Diagnosis and management of serpiginous choroiditis

Abstract

Purpose: Serpiginous choroiditis (SC) is a rare, bilateral, idiopathic inflammatory disorder that causes geographic destruction of the retina and choroid often in healthy middle-aged patients. Symptoms include blurred vision, central and paracentral scotomas. SC is part of a group termed white dot syndromes which involves inflammation of the retina and choroid with the appearance of white dots in the fundus. SC requires a thorough workup prior to treatment to rule out other inflammatory autoimmune or infectious etiologies. Early stages are challenging to diagnose. Treatment includes corticosteroids and immunosuppressive therapy. The exact cause of Serpiginous Choroiditis is not known, yet HLA-B7 is typically positive indicating an autoimmune response in these patients. Choroidal neovascularization and cystoid macula edema are side effects, so close monitoring is important. This case presents a holistic clinical picture of SC to aid in better clinical diagnosis and understanding of this condition for the primary care eye clinician. Results: Presentation of a young otherwise healthy 37-year-old male patient to our clinical service revealed a serpentine shaped retina presentation absent of anterior chamber or vitreous inflammation. Fundus photos, fluorescein angiography, and ocular coherence tomography revealed serpiginous choroiditis. Primary care workup to rule out systemic conditions was unremarkable. Initial treatment with monitoring included intravitreal triamcinolone 2 mg/0.05 mL OU with oral chlorambucil therapy immediately afterwards. Active lesions resolved over the next 3 weeks, yet visual acuity showed no improvement as the foveal was already involved. The goal of chlorambucil therapy for 3 months was to induce sustained remission. Conclusion: Prognosis of SC is less favorable when the macula is involved (macular SC). Upon clinical presentation of serpiginous choroiditis, it is important to establish etiology of non-infectious autoimmune disorders or infectious disorders before classification of idiopathic SC and aggressive treatment to address underlying systemic conditions.