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ORIGINAL ARTICLE
Year : 2002  |  Volume : 50  |  Issue : 3  |  Page : 189-196
 

Macular serpiginous choroiditis


Retina-Vitreous Services, Manipal Hospital, Airport Road, Bangalore-560 017, India

Correspondence Address:
Dinesh K Sahu
Retina-Vitreous Services, Manipal Hospital, Airport Road, Bangalore-560 017
India
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PMID: 12355692

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  Abstract 

Purpose: To report a variant form of serpiginous choroiditis, that initially or predominantly involved the macular area. Methods: Nine eyes of 6 patients with the macular form of serpiginous choroiditis were evaluated clinically and angiographically in a longitudinal fashion for a period of 12-36 months. The active stage and the recurrences were treated by oral and periocular cortico steroids; and two patients were supplemented with oral azathioprine. Most of these patients were referred to our center with varied diagnoses. Results: In this group, 4 were male and 2 were female with an average age of 30.5 years. Three patients had bilateral macular lesions, two had typical serpiginous choroiditis in the fellow eye and the remaining one had unilateral macular involvement alone. The initial visual acuity was 6/60 or less in 60% eyes whereas the final visual acuity was 6/18 or better in 66% eyes. Angiographic findings were typical of serpiginous choroiditis characterised by early hypofluorescence followed by leakage and staining of the borders and the lesion itself without any evidence of choroidal ischaemia or retinal vascular abnormalities. Conclusion: The macular variant of serpiginous choroiditis can mimic many other macular pathologic lesions, thus posing a diagnostic dilemma. Because of its relentless destructive course, early diagnosis and prompt treatment is required to prevent sight-threatening complications.


Keywords: Macular serpiginous choroiditis, geographical helicoid peripapillary choroidopathy, subretinal neovascularisation, immunosuppressants


How to cite this article:
Sahu DK, Rawoof A, Sujatha B. Macular serpiginous choroiditis. Indian J Ophthalmol 2002;50:189-96

How to cite this URL:
Sahu DK, Rawoof A, Sujatha B. Macular serpiginous choroiditis. Indian J Ophthalmol [serial online] 2002 [cited 2014 Nov 23];50:189-96. Available from: http://www.ijo.in/text.asp?2002/50/3/189/14788


Geographic Helicoid Peripapillary Choroidopathy (GHPC) is a rare, usually bilateral, chronic, progressive and recurrent disease of unknown aetiology affecting the retinal pigment epithelium, choriocapillaris and choroid. It characteristically starts at the juxtapapillary or peripapillary region and progresses centrifugally from the disc to involve the macula area.[1][2][3] It is also known as serpiginous choroiditis because of its peculiar extension in a serpiginous fashion. The disease has a relentless destructive course. As the lesions resolve, retinal pigment epithelial and choroidal degeneration begin, leading to fibrous scarring and pigment hyper or hypoplasia.[1-6] Subretinal neovascular membrane (SRNVM) formation may occur after a chronic course.[7][8][9][10] Recurrences at intervals of months to years are a rule and new lesions start from the inactive borders of old lesions.[1][2][3]

On rare occasions, serpiginous choroiditis may present initially as lesions involving the macula exclusively.[11],[12] We describe an additional six such patients who presented to us predominantly with macular lesions. This report describes the clinical profile, fluorescein angiographic features and course of the disease in Indian patients, and emphasizes its early recognition to avoid inappropriate management. This is also the first report of any form of serpiginous choroiditis described in Indian patients (Medline search).


  Materials and Methods Top


The study included 6 patients with serpiginous choroiditis with predominantly macular involvement. They were seen in the retina-vitreous unit of our hospital between January 1998 and December 2000 and their charts were reviewed retrospectively. We included in this study only those patients who were observed clinically and angiographically to have serpentine or geographical lesions in the macular area; and/ or showed one or more recurrences. Each of these patients provided a detailed medical and ocular history. Each patient underwent a complete ophthalmic examination including best corrected visual acuity, slitlamp biomicroscopy, intraocular pressure, and fundus evaluation by indirect ophthalmoscopy and with a +90D lens. In addition, fundus photographs were taken, fluorescein angiography was performed; and B-scan was done wherever indicated. At the follow-up visits, all procedures were repeated.

All the cases were treated with oral prednisolone (1mg/ kg.body wt.) tapered over a 3-month period, and 3-6 posterior subtenon (PST) injections of triamcinolone (20mg) given at two-weekly intervals. The PST was given by a 26-gauge needle after anaesthetizing the ocular surface with topical lidocaine 4%. Eye speculum was used for the proper exposure and the patient was then asked to look in the infero-nasal direction (in relation to the eye being treated). The needle was passed through the bulbar conjunctiva in the supero-temporal quadrant into the sub-Tenon space keeping the bevel edge towards the globe. The needle was then advanced posteriorly with a side-to-side movement while maintaining the needle tip adjacent to the globe. It was advanced posteriorly till the hub was adjacent to the entry point in the bulbar conjunctiva and the drug was then injected into the posterior subtenon space.

Two patients also received oral azathioprine (2mg/ kg.body wt.) which was tapered over 6 months. These two patients did not show any significant improvement with corticosteroid treatment alone in a 2-4 week period. These cases were monitored constantly by an internist to evaluate any systemic side effects of the drugs while under treatment. The monitoring was done by evaluating complete blood counts and peripheral smear at weekly intervals for the first month and at two-weekly intervals thereafter; and liver function tests at monthly intervals with the baseline values taken at the initiation of the therapy.

The course of the disease was monitored using Amsler's chart, fundoscopy and fluorescein angiography wherever required. The patients were diagnosed to have recurrence when there was defective vision of acute onset or any sign of activity noticed clinically and/or angiographically. These recurrences were treated with oral prednisolone (1 mg/kg body wt) tapered over 10 - 12 weeks, and 1-2 posterior subtenon injections of triamcinolone (20mg) at two-weekly intervals.

An extensive search was made for any underlying systemic cause. The internist and rheumatologist's opinions were sought. The laboratory investigations included routine blood investigations, Mantoux test, serology for tuberculosis, syphilis, sarcoidosis, toxoplasmosis and a chest X-ray. Additional tests included serum assay for antinuclear antibody, LE cells, rheumatoid factor, and ELISA for HIV, herpes and cytomegaloviruses. Most of these patients were referred to our center with varied diagnoses for further evaluation and management [Table - 1].


  Results Top


All the 6 patients showed lesions in one or both eyes that were similar in character to those of typical serpiginous choroiditis [Figure:1a] except that they were located at the macula initially or exclusively [Figure:1b]-[Figure:1e], [Figure:2a]-[Figure:2d]. These lesions were sharply demarcated, greyish-yellow, discoid or confluent translucent patches with serpentine-like borders, situated deep to the retina in the macular area. The retinal vessels were unaffected but the overlying retina was oedematous [Figure:1b], [Figure:2a]. Angiographic features included hypofluorescence of the lesion in the early-phase; and hyperfluorescent borders with mild leakage in the mid-phase. The late-phase showed a patchy or stippled hyperfluorescent pattern inside the lesion and later the entire lesion became hyperfluorescent with fuzzy borders [Figure:1c], [Figure:1d], [Figure:2b], [Figure:2c].

Of the 6 patients, three had bilateral involvement; two had unilateral macular lesion with old lesion of typical serpiginous choroiditis in the fellow eyes, and the remaining patient had unilateral macular involvement only. Two of 3 bilateral cases had simultaneous bilateral involvement whereas in one case the fellow eye developed the disease five months after the cessation of treatment for the affected eye. Thus a total of 9 eyes were found to have macular serpiginous choroiditis. [Table - 1] shows the clinical profile, angiographic features and course of the disease in the present series.

The age of the patients ranged from 16 - 45 years (average 30.5 years). Four patients were male and two were female. The chief presenting complaints were metamorphopsia, rapid blurring of vision or central scotoma. The duration of the symptoms at the time of presentation to our hospital varied from one to three weeks as most were referral cases. The visual acuity at the time of presentation ranged from 6/24 to 4/60. The IOP was within normal limits, the anterior segment examination was unremarkable while scanty cellular reaction was seen in the vitreous cavity. Fundus examination revealed well circumscribed, yellow-white to grey, discoid or geographic lesions with pseudopodia-like borders deep to the retina involving the macular area with characteristic angiographic features of GHPC [Figure:1b], [Figure:1d], [Figure:2b], [Figure:2c]. The retinal vessels were unaffected but the overlying retina was oedematous [Figure:1b], [Figure:2a].

