- Email: [email protected]
Terrien's Marginal Degeneration Associated With Vernal Conjunctivitis
Letters to the Journal
Vol. I l l , No. 4
517
Terrien's Marginal Degeneration A s s o c i a t e d W i t h Vernal Conjunctivitis Israel Kremer, M.D. Department of Ophthalmology, Beilinson Medical Center, Petah Tiqva, Sackler School of Medicine, Tel Aviv University. Inquiries to Israel Kremer, M.D., Department of Oph thalmology, Beilinson Medical Center, Petah Tiqva 49100, Israel.
Figure (De Keyser, Herroelen, and Van Langen hove). Perimetry carried out with the head in hyperextension. of the l e s i o n l e a v e l i t t l e d o u b t t h a t the v e r t e b r a l artery occlusion was primarily caused by m e c h a n i c a l i n j u r y or c o m p r e s s i o n o f t h e v e r t e b r a l a r t e r y a c r o s s the c r a n i o v e r t e b r a l j u n c t i o n , caused by cervical hyperextension. Similar c a s e s o f v e r t e b r a l a r t e r y i n j u r y have b e e n d e s c r i b e d after y o g a , ' g y m n a s t i c exercises,^ o v e r head work,' and neck manipulation.'' Clinic personnel should be instructed about the p o t e n t i a l h a z a r d s of c e r v i c a l h y p e r e x t e n sion d u r i n g d i a g n o s t i c p r o c e d u r e s .
References 1. Hanus, S. H., Homer, T. D., and Harter, D. H.: Vertebral artery occlusion complicating yoga exer cise. Arch. Neurol. 34:574, 1977. 2. Nagler, W.: Vertebral artery obstruction by hy perextension of the neck. Report of three cases. Arch. Phys. Med. Rehabil. 54:237, 1 9 7 3 . 3. Okawara, S., and Nibbelinck, D.: Vertebral ar tery occlusion following hyperextension and rotation of the neck. Stroke 5:640, 1974. 4. Mehalic, T., and Farhat, S. M.: Vertebral artery injury from chiropractic manipulation of the neck. Surg. Neurol. 2:125, 1974.
T e r r i e n ' s d i s e a s e o f the p e r i p h e r a l c o r n e a is characterized by a slowly progressive, nonin flammatory, marginal corneal furrowing and e c t a s i a o f the s u p e r i o r p e r i p h e r a l c o r n e a . ' S u v e g e s , L e v a i , a n d A l b e r t ' n o t e d p h a g o c y t o s i s of corneal stroma by cells resembling histiocytes a s s o c i a t e d with p e r i p h e r a l c o r n e a l b l o o d v e s s e l s in the e c t a t i c a r e a s . T h e o r i g i n o f T e r r i e n ' s d i s e a s e is still u n k n o w n , but the p r e s e n c e o f lipid a s s o c i a t e d w i t h the f u r r o w i n g s u g g e s t s a degenerative process. Patients with Terrien's disease are usually asymptomatic, unless they have severe irregu lar a s t i g m a t i s m . M o s t p a t i e n t s do n o t have significant associated ocular inflammation. A u s t i n a n d Brown,^ h o w e v e r , d e s c r i b e d six p a tients with a combination of severe recurrent e p i s o d e s o f painful o c u l a r i n f l a m m a t i o n a n d c o r n e a l findings t y p i c a l o f T e r r i e n ' s d i s e a s e . Binder, Zavala, and S t a i n e r ' s i m i l a r l y described a p a t i e n t w h o h a d b o t h t h e c o r n e a l c h a n g e s of Terrien's degeneration and moderately severe ocular inflammation. I treated a patient who had chronic limbal vernal conjunctivitis and developed Terrien's degeneration. A 40-year-old man had severe vernal con junctivitis, both palpebral and limbal, during childhood. He was treated with corticosteroid a n d d i s o d i u m - c r o m o g l y c a t e e y e d r o p s for al m o s t ten y e a r s , a n d at t h e a g e of 1 7 y e a r s , h e w a s free of s y m p t o m s . S u b s e q u e n t l y , h e b e g a n having visual disturbances. Refraction dis c l o s e d 4 d i o p t e r s of a g a i n s t the r u l e a s t i g m a tism in a d d i t i o n to — 4 . 0 s p h e r e s in b o t h e y e s . S l i t - l a m p e x a m i n a t i o n d i s c l o s e d p e r i p h e r a l su p e r i o r c o r n e a l o p a c i f i c a t i o n w i t h m i l d , superfi cial v a s c u l a r i z a t i o n . C o r n e a l t h i n n i n g w a s a l s o n o t e d in t h a t a r e a . T h e c o r n e a l findings p r o g r e s s e d d u r i n g t h e f o l l o w i n g y e a r s , a n d the superior stromal thinning continued gradually, extending peripherally and centrally (Figure), w h i c h l e d to e c t a s i a , i n c r e a s e d a g a i n s t the rule astigmatism, and diminution of visual acuity.
