郝晓军（翻译） 尖峰眼科
Background
Purtscher retinopathy is a hemorrhagic and vasoocclusive vasculopathy, which, in 1912, was first described as a syndrome of sudden blindness associated with severe head trauma. These patients had findings of multiple white retinal patches and retinal hemorrhages that were associated with severe vision loss.
Since its original description, Purtscher retinopathy has been associated with traumatic injury, primarily blunt thoracic trauma and head trauma, and numerous nontraumatic diseases.
远达性视网膜病变是一种以视网膜出血和血管闭塞为表现的血管病变，1912年首次发现，定义为伴随严重头部创伤导致突然失明的综合征，病人视网膜上有多个白色斑块及出血，伴有严重的视力丧失。自从被发现以来,远达性视网膜病变主要是和胸部挤压伤和头外伤等外伤有关，也见于许多非创伤性疾病。
Pathophysiology
The original findings of white lesions in the retina associated with intraretinal and preretinal hemorrhages and papillitis were believed to be caused by lymphatic extravasation from trauma. These lesions are known as Purtscher flecken (larger infarcts of the retinal capillary bed) and cotton-wool spots (small retinal microinfarcts at the level of the nerve fiber layer). Fluorescein leakage in Purtscher retinopathy suggests that an acute endothelial cell injury is caused by trauma, possibly predisposing the retinal vessels to occlusion.
病理机制：
原先的研究认为，视网膜上的白色病灶伴随视网膜层间、视网膜前出血、视神经乳头炎，是由于创伤引起的淋巴液外渗所致，这些病变称为Purtscher斑(视网膜毛细血管网的大范围梗死)，和棉绒斑(视网膜神经纤维层的小范围梗死)。远达性视网膜病变中有荧光素渗漏，表明创伤是引起急性内皮细胞损伤的主要因素，而后者常常是视网膜血管闭塞的先兆。
The exact pathophysiology remains somewhat controversial, and different mechanisms have been proposed.
The most accepted mechanism is leukoembolization that causes arterial occlusion and infarction of the microvascular bed. Leukocyte aggregation, which is induced by complement C5a, is believed to be the most likely mechanism of embolization because of its known association with trauma, acute pancreatitis, and vasculitic diseases.
准确的病理机制仍有争议，有人提出一些不同的机理。被广泛接受的机理是白细胞栓塞，导致动脉闭塞和微血管网梗死。C5a补体诱导的白细胞聚集被认为是最可能导致血管闭塞的机理，因为其与创伤、急性胰腺炎、血管炎性疾病相关。
Other possible sources of emboli include fat emboli in cases of long bone fractures and perhaps pancreatitis from enzymatic digestion of omental fat, amniotic fluid embolization during childbirth and postpartum, air emboli from traumatic chest compression, and granulocyte aggregation resulting from complement activation.
Other proposed mechanisms of vascular occlusion include angiospasm resulting from an acute rise in venous pressure from compressive chest injuries or possibly acute head injuries and endothelial cell damage resulting from acutely increased intraluminal pressure. A recent paper modeling shear stress in the retina suggests that Purtscher retinopathy may be a rheological event at a retinal posterior pole foci of vascular endothelial dysregulation, followed by downstream endothelin-induced vasculopathy.
其他可能的栓子的来源包括，长骨骨折时产生的脂肪栓子，胰腺炎时网膜脂肪在酶作用下的消解，分娩和产后的羊水栓塞，胸部挤压伤时的空气栓子，补体激活产生的中性粒细胞聚集。血管闭塞的其他可能的机制包括，胸部挤压伤或急性脑外伤时静脉压力急剧上升引起的血管痉挛，以及血管内压力急骤升高导致的内皮细胞损伤。最近的一篇关于模拟视网膜切应力的论文表明，远达性视网膜病变可能是后极部视网膜血管内皮失调，内皮素作用下的分支血管病变。
Mortality/Morbidity
Bilateral manifestations are seen most commonly, but unilateral findings have been reported.
Decreased vision occurs in the affected eyes, generally in the range of 20/200 to counting fingers. Vision often improves over several months to a range of 20/30 to 20/200, depending on the severity of the retinal findings. Case reports of Purtscher retinopathy associated with preeclampsia have demonstrated poor visual outcomes in more than one half of these patients.
