Giran Mannat, Jadhav Vishal, Patel Anamika, Kelgaonkar Anup, Pathengay Avinash
Uveitis, Vitreous and Retina Services, LV Prasad Eye Institute, Mithu Tulsi Chanrai Campus, Bhubaneswar, Odisha, 751024, India.
Uveitis, Vitreous and Retina Services, LV Prasad Eye Institute, GMR Varalaxmi Campus, Visakhapatnam, Andhra Pradesh, India.
Int Ophthalmol. 2025 Jun 20;45(1):250. doi: 10.1007/s10792-025-03634-y.
To report a case of bilateral, recurrent, Cytomegalovirus (CMV) retinitis in Dyskeratosis Congenita (DKC) healing with rapid calcifications.
a case report.
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.
This case highlights recurrent CMV retinitis with rapid presumed dystrophic calcification in Dyskeratosis congenita with progressive bone marrow hypoplasia.
报告1例先天性角化不良（DKC）伴双侧复发性巨细胞病毒（CMV）视网膜炎且愈合过程中出现快速钙化的病例。
病例报告。
病例报告：一名25岁男性，诊断为DKC，伴有严重骨髓发育不全，双眼CMV视网膜炎复发。4个月前，他接受了全身及双侧玻璃体腔内抗病毒治疗8周，以治疗CMV视网膜炎。散瞳眼底检查显示大片视网膜脉络膜瘢痕形成，伴有折射性钙化沉积物，提示愈合的视网膜炎病变内存在钙化性营养不良。在这些愈合病变的边缘，可见新的颗粒状视网膜炎病灶。重新开始玻璃体腔内抗病毒治疗后，这些病灶迅速产生反应，并在几周内愈合，伴有视网膜萎缩和新的钙化沉积物。在光学相干断层扫描中，这些钙沉积物在萎缩的视网膜层中表现为高反射性沉积物。尽管病情得到缓解，但由于视网膜炎病变钙化，最终视力仍伴有视觉损害。他发生了感染性休克和弥散性血管内凝血，导致在治疗期间不幸死亡。
本病例突出了先天性角化不良伴进行性骨髓发育不全时复发性CMV视网膜炎及快速推测性营养不良性钙化的情况。
