Br J Ophthalmol 1999;83:753–758 753

47–83 Boulevard de l’Hôpital, 75651 Paris Cedex 13, France. LETTERS TO Accepted for publication 3 December 1998 Br J Ophthalmol: first published as 10.1136/bjo.83.6.753c on 1 June 1999. Downloaded from THE EDITOR 1 Helm C, Holland GN. Ocular tuberculosis. Surv Ophthalmol 1993;38:229–56. 2 Espinasse MA, Perrenoud F, Gaudric A, et al. A propos de deux cas de tubercules de Bouchut (étude angiographique). Bull Soc Ophtalmol Fr 1984;5:537–40. Indocyanine green angiography in 3 Grewal A, Kim R, Cunningham ET. Miliary choroidal tuberculomas tuberculosis. Arch Ophthalmol 1998;116:953–4. 4 Berinstein DM, Gentile RC, McCormick SA, et EDITOR,—An 85 year old white woman al. Primary choroidal tuberculoma. Arch Oph- presented with progressive asthenia, fever, thalmol 1997;115:430–1. coughing, and dyspnoea. Chest roentgeno- gram showed interstitial pulmonary infiltrates Diagnosis of an atypical case of ocular and right pleural eVusion. Cultures of the toxoplasmosis using the demonstration bronchoalveolar lavage fluid subsequently of intraocular antibody production and confirmed the presence of Mycobacterium the polymerase chain reaction tuberculosis. On admission, best corrected visual acuity EDITOR,—Ocular toxoplasmosis is the most was 20/400 in a right amblyopic eye and 20/50 frequent infectious cause of chorioretinal in the left eye. Biomicroscopic examination inflammation in immunocompetent revealed no sign of anterior or posterior individuals.1 Diagnosis is usually made by inflammation. Multiple choroidal lesions (Fig observing the typical fundus lesion, by detect- 1) were present in both eyes. The choroidal ing the presence of anti-Toxoplasma antibodies lesions were deep, white-yellowish, with indis- in the serum, and by excluding other causes of tinct borders. Fluorescence angiography (FA) necrotising fundus lesions.2 In unusual cases, revealed early nodular hypofluorescence, and invasive procedures may be required to aid diagnosis.3

CASE REPORT Figure 3 ICG angiograms reveal early (A) A 17 year old white male presented complain- and late (B) phase blockage by the choroidal ing of floaters and reduced visual acuity in the granulomas. left eye. Visual acuity was 6/9 in the left eye, 6/6 in the right. Examination revealed moder- late moderate hyperfluorescence (Fig 2). ate anterior chamber activity, marked vitritis, Indocyanine green (ICG) angiography re- and an active retinochoroiditis adjacent to an vealed prolonged hypofluorescence and in the area of old chorioretinal scarring inferonasal late stage images, moderate delineation of the to the optic disc. A diagnosis of ocular lesions by a peripheral hyperfluorescent ring toxoplasmosis was suspected, and topical and (Fig 3). oral steroids, and oral clindamycin were com- menced. Peripheral blood anti-Toxoplasma IgG antibodies, measured using the dye test, Figure 1 Multiple choroidal granulomas in the COMMENT were positive (16 IU/ml). Despite treatment, left posterior pole. Ocular tuberculosis may occur by haema- the ocular inflammatory signs increased and 5 http://bjo.bmj.com/ togenic spread from a pulmonary focus. weeks following initial presentation he devel- Choroidal tuberculomas are rare ophthalmic 1 oped a confluent area of retinal necrosis in the findings even in miliary tuberculosis. Previ- peripheral retina leading to a superotemporal ous reports indicate that these lesions have retinal detachment. This was distinct from the prolonged hypofluorescence in FA, and late 23 original area of inflammation. The presence of mild hyperfluorescence. severe vitreous inflammation and peripheral Only one description of ICG angiography retinal necrosis suggested a unilateral acute in a case with presumed ocular tuberculosis retinal necrosis syndrome.4 Three port trans 4 on October 2, 2021 by guest. Protected copyright. has been reported previously in the literature. pars plana vitrectomy with perfluorocarbon We found similar angiographic characteristics liquid and fluid/silicone exchange was per- in our case, which represents, to our knowl- formed. At vitrectomy, vitreous humour was edge, the first ICG angiography description of taken for anti-Toxoplasma and antiviral anti- multiple choroidal tuberculomas in microbio- body levels and a retinal biopsy was also logically confirmed miliary tuberculosis.Hypo- obtained. Postoperatively, he was commenced fluorescence in ICG images may be due to a on sulphadiazine, pyrimethamine, and folinic masking eVect of the choroidal vessels by the acid and continued on oral steroid medi- overlying granulomas. cation. Levels of IgG, IgA, and IgM were Ophthalmic examination may be contribu- measured in serum and vitreous aspirate at the tive when disseminated tuberculosis is sus- same time. The Goldmann–Witmer coef- pected. In this case ICG angiography, which ficient using IgG was greater than 59, using was performed to assess the choroidal involve- IgA greater than 45, and using IgM greater ment, showed prolonged hypofluorescence. than 65. This is evidence of intraocular DAN MILEA antibody production. Samples were negative CHRISTINE FARDEAU for antiviral antibodies. Intraocular Toxo- LIVIA LUMBROSO plasma DNA was demonstrated by a polymer- Department of Ophthalmology, Hôpital de la ase chain reaction (PCR) assay using primers Pitié-Salpêtrière, Paris, France for the P30 gene. PCR testing for viral DNA THOMAS SIMILOWSKI was negative. InsuYcient material was ob- Department of Respiratory and Intensive Care tained to attempt to isolate the parasite using Medecine, Hôpital de la Pitié-Salpêtrière, Paris, France tissue culture or animal inoculation. Retinal PHUC LEHOANG biopsy demonstrated a mixed inflammatory Department of Ophthalmology, Hôpital de la response without a specific infective agent. Figure 2 (A) Early prolonged blockage and Pitié-Salpêtrière, Paris, France The patient subsequently responded to treat- (B) late moderate hyperfluorescence of the Correspondence to: Phuc Le Hoang, MD, Service ment and the intraocular inflammatory signs choroidal lesions on fluorescein angiography. d’Ophtalmologie, Hôpital de la Pitié-Salpêtrière, subsided. 754 Letters

COMMENT Protein C and protein S deficiency

Ocular toxoplasmosis is a common cause of associated with retinal, optic nerve, and Br J Ophthalmol: first published as 10.1136/bjo.83.6.753c on 1 June 1999. Downloaded from retinochoroiditis, and can usually be diag- cerebral ischaemia nosed clinically. Rarely is it possible to obtain vitreous and retinal biopsies to aid diagnosis, EDITOR,—Deficiencies in the vitamin K de- but in doubtful cases, it may be appropriate to pendent factors protein C and protein S can perform anterior or posterior chamber aspi- lead to arterial or venous thrombosis. Branch rate to confirm the diagnosis. The assessment and central retinal arterial and venous of Toxoplasma antibodies in serum is of limited occlusions1–4 have been associated with defi- use, unless rising titres can be demonstrated, ciencies in these plasma proteins, as have 5 6 since the incidence of Toxoplasma infection in amaurosis fugax and stroke. We report, to the general population is high. The demon- the best of our knowledge, the first case of stration of antibody production within the eye ischaemic optic neuropathy associated with is particularly valuable in the diagnosis of dif- combined protein C and protein S deficiency. Figure 3 Six weeks after initial presentation. Note pale disc with narrowing of the retinal ficult cases. The finding of higher anti- arterioles and an overall reduction in venous Toxoplasma antibody levels in the aqueous CASE REPORT calibre and tortuosity. humour than in the serum (the Goldmann– A 47 year old woman with non-insulin Witmer coeYcient) indicates intraocular anti- dependent diabetes mellitus with documented painless loss of vision to the level of hand body production.5 A further investigation absence of previous retinopathy presented movements in the right eye. Fundus examina- which is extremely useful is the demonstration with blurring of vision and bright flashing tion 6 weeks later revealed a pale optic disc of parasite DNA within ocular fluid by PCR.6 lights in her right eye for 2 weeks, associated with both generalised and focal narrowing of With PCR a sequence of DNA is amplified with vague periocular discomfort and left the retinal arterioles, and an overall reduction from minuscule amounts of DNA making it sided facial and leg numbness. Best corrected in venous calibre and tortuosity (Fig 3). Three amenable to direct analysis.78 De Boer et al visual acuity was 20/30 right eye and 20/25 left months later, at which time the visual acuity used a combination of the demonstration of eye. The anterior segment examination was remained at hand movements, electroretino- intraocular antibody production and PCR unremarkable and the intraocular pressures graphy (ERG) was performed to distinguish analysis in the diagnosis of a variety of were 15 mm Hg right eye and 14 mm Hg left retinal vascular pathology from optic nerve infectious uveitis cases.9 In this case we eye. A large cotton wool spot was present embarrassment. The right eye exhibited mod- initially made a diagnosis of ocular toxoplas- inferotemporal to the right optic disc (Fig 1). est reductions in scotopic b-wave amplitudes mosis, but the disease progressed clinically The overlying vitreous was clear. The retinal in response to dim white flash (33%) and to and did not respond to treatment. The patient venules appeared moderately tortuous but bright white flash (20%) compared with the was compliant with prescribed medication, undilated. Fluorescein angiography revealed left eye. Cone b-wave implicit time on 30 Hz and had no evidence of immunocompromise. normal arterial filling but markedly delayed flicker testing was only slightly longer in the Retinal detachment is unusual in ocular arteriovenous filling and late disc hyperfluo- right eye compared with the left eye (30.5 ms toxoplasmosis,10 but is typical of acute retinal rescence. When she returned 2 weeks later, versus 29.5 ms). Oscillatory potential ampli- necrosis syndrome, suggesting an alternative this cotton wool spot was smaller, but other tudes were normal in both eyes. These results diagnosis in this case. We were, however, able cotton wool spots superior to the disc had were interpreted as showing insuYcient evi- to confirm the diagnosis of toxoplasmosis by appeared (Fig 2). The patient underwent dence for ischaemic retinal damage as an evidence of intraocular antibody production carotid Doppler and cerebral angiography explanation for her profound loss of vision. and by positive PCR amplification. studies which revealed near complete occlu- The patient was diagnosed with ischaemic sion of the right internal carotid artery. optic neuropathy in the right eye based on M MINIHAN P E CLEARY Coumadin therapy was instituted and exten- clinical findings and the ERG results. Labora- Department of Ophthalmology, Cork University sive diagnostic evaluation was pursued. She tory testing revealed that protein C antigen Hospital and University College, Cork returned 2 weeks later and all the cotton wool was 47% and protein S antigen 46% of spots were resolving. normal levels. Activated protein C and anti- BCRYAN thrombin levels were normal, and no lupus http://bjo.bmj.com/ Department of Medical Microbiology, Cork University Three days later she was admitted to the Hospital and University College, Cork hospital with syncope and left hemiparesis due anticoagulant activity was detected. to an infarct in the territory of the right middle R HOLLIMAN cerebral artery. She also suVered sudden, Toxoplasma Reference Unit, Public Health Laboratory, COMMENT St George’s Hospital, Blackshaw Road, London This patient, with combined protein C and Correspondence to: Ms Minihan. protein S deficiency, suVered ipsilateral reti- Accepted for publication 9 December 1998 nal, optic nerve, and cerebral ischaemia within a period of 6 weeks. The rapid changes in the

appearance of cotton wool spots over a period on October 2, 2021 by guest. Protected copyright. 1 Henderly DE, Gentsler AJ, Smith RE, et al. of several days, which is not consistent with Changing patterns of uveitis. Am J Ophthalmol their natural course in diabetic retinopathy,7 1987;103:131. 2 Rothova A. Ocular involvement in toxoplasmo- combined with neurological symptoms sis. Br J Ophthalmol 1993;77:371–7. prompted us to search for systemic causes of 3 Nussenblatt RB, Belfort R. Ocular toxoplasmo- ischaemia, including evaluation for hyperco- sis. An old disease revisisited. JAMA 1994;271: agulable states. We suggest that new cotton 304–7. 4 Holland GN and the Executive Committee of the wool spots in a patient free of other signs of American Uveitis Society. Standard diagnostic vascular retinopathy such as microaneurysms criteria for the acute retinal necrosis syndrome. or retinal haemorrhages should raise the spec- Am J Ophthalmol 1994;117:663–6. Figure 1 Initial large cotton wool spot tre of a systemic basis for the ischaemia. As the 5 Desmonts G. Definitive serological diagnosis of inferotemporal to right optic disc. ocular toxoplasmosis. Arch Ophthalmol 1966;76: ERG was not compatible with occlusion of the 383–6. ophthalmic or central retinal arteries, demon- 6 Holliman RE, Stevens PJ, DuVyKT,et al. The strating only mild retinal ischaemia, we serological investigation of ocular toxoplasmo- ascribed the sudden visual loss in the face of sis. Br J Ophthalmol 1991;75:353–5. 7 Kijlstra A, Luyendjik L, Baarsma GS, et al. diVuse disc pallor to optic nerve ischaemia, Aqueous humor analysis as a diagnostic tool in perhaps from occlusion of multiple ciliary ves- toxoplasma uveitis. Int Ophthalmol 1989;6:383– sels. Ischaemic optic neuropathy has, to our 6. 8 Chan C, Palestine AG, Li Q, et al. Diagnosis of knowledge, not previously been associated ocular toxoplasmosis by the use of immunocy- with protein C or protein S deficiency, and tology and the polymerase chain reaction. Am J expands the spectrum of ophthalmic manifes- Ophthalmol 1996;117:803–5. tations of the hypercoagulable state. 9 De Boer JH, Verhagen C, Bruinenberg M, et al. Serologic and polymerase chain reaction analy- sis of intraocular fluids in the diagnosis of infec- Supported, in part, by the Heed Ophthalmic tious uveitis. Am J Ophthalmol 1996;121:650–8. Foundation (Dr Ambati) and an unrestricted grant 10 De Boer JH, Luyendijk L, Rothova A, et al. Figure 2 Initial cotton wool spot along the from Research to Prevent Blindness, Inc, New York, Detection of intraocular antibody production to NY (University of Rochester), USA. herpesviruses in acute retinal necrosis syn- inferotemporal vessel resolving 2 weeks later with drome. Am J Ophthalmol 1994;117:201–10. appearance of new cotton wool spots superiorly. JAYAKRISHNA AMBATI Letters 755

OMAR E HANUCH Evaluation of the patient 4 weeks after sur- both human and monkey eyes following YAG GEORGE H BRESNICK gery revealed an improvement of visual acuity posterior capsulotomy.4 Br J Ophthalmol: first published as 10.1136/bjo.83.6.753c on 1 June 1999. Downloaded from Department of Ophthalmology, University of Rochester in the left eye to the level of 20/25. Visual acu- School of Medicine and Dentistry, Rochester, USA ity 6 months after surgery remained at the The authors have no proprietary interest in any of Correspondence to: Dr J Ambati, Retina Service, level of 20/25 with the macular hole closed. the products mentioned in this article. Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114, USA. RIPAN CHAUDHARY Accepted for publication 11 December 1998 COMMENT Department of Ophthalmology, The most common complication of extracap- University of Alberta, sular methods is a late opacification of the Edmonton, Canada 1 Nelson ME, Talbot JF, Preston FE. Recurrent posterior capsule. Surgically opening the pos- TOM SHEIDOW multiple-branch retinal arteriolar occlusions in a terior capsule has been shown in several stud- JOHN R GONDER patient with protein C deficiency. Graefes Arch ies to increase the incidence of both cystoid MOHAN M MERCHEA Clin Exp Ophthalmol 1989;227:443–7. Department of Ophthalmology, 2 Greven CM, Weaver RG, Owen J, et al. Protein S macular oedema and retinal detachment.45 University of Western Ontario, deficiency and bilateral branch retinal artery With the advent of the Nd:YAG laser, the ease occlusion. 1991; :33–4. London, Canada Ophthalmology 98 of posterior capsulotomy has been greatly 3 Golub BM, Sibony PA, Coller BS. Protein S Correspondence to: Dr Sheidow. deficiency associated with central retinal artery simplified. Retinal complications following Accepted for publication 11 December 1998 occlusion. Arch Ophthalmol 1990;108:918. YAG laser capsulotomy are well 4 Chung MM, Trese MT, Hong YJ. Protein C lev- documented.45Winslow and Taylor4 reported els in retinal vein occlusions. Invest Ophthalmol one retinal flap, two macular holes, six cases of 1 Aaberg TM. Macular holes: a review. Surv Oph- Vis Sci 1989;30:477. thalmol 1970;15:139. 