发病率
双眼病变最为常见，单眼发病的情况也有。
受累眼视力下降，0.1到指数不等。尽管视网膜病情严重程度不一，几个月后，视力往往可以改善至20/30～20/200。发生子痫前期的远达性视网膜病变者，超过一半以上的病人视力预后较差。
History
Patients with traumatic Purtscher retinopathy present with a recent history of blunt chest trauma or head trauma. The severity of chest trauma is not correlated directly with the incidence or severity of retinopathy, which is observed in these patients.
Patients may present with unilateral or bilateral vision loss (possibly severe) generally within 2 days.
病史：
创伤性远达性视网膜病变患者，有近期的胸部钝伤或头部外伤史。临床发现，胸部外伤的严重程度，与视网膜病变是否发生及其严重程度并不直接相关。单侧或双侧视力下降，或严重下降，大多发生在两天之内。
Macular cotton-wool spots and intraretinal hemorrhages in patients with this history of trauma are diagnostic of the condition. Larger capillary bed infarcts (Purtscher flecken) are also seen in the macula and region surrounding the optic nerve. Typically, there is sparing of the retina whitening immediately adjacent to the larger retinal vessels.
Patients also may present with a Purtscher-like retinopathy in the absence of trauma. Various systemic conditions have been associated with developing the following characteristic retinal findings:
诊断标准包括外伤史，黄斑区棉绒斑，和视网膜内出血，黄斑区和视神经周围可见大片毛细血管网梗死（Purtscher斑）。而紧邻较粗视网膜血管的视网膜常常并不出现苍白。没有外伤史的患者也可能有Purtscher样视网膜病变，而没有外伤史，此与多种全身性疾病有关:
· Patients with acute pancreatitis are at risk for developing Purtscher-like retinopathy.
• Patients with long bone fractures are at risk for developing fat embolization and Purtscher-like retinopathy.
急性胰腺炎患者是发生Purtscher样视网膜病变的危险因素。
长骨骨折患者有发生脂肪栓塞和Purtscher样视网膜病变的危险。
•\tChildbirth
•\tPurtscher-like retinopathy may be seen in the postpartum period.
•\tPurtscher-like retinopathy may be due to amniotic fluid embolization with a catastrophic presentation, including shock and disseminated intravascular coagulopathy (DIC).
•\tRetinal arterial occlusions with microvascular infarcts and labor-associated hemorrhage have been reported in patients with preeclampsia and Purtscher-like retinopathy.
•\tProtein C and protein S deficiency have been reported.
分娩。
Purtscher样视网膜病变可见于产后。
羊水栓塞可导致包括Purtscher样视网膜病变在内的多种严重并发症，如休克和弥散性血管内凝血症(DIC)。
子痫前期出现Purtscher样视网膜病变的患者，有发生视网膜动脉阻塞伴微血管梗死，和分娩出血的报道。
C蛋白和S蛋白缺陷。
（presentation指的是产位）
•\tPurtscher-like retinopathy has been observed in patients with certain types of systemic vasculitides.
•\tThe most likely etiology is microvascular infarction due to complement C5a-induced leukocyte aggregation.
•\tPurtscher-like retinopathy has been reported in diverse vasculitic and related diseases, including thrombocytopenia purpura, cryoglobulinemia, hemolytic uremic syndrome, juvenile dermatomyositis, and multiple myeloma.
•\tUnexplained vision loss in patients with these conditions (eg, systemic lupus erythematosus, dermatomyositis, scleroderma) should raise the possibility of Purtscher-like retinopathy.
某些类型的系统性血管炎患者可发生Purtscher-like视网膜病变。
最可能的病因是C5a补体诱导的白细胞聚集，从而导致微血管梗死。
Purtscher-like视网膜病变亦见于多种类型的血管炎及相关疾病，包括血小板减少性紫癜，冷球蛋白血症，溶血性尿毒症，青少年型皮肌炎和多发性骨髓瘤。
发生于这些患者（如系统性红斑狼疮、皮肌炎、硬皮病）的原因不明的视力丧失，应该怀疑Purtscher-like视网膜病变发生的可能。
Purtscher-like retinopathy in a patient with systemic lupus erythematosus with microvascular encephalopathy and retinopathy. Multiple cotton-wