Uncommon Causes of Treatable Maculopathy
GS Bajwa & Nidhi Mittal

The main symptom of maculopathy is impairment of central vision.  The patient complains of positive scotomas, metamorphopsia, micropsia and macropsia.  The common cause encountered are central serous retinopathy, age related macular degeneration, cystoid macular edema and myopic maculopathy.  Four patients presented to us with symptoms of maculopathy, but were found to hve uncommon manifestations.    

CASE I
A male patient aged 27 years presented with sudden diminution of vision in left eye.  Patient had loss of vision in right eye 1 year back, which recovered completely.  On examination the patient had visual acuity of 6/36 in left eye.  Fundus examination showed serous retinal detachment in left eye.  Right eye showed two lesions- grey, yellow, indistinct invloving inner choroid and retinal pigment epithelium (RPE).

Fundus fluorescein angiography of left eye showed hyperfluorescence just above the disc which increased in intensity in thelater phase and small pinhead size area of hyperfluorescence temporal to fovea.  Right eyes showed two big lesions of one disc diameter.

This patient was diagnosed as punctate inner choroidopathy and was put on steroids 60 mg orally and gradually tapered off.  Vision improved to 6/6.

Punctated inner choroidopathy forms a part of Multiple Evanescent White Dot Syndrome.  Clinically and angiographically the disease process involves RPE and outer retina1. The electrophysiologic results also confirm the abnormality at the level of RPE- photoreceptor complex.  It is suggested to be inflammatory disease of choroid2, particularly of intermediate choroidal layer situated between the choriocapillaris and large choroidal vessels, that may alter the blood flow. The major impact of disease on vision is attributable to dysfunction at the level of photoreceptor outer segment3.

CASE II
A male patient aged 36 years presented with history of loss of vision in left eye one month back.  Visual acuity was less than 6/60.  Fundus examination showed irregular whitish subretinal lesion.  FFA showed two hyper-fluorescent spots around grayish lesions in early stage while late stage showed hyperfluorescent staining of sub retinal lesion.  This patient was diagnosed as sub retinal fibrosis and was put on steroids and gradually tapered off over 4 weeks period.  Vision improved to 6/9.

Fig.2: Fundus photograph showing Subretinal fibrosis.

Subretinal fibrosis is a rare distinct disorder4 characterized by multiple small whitish yellow retinal pigment epithelial or choroidal lesions in the posterior pole and mid periphery in early stage and progressive fibrosis in late stage.  Sub retinal fibrosis may be observed as last stage in the spectrum of the disorder termed multifocal choroiditis5, rather than being a unique entity itself.  This is probably due to localized autoimmune antibody mediated inflammation with destruction of the RPE 4,6,7. The antibodies may be produced by plasma cells, which then destroy the retinal pigment epithelium and produce subretinal fibrosis.

CASE III
A male patient aged 45 years presented with sudden loss of vision in left eye.  Patient was able to see peripheral hands but face could not be detected.  Visual acuity was 6/36. Fundus examination revealed a reddish lesion of approximately 4 disc diameter size below the left disc while fovea showed serous detachment of retina.  FFA showed hyperfluorescence below the disc, surrounded by a rim of hypofluorescence. A small area showed few spots of hyperfluorescence temporal to the disc.

This patient was diagnosed as choroidal haemangioma and photo coagulation was done surrounding the lesion.  Girdle was created between the tovea and the lesion.  After six weeks vision improved to 6/12.

Choroidal Haemangioma is a rare benign tumour which presents in adults with unilateral visual impairment or may be an incidental finding.

It is observed as smooth elevated dome shaped or placoid red choroidal mass which blends with the surrounding choroid, most commonly located as posterior pole.  Macular changes result from retinal detachment form tumour and in case of subfoveal tumour from degeneration of overlying retina 8,9. Other macular changes in form of hard exudates, RPE changes, epiretinal membrane formatio, chronic cystic changes, secondary cystoid retinal degeneration and exudative retinal detachment may be seen.