5 Smith DB, Ens GE. Protein C deficiency: a cause cystoid macular oedema, and 10 retinal 2 Gordon LW, Glaser BM, Le D, et al. Full of amaurosis fugax. J Neurol Neurosurg Psychia- detachments following YAG laser cap- thickness macular hole formation in eyes with a try 1987;50:361. sulotomy. In this series, macular hole forma- pre-existing complete posterior vitreous detach- 6 Martinez HR, Rangel-Guerra RA, Marfil LJ. ment. Ophthalmology 1995;102:1702–5. Ischemic stroke due to deficiency of coagulation tion occurred 1 and 3 months after cap- 3 Gass JDM. Idiopathic senile macular holes. Its inhibitors. Report of 10 young adults. Stroke sulotomy while in our case it occurred within early stages and pathogenesis. Arch Ophthalmol 1993; :19–25. 24 2 weeks. 1988;106:629–39. 7 Kohner EM, Dollery CT, Bulpitt CJ. Cotton- 4 Winslow RL, Taylor BC. Retinal complications wool spots in diabetic retinopathy. Diabetes Over the years, several mechanisms have following YAG laser capsulotomy. Ophthalmol- 1969;18:691–704. been proposed to explain the increased ogy 1985;92:785–9. incidence of retinal complications following 5 Steinert RF, Puliafito CA, Kumar SR, et al. Cyst- oid macular edema, retinal detachment, and Macular hole following YAG posterior capsulotomy including increased glaucoma after Nd:YAG laser posterior cap- vitreous liquefaction, changes in vitreous sulotomy. 1992; :373–8. capsulotomy Am J Ophthalmol 112 composition, acoustic transients, and direct 6 Osterlin S. Changes in the macromolecular com- 6 position of the vitreous produced by removal of EDITOR,—Since the initial identification of retinal damage. Osterlin reported a greater the lens. Concillium Ophthalmologicum 1971;222: macular holes as pathological entities in the decline in the hyaluronic acid content in vitre- 1620–3 as cited in Winslow, Taylor.4 middle of the 19th century,1 there has been ous samples from monkey eyes having 7 Miyake K. Prophylaxis of aphakic cystoid macu- lar edema using topical indomethacin. Am an evolution in the understanding of their undergone intracapsular cataract extraction as Intraocul Implant Soc J 1978;4:174–9 as cited in aetiology. Tangential macular traction by peri- opposed to extracapsular cataract extraction. Winslow, Taylor.4 foveal vitreous cortex is now accepted as the He postulated that in the eyes that had 8 Lerman S, Thrasher B, Morgan M. Vitreous changes after neodymium:YAG laser irradiation causative factor in the development of idio- undergone intracapsular cataract extraction, of the posterior lens capsule or mid-vitreous. pathic macular holes.23 hyaluronic acid in the vitreous had diVused Am J Ophthalmol 1984;97:470–5. With the widespread use of extracapsular anteriorly, resulting in the vitreous instability 9 Thach AB, Lopez PF, Snedy-McCoy LC, et al. cataract extraction procedures, posterior cap- and subsequent retinal complications. Thus, Accidental Nd:YAG laser injuries to the macula. Am J Ophthalmol 1995;119:767–73. sule opacification is a frequent complication. the intact capsule acts as a diVusion barrier for 10 Blacharski PA, Newsome DA . Bilateral macular YAG laser capsulotomy, although a non- hyaluronic acid. This concept of a diVusion holes after Nd:YAG laser posterior cap- invasive procedure, has been associated with a barrier was again employed by Miyake7 to sulotomy. Am J Ophthalmol 1988;105:417–8. number of complications, including retinal theorise a role for the posterior capsule in the http://bjo.bmj.com/ development of cystoid macular oedema due detachment, cystoid macular oedema, and Magnetic resonance imaging of raised intraocular pressure (IOP).45 A much to iris synthesised prostaglandins. colobomatous optic hypoplasia rarer complication of YAG capsulotomy Significant liquefaction of the vitreous, pos- herein reported is the formation of a macular tulated to be the result of acoustic transients DITOR 4 E ,—Retinochoroidal coloboma is a hole after YAG capsulotomy. accompanying the laser irradiation, has been common ocular malformation that can occur documented in monkey and rabbit eyes as an isolated finding in an otherwise healthy CASE REPORT following Nd:YAG laser irradiation of the individual or as part of a complex malforma- 8 A 71 year old woman underwent an uncom- posterior capsule. Other more direct injuries tion syndrome.1 Histologically, it is a well on October 2, 2021 by guest. Protected copyright. plicated extracapsular cataract extraction with to the retina with the formation of macular demarcated, excavated, infrapapillary area of posterior chamber lens implantation in her left holes have been reported in industrial acci- absent retina, pigment epithelium, Bruch’s 9 eye. Her ocular history was significant for dents involving the Nd:YAG laser. membrane, and choriocapillaris, with variable chronic open angle glaucoma. In the immedi- In a case report by Blacharski and attenuation of the choroid.2 Some retino- 10 ate postoperative period, there was an acute Newsome, bilateral macular holes were choroidal colobomas incorporate the optic rise in IOP to 40 mm Hg that responded to reported following Nd:YAG laser posterior disc and cause the inferior aspect of the optic Diamox (acetazolamide) orally. Three months capsulotomies. In the first eye, a macular hole disc to appear retruded or absent within the postoperatively, best corrected visual acuity formed 21 days after capsulotomy in the excavation.3 The purpose of this study was to was 20/20 in both eyes with IOPs of 17 mm absence of vitreous prolapse or an elevated determine whether such malformations are Hg in the right eye and 13 mm Hg in the left. IOP post laser. In the second eye, careful associated with hypoplasia of the intracranial Two years later, the best corrected visual biomicroscopy was performed before optic nerve. acuity was found to have decreased to 20/80 in Nd:YAG capsulotomy and despite the ab- Five patients with unilateral retinochoroidal the left eye attributable to significant posterior sence of complication, a macular hole formed coloboma involving the optic disc underwent