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Juvenile hemorrhagic macular choroidopathy
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CURRENTOPHTHAlMOL0GY
cidence of ocular signs and symptoms of much less than 50%. Of course, the incidence of ocular findings dependsupon the physician consulted by the patient. Obviously, in a rheumatoid disease clinic, most patients will present with signs of joint and muscle aching and stiffness whereas the patient with giant cell arteritis consulting an ophthalmologist will generally have ocular involvement. All in all, this article should be read in full by all ophthalmologists treating patients with giant cell arteritis. PAUL HENKIND
JuvenileHemorrhagicMacular Choroidopathy,by J Frangois, JJ deLaey and M Dakir. Bull Sot Belge Ophtalmol 167:664-678, 1974 Although histoplasmosis is exceptional in Europe, “presumed ocular histoplasmosis” is far from being rare. In three years, we studied 23 cases of hemorrhagic macular choroidopathy. The age of the patients varied between 12 years and 51 years. The lesions showed the following characteristics. 1. The macular lesion was usually unilateral (22 cases out of 23). 2. The disciform macular lesion was usually rather small (% to $5disc diameter) and presented with a choroidal neovascularization surrounded by retina1 and subretinal hemorrhages as well as by a localized detachment of the neuroepithelium. 3. In 13 cases, small chorioretinitis scars were found near the optic disc, around the macula or even more peripherally. The number of these scars, which were very similar to the “histospots” described in the American literature, varied between 1 and 8 in a single eye. In 5 cases, they were found in both eyes. In 8 cases, serological or skin tests for histoplasma capsulatum were performed. They were all negative, except in a young American woman with bilateral involvement. Three patients had positive skin tests for tuberculin. The fundus lesions in most of these patients seemed thus unrelated to histoplasmosis. However, the disciform macular response is probably a late consequence of an inflammatory disease of the choroid with localized destruction of Bruch’s membrane. Whereas the histoplasma capsulatum might be the causal organism in a number of cases in areas where histoplasmosis is endemic, it is certainly not the only cause able to reproduce a similar clinical aspect. (Abstract by J. Fragois)
Comment Cases of the presumed ocular histoplasmosis syndrome have been documented from England (Braunstein RA, Rosen DA, Bird AC: Ocular histoplasmosis syndrome in the United Kingdom. Br J Ophfhalmol58:893, 1974). the Netherlands (Deutman AF: Fluorescein angiography in macular disease, in Henkes HE: Photography Electra-ophthalmof and Echo-ophthalmology in Ophthalmic Practice, Vol. 3, Haag, Junk, 1973, p. 210), as well as in Belgium, as depicted in this article. These reports indicate that the syndrome of presumed ocular histoplasmosis is not rare, but it is much more common in the endemic areas of the United States. Thirteen of Francois’ patients had the typical syndrome, including disseminated histospots and changes around the optic nerve. The other patients might be classified as either atypical ocular histoplasmosis or idiopathic subretinal neovascularization. I would estimate that in England, the Netherlands, and Belgium, most of the cases seen have no relation at all to benign systemic histoplasmosis. However, in the endemic area of the United States, I estimate that 85% of the cases do have such a relationship. I have now personally seen 1023 cases of the syndrome of presumed ocular histoplasmosis and 184 patients classified as atypical. Thus, 85% of my cases have been typical and are probably related to benign systemic histoplasmosis. The other 15% may be due to some other agent or agents. We have no clues as to the etiology of those cases which are not associated with benign systemic histoplasmosis. Many have been puzzled by the fact that symptomatic pulmonary histoplasmosis has not been associated with the ocular picture. This may be simply a matter of frequency. The benign disease is 1000 times as common as the symptomatic. In my experience with slightly over a thousand ocular cases, I have seen one patient with past proved symptomatic pulmonary histoplasmosis. Thus, the absence of the ocular syndrome after symptomatic pulmonary histoplasmosis may be related entirely to the rarity of the symptomatic compared to the benign variety of systemic histoplasmosis. TF SCHLAEGEL, JR
