Delhi Journal of Ophthalmology

Clinical Challenge

Pradeep Kumar1y, Co, Gaurav Verma1, Rakesh Maggon1, Shishir Narain2, Padmamalini Mahendradas3
1Dept of Ophthalmology, Command Hospital, Chandigarh, India;
2Shroff Eye Centre, New Delhi, India; 3Narayana Nethralya, Bangaluru, India

Corresponding Author:

Pradeep Thakurella MS, DNB (Ophthalmology)
Dept of Ophthalmology,
Command Hospital, Chandigarh, India
Email id: eyepradeep@yahoo.com

Received: 15-JUL-2019

Accepted: 18-AUG-02019

Published Online: 27-DEC-2019

DOI:http://dx.doi.org/10.7869/djo.491

Abstract

Keywords :

The following case is submitted by Dr. Pradeep and commented upon by Dr. Padmamalini Mahendradas and Dr. Shishir Narain.

Case Report

A 23-year-old male Asian Indian patient presented with bilateral progressive diminution of vision of five days duration associated with photopsia and global headache of mild severity. There was no history of redness of eyes, photophobia, preceding flu-like illness, skin rash, arthritis, backache, dysuria and contact with tuberculosis patients. The best-corrected visual acuity (BCVA) of right eye (RE) was 6/12 and left eye (LE) was 6/24. The pupillary reactions of both eyes were normal. Fine keratic precipitates with +2 anterior chamber cells and flare were seen. The optic disc in both eyes were hyperemic and edematous with blurring of the margins. The retinal vessels were normal. Multiple discrete whitish-yellow choroidal lesions were seen, extending from posterior pole to equator seen symmetrically in both eyes (BE). Minimal pre-retinal vitritis was seen. Spectral domain optical coherence tomography (OCT) of the lesions showed loss of ellipsoid zone, retinal pigment epithelium (RPE) hyper-reflectivity and homogeneity of the underlying choroid. The fovea of both eyes was involved with minimal ellipsoid zone disruptions. Fundus fluorescein angiography (FFA) showed early hypofluorescence and late hyperfluorescence at site of lesions with optic disc leakage in late phases. On short-wave fundus autofluorescence (FAF), the lesions appeared hyperautofluorescent centrally with hypoautofluorescent edges.
Based on the young age of patient, short disease duration of five days, associated photopsia and headache, bilateral presentation with mild visual loss, anterior uveitis,creamy choroidal lesions and disc leakage, a differential diagnosis of white dot syndrome versus multifocal choroiditis was further investigated. The haemogram and C-reactive protein levelswere normal. QuantiFERON-Tuberculosis (TB) Gold test levels were negative and contrast-enhanced computed tomography scan of chest was normal. The patient was negative for Human immunodeficiency virus (HIV), Syphilis, Lyme’s, Leptospirosis, toxoplasma, toxocara, herpes simplex virus, varicella zoster virus, Hepatitis B and Hepatitis C serology. Pulmonary function tests and serum angiotensin converting enzyme (ACE) levels were normal. Rheumatoid factor, anti-nuclear antibody (ANA) and cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) levels were normal.
Two days after the initial presentation, the number and size of lesions had increased and BCVA worsened by one line in both eyes. At this stage,the patient was started on intravenous methylprednisolone (IVMP) 1 gram per day for three consecutive days followed by oral prednisolone 1.5 mg/kg/ day. Seven days after starting treatment the BCVA improved to 6/9 in RE and 6/12 in LE. The anterior chamber reaction and vitritis got resolved in BE. Disc edema and hyperemia had reduced in BE. Choroidal lesions in BE became more well defined and started developing pigmentation of the edges. Autofluorescence imaging showed hyperautofluorescence of edges of lesions. The dose of oral steroids was reduced at 0.2 mg/kg/day every week. Over the next eight weeks patient received tapering oral steroids and showed improvement in symptoms.
At the end of 12 weeks from onset, BCVA BE was 6/9 with contrast sensitivity of 1.52 (mild reduction on Pelli Robson chart) and non-specific field defects spread over entire 30-2 perimetry (Humphrey’s visual filed). The vitritis did not recur after stopping steroids. The optic discs in BE had gliosis at margins. The choroidal lesions were faint, pigmented and well defined. The ellipsoid zone disruption at the site of lesions persisted.