capsule opacification. Posterior capsulotomy 10 days after treatment. These authors magnetic resonance imaging (MRI) of the was performed with a Nd:YAG laser (4.1 believed it unlikely that the shock wave gener- head to rule out associated intracranial mJ/pulse, total energy 109.5 mJ). Postopera- ated by the pulse directly caused the macular malformations. Patients consisted of two tively, there was no increase in IOP and no vit- hole as relatively low energies were used on males and three females with ages at the time reous prolapse into the anterior chamber. Two both occasions (18 mJ and 29 mJ). of MRI ranging from 2 weeks to 4 years. All weeks after the Nd:YAG laser capsulotomy, In our case, we propose that the macular patients had large unilateral retinochoroidal the patient noted a decrease in visual acuity, hole was formed secondary to the perifoveal colobomas that incorporated the optic disc along with a black spot in her central vision. vitreous contraction initiated by the YAG cap- (Fig 1). On examination, a stage 3 macular hole was sulotomy. The possible mechanisms of MRI consisted of sagittal T1 weighted seen with best corrected visual acuity 20/400 Nd:YAG laser initiation of vitreous contrac- images, axial T2 weighted images, and coronal left eye. Retinal consultation confirmed the tion could include the well documented T1 weighted thin section images (with 3 mm diagnosis and the patient underwent a pars acoustic transients generated by a YAG laser slice thickness and 0.3 mm gaps) through the plana vitrectomy, with C3F8 gas instillation pulse, as well as vitreous instability secondary chiasm, intracranial optic nerves, and orbits. and facedown positioning. to the vitreous liquefaction demonstrated in T1 weighted coronal MR images of the 756 Letters

nised, reflects the timing of colobomatous dysembryogenesis early in gestation and im- Br J Ophthalmol: first published as 10.1136/bjo.83.6.753c on 1 June 1999. Downloaded from plicates a primary developmental failure of inferior retinal ganglion cells.4 MRI of other segmental optic disc malformations (for ex- ample, congenital tilted disc syndrome, unilat- eral high myopia) may disclose similar reduc- tions in intracranial optic nerve size. Figure 2 Coronal T1 weighted magnetic Supported in part by a grant from Research to resonance image demonstrating intraconal orbital Prevent Blindness, Inc. mass compressing the globe. MICHAEL C BRODSKY University of Arkansas for Medical Sciences, Little cence in the area of the mass (Fig 1). B-scan Rock, Arkansas ultrasonography showed an echolucent mass Correspondence to: Arkansas Children’s Hospital, compressing the sclera, measuring 16×16×12 800 Marshall, Little Rock, AR 72202, USA. mm. Based on these findings, an orbital Accepted for publication 11 January 1999 tumour producing globe compression was suspected. 1 Pagon RA. Ocular coloboma. Surv Ophthalmol Magnetic resonance imaging was per- 1981;25:223–36. formed to more clearly delineate the soft 2 Mann I. Developmental abnormalities of the eye. tissue mass. A well circumscribed intraconal 2nd ed Philadelphia: JB Lippincott, 1957:74– mass was found adjacent to the sclera 91. 3 Gopal L, Badrinath SS, Kumar KS, et al. Optic inferomedially, producing globe compression disc in fundus coloboma. Ophthalmology 1996; and inferior rectus displacement (Fig 2). On 103:2120–7. T1 weighted images, the lesion was isointense 4 Novakovic P, Taylor DSI, Hoyt WF. Localizing patterns of optic nerve hypoplasia retina to optic and on T2 weighted images, hyperintense nerve. Br J Ophthalmol 1988;72:176–82. with respect to muscles. Marked enhancement 5 Brodsky MC, Glasier CM, Pollock SC, et al. of the lesion with gadolinium was found. Our Figure 1 (A) Retinochoroidal coloboma Optic nerve hypoplasia: identification by mag- diVerential diagnosis included orbital cavern- netic resonance imaging. Arch Ophthalmol 1990; incorporating the segmentally hypoplastic right 108:1562–7. ous haemangioma, neurofibroma, schwan- optic disc (open arrows). The major retinal noma, fibrous histiocytoma, and haemangio- vessels delimit the lower margin of the optic disc. pericytoma. (B) T1 weighted coronal MR image (case 1) Orbital haemangiopericytoma The patient underwent transconjunctival demonstrating hypoplasia of the right optic nerve (small arrow). The area of the right optic nerve simulating an intraocular mass excisional biopsy. The pink encapsulated mass is approximately half the size of the normal left was composed of spindle cells with moderate optic nerve (large arrow). EDITOR,—Most patients with orbital tumours mitotic activity. Staghorn vascular channels present with proptosis.12 It is uncommon for were evident, and in several areas the tumour an orbital mass to cause symptoms and signs cells invaded the pseudocapsule. intracranial optic nerves were examined to simulating intraocular disease. We report the The histopathological diagnosis was benign compare the size of the intracranial optic case of a patient with an orbital tumour that haemangiopericytoma. The patient has been nerve corresponding to the colobomatous eye was initially suspected to be an intraocular followed for 1 year without further problems. with that corresponding to the normal eye. tumour. Cases 1–3 had no associated systemic or intracranial malformations. Case 4 had Gold- COMMENT enhar syndrome with hemifacial microsomia, CASE REPORT Haemangiopericytoma is a rare vascular cerebral hemispheric asymmetry without dis- A 71 year old woman noted a photopsia, tumour derived from an abnormal prolifera- organisation, and colobomatous microphthal- diplopia, and peripheral scotoma in her left tion of pericytes. It rarely occurs in the orbit, http://bjo.bmj.com/ mos on the involved side. Case 5 had Aicardi eye. She was