518
April, 1991
AMERICAN JOURNAL OF OPHTHALMOLOGY
a l s o f o u n d in the p a t i e n t C a m e r o n , A l - R a j h i , Badr.^
described
by
References
Figure (Kremer). Slit-lamp photograph of a superi or band of corneal opacification and some vasculariza tion, in addition to localized thinning with superior corneal ectasia. Keratometry disclosed R.E.: 5 2 diopters hori zontal and 44 diopters vertical and L.E.: 51 d i o p t e r s h o r i z o n t a l a n d 4 3 d i o p t e r s v e r t i c a l . In both eyes, a typical progression of Terrien's d e g e n e r a t i o n was f o u n d , w h i c h c o n s i s t e d o f superior peripheral corneal opacification, mild v a s c u l a r i z a t i o n , t h i n n i n g , a n d e c t a s i a . T h e pa t i e n t was i n t o l e r a n t to c o n t a c t l e n s e s , b e c a u s e he d e v e l o p e d g i a n t p a p i l l a r y c o n j u n c t i v i t i s , a n d t h e r e f o r e h a d to w e a r s p e c t a c l e s . B e s t corrected visual acuity was 2 0 / 4 0 bilaterally. U n l i k e the a s s o c i a t i o n of k e r a t o c o n u s w i t h v e r n a l conjunctivitis,* o t h e r t y p e s o f n o n i n flammatory c o r n e a l e c t a s i a , s u c h as s u p e r i o r corneal thinning and pellucid marginal degen e r a t i o n , have b e e n r e p o r t e d to b e a s s o c i a t e d with v e r n a l c o n j u n c t i v i t i s . C a m e r o n , A l - R a j h i , a n d Badr^ s t u d i e d 6 1 p a t i e n t s w i t h different corneal ectasia and vernal keratoconjunctivitis. F i f t y - t h r e e p a t i e n t s h a d k e r a t o c o n u s , five h a d pellucid marginal degeneration, two had keratoglobus, and only one patient had superior c o r n e a l t h i n n i n g . In v e r n a l k e r a t o c o n j u n c tivitis, eye r u b b i n g is c o m m o n b e c a u s e o f the intense itching; several authors have c o m m e n t ed on the role of c h r o n i c e y e r u b b i n g in k e r a t o c o n u s . T h e v a r i e t y of c o r n e a l t h i n n i n g p a t t e r n s in a s s o c i a t i o n with v e r n a l k e r a t o c o n j u n c t i v i t i s found in this report^ s u p p o r t s e y e r u b b i n g a s o n e o f the m a j o r factors in t h e c a u s e o f c o r n e a l e c t a s i a . In my o p i n i o n , the a c t i v e l i m b a l v e r n a l k e r a t i t i s per s e , with the e o s i n o p h i l i c infiltra tion r e a c h i n g the p e r i p h e r a l c o r n e a , m a y b e a n o t h e r i m p o r t a n t p a t h o g e n e t i c factor in my patient. Limbal vernal keratoconjunctivitis was
1. Suveges, I., Levai, C , and Albert, B.: Pathology of Terrien's disease. Histochemical and electron mi croscopic study. Am. J. Ophthalmol. 74:1191, 1972. 2. Austin, P., and Brown, S. I.: Inflammatory Ter rien's marginal corneal disease. Am. J. Ophthalmol. 92:189, 1 9 8 1 . 3. Binder, P. S., Zavala, E. Y., and Stainer, G. Α.: Noninfectious peripheral corneal ulceration. Morren's ulcer or Terrien's marginal degeneration? Ann. Ophthalmol. 14:425, 1982. 4. Khan, M. D., Kundi, N., Saeed, N., Gulab, Α., and Nazeer, A. F.: Incidence of keratoconus in spring catarrh. Br. J. Ophthalmol. 72:41, 1 9 8 8 . 5. Cameron, J. Α., Al-Rajhi, A. Α., and Badr, I. Α.: Corneal ectasia in vernal keratoconjunctivitis. Oph thalmology 96:1615, 1989.
