Frosted Branch Angiitis in a Patient With the Acquired Immunodeficiency Syndrome

Frosted Branch Angiitis in a Patient With the Acquired Immunodeficiency Syndrome

LETTERS TO THE JOURNAL Frosted Branch Angiitis in a Patient With the Acquired Immunodeficiency Syndrome sion or stasis of the s h e a t h e d vessels...

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LETTERS TO THE JOURNAL Frosted Branch Angiitis in a Patient With the Acquired Immunodeficiency Syndrome

sion or stasis of the s h e a t h e d vessels; (4) fluo­ rescein l e a k a g e from t h e s h e a t h e d vessels oc­ c u r r i n g in the later stages of a n g i o g r a p h y ; (5) o t h e r w i s e h e a l t h y p a t i e n t s ; a n d (6) p r o m p t r e ­ s p o n s e to c o r t i c o s t e r o i d s . We e n c o u n t e r e d a case w i t h t h e typical clini­ cal a p p e a r a n c e of u n i l a t e r a l frosted b r a n c h a n ­ giitis in a p a t i e n t w i t h a c q u i r e d i m m u n o d e f i ­ ciency s y n d r o m e , c y t o m e g a l o v i r u s retinitis, a n d cerebral t o x o p l a s m o s i s . A 4 9 - y e a r - o l d m a n w a s referred to o u r h o s p i ­ tal b e c a u s e of a o n e - w e e k h i s t o r y of d e c r e a s e d a n d b l u r r e d vision in t h e r i g h t eye. H e t e s t e d positive for h u m a n i m m u n o d e f i c i e n c y v i r u s (HIV) in 1985, a n d cerebral t o x o p l a s m o s i s w a s d i a g n o s e d in 1989. O n initial e x a m i n a t i o n , vis­ ual acuity w a s R.E.: 2 0 / 4 0 a n d L.E.: 2 0 / 2 0 . The i n t r a o c u l a r p r e s s u r e s w e r e w i t h i n n o r m a l lim­ its. Filiform keratic p r e c i p i t a t e s , a m o d e r a t e flare in the a n t e r i o r c h a m b e r , a n d a slightly h a z y v i t r e o u s w e r e s e e n in t h e r i g h t eye. Biomicroscopic e x a m i n a t i o n d i s c l o s e d t h a t t h e left eye w a s n o r m a l . O p h t h a l m o s c o p i c e x a m i n a t i o n of t h e r i g h t eye d i s c l o s e d severe s h e a t h i n g a l o n g t h e r e t i n a l v e i n s w i t h special e m p h a s i s on the p o s t e r i o r p o l e (Fig. 1). In t h e inferior fundus periphery, a tongue-shaped retinopathy w i t h c e n t r a l n e c r o s i s a n d a h e m o r r h a g i c , yel­ low m a r g i n w a s s e e n . The left eye s h o w e d n o e v i d e n c e of i n f l a m m a t i o n or r e t i n a l m i c r o v a s culopathy. F u n d u s fluorescein a n g i o g r a p h y w a s per­ f o r m e d at the initial e x a m i n a t i o n . An early p h a s e a n g i o g r a m of t h e r i g h t eye d i s c l o s e d a discrete m i c r o v a s c u l o p a t h y s u p e r i o r a n d n a s a l to the fovea. N o l e a k a g e o c c u r r e d a l o n g the