evaluated and underwent laser accounting for only 1% of all orbital syndrome with agenesis of the corpus callo- treatment for suspected retinal hole at the biopsies.1–5 Orbital haemangiopericytoma oc- sum and bilateral grey matter heterotopia. margin of a presumed retinal detachment. curs as a painless, unifocal tumour often in the In all patients, coronal MRI showed a After non-resolution of the “detachment”, a muscle cone.1–5 The majority of cases are rec- smaller intracranial optic nerve on the side second ophthalmologist raised the possibility ognised between the ages of 20–70 years.1–5 In corresponding to the retinochoroidal colo- that the fundus lesion was a choroidal most cases there is progressive proptosis. boma (Fig 1). The degree of intracranial optic melanoma. The patient was then referred to However, in our case mild proptosis but nerve hypoplasia varied according to the oph- the oncology service for further management. marked compression of the globe was seen. on October 2, 2021 by guest. Protected copyright. thalmoscopic configuration of the optic disc. Ocular examination revealed corrected visual Orbital haemangiopericytoma poses a risk for When the inferior aspect of the optic disc was acuity of 6/7.5 in both eyes. Proptosis of 3 mm recurrence and metastasis, especially when the present but retruded posteriorly within the with minimal limitation of supraduction and tumour invades beyond the pseudocapsule.3–5 colobomatous defect (case 1), the correspond- infraduction was noted. Fundus examination Orbital haemangiopericytoma generally is a ing intracranial optic nerve was only slightly showed an elevated choroidal mass with slow growing tumour that has an ocular and diminished in size relative to the normal optic normal appearing retinal and choroidal vessels systemic prognosis.1–5 There is a risk for recur- nerve. When only the inferior aspect of the overlying the mass. The mass did not shift rence and metastasis when the pseudocapsule optic disc was absent within the colobomatous with eye position. Fluorescein angiography is breached.4 In one series, a 30% recurrence defect (cases 2–5), a moderate reduction in demonstrated retinal and choroidal isofluores- rate was noted with recurrences generally intracranial optic nerve size was seen. occurring 1 month to 7 years after surgery.3 In 1988, Novakovic et al demonstrated that Our patient may be at risk of developing focal retinal lesions can produce segmental orbital recurrence in the future because there hypoplasia of corresponding sectors of optic was invasion of the pseudocapsule. disc.4 In 1990, Brodsky et al showed that T1 Orbital tumours should be included in the weighted MRI can be used to confirm the diVerential diagnosis of a solid intraocular clinical diagnosis of optic nerve hypoplasia by mass. Those orbital tumours that arise in the showing a reduction in size of the intracranial muscle cone adjacent to the sclera may optic nerve(s).5 In the present study, MRI produce these confusing clinical features. showed that colobomatous retinochoroidal malformations involving the optic disc are Dr Ralph C Eagle Jr performed the interpretation of consistently associated with hypoplasia of the the histopathology. ipsilateral intracranial optic nerve, corre- Supported by the Eye Tumor Research Founda- tion, Philadelphia, PA and the Paul Kayser Inter- sponding to the inferior segmental hypoplasia national Retina Research Fund Houston, Texas (Dr observed ophthalmoscopically. The nosologi- J Shields), and the Macular Foundation, New York cal overlap between colobomatous derange- Figure 1 Late venous phase of fluorescein (Dr C Shields). ment of the optic nerve and segmental angiography showing retinal and choroidal WALTENIO V DINIZ hypoplasia, which has gone largely unrecog- isofluorescence. CAROL L SHIELDS Letters 757

JERRY A SHIELDS of the haemorrhage is unknown but has not KAAN GUNDUZ been previously reported as a risk factor. This Br J Ophthalmol: first published as 10.1136/bjo.83.6.753c on 1 June 1999. Downloaded from Ocular Oncology Service of the Wills Eye Hospital, report emphasises that, while needling of Thomas JeVerson University, Philadelphia, USA trabeculectomy blebs is usually a safe proce- ALAN BRACKUP dure, severe complications may arise and Saint Morries Hospital, Langhorne, PA, USA these need to be taken into consideration, Correspondence to: Jerry A Shields, MD, Ocular especially when managing high risk patients. Oncology Service, Wills Eye Hospital, 900 Walnut LUCY J HOWE Street, Philadelphia, PA 19107, USA. PHILIP BLOOM Accepted for publication 16 December 1998 The Western Eye Hospital, Marylebone Road, London NW1 5YE 1 Shields JA. Vasculogenic and tumors malforma- Figure 2 B scan ultrasound examination of the Correspondence to: Lucy Howe. tions. In: Diagnosis and management of orbital same eye 4 months later showing a fixed funnel Accepted for publication 22 December 1998 tumors. Philadelphia: WB Saunders, 1989:132– retinal detachment. 34. 2 Shields JA, Shields CL. Orbital hemangioperi- 1 Mardelli PG, Lederer CM, Murray PL, Slit- cytoma. Atlas of orbital tumors. Philadelphia: et al. Lippincott, Williams and Wilkins, 1999. The needling was performed at the slit lamp lamp needle revision of failed filtering blebs using 3 Croxatto JO, Font RL. Hemangiopericytoma of with immediate development of a shallow mitomycin C. Ophthalmology 1996;103:1946–55. the orbit: a clinicopathologic study of 30 cases. bleb. The anterior chamber was well main- 2 Greenfield DS, Miller MP, Suner IJ, et al. Needle 1982; :210–18. elevation of the scleral flap for failing filtration Human Pathol 13 tained with an IOP of 4 mm Hg. Subconjunc- blebs after trabeculectomy with mitomycin C. 4 Jakobiec FA, Howard GM, Jones IS, et al. Hemangiopericytoma of the orbit. tivally, 5 mg of 5-fluorouracil was adminis- Am J Ophthalmol 1996;122:195–204. Am J Oph- 3 Manschot WA. The pathology of expulsive hem- thalmol 1974;78:816–34. tered and the patient was discharged with orrhage. Am J Ophthalmol 1955;40:15–24. 