Identification of A m i o d a r o n e in Corneal Deposits Stephen J. Haug, M.D., and Alan H. Friedman, M . D . Department of Ophthalmology, Mount Sinai Medical Center. This study was supported in part by an unrestricted grant from Research to Prevent Blind ness, Inc. Inquiries to Alan H. Friedman, Box 1183, Mount Sinai Medical Center, One Gustave I. Levy Place, New York, NY 10029-6574. A m i o d a r o n e h y d r o c h l o r i d e is an effective drug for the t r e a t m e n t of a n g i n a a n d v e n t r i c u lar a r r h y t h m i a s . Its u s e h a s b e e n a s s o c i a t e d with d e p o s i t s in v a r i o u s t i s s u e s , i n c l u d i n g s k i n , nerves, and c o r n e a . ' ' Amiodarone has been identified in d e r m a l m a c r o p h a g e s b y e n e r g y d i s p e r s i v e x-ray m i c r o a n a l y s i s . ^ We u s e d this t e c h n i q u e on a m i o d a r o n e - r e l a t e d c o r n e a l d e posits. A 74-year-old man was given 4 0 0 mg o f a m i o d a r o n e per day after c a r d i o v e r s i o n for r e fractory v e n t r i c u l a r t a c h y c a r d i a . S e v e n m o n t h s later ophthalmologic consultation was ob t a i n e d after h o s p i t a l a d m i s s i o n b e c a u s e o f the amiodarone dosage. The interior portion o f each cornea contained golden-brown deposits
Vol. I l l , No. 4
517
Terrien's Marginal Degeneration A s s o c i a t e d W i t h Vernal Conjunctivitis Israel Kremer, M.D. Department of Ophthalmology, Beilinson Medical Center, Petah Tiqva, Sackler School of Medicine, Tel Aviv University. Inquiries to Israel Kremer, M.D., Department of Oph thalmology, Beilinson Medical Center, Petah Tiqva 49100, Israel.
Figure (De Keyser, Herroelen, and Van Langen hove). Perimetry carried out with the head in hyperextension. of the l e s i o n l e a v e l i t t l e d o u b t t h a t the v e r t e b r a l artery occlusion was primarily caused by m e c h a n i c a l i n j u r y or c o m p r e s s i o n o f t h e v e r t e b r a l a r t e r y a c r o s s the c r a n i o v e r t e b r a l j u n c t i o n , caused by cervical hyperextension. Similar c a s e s o f v e r t e b r a l a r t e r y i n j u r y have b e e n d e s c r i b e d after y o g a , ' g y m n a s t i c exercises,^ o v e r head work,' and neck manipulation.'' Clinic personnel should be instructed about the p o t e n t i a l h a z a r d s of c e r v i c a l h y p e r e x t e n sion d u r i n g d i a g n o s t i c p r o c e d u r e s .
References 1. Hanus, S. H., Homer, T. D., and Harter, D. H.: Vertebral artery occlusion complicating yoga exer cise. Arch. Neurol. 34:574, 1977. 2. Nagler, W.: Vertebral artery obstruction by hy perextension of the neck. Report of three cases. Arch. Phys. Med. Rehabil. 54:237, 1 9 7 3 . 3. Okawara, S., and Nibbelinck, D.: Vertebral ar tery occlusion following hyperextension and rotation of the neck. Stroke 5:640, 1974. 4. Mehalic, T., and Farhat, S. M.: Vertebral artery injury from chiropractic manipulation of the neck. Surg. Neurol. 2:125, 1974.