The medical examination and laboratory investigations were inconclusive of any underlying cause. All the patients were treated with oral prednisolone, and 3-6 PST injections of triamcinolone given at two-weekly intervals. Two patients also received oral azathioprine. These two patients did not show any significant improvement with steroid treatment alone in a 2-4 week period. All patients responded well to the above therapeutic regimen during follow-ups. There was no significant change in the IOP in any of the eyes while under treatment and it remained within normal limits. The treatment end point was complete resolution of the lesions observed clinically or angographically; and/or stabilisation of the visual acuity for consecutive 3 to 4 weeks. The patients were monitored closely by an internist while under treatment, but they did not show any significant side effects of the drugs.

As the lesions resolved, chorioretinal degeneration ensued and substantial amount of RPE degeneration with or without fibrosis was observed. The cases were followed up for 12-36 months (average 18 months) after the resolution of the first episode and during this period 5 (56%) eyes showed recurrences. The new area of activity started either as extensions from the inactive edges of the old lesion or inside the lesion itself. In two of them, new lesions extended towards the disc [Figure:2d] whereas one eye had recurrence with disc oedema and peripapillary vasculitis [Figure:1e]. These recurrences were treated with oral corticosteroids and PST injections of triamcinolone and responded well with complete resolution of the lesions within 2-6 weeks. However, the final visual acuity was marginally less in these eyes compared to the eyes without recurrences [Table - 1]. None of the eyes showed any evidence of subretinal neovascular membrane formation clinically or angiographically during follow-ups. Six eyes (66%) attained a visual acuity of 6/18 or better at the final visit, whereas three eyes (33%) attained only 6/60 or less due to extensive macular degeneration and fibrosis [Table - 1].


  Discussion Top


Serpiginous choroiditis is a rare, chronic, progressive, recurrent disease affecting young to middle aged, otherwise healthy individuals. It is usually bilateral.[1][2][3],[13],[14] Active disease is characterized by grey-yellow discoid lesions deep to the retina with irregular borders. In its typical form it progresses centrifugally from the disc and impinges on the macula[1][2][3][4][5][6] and was observed in the fellow eye of case 3 as old lesions [Figure:1a]. Sometimes the disease may commence at the macula and progress centripetally. This variant form of the disease is termed as macular serpiginous choroiditis[11],[12] and was seen in 9 eyes of the present series.

Because of its location, the macular variant of serpiginous choroiditis differed from the typical form in many respects. Since it starts at the macular area it causes sudden loss of vision and hence the patient reports early in the course of the disease. In the typical form, the patient may present late in the course of the disease only when the macula is involved. The lesions in the macular form are often subtle and resemble many other macular pathological conditions whereas lesions of the typical type are classical and rarely pose any difficulty in establishing the diagnosis. The visual prognosis in the macular form is less favorable than in the typical form because of early involvement of the fovea. The features shared by both the forms of the disease are the age at onset; absence of gender or familial predilection; laterality, progression, good response to corticosteroids and/or immunosupressants; recurrences; and extensive RPE degeneration and scarring on resolution of the disease. Recurrences are frequent and new lesions are seen at the inactive borders of old lesions or within the lesion itself. Chronic cases may show subretinal neovascular membrane formation which may require laser photocoagulation.[7][8][9][10] Five eyes in our series showed recurrences but none of them developed SRNVM during the follow-up visits [Figure:1e], [Figure:2d].

The exact aetiology of the disease is unknown though it has been associated with various causes.[15][16][17][18] Serpiginous choroiditis appears to be an inflammatory disorder affecting the RPE and choroid, with an immune response produced by tissue or microbial agents and recurrences due to reactivation of these agents.[1][2][3],[7],[8],[16] In our series the medical history and laboratory investigations were inconclusive of any underlying aetiology.