5 Henderson JW, Farrow GM. Primary orbital topical steroids and antibiotics. When he bent hemangiopericytoma: an aggressive and poten- 4 Frenkel REP, Shin DH. Prevention and manage- tially malignant neoplasm. over 7 hours later he experienced sudden pain ment of delayed suprachoroidal hemorrhage Arch Ophthalmol after filtration surgery. 1986; 1978;96:666–73. in his left eye with immediate reduction of Arch Ophthalmol 104:1459–63. vision. He presented for examination the 5 Potash SD, Ritch R, Liebmann J. Ocular following day when the visual acuity was hypotony and choroidal eVusion following bleb Delayed suprachoroidal haemorrhage noted to be reduced to hand movements with needling. Ophthalmic Surg 1993;24:279–8. following trabeculectomy bleb needling a left relative aVerent pupillary defect (RAPD). There was a large subconjunctival Spontaneous intracorneal haemorrhage EDITOR,—Transconjunctival needling of trab- haemorrhage, a total hyphaema, and IOP of 7 eculectomy blebs is a relatively safe, simple mm Hg. There was no fundal view but B scan outpatient procedure that can successfully EDITOR,—Spontaneous intracorneal haemor- ultrasound showed vitreous haemorrhage and rhage leading to corneal discoloration is an re-establish aqueous flow in failed haemorrhagic choroidal detachments (Fig 1). 12 uncommon occurrence. The few such reported trabeculectomies. We report a severe de- Further direct questioning revealed that the layed suprachoroidal haemorrhage occurring cases of spontaneous intracorneal haemorrhage patient was taking 75 mg of aspirin “for his have been due to contact lens related deep stro- secondary to this procedure in an aphakic heart” on his family doctor’s advice. patient receiving aspirin therapy. mal neovascularisation, erosion of a vessel due The patient was managed conservatively to corneal ulceration, and rupture of reopened with serial ultrasound examinations. Despite ghost vessels in a patient with interstitial kerati- CASE REPORT initial subjective improvement in vision, the tis and systemic hypertension. Corneal blood Fifteen years previously a 75 year old myopic sight remained reduced at hand movements staining clinically represents a reddish-brown, man underwent bilateral intracapsular cata- with a persistent RAPD and a soft eye. B scan or greenish-yellow discoloration of the cornea ract extractions. He developed secondary ultrasound 4 months after needling showed an resulting from blunt trauma and subsequent open angle glaucoma but was intolerant of open funnel retinal detachment (Fig 2) which, hyphaema with raised intraocular pressures or topical â blockers because of bradycardia. He in view of the poor visual prognosis, was not less commonly by intrastromal haemorrhage in was managed on pilocarpine drops 4% four felt to be amenable to vitreoretinal surgery. the presence of corneal vascularisation.1 The times daily but control of intraocular pressure term “corneal blood staining” has been used to http://bjo.bmj.com/ (IOP) was inadequate with deterioration in COMMENT refer only to the latter in this case report—a visual fields. Twelve years after the cataract Delayed suprachoroidal haemorrhage is a well case of spontaneous intracorneal haemorrhage extractions he underwent bilateral trabeculec- recognised but fortunately rare complication related to acne rosacea associated corneal tomies with postoperative 5-fluorouracil. of all forms of intraocular surgery, especially vascularisation. Three years later the left visual acuity was 6/18 filtering procedures. Pathological study of eyes with an IOP of 22 mm Hg despite pilocarpine. enucleated within hours of the haemorrhage In view of progressive cupping of the left optic CASE REPORT occurring have suggested the cause to be rup- A 72 year old man was seen at the eye casualty disc in association with this pressure, the ture of necrotic posterior ciliary arteries.3 A unit witha3weekhistory of reduced vision on October 2, 2021 by guest. Protected copyright. patient was oVered needling of the left number of risk factors for delayed supra- filtering bleb. and ocular discomfort in his left eye. There choroidal haemorrhage have been reported was no history of trauma. including aphakia, high myopia, a large perop- His ocular history included chronic posterior erative reduction in IOP, postoperative hypo- 34 blepharitis and peripheral corneal ulcers (upper tony, and systemic vascular disease. The cornea) in the left eye related to acne rosacea. patient reported here was myopic, aphakic, This patient had also had an uneventful had ischaemic heart disease and additionally cataract surgery in the same eye some 3 years was on aspirin. previously and had not been seen in the eye Two cases of haemorrhagic choroidal de- 12 department since. Relevant medical history tachments have been reported after bleb includes treatment for paroxysmal atrial fibril- needling with adjunctive mitomycin C. Pre- lation with sotalol. The patient had been taking cise details of the individual cases were not supplied, however, so it is not clear if these patients had predisposing risk factors or the result of their final visual outcome. A large choroidal eVusion occurring after bleb nee- dling has been reported in a pseudophakic patient,5 the eVusion resolving after surgical reformation of the anterior chamber. Our patient was managed conservatively owing to early subjective improvement in his visual Figure 1 B scan ultrasound examination of the acuity. It is possible, however, that the patient’s left eye 24 hours after trabeculectomy outcome may have been improved with surgi- bleb needling and 17 hours after he noticed sudden pain and loss of vision in that eye. cal drainage of the suprachoroidal haemor- Findings are consistent with vitreous rhage at an early stage, as has been advocated haemorrhage and haemorrhagic choroidal by some authors.4 The contribution that aspi- detachments. rin played in the development or exacerbation Figure 1 Left eye on presentation. 758 Letters

