Bilateral, recurrent Cytomegalovirus retinitis in Dyskeratosis congenita healing with extensive dystrophic calcification.

PURPOSE: To report a case of bilateral, recurrent, Cytomegalovirus (CMV) retinitis in Dyskeratosis Congenita (DKC) healing with rapid calcifications.

METHODS

a case report.

RESULTS

Case report: A 25-year-old man diagnosed with DKC, having severe bone marrow hypoplasia, presented with recurrence of CMV retinitis in both eyes. He was treated four months back for CMV retinitis with systemic and bilateral intravitreal antiviral therapy for eight weeks. Dilated fundus examination showed large areas of retino-choroidal scarring with refractile calcific deposits suggestive of calcific dystrophy within healed retinitis lesions. At the margins of these healed lesions, eruptions of new granular retinitis were seen. After restarting intravitreal anti-viral therapy, these lesions showed a rapid response and healed with retinal atrophy and new calcific deposits within a few weeks. These calcium deposits were visualised as hyper-reflective deposits in atrophic retinal layers on optical coherence tomography. Despite resolution, the final visual acuity was associated with visual morbidity owing to calcification of retinitis lesions. He developed septic shock and disseminated intravascular coagulation, leading to his unfortunate demise during treatment.

CONCLUSION

This case highlights recurrent CMV retinitis with rapid presumed dystrophic calcification in Dyskeratosis congenita with progressive bone marrow hypoplasia.