Angiographic features of the active lesions are characteristic which reveals early hypofluorescence with hyperfluorscent borders. The late-phase shows patchy hyperfluorescence and staining of the whole lesion [Figure:1c], [Figure:1d], [Figure:2b], [Figure:2c]. Sometimes large choroidal vessels are also visible which may be due to the loss of retinal pigment epithelium and choriocapillaris [Figure:1c]. Since the lesions of macular serpiginous choroiditis are often subtle, angiography seems to be an important diagnostic tool which reveals the classical pattern of the disease.

There are a variety of macular lesions, that can mimic this variant form of serpiginous choroiditis. These include age-related macular degeneration, idiopathic subretinal neovascularization, idiopathic central serous retinopathy with exudation, retinal pigment epithelitis, posterior scleritis, toxoplasmic retinitis, presumed ocular histoplasmosis syndrome, tuberculosis, sarcoidosis, acute multifocal posterior placoid pigment epitheliopathy (APMPPE) and viral diseases.[19][20][21][22][23][24][25][26][27][28] [Table - 2] describes the differentiating features of macular serpiginous choroiditis.

Many therapeutic modalities have been suggested for the treatment of serpiginous choroiditis. Oral and periocular corticosteroids form the mainstay of treatment. Newer reports advocate immunosuppressive for rapid remission of the disease with minimal residual scarring.[29][30][31] To date, there has been no randomized controlled study to prove the efficacy of immuno-suppressants over oral corticosteroids. All our cases responded well to oral and periocular corticosteroids; and azathioprine was supplemented in two cases.

The disease should be considered in the differential diagnosis of inflammatory disorders of the posterior pole of the retina since the disease has a relentless, destructive course and can cause severe visual impairment. At the same time, it may pose a diagnostic dilemma because the lesions are subtle and may mimic many other macular pathological conditions [Table - 2]. Its early recognition, appropriate treatment and careful follow-ups are important to avoid sight-threatening complications.