cornea. The contribution of systemic factors hyphaema, therapeutic eVorts are directed 2

such as diabetes or hypertension is unclear. towards prevention of corneal blood Br J Ophthalmol: first published as 10.1136/bjo.83.6.753c on 1 June 1999. Downloaded from Acne rosacea is known to cause peripheral staining—for example, treating the corneal vascularisation especially involving the infero- ulcer vigorously, correction of entropion or nasal and inferotemporal quadrant. These treatment of systemic hypertension.27 The vessels are known to progress in the absence of presence of deep stromal vascularisation acute symptoms.3 In our patient, the corneal secondary to any cause must be watched care- blood staining was a result of direct bleeding fully and managed as a potentially vision into the corneal stroma from the deep stromal threatening complication especially in contact vessels. The deep stromal vascularisation lens wearers. Once intracorneal bleeding has appears to have developed insidiously as in occurred, Giessler et al advise waiting for a similar cases reported subsequent to contact spontaneous clearing, although it may take 2 lens wear.23 Corneal blood staining either or 3 years or more.7 In the absence of severe Figure 2 The same eye 7 months later showing from persisting hyphaema or deep intra- associated pathology, corneal blood staining shrinkage of the area of staining and underlying corneal haemorrhage represents deposition of has been noted to clear without permanent stromal vessels. haemoglobin and its breakdown products corneal opacity changes.4 Penetrating kerato- within the cornea.4 A histopathological analy- warfarin until 2 months before the original plasty may be considered. sis of blood stained corneas, most of which presentation. The clinical findings were left eye were associated with raised intraocular pres- V SUDHA visual acuity −6/60 (Sn) improving to 6/12p S E P BURGESS sures, indicated a gradient of haemoglobin with a pinhole; circumciliary congestion and an Department of Ophthalmology, Princess Margaret degradation from the posterior to the anterior area of reddish-brown discoloration (6.0–6.5 Hospital, Swindon mm) inferotemporally on the cornea, clinically corneal stroma, extracellular haemoglobin particles being concentrated more posteriorly Correspondence to: Miss S E P Burgess, Princess resembling corneal blood staining. The in- Margaret Hospital, Okus Road, Swindon, Wiltshire traocular pressure was within normal limits and while haemosiderin laden keratocytes pre- SN1 4JU. 1 no other ocular abnormality was detected. The dominated anteriorly. Animal model experi- Accepted for publication 8 February 1999 other eye had a visual acuity of 6/6p with ments in rabbits utilising total persistent pinhole and appeared to be normal. On review, hyphaema with sustained increased intraocu- 2 months after initial presentation, he was lar pressures have also revealed similar 1 Messmer EP, Gottsch J, Font RL. Blood staining noted to have shrinkage of the area of results.5 Endothelial degeneration accompa- of the cornea; A histopathologic analysis of 16 discoloration revealing underlying prominent nies corneal blood staining and keratocytes cases. Cornea 1984;3:205–12. 2 Donnenfield ED, Ingraham H, Perry HD, et al. superficial and deep stromal corneal vessels appear to be actively involved in haemoglobin Contact lens-related deep stromal haemor- 5 adjacent to the area of discoloration, and some degradation. Porphyrin induced photosensi- rhage. Ophthalmology 1991;98:1793–6. lipid deposition close to the deeply vascularised tivity producing cytotoxic oxygen species 3 Kanski JJ, ed. Clinical ophthalmology, a systematic limbus. On further follow up 5 months later, within the blood stained cornea have also been approach. 3rd ed. Oxford: Butterworth- the patient had retained the same visual acuity considered as contributing to endothelial and Heinemann, 117–18. 6 4 McDonnell PJ, Green WR, Stevens RE, et al. of 6/60 (Sn) improving to 6/12p with pinhole. keratocyte degeneration. Clearing of blood Blood staining of the cornea: light microscopic Though the ciliary congestion persisted, the staining is thought to be a result of the phago- and ultrastructural features. Ophthalmology patient was not in any discomfort. The area of cytic action of the keratocytes and from a dif- 1985;92:1668–74. discoloration had a greenish-yellow tinge now 5 Gottsch JD, Messmer EP, McNair DS, et al. fusion of haemoglobin into the conjunctival Corneal bloodstaining. An animal model. and measured 5.7–4.2 mm. 7 circulation and the anterior chamber. The Ophthalmology 1986;93:797–802. pattern of peripheral, posterior, and anterior 6 McDonnell PJ, Gritz DC, McDonnell JM, et al. COMMENT stromal clearing observed seems consistent Fluorescence of blood-stained cornea. Cornea with diVusion of haemoglobin breakdown 1991;10:445–9. Deep intracorneal haemorrhage is most often 7 Giessler S, Gross M, Struck HG. [Spontaneous seen after intraocular surgery, after direct, products out of the cornea as the primary hematocornea after keratitis of various aetiology.] blunt ocular trauma, and in a vascularised mechanism of clearing.4 In the absence of a Klin Monatsbl Augenheilkd 1997;211:65–7. http://bjo.bmj.com/ on October 2, 2021 by guest. Protected copyright.