Brit. J. Ophthal. (I975) 59, 409

Acute presumed histoplasmosis of the optic nerve head

ROGER C. HUSTED AND JOHN P. SHOCKFrom the Ophthalmology Service, Letterman Army Medical Center,Presidio ofSan Francisco, California

The classic fundus findings ofpresumed histoplasmosisare well known and include peripheral chorio-retinalscars, haemorrhagic disciform disease of the macula,and peripapillary chorio-retinal scarring (Woodsand Wahlen, I 960; Walma and Schlaegel, I 964).The development of new peripheral chorio-retinalscars and disciform macular lesions has been observedclinically. However, to the best of our knowledge thedevelopment of the typical diffuse peripapillaryatrophy has not been described. A case is reported inthis paper in which the development of peripapillaryatrophy and peripheral chorio-retinal lesions ,re

Address for reprints: Dr R. C. Husted, Ophthalmology Clini.,4*ter-man Army Medical Center, Presidio of San Francisco,' lfornia94I29, USA

The opinions or assertions contained herein are the private views ofthe authors and are not to be construed as official or as reflecting theviews of the Department of the Army or the US Department ofDefense.

documented in the normal right eye of a patient withtypical presumed ocular histoplasmosis lesions in theleft eye.

C;se report

A 38-year-old man was referred to Letterman ArmyMedical Center with sudden loss of vision in the rightinferior temporal field. The visual loss had been precededby a moderately severe right frontal headache of severalhours' duration. The patient had spent his early childhoodin the Ohio Valley area.

Except for the ocular findings, the physical examinationwas unremarkable. The best visual acuity was 20/25 righteye, 20/20 left eye. Tension by applanation was I6 mm Hgin both eyes. The pupillary responses and slit-lampexarnination were normal. There was a right lower tem-poral field loss by confrontation and Goldmann perimetry(Fig. i). Fundus examination of the right eye revealed a

' X.%

FIG. I Goldmann field, right eye, demonstratzng right, lower temporal field loss

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4IO British journal of Ophthalmology

segmental nodular swelling at the nasal side of the opticdisc (Fig. 2). The disc margins were blurred with over-lying hyperaemia and small splinter haemorrhages. Therewas adjacent retinal oedema and mild engorgement of theretinal vessels. Examination with the Hruby lens revealed

FIG. 2 Acute nodular swelling of right optic disc at thenasal side with overlying haemorrhage

FIG. 3 Diffuse peripapillary atrophy of left optic disc

a few vitreous cells overlying the nodule. The maculawas normal. Extensive indirect ophthalmoscopic examina-tion revealed a normal peripheral retina. Examination ofthe fundus of the left eye revealed extensive, diffuse,peripapillary atrophy and scarring (Fig. 3). In addition,there were several equatorial, chorio-retinal, punched-outscars typical of presumed ocular histoplasmosis (Fig. 4).Visual field and macular examination of the left eye wasnormal. Fluorescein angiography revealed leakage of dyefrom the right disc in the arterio-venous phase (Fig. 5)and late staining (Fig. 6).

FIG. 4 One of several peripheral, chorio-retinal,punched-out scars seen in left eye

FIG. 5 Fluorescein angiography of right disc, revealingleakage of dye in arterio-venous phase

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Acute presumed histoplasmosis of the optic nerve head 411

The patient was given one subtenon's injection of 40mg of triamcinolone acetonide (Kenalog®) and followedclosely by dilated fundus examinations and fundus photo-graphy.Three weeks after the initial examination the disc

swelling had decreased by 50 per cent and the haemor-rhages had begun to resolve (Fig. 7). Eight weeks laterthere was little or no disc swelling, but a definite peri-papillary atrophy was noted (Fig. 8). By 4 months therewas marked peripapillary chorio-retinal scarring of the

FIG. 6 Fluorescein angiography of right disc showinglate staining

All laboratory tests were normal except a histoplasminintradermal skin test which was positive, with a 14 X 12mm area of induration at 48 hours. Tine test for tuber-culosis and coccidiosis intradermal skin test were negative.