Fig.3: Fundus photograph showing Choroidal Haemangioma

CASE IV
A male patient aged 42 years presented with diminution of vision in left eye since one month.  Visual acuity was 6/60.  Fundus examination showed serous detachment of fovea.  FFA showed hyperfluorescence at two spots half disc diameter in size with finger like projections nasally pointing to the diagnosis of idiopathic polypoidal choroidal vasculopathy.

Idiopathic polypoidal choroidal vasculopathy is a pecuilar10 haemorrhagic disorder of the macula, earlier classified as posterior uveal bleeding syndrome11 and multiple recurrent RPe detachment12. Although pathogenesis is unknown, the primary abnormality involves choroidal circulation.  The characteristic lesion is a inner choroidal vascular network of vessels ending in an aneurysmal bulge, visible clearly as reddish orange spheroid polyp like structure.  The disorder is characterized by multiple serosanguinous detachments of the pigment epithelum and neurosensory retina, secondary to leakage and bleeding from the peculiar choroidal vascular abnormality.  Vitreous haemorrhage, minimal fibrous scarring, absence of drusen, retinal vascular disease and signs of intra ocular inflammation are other common features of this maculopathy.

Fig.4: Fundus photograph showing Idiopathic polypoidal choroidal vasculopathy.   

Many of the patients of maculopathy who present with serous retinal detachment / central serous retinopathy like picture, should be looked for associated features like haemangioma, space occupying lesion, multiplicity of  lesions as discussed as above.  These may be uncommon features but are readily treatable and can lead to improvement of vision in many cases.  As seen above four patients of maculopathy presented to us with such uncommon manifestation and were treated with steroids and photo coagulation with favourable outcome.  

REFERENCES

  1. Watzke RC, Parker AJ, Folk JC et al. Punctate inner choroidopathy. Am J. Ophthalmol 1984; 198:572.

  2. Obana K, Kusumi M, Yamaguchi M, Miki T. Indocyanine green angiographic aspects of multiple evanescent white dot syndrome.  Retina 1996; 16: 97-104.

  3. Horiguchi M, Miyakie Y, Nakamura M, Fujii Y. Focal elec troretinogram and visual field defect in MEWDS. Br. J. Ophthalmol 1993; 77:452-455.

  4. Palestine AG, Nussenbdatt RB, Parver LM< Knox DL Progressive sub retinal fibrosis.  Br. J Ophthalmol 1984; 168:667-673.

  5. Singerman LJ. Discussion of Morgam CM, Schatz H: recurrent multifocal choroiditis.  Ophthalmology 1986; 93:1143-1147.

  6. Kim MK, Chan CL, Belfort R et al. Histopathologic and immuno-histopathologic features of sub retinal fibrosis and uveitis syndrome.  Am J Ophthalmol 1987; 4: 15-23.

  7. Cantrill HL, Folk JC. Multifocal choroditis associated with progressive sub retinal fibrosis.  Am J Ophthalmol 1986; 170-180.

  8. Witschel H, Font RL.  Haemangioma of the choroids.  A clinicopathologic study of 71 cases and a review of literature. Surv. Ophthalmol 1976; 20-415.

  9. Mc Clean AL, Maumenee AE.  Haemangioma of choroid.  Am J. Ophthalmol 1960; 50:3.

  10. Yannuzzi LA. Idiopathic polypoidal choroidal vasculopathy presented at 1982 Macular Society Meeting, Miami, Florida.

  11. Kleiner RC, Brucker AJ, Johnston RL.  Posterior uveal bleeding syndrome.  Ophthalmology 1984; 91 (Suppl 9): 110.

  12. Stem RM, Zakov N, Zegarra H, et al. Multiple recurrent serous sanguinous retinal pigment epithelial detachments in black women.  Am J. Ophthalmol 1985; 100: 560-569. 

Address for Correspondence
Dr. G.S. Bajwa, Deptt. of Ophthalmology,
Dayanand Medical College & Hospital, Ludhiana.