T e r r i e n ' s d i s e a s e o f the p e r i p h e r a l c o r n e a is characterized by a slowly progressive, nonin flammatory, marginal corneal furrowing and e c t a s i a o f the s u p e r i o r p e r i p h e r a l c o r n e a . ' S u v e g e s , L e v a i , a n d A l b e r t ' n o t e d p h a g o c y t o s i s of corneal stroma by cells resembling histiocytes a s s o c i a t e d with p e r i p h e r a l c o r n e a l b l o o d v e s s e l s in the e c t a t i c a r e a s . T h e o r i g i n o f T e r r i e n ' s d i s e a s e is still u n k n o w n , but the p r e s e n c e o f lipid a s s o c i a t e d w i t h the f u r r o w i n g s u g g e s t s a degenerative process. Patients with Terrien's disease are usually asymptomatic, unless they have severe irregu lar a s t i g m a t i s m . M o s t p a t i e n t s do n o t have significant associated ocular inflammation. A u s t i n a n d Brown,^ h o w e v e r , d e s c r i b e d six p a tients with a combination of severe recurrent e p i s o d e s o f painful o c u l a r i n f l a m m a t i o n a n d c o r n e a l findings t y p i c a l o f T e r r i e n ' s d i s e a s e . Binder, Zavala, and S t a i n e r ' s i m i l a r l y described a p a t i e n t w h o h a d b o t h t h e c o r n e a l c h a n g e s of Terrien's degeneration and moderately severe ocular inflammation. I treated a patient who had chronic limbal vernal conjunctivitis and developed Terrien's degeneration. A 40-year-old man had severe vernal con junctivitis, both palpebral and limbal, during childhood. He was treated with corticosteroid a n d d i s o d i u m - c r o m o g l y c a t e e y e d r o p s for al m o s t ten y e a r s , a n d at t h e a g e of 1 7 y e a r s , h e w a s free of s y m p t o m s . S u b s e q u e n t l y , h e b e g a n having visual disturbances. Refraction dis c l o s e d 4 d i o p t e r s of a g a i n s t the r u l e a s t i g m a tism in a d d i t i o n to — 4 . 0 s p h e r e s in b o t h e y e s . S l i t - l a m p e x a m i n a t i o n d i s c l o s e d p e r i p h e r a l su p e r i o r c o r n e a l o p a c i f i c a t i o n w i t h m i l d , superfi cial v a s c u l a r i z a t i o n . C o r n e a l t h i n n i n g w a s a l s o n o t e d in t h a t a r e a . T h e c o r n e a l findings p r o g r e s s e d d u r i n g t h e f o l l o w i n g y e a r s , a n d the superior stromal thinning continued gradually, extending peripherally and centrally (Figure), w h i c h l e d to e c t a s i a , i n c r e a s e d a g a i n s t the rule astigmatism, and diminution of visual acuity.
518
April, 1991
AMERICAN JOURNAL OF OPHTHALMOLOGY
a l s o f o u n d in the p a t i e n t C a m e r o n , A l - R a j h i , Badr.^
described
by
References
Figure (Kremer). Slit-lamp photograph of a superi or band of corneal opacification and some vasculariza tion, in addition to localized thinning with superior corneal ectasia. Keratometry disclosed R.E.: 5 2 diopters hori zontal and 44 diopters vertical and L.E.: 51 d i o p t e r s h o r i z o n t a l a n d 4 3 d i o p t e r s v e r t i c a l . In both eyes, a typical progression of Terrien's d e g e n e r a t i o n was f o u n d , w h i c h c o n s i s t e d o f superior peripheral corneal opacification, mild v a s c u l a r i z a t i o n , t h i n n i n g , a n d e c t a s i a . T h e pa t i e n t was i n t o l e r a n t to c o n t a c t l e n s e s , b e c a u s e he d e v e l o p e d g i a n t p a p i l l a r y c o n j u n c t i v i t i s , a n d t h e r e f o r e h a d to w e a r s p e c t a c l e s . B e s t corrected visual acuity was 2 0 / 4 0 bilaterally. U n l i k e