Mavrikakis Ioannis, Heran Manraj K S, White Valerie, Rootman Jack
Department of Ophthalmology and Visual Sciences, Vancouver General Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
Ophthalmology. 2009 Jun;116(6):1216-24. doi: 10.1016/j.ophtha.2009.01.018. Epub 2009 Apr 19.
To describe venous thrombosis as a mechanism of clinical change in venous and combined venous lymphatic malformations of the orbit and to attempt histopathologically to distinguish the various vascular components of these lesions using immunohistochemistry with CD31 and D2-40 antibodies.
Retrospective, comparative, interventional case series.
Twelve patients with clinically and radiologically well-documented episodes of thrombosis in venous malformations (n = 7; group A) and combined venous lymphatic malformations (n = 5; group B).
Surgical excision of lesion in selected patients, 2 from group A and 5 from group B.
Age at presentation, gender, onset, symptoms and signs, investigative findings (imaging and histopathologic review), management, and outcome.
In group A, 4 patients were male and 3 were female, and in group B, 4 patients were female and 1 was male. The mean age+/-standard deviation at presentation was 57.6+/-10.9 years (range, 45-71 years) and 11+/-11.6 years (range, 1.5-26 years), respectively. The pattern of onset was acute in all cases. The most common signs and symptoms in group A were pain (n = 7), proptosis (n = 6), and nausea (n = 5), whereas in group B they were periorbital swelling (n = 5), proptosis (n = 5), and ecchymosis (n = 4). The immunohistochemistry results were positive for vascular (CD31) and lymphatic (D2-40) endothelium in all of the specimens. The combined venous lymphatic lesions divided themselves into 2 main categories based on the D2-40 findings in relationship to percentage of lymphatic vessels. These were lesions that were either lymphatic dominant (n = 3) or venous dominant (n = 2).
Clinically, the 2 groups behave differently. Group A lesions present in adults with acute pain, proptosis, and nausea and may resolve spontaneously on follow-up. Intervention may be required in cases of severe pain, proptosis, or dysfunction. Group B lesions present early in life with frequent bouts of periorbital swelling, progressive proptosis, and ecchymosis. Therefore, early intervention is advised. Finally, when requesting imaging for such lesions, early- and late-phase contrast imaging should be used because thrombosis typically is better demonstrated in the late phase.
FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
描述静脉血栓形成作为眼眶静脉性和静脉淋巴混合性畸形临床变化的一种机制，并尝试使用CD31和D2 - 40抗体进行免疫组织化学，从组织病理学上区分这些病变的各种血管成分。
回顾性、对比性、干预性病例系列研究。
12例临床和影像学资料充分的静脉畸形（n = 7；A组）和静脉淋巴混合性畸形（n = 5；B组）血栓形成患者。
对部分患者进行病变切除手术，A组2例，B组5例。
就诊年龄、性别、起病情况、症状和体征、检查结果（影像学和组织病理学检查）、治疗及预后。
A组4例男性，3例女性；B组4例女性，1例男性。就诊时的平均年龄±标准差分别为57.6±10.9岁（范围45 - 71岁）和11±11.6岁（范围1.5 - 26岁）。所有病例起病方式均为急性。A组最常见的症状和体征为疼痛（n = 7）、眼球突出（n = 6）和恶心（n = 5）；而B组为眶周肿胀（n = 5）、眼球突出（n = 5）和瘀斑（n = 4）。所有标本的免疫组织化学结果显示血管（CD31）和淋巴管（D2 - 40）内皮均呈阳性。根据D2 - 40结果及淋巴管百分比，静脉淋巴混合性病变可分为2大类。即淋巴管为主型（n = 3）或静脉为主型（n = 2）。
临床上，两组表现不同。A组病变见于成年人，表现为急性疼痛、眼球突出和恶心，随访时可能自行缓解。严重疼痛、眼球突出或功能障碍时可能需要干预。B组病变在生命早期出现，常伴有眶周肿胀、进行性眼球突出和瘀斑。因此，建议早期干预。最后，对此类病变进行影像学检查时，应采用早期和晚期对比成像，因为血栓形成通常在晚期显示更佳。
作者对本文讨论的任何材料均无专利或商业利益。