CURRENTOPHTHAlMOL0GY
cidence of ocular signs and symptoms of much less than 50%. Of course, the incidence of ocular findings dependsupon the physician consulted by the patient. Obviously, in a rheumatoid disease clinic, most patients will present with signs of joint and muscle aching and stiffness whereas the patient with giant cell arteritis consulting an ophthalmologist will generally have ocular involvement. All in all, this article should be read in full by all ophthalmologists treating patients with giant cell arteritis. PAUL HENKIND
JuvenileHemorrhagicMacular Choroidopathy,by J Frangois, JJ deLaey and M Dakir. Bull Sot Belge Ophtalmol 167:664-678, 1974 Although histoplasmosis is exceptional in Europe, “presumed ocular histoplasmosis” is far from being rare. In three years, we studied 23 cases of hemorrhagic macular choroidopathy. The age of the patients varied between 12 years and 51 years. The lesions showed the following characteristics. 1. The macular lesion was usually unilateral (22 cases out of 23). 2. The disciform macular lesion was usually rather small (% to $5disc diameter) and presented with a choroidal neovascularization surrounded by retina1 and subretinal hemorrhages as well as by a localized detachment of the neuroepithelium. 3. In 13 cases, small chorioretinitis scars were found near the optic disc, around the macula or even more peripherally. The number of these scars, which were very similar to the “histospots” described in the American literature, varied between 1 and 8 in a single eye. In 5 cases, they were found in both eyes. In 8 cases, serological or skin tests for histoplasma capsulatum were performed. They were all negative, except in a young American woman with bilateral involvement. Three patients had positive skin tests for tuberculin. The fundus lesions in most of these patients seemed thus unrelated to histoplasmosis. However, the disciform macular response is probably a late consequence of an inflammatory disease of the choroid with localized destruction of Bruch’s membrane. Whereas the histoplasma capsulatum might be the causal organism in a number of cases in areas where histoplasmosis is endemic, it is certainly not the only cause able to reproduce a similar clinical aspect. (Abstract by J. Fragois)
Comment Cases of the presumed ocular histoplasmosis syndrome have been documented from England (Braunstein RA, Rosen DA, Bird AC: Ocular histoplasmosis syndrome in the United Kingdom. Br J Ophfhalmol58:893, 1974). the Netherlands (Deutman AF: Fluorescein angiography in macular disease, in Henkes HE: Photography Electra-ophthalmof and Echo-ophthalmology in Ophthalmic Practice, Vol. 3, Haag, Junk, 1973, p. 210), as well as in Belgium, as depicted in this article. These reports indicate that the syndrome of presumed ocular histoplasmosis is not rare, but it is much more common in the endemic areas of the United States. Thirteen of Francois’ patients had the typical syndrome, including disseminated histospots and changes around the optic nerve. The other patients might be classified as either atypical ocular histoplasmosis or idiopathic subretinal neovascularization. I would estimate that in England, the Netherlands, and Belgium, most of the cases seen have no relation at all to benign systemic histoplasmosis. However, in the endemic area of the United States, I estimate that 85% of the cases do have such a relationship. I have now personally seen 1023 cases of the syndrome of presumed ocular histoplasmosis and 184 patients classified as atypical. Thus, 85% of my cases have been typical and are probably related to benign systemic histoplasmosis. The other 15% may be due to some other agent or agents. We have no clues as to the etiology of those cases which are not associated with benign systemic histoplasmosis. Many have been puzzled by the fact that symptomatic pulmonary histoplasmosis has not been associated with the ocular picture. This may be simply a matter of frequency. The benign disease is 1000 times as common as the symptomatic. In my experience with slightly over a thousand ocular cases, I have seen one patient with past proved symptomatic pulmonary histoplasmosis. Thus, the absence of the ocular syndrome after symptomatic pulmonary histoplasmosis may be related entirely to the rarity of the symptomatic compared to the benign variety of systemic histoplasmosis. TF SCHLAEGEL, JR