the a s s o c i a t i o n of k e r a t o c o n u s w i t h v e r n a l conjunctivitis,* o t h e r t y p e s o f n o n i n flammatory c o r n e a l e c t a s i a , s u c h as s u p e r i o r corneal thinning and pellucid marginal degen e r a t i o n , have b e e n r e p o r t e d to b e a s s o c i a t e d with v e r n a l c o n j u n c t i v i t i s . C a m e r o n , A l - R a j h i , a n d Badr^ s t u d i e d 6 1 p a t i e n t s w i t h different corneal ectasia and vernal keratoconjunctivitis. F i f t y - t h r e e p a t i e n t s h a d k e r a t o c o n u s , five h a d pellucid marginal degeneration, two had keratoglobus, and only one patient had superior c o r n e a l t h i n n i n g . In v e r n a l k e r a t o c o n j u n c tivitis, eye r u b b i n g is c o m m o n b e c a u s e o f the intense itching; several authors have c o m m e n t ed on the role of c h r o n i c e y e r u b b i n g in k e r a t o c o n u s . T h e v a r i e t y of c o r n e a l t h i n n i n g p a t t e r n s in a s s o c i a t i o n with v e r n a l k e r a t o c o n j u n c t i v i t i s found in this report^ s u p p o r t s e y e r u b b i n g a s o n e o f the m a j o r factors in t h e c a u s e o f c o r n e a l e c t a s i a . In my o p i n i o n , the a c t i v e l i m b a l v e r n a l k e r a t i t i s per s e , with the e o s i n o p h i l i c infiltra tion r e a c h i n g the p e r i p h e r a l c o r n e a , m a y b e a n o t h e r i m p o r t a n t p a t h o g e n e t i c factor in my patient. Limbal vernal keratoconjunctivitis was
1. Suveges, I., Levai, C , and Albert, B.: Pathology of Terrien's disease. Histochemical and electron mi croscopic study. Am. J. Ophthalmol. 74:1191, 1972. 2. Austin, P., and Brown, S. I.: Inflammatory Ter rien's marginal corneal disease. Am. J. Ophthalmol. 92:189, 1 9 8 1 . 3. Binder, P. S., Zavala, E. Y., and Stainer, G. Α.: Noninfectious peripheral corneal ulceration. Morren's ulcer or Terrien's marginal degeneration? Ann. Ophthalmol. 14:425, 1982. 4. Khan, M. D., Kundi, N., Saeed, N., Gulab, Α., and Nazeer, A. F.: Incidence of keratoconus in spring catarrh. Br. J. Ophthalmol. 72:41, 1 9 8 8 . 5. Cameron, J. Α., Al-Rajhi, A. Α., and Badr, I. Α.: Corneal ectasia in vernal keratoconjunctivitis. Oph thalmology 96:1615, 1989.
Identification of A m i o d a r o n e in Corneal Deposits Stephen J. Haug, M.D., and Alan H. Friedman, M . D . Department of Ophthalmology, Mount Sinai Medical Center. This study was supported in part by an unrestricted grant from Research to Prevent Blind ness, Inc. Inquiries to Alan H. Friedman, Box 1183, Mount Sinai Medical Center, One Gustave I. Levy Place, New York, NY 10029-6574. A m i o d a r o n e h y d r o c h l o r i d e is an effective drug for the t r e a t m e n t of a n g i n a a n d v e n t r i c u lar a r r h y t h m i a s . Its u s e h a s b e e n a s s o c i a t e d with d e p o s i t s in v a r i o u s t i s s u e s , i n c l u d i n g s k i n , nerves, and c o r n e a . ' ' Amiodarone has been identified in d e r m a l m a c r o p h a g e s b y e n e r g y d i s p e r s i v e x-ray m i c r o a n a l y s i s . ^ We u s e d this t e c h n i q u e on a m i o d a r o n e - r e l a t e d c o r n e a l d e posits. A 74-year-old man was given 4 0 0 mg o f a m i o d a r o n e per day after c a r d i o v e r s i o n for r e fractory v e n t r i c u l a r t a c h y c a r d i a . S e v e n m o n t h s later ophthalmologic consultation was ob t a i n e d after h o s p i t a l a d m i s s i o n b e c a u s e o f the amiodarone dosage. The interior portion o f each cornea contained golden-brown deposits