Stephan A. Geier, M.D., Joachim N a s e m a n n , M.D., Volker Klauss, M.D., Ursula Kronawitter, M.D., and Frank D . G o e b e l , M . D . University Eye Hospital Munich (S.A.G., J.N., V.K.) and Medical Policlinic of the University of Munich (U.K., F.D.G.). Supported by Bundesministerium fur Forschung und Technik, Germany, grant FKZ BGA III-002-089. Inquiries to Stephan A, Geier, M.D., University Eye Hospital, Mathildenstr. 8, W-8000 Munich 2, Germany. Frosted b r a n c h angiitis of t h e r e t i n a w a s first d e s c r i b e d in a 6-year-old b o y in 1976 b y Ito a n d associates. 1 The d e v e l o p m e n t of a severe sheathing along the retinal veins created the a p p e a r a n c e of frosted tree b r a n c h e s , w h i c h p r o m p t e d the n a m e " f r o s t e d b r a n c h a n g i i t i s " for this p e c u l i a r form of a c u t e r e t i n a l vasculitis. Recently two cases of u n i l a t e r a l frosted b r a n c h angiitis w e r e r e p o r t e d in N o r t h America. 2 Little is k n o w n a b o u t the cause of this d i s e a s e . In all p r e v i o u s l y r e p o r t e d cases, n o u n d e r l y i n g sys­ temic d i s e a s e or infection h a s b e e n f o u n d . The characteristic features of frosted b r a n c h angiitis can be s u m m a r i z e d as follows: (1) se­ vere s h e a t h i n g of the r e t i n a l vessels a p p e a r i n g like frosted b r a n c h e s of a t r e e ; (2) a c u t e visual d i s t u r b a n c e associated w i t h s i g n s of v i t r e o u s a n d a n t e r i o r c h a m b e r i n f l a m m a t i o n ; (3) f u n d u s fluorescein a n g i o g r a p h y t h a t s h o w s n o occlu­

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AMERICAN JOURNAL OF OPHTHALMOLOGY

Fig. 1 (Geier and associates). Initial fundus examination of the right eye showing sheathing along the retinal veins with special emphasis on the posterior pole.

retinal veins. However, in the late phase angiogram an optic disk edema developed accompanied by pronounced leakage from most, but not all, of the inflamed vessels of the posterior pole (Fig. 2). No occlusion or stasis was found. No leaking vessels or papilledema could be detected in the left eye. The diagnosis of cytomegalovirus retinitis and frosted branch like angiitis of the right eye was made. The patient was given ganciclovir intravenously, 10 mg/kg of body weight per day. Five days later the patient's visual acuity had decreased in the right eye to 20/200. Treatment with oral Fluocortolone (100 mg/day) was started. After two weeks sheathing disappeared and cytomegalovirus retinitis showed regression. Visual acuity in the right eye improved to 20/30. Fluocortolone was decreased stepwise to 20 mg/ day and ganciclovir to 6 mg/kg of body weight per day. Retinal periphlebitis has been reported in association with cytomegalovirus retinitis,3 HIV infection,4 and other infectious and systemic diseases including syphilis, tuberculosis, adenovirus infection, human T-cell lymphotropic virus type I infection, multiple sclerosis, sarcoidosis, and Crohn's disease. Severe retinal periphlebitis, as described here, is unique in cytomegalovirus retinitis and HIV infection. Differential diagnosis includes peripheral uveitis, Eales' disease, and acute retinal necrosis

Fig. 2 (Geier and associates). Late phase fundus fluorescein angiogram of the right eye showing pronounced leakage from inflamed vessels of the posterior pole. No occlusion or stasis can be seen.

(Kirisawa-type uveitis and bilateral acute retinal necrosis), the latter showing severe obliteration of the retinal capillaries and vessels. The adjunctive treatment with corticosteroids in patients with AIDS was expected to be beneficial in our patient. Corticosteroid-related development of Kaposi's sarcoma in AIDS patients has been reported but beneficial effects of corticosteroid therapy have been reported for Pneumocystis carinii pneumonia,5 HIV-related immune thrombocytopenia, toxoplasmosis-induced cerebral edema, and severe HIV-associated myositis. The periphlebitis in our patient met five of the six previously mentioned criteria for diagnosis of frosted branch angiitis: the patient had sheathing of retinal veins and mild anterior chamber and vitreous involvement (criteria 1 and 2), fluorescein angiography showed no occlusion or stasis and leakage occurred in the later stages (criteria 3 and 4), and response to corticosteroids was prompt (criterion 6). Only criterion 5 was not met because the patient suffered from AIDS, cytomegalovirus retinitis, and toxoplasmosis.

References 1. Ito, Y., Nakano, M., Kyu, N., and Takeuchi, M.: Frosted branch angiitis in a child. Jpn. J. Clin. Ophthalmol. 30:797, 1976.