Dr. Padmamalini Mahendradas
Brief Case summary: A 23 year Indian male presented with progressive bilateral vision loss associated with headache. Clinical evaluation revealed bilateral posterior uveitis (multifocal dendritic pattern of choroiditis lesions in the posterior pole with multiple choroiditis lesions in the midperiphery of the fundus ) with spill over inflammation into the anterior segment without any associated systemic disease. Clinical Diagnosis: Bilateral serpiginous-like choroiditis (SLC) Causes: Commonest etiology in our country is Tuberculosis, rarely viral& immune mediated diseases & very rarely fungal and toxoplasmosis can also cause SLC.

Laboratory Investigations:
  • Tuberculin skin reaction (Mantoux test), chest imaging (high resolution contrast enhanced computed tomography, HRCT), QuantiFERON-TB Gold test, Anterior chamber (AC) tap- polymerase chain reaction (PCR)- for mycobacterioum tuberculosis, endobronchial ultrasound (EBUS)may be ordered to rule out tuberculosis.
  • AC tap- PCR for viral genomes to rule out herpetic infections
  • Venereal disease research laboratory (VDRL) or rapid plasma reagin (RPR) along with Treponema pallidum haemagglutination (TPHA) or Fluorescent Treponema antibody absorption (FTA-ABS) to rule out syphilis.
  • Complete blood count (CBC), random blood sugar (RBS), urine microscopy, erythrocyte sedimentation rate (ESR) to assess the systemic status of the patient.
Ocular Investigations:
  • FAF of the fundus at presentation and during follow up visits to titrate the treatment till the lesions are completely hypoautofluorescent.
  • FFA at the time of presentation or in doubt when there is worsening of inflammation to assess the activity of the lesions.
  • Indocyanine green angiography can be done to assess the choroidal involvement.
  • OCT– Periodic follow up to monitor the changes in the outer retina and choroid.
  • Optical coherence tomography angiography (OCTA) can be done when we suspect the development of choroidal neovascular membrane ( CNVM)
  • Ultrasound B scan may be done to look for other optic nerve head pathologies.
  • Visual fields and color vision may also be done in this case in view of disc edema.
Management: After ruling out TB & viral infections, systemic steroid therapy is used to control the inflammation. The authors have used IVMP followed by oral steroids for three months. The fact that the patient responded well to treatment suggest the possibility of immune-mediated component of the disease. Day 56 fundus photograph shows healing lesions. Autofluorescence imaging would help assess the healing pattern in addition to clinical examination. The systemic steroids/ immunosuppressive therapy with or without anti-tubercular therapy may be required to control the choroiditis lesions in this case.



Dr. Shishir Narain

Salient features for aid in the diagnosis in this case:
  1. Young age
  2. Asian Indian Origin
  3. Bilaterality of involvement
  4. Panuveitis with predominant involvement at the level of the RPE and inner choroid
  5. Autoimmune as well as infectious serological workup is negative
  6. Good response to systemic steroids
Features supporting a diagnosis of Multifocal Choroiditis (MFC) are
  1. Dramatic response to steroids 2
  2. Relative asymmetry in distribution and simultaneous involvement of very large areas of mid periphery as well. A classic White Dot Syndrome (WDS) would be largely limited to the posterior pole.
  3. Autofluorescence pattern is classical of choroiditis lesions with RPE involvement. WDS would have had more numerous and finer multiple lesions rather than well defined larger geographical lesions seen here. Also varying patterns of different stages of lesions in the same eye are more suggestive of simultaneous healing and newer crop of choroiditis. MWS would have had a more uniform FAF pattern.
Further investigations
ICG Angiography would have provided further information. MWD would show a much larger number of lesions since the disease is more subclinical and self limiting. MFC would show patchy focal pattern of involvement similar to the fluorescein angiography pattern.

