血友病表现为复发性眼出血。

GMS ophthalmology cases Pub Date : 2020-03-18 eCollection Date: 2020-01-01 DOI:10.3205/oc000142
Luis Miguel Aquino, Felice Katrina Ranche
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引用次数: 2

摘要

目的:遗传性出血性疾病患者很少以眼内或眼眶出血为首发症状。在考虑眼科手术时,这种情况很容易被忽视,并可能引起术中和术后并发症。了解这些情况可以改善手术决策。方法:这是一个8岁的菲律宾男性谁持续钝性创伤,他的右眼,造成外伤性全前房积血角膜染色的病例报告。超声示视网膜下出血。患者行前房冲洗术及暂时性角膜假体及玻璃体平滑肌切除及硅油填塞术。术后注意到前房积血的清除。然而,在19天后的随访中,患者出现前房积血复发,新发突出和球周出血。结果:眼眶影像学显示新发假突出伴眼内及球周出血。此时怀疑是出血性疾病。进一步的调查揭示了家族史延长出血时间的x连锁遗传模式跨越三代。实验室检测凝血酶原、部分凝血活酶和因子分析,发现因子VIII缺乏,诊断血友病a。患者接受新鲜冷冻血浆输注,出血得以缓解。结论:出血性疾病是外科治疗患者的难题。在外伤性出血的情况下,应该做充分的病史和体格检查,以排除这些可能性。血友病患者的手术效果可以通过术前预防性治疗和术后密切的监测和护理得到改善。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Hemophilia presenting as recurrent ocular hemorrhage.

Hemophilia presenting as recurrent ocular hemorrhage.

Hemophilia presenting as recurrent ocular hemorrhage.

Hemophilia presenting as recurrent ocular hemorrhage.

Objective: Patients with hereditary bleeding disorders rarely present with intraocular or orbital hemorrhage as the initial symptom. The presence of such a condition can be easily overlooked when contemplating ophthalmic surgery, and can give rise to intraoperative and postoperative complications. Awareness of such conditions can improve surgical decisions. Methods: This is a case report of an eight-year-old Filipino male who sustained blunt trauma to his right eye, causing traumatic total hyphema with corneal staining. Subretinal hemorrhage was seen on ultrasound. The patient underwent anterior chamber washout with temporary keratoprosthesis and pars plana vitrectomy with silicone oil tamponade. Clearance of the hyphema was noted postoperatively. However, on follow-up after 19 days, the patient presented with recurrence of hyphema, new onset proptosis and peribulbar hemorrhage. Results: Imaging of the orbit revealed new-onset pseudoproptosis with intraocular and peribulbar hemorrhage. A bleeding disorder was suspected at this point. Further probing revealed a family history of prolonged bleeding time in an X-linked genetic inheritance pattern spanning three generations. Laboratory testing of prothrombin, partial thromboplastin, and factor assays were done, which revealed factor VIII deficiency, diagnostic of hemophilia A. No further surgery was done. The patient was given transfusions of fresh frozen plasma, which resolved the hemorrhage. Conclusions: Bleeding disorders present a dilemma in the surgical management of patients. In cases of traumatic hemorrhage, adequate history and physical examination should always be done to rule these out. Surgical outcomes in hemophiliacs can be improved with preoperative prophylactic treatment and close postoperative monitoring and care.

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