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Letters to The Journal

2. Sugin, S. L., Henderly, D. E., Friedman, S. M., Jampol, L. M, and Doyle, J. M.: Unilateral frosted branch angiitis. Am. J. Ophthalmol. 111:682, 1991. 3. Kestelyn, P., Lepage, P., and Van de Perre, P.: Perivasculitis of the retinal vessels as an important sign in children with AIDS-related complex. Am. J. Ophthalmol. 100:614, 1986. 4. Culbertson, W. W.: Infections of the retina in AIDS. Int. Ophthalmol. Clin. 29:108, 1989. 5. The National Institutes of Health-University of California Expert Panel for Corticosteroids as Adjunctive Therapy for Pneumocystis Pneumonia: Con­ sensus statement on the use of corticosteroids as adjunctive therapy for Pneumocystis pneumonia in the acquired immunodeficiency syndrome. N. Engl. J. Med. 323:1500, 1990.

Treatment of Endogenous Fungal Endophthalmitis With Systemic Fluconazole With or Without Vitrectomy Leila Laatikainen, M.D., Mikko Tuominen, M.D., and Kai von Dickhoff, M . D . Department of Ophthalmology, University of Oulu. Inquiries to Leila Laatikainen, M.D., Department of Ophthalmology, Oulu University Hospital, 90220 Oulu, Finland. Fungal infections are an increasing problem, particularly in immunocompromised patients, because the diagnostic and treatment processes of fungal infections may be difficult. Sensitivity measurements in vitro may not correspond to the clinical effects of treatment in vivo, and most of the drugs available are toxic and diffi­ cult to use. In the treatment of Candida endophthalmitis, amphotericin B is considered to be the most effective agent but it should be used intraocularly. Amphotericin B can be administered by intravenous route but, according to Barrie, 1 it does not penetrate into the vitreous. It is also highly toxic when given intravenously. Flucytosine (5-fluorocytosine) may also be effective but resistance to this drug by Candida organisms may be a problem. Of the new azole derivatives, fluconazole has a good antifungal activity in vitro. It is watersoluble, and 10% to 20% will be bound to plasma proteins. Therefore, the distribution of fluconazole in the organism is wide, and the

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penetration into the eye is good. 2,3 Additional­ ly, fluconazole is easily absorbed after oral administration, it is excreted renally, and it has a long half-life and low toxicity. Thus, theoreti­ cally, fluconazole should be a welcome antifun­ gal agent in ophthalmic use. The information on its effectiveness in endophthalmitis is scarce.4,5 Our patient was a 63-year-old man who had carcinoma of the rectum with abdominal and lung metastasis. He had had an abdominal operation with gut perforation, which led to broad spectrum antibiotic medication, and he was on a regimen of chemotherapy (5-fluorouracil). He developed Candida stomatitis, but other manifestations of fungal infection had not been diagnosed. Ophthalmic examination was carried out be­ cause of blurred vision in the patient's right eye. The visual acuity was 20/50, there were cells in the anterior chamber, and diffuse haze and cells in the vitreous. A fairly dense, white infiltrate was observed on the right optic disk (Fig. 1, left). The left eye was amblyopic, and visual acuity was 20/60. The anterior chamber was not in­ flamed, but there were also cells in the vitreous, and an active white vitreoretinal lesion was observed in the midperiphery (Fig. 1, right). On the basis of the history of malignancy and chemotherapy as well as previously diagnosed C. albicans stomatitis and the fundus lesions, the diagnosis of fungal endophthalmitis was proposed. In the vitreous sample of the right eye, yeast spots were observed on microscopy. The bacterial culture was negative. Following vitreous tap, 5 u-g of amphotericin B was injected intravitreally. Because of more intense inflammatory signs in both the anterior chamber and vitreous, pars plana vitrectomy was performed three days later followed again by 5 u.g of amphotericin B intravitreally. In the left eye, no treatment was given locally. At the time of the first vitreous tap systemic treatment with fluconazole was started. Be­ cause of renal insufficiency, the treatment was given every second day, initially with 200 mg of fluconazole intravenously during the first two weeks. From the third week the daily dose was 300 mg of fluconazole orally for another six weeks. The treatment was well tolerated with­ out any side effects. After four months of follow-up the eyes were symptom-free and the vision had improved. The visual acuity was 20/30 in the right eye. In the left amblyopic eye, the visual acuity was