 
  References Top

1.Schatz H, Maumence AE, Patz A. Geographical helicoid peripapillary choroidopathy: Clinical presentation and fluorescein angiographic findings. Trans Am Acad Ophthalmol Otolaryngol 1974;78:747-61.  Back to cited text no. 1    
2.Hamilton AM, Bird AC. Geographical choroidopathy. Br J Ophthalmol 1974;58:784-97.  Back to cited text no. 2  [PUBMED]  
3.Laatikainen L, Erkkila H. Serpiginous choroidits. Br J Ophthalmol 1974;58:777-83.  Back to cited text no. 3  [PUBMED]  
4.Chisholm IH, Gass JDM, Hutton WL. The late stage of serpiginous (geographic) chroiditis. Am J Ophthalmol 1976;82:343-51.  Back to cited text no. 4    
5.Weiss, Annesley WH JR, Shields JA, Tomer T, Christopherson K. The clinical course of serpiginous choroidopathy. Am J Ophthalmol 1979;87:133-42.  Back to cited text no. 5    
6.Laatikainen L, Erkkila H. A follow up study on serpiginous choroiditis. Acta Ophthalmol 1981;59:707-12.  Back to cited text no. 6  [PUBMED]  
7.Jampol LM, Orth D, Daily MJ, Rabb MF. Subretinal neovascularisation with geographic (serpiginous) choroiditis. Am J Ophthalmol 1979;88:683-89.  Back to cited text no. 7  [PUBMED]  
8.Bluemankranzh MS, Gass JDM, Clarkson JG. Atypical serpiginous choroiditis. Arch Ophthalmol 1982;100:1773-75.  Back to cited text no. 8    
9.Laatikainen L, Erkkila H. Subretinal and disc neovascularisation in serpiginous choroiditis. Br J Ophthalmol 1982;66:326-31.  Back to cited text no. 9  [PUBMED]  
10.Wu JS, Lewis H, Fine SL, Grover DA, Green DR. Clinicopathologic findings in a patient with serpiginous choroiditis and treated choroidal neovasularisation. Retina 1989; 9:292-301.  Back to cited text no. 10    
11.Hardy RA, Schatz H. Macular geographic helicoid choroidopathy. Arch Ophthalmol 1987;105:1237-42.  Back to cited text no. 11  [PUBMED]  
12.Mansour AM, Jampol LM, Packo KH, Hrismalos NF. Macular serpiginous choroidits. Retina 1988; 8:125-31.  Back to cited text no. 12    
13.Schatz H, McDonald HR. Geographic helicoid peripapillary choroidopathy (GHPC): Serpiginous choroiditis. In: Ryan SS, editor. Retina, 1st ed. St. Louis: CV Mosby, 1989. pp 705-13.  Back to cited text no. 13    
14.Nussenblatt RB, Whitcup MC, Palestine AG. Serpiginous choroiditis. In: Nussenblatt RB, editor. Uveitis: Fundamental and Clinical Practice, 2nd ed. St. Louis: Mosby Year Book Inc, 1996. pp 364-68.  Back to cited text no. 14    
15.Erkkila H, Laatikainen L, Jokinen E. Immunological studies on serpiginous choroiditis (Abstract). Graefes Arch Clin Exp Ophthalmol 1982;219:131.  Back to cited text no. 15  [PUBMED]  
16.Wojno T, Meredith TA. Unusual findings in serpiginous choroiditis. Am J Ophthalmol 1982;94:650-55.  Back to cited text no. 16  [PUBMED]  
17.Mulder CJJ, Pena AS, Jansen J, Oosterhuis JA. Celiac disease and geographic (serpiginous) choroidopathy with occurence of thrombocytopenic purpura. Arch Int Med 1983;143:842-44.  Back to cited text no. 17    
18.King DG, Grizzard WS, Sever RJ, Espinoza T. Serpiginous choroidopathy associated with elevated factor VIII von Willebrand factor antigen. Retina 1990;10:97-101.  Back to cited text no. 18    
19.Gass JDM. Pathogenesis of disciform detachment of neuro epithelium (Part I and III). Am J Ophthalmol 1967;63:573-711.  Back to cited text no. 19    
20.Green WR, Wilson DJ. Choroidal neovascularization. Ophthalmol 1986;93:1169-75.  Back to cited text no. 20    
21.Gass JDM. Bullous retinal detachment - An unusual manifestation of idiopathic central serous retinopathy. Am J Ophthalmol 1973;75:810-12.  Back to cited text no. 21    
22.Sahu DK, Namperumalswamy P, Hilton GF, de Sousa NF. Bullous variant of idiopathic central serous chorioretinopathy. Br J Ophthalmol 2000;84:485-92.  Back to cited text no. 22    
23.Watson DG. Doyne memorial lecture: The nature and treatment of scleral inflammation. Trans Ophthalmol Soc UK 1982;102:257-81.  Back to cited text no. 23    
24.Krill AE, Deutman AF. Acute retinal pigment epitheliitis. Am J Ophthalmol 1972;74:193-205.  Back to cited text no. 24  [PUBMED]  
25.Woods AC, Wahlen HE. The probable role of benign histoplasmosis in the etiology of granulomatous uveitis. Trans Am Ophthal Soc 1959;57:318-43.  Back to cited text no. 25    
26.Hogan MJ. Ocular toxoplasmosis : Clinical and laboratory diagnosis; evaluation of immunological tests; treatment. Arch Ophthalmol 1956;55:333-45.  Back to cited text no. 26  [PUBMED]  
27.Lyness AL, Bird AC. Recurrences of acute posterior placoid pigment epitheliopathy. Am J Ophthalmol 1984;98:263-67.  Back to cited text no. 27  [PUBMED]  
28.Gass JDM. Acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol 1968;80:177-85.  Back to cited text no. 28    
29.Secchi AG, Tognon MS, Maselli C. Cyclosporine A in the treatment of serpiginous choroiditis. Int Ophthalmol 1990;14:395-99.  Back to cited text no. 29  [PUBMED]  
30.Laatikainen L, Tarkkanen A. Failure of cyclosporine A in serpiginous choroiditis. Ocular therapy and Surg 1984;3:280-82.  Back to cited text no. 30    
31.Hooper PL, Kalpan HJ. Triple agent immunosuppresion in serpiginous choroidopathy. Ophthalmology 1991;98:944-51.  Back to cited text no. 31    


    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9]

    Tables

[Table - 1], [Table - 2]


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