Diagnosis
Morphological diagnosis is Idiopathic Bilateral Panuveitis with Multifocal Serpiginoid (Serpiginous-like) Choroiditis. Considering the fact that this patient was of Asian- Indian origin and with the phenotypic presentation of a lesions coalescing in a serpiginous-like pattern, Multifocal Serpiginoid choroiditis/Serpiginous-like choroiditis would be first on the list of differentials. The RPE is the primary site of pathology here with concurrent inflammation of the outer retinal elements as seen on the OCT and uniform choroidal thickness without focal granulomas or diffuse thickening. The RPE has characteristics of mononuclear phagocytic system (MPS) with expression of Toll-like receptors and capability of engulfing organisms and therefore an infectious basis with local hypersensitivity is a possible mechanism of uveitis here.
Etiologic diagnosis is more challenging here. Workup for TB is negative, and so is it for other infective etiologies. PCR of aqueous obtained by AC tap for TB and other infectious organisms is an option especially in few cases with significant anterior chamber inflammation and/or if there are suspicious signs in HRCT/QuantiFERON-TB Gold/Mantoux test or contact history. Serology for herpetic viruses may not help much in the diagnosis.

Management:
I agree with the present management with initial IVMP to save central vision followed by full dose systemic steroids with a slow taper, given that it has had an excellent response to steroids. The authors should followup the patient on serial AF till all lesions show uniform hypoautofluorescence consistent with complete healing. An Amsler chart should be given to the patient especially for the left eye for possible reactivation and/or development of inflammatory choroidal neovascular membrane. If there is a recurrence, re-investigations/AC tap for various infectious etiologies including TB should be performed, and if ruled out can put the patient on long term steroid-sparing immunosuppressive therapy.




Section Editor (Dr Devesh Kumawat) Note
Dr. Pradeep presents a case of young healthy male developing bilateral simultaneous extensive multi-focal choroiditis lesions which showed a prompt resolution with systemic steroid treatment. Dr. Padmamalini Mahendradas and Dr. Shishir Narain present their views on this case.
Both the commenter’s believe that in the presence of features like bilateral disease, multifocal geographical choroiditis lesions in different stages of evolution, and panuveitis, a diagnosis of Multifocal Serpiginous or Serpiginous-like Choroiditis (SLC) is appropriate. On the other hand, the white dot syndromes will have uniform sized and shaped distinct lesions involving predominantly the posterior pole. The ocular investigations in such cases should include OCT, FAF, FFA and ICGA if deemed necessary, not only for the diagnosis but also for monitoring the treatment response. OCT shows disturbance of the outer retinal layers, RPE and choriocapillaris. FFA shows early hypofluorescence and late hyperfluorescence. Both the commenter’s believe that FAF is of utmost importance. The lesion activity correlates with the FAF response and with healing the lesions become hypoautofluorescent.
The consensus is to perform a thorough systemic work-up in cases of SLC to determine the etiology as well as to rule out systemic infective foci before starting immunosuppressive treatment. In case the systemic investigations turn out to be normal, or there is high suspicion, or the disease is recurrent, then ocular fluids such as AC tap may be used for TB and viral PCR.
Both commenters’ are clear in their assertion that SLC is usually an immune response to a systemic TB infection in our country. Therefore they recommend the line of treatment is systemic immunosuppression with steroids with or without anti-tubercular treatment. The steroids need to be tapered slowly depending upon the clinical response and FAF activity. Recurrent/ chronic cases may be managed with steroid sparing agents.
We thank Dr. Pradeep for the case, and Dr. Dr. Padmamalini Mahendradas and Dr. Shishir Narain for their expert opinion.

CITE THIS ARTICLE

Kumar P, Verma G, Maggon R, Narain S, Mahendradas PClinical Challenge.DJO 2019;30:79-82

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Kumar P, Verma G, Maggon R, Narain S, Mahendradas PClinical Challenge.DJO [serial online] 2019[cited 2020 Jan 25];30:79-82. Available from: http://www.djo.org.in/articles/30/1/case-report-2019-30-1_23.html