马凡综合征患儿伴巨大视网膜裂孔的孔源性视网膜脱离：一种罕见的眼部急症。
Rhegmatogenous retinal detachment with giant retinal tear in a child with Marfan's syndrome: a rare ocular emergency.
机构信息
Ophthalmology, Government Medical College and Hospital, Chandigarh, India.
Ophthalmology, Government Medical College and Hospital, Chandigarh, India
出版信息
BMJ Case Rep. 2021 Jun 2;14(6):e241354. doi: 10.1136/bcr-2020-241354.
A 7-year-old boy with Marfanoid habitus presented with sudden and painless decrease in the vision of the right eye. Ocular examination revealed rhegmatogenous retinal detachment with 360° giant retinal tear in the right eye and small peripheral retinal breaks with lattice degeneration in the left eye. The patient underwent a 23-gauge pars plana vitrectomy with scleral buckling in the right eye and laser around the breaks in the left eye. At 1-week follow-up visit, the child presented with similar complaints in the left eye as were seen in the right eye. This was later managed effectively with 23-gauge pars plana vitrectomy only. So, with our case report, we would like to highlight the need for aggressive screening in children who are diagnosed with Marfan's syndrome and the need for prophylactic treatment in the unaffected eye.
一位 7 岁男孩，具有马凡氏体型外观，突发无痛性右眼视力下降。眼部检查发现右眼孔源性视网膜脱离伴 360°巨大视网膜裂孔，左眼周边小视网膜裂孔伴格子状变性。患者右眼行 23G 经睫状体平坦部玻璃体切割联合巩膜扣带术，左眼行裂孔周围激光光凝。术后 1 周复诊时，患儿左眼出现与右眼类似的症状。随后仅行 23G 经睫状体平坦部玻璃体切割术治疗，效果良好。因此，通过本病例报告，我们想强调对诊断为马凡氏综合征的儿童进行积极筛查的必要性，以及对未受影响的眼睛进行预防性治疗的必要性。
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