Euretina Meeting 2013 Hamburg
Miles StanfordMedical Eye Unit
St Thomas’ HospitalLondon
Serpiginous choroiditis
Serpiginous choroiditis
• Rare
• Bilateral
• 40-60 years
• Mainly caucasian
• Slight preponderance for men
Serpiginous choroiditis - pathology
• Little available
• Widespread atrophy of photoreceptors, RPE and choriocapillaris
• Lymphocytic infiltration of the choroid
• Secondary choroidal neovascularisation
Serpiginous choroiditis – clinical features
• Unilateral decrease in central vision, metamorphopsia or scotoma
• Little anterior segment reaction• Lesions classically peripapillary and then
spread outwards• Disease progression is stepwise and
asymmetric• Eventually permanent scar and subretinal
fibrosis
Serpiginous – progression over 6 months
Serpiginous choroiditis – stepwise progression over 18 months
Fluorescein angiography showing early masking and late staining on the edge of a old
scar
Serpiginous – FFA staining at the edge of an old scar. These changes may be more evident on ICG
Serpiginous choroiditisdifferential diagnosis
• APMPPE• Myopia• Choroidal ischaemia• Sarcoidosis• Toxoplasma• Tuberculosis/syphilis• Metastases/lymphoma• Retinochoroidal dystrophies
Ampiginous choroiditis (mantoux 20mm, subsequently developed Eales’ disease)
Serpiginous-like choroiditis and TB• Presumed uveitis due to TB: All patients with 1 year
follow up, exclusion of other infections, +ve Mantoux, no recurrence after full anti TB treatment
• 26/192 (15%) patients with presumed TB-related posterior uveitis had serpiginous like choroiditis (OR 26; 95% CI 7.4-91.4)
• Sensitivity 14%: specificity 98%: positive predictive value 56%
• Not a good sign for screening but makes diagnosis 90% certain if positive
Gupta A et al Am J Ophthalmol 2010 149:562
Serpiginous-like choroiditis and TB
• 11/21 (52%) patients tested +ve with Quantiferon compared to 9% HC and 13% uveitis controls
• 3/11 improved with specific anti-TB treatment
• ?directly due to bacteria or allergic response
Mackensen F et al Am J Ophthalmol 2008 146;761
Serpiginous-like choroiditis and TB
• Comparison of 5 patients with serpiginous like (SLC) and classical serpiginous (SC)
• Patients with SLC were: - most likely to have come from a country
where TB endemic - To have unilateral multifocal disease with
significant vitritis - to have a positive PPD - to respond to tuberculostatic therapy
Arch Ophthalmol 2010 128: 853
Serpiginous choroiditisInvestigations
• FFA
• ICG
• OCT
• Electrodiagnostics
• Visual fields
• Mantoux/IFN gamma
Serpiginous choroiditis - complications
• CNVM occurs in 15-35%
• Usually arises from the edge of a scar but may be peripapillary
• Serous retinal or RPE detachments
• Subretinal fibrosis
• Rarely, CMO or NVs
Serpiginous choroiditisTreatment
• Goals of therapy are to control active lesions rapidly and to prevent further recurrences and progression
• Steroids – oral or pulsed
• Other immunosuppressives
• Infliximab
• Treatment for secondary neovascularisation
Serpiginous choroiditis - prognosis
• Very few long term studies
• Chronic, progressive disease in a stepwise manner
• Active lesions usually resolve over 3-6 months but may take longer
• Extrafoveal lesions may not give rise to symptoms and so pass unrecognised
Serpiginous choroiditis - Conclusions
• Rare, progressive disease of the middle-aged
• Must exclude TB especially if patient from endemic area
• Treat with standard immunosuppressives to control active lesions and prevent progression
• Potential for secondary CNVM