FIG. 8 Complete resolution of right optic disc swellingat 8 weeks

FIG. 7 Partial resolution of right optic disc swelling3 weeks after initial examination

FIG. 9 Marked peripapillary atrophy of right optic disc4 months after initial examination

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412 British Journal of Ophthalmologv

F I G. i o Equatorial punched-out lesion which developedin right eye during first 8 weeks

right disc (Fig. 9) similar to that noted initially of the leftdisc. At 8 weeks, two faint equatorial punched-out scarswere noted which became more distinctive over the nextfew weeks (Fig. io).At 8 months no new findings were noted except for early

posterior subcapsular cataracts of which the right one wasworse. Visual acuity was 20/20 in both eyes, but there wasa persistence of the field loss in the right eye.

Discussion

No acceptable aetiological relationship betweenHistoplasmosis capsulatum and the characteristic findingsof presumed ocular histoplasmosis has so far beenestablished. The diagnosis of presumed ocularhistoplasmosis is based mainly on the relationshipbetween characteristic chorio-retinal findings and apositive histoplasmin skin test (Woods and Wahlen,I960; Walma and Schlaegel, i964). Reid, Scherer,Herbut, and Irving (1942) described one case ofocular involvement in the fatal form of human histo-plasmosis, but unfortunately the eyes were notobtained at necropsy. Klintworth,' 'Hollingsworth,Lusman, and Bradford '4973) recently described afatal human case of disseminate;d histoplasmosis inwhich they identified Histoplasma capsulatum in choroi-

dal lesions. Although the cliniq$mfindings were nottypical of presumed ocular histoplasmosis, it never-theless was the first time that the fungus has beenidentified in a human choroid. Numerous investiga-tors have reproduced the peripheral chorio-retinallesions in a variety of experimental animals; however,most of these studies involved direct intraocularinjection of the organisms. Naturally acquiredsystemic histoplasmosis seems to be secondary to theinhalation of spores with subsequent haematogenousspread to susceptible organs such as the eye. Smith,O'Conrfor, Halde, Scalarone, and Easterbrook (I 973)produced the choroidal lesions in rabbits by intra-carotid injection of the histoplasma organisms. Thedeveldpment of diffuse peripapillary atrophy was notone of1 the sequelae noted.

Peripapillary atrophy was first reported bySchlaegel and Kenney (i966), who noted it in 85 percent of their cases. It is thought to be primarily due toa choroiditis. The presentation of our patient with atypical nodular papillitis with overlying vitreous cellsdoes not substantiate this theory yet the final clinicalappearance of the disc was identical to that typicallyseen. In certain cases the peripapillary atrophy asso-ciated with presumed ocular histoplasmosis may besecondary to a retinitis rather than to a choroiditis.Since the development of the peripapillary atrophyhas not been described previously, this case maybe helpful in determining the natural history of thedisease. It suggests that, when examining eyes histo-pathogenically for histoplasmin organisms, a morecareful search of the retina may be indicated.

Summary

A 38-year-old man from the Ohio Valley area pre-sented to the Ophthalmology Clinic at a west coasthospital (USA) because of sudden loss of the rightinferior temporal visual field after severe rightfrontal headache of several hours' duration. Duringthe following months, diffuse peripapillary atrophyand peripheral punched-out lesions developed. Whenhe had been seen initially, the fundus of the left eyehad also revealed extensive, diffuse, peripapillaryatrophy and scarring. All laboratory tests were nor-mal except a histoplasmin intraderqnal skin test. Theclinical appearance of both eyes B months after theacute episodlein the right eye supports the diagnosis ofpresumed ocular histoplasmosis.

References

KLINTWORTH, G. K., HOLLINGSWORTH, A. S., LUSMAN, P. A., and BRADFORD, W. D. (1973) Arch. Ophthal., go, 45

REID, J. D., SCHERER, J. H., HERBUT, P. A., and IRVING, H. (1942) J. Lab. clin. Med., 27, 419SCHLAEGEL, T. F., and KENNEY, D., (i966) Amer. j. Ophthal., 62, 454SMITH, R. E., O'CONNOR, G. R., HALDE, C. J., SCALARONE, M. A., and EASTERBROOK, W. M. (I973) Ibid., 76, 284WALMA, D., and SCHLAEGEL, T. F. (I964) Ibid., 57, I07WOODS, A. c., and WAHLEN, H. E. (I960) Ibid., 49, 205

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