski .!1: Terrien's marginal degeneration. 3. Binder PS, za,,a/a EY, Stainer GA: In Kanski JJ (ed): Clinical Ophthalmology. A Noninfectious peripheral corneal ulceration: Systemic Approach. Buflerworths, London Mo01·en's ulcer or Terrien's marginal 1989. pp. 108-109. degeneration? Ann Ophthalmol 14:425-435, 9. Mw11zis MJ: Terrien's marginal degeneration. 1982. In Fraunfelder FT. Roy T (eds): Current 4. Caldwell DR, fns/er MS, Boutros G, Hawk T: Ocular Therapy. W .B. Saunders, Primary surgical repair of severe peripheral Philadelphia 1990. p 426. marginal ectasia in Terrien's m.arginal 10. Robin JB, Schanzlin DJ, Verity SM. et at: degeneration. Am J Ophthalmol 97:332-336, P eripheral corneal disorders. Surv 1984. Ophthalmo/31: 1-36,1986. 5. Goldman KN, Kaufman HE: Atypical 11 . Suveges !, Levai G. A/berth B: Pathology of A clinical feature of Terrien's Terrien's disease: histochemical and electron marginal degeneration. Arch Ophthalmol microscopic study. Am J Oplzthalmol 74: 96:1027-1029, 1978. 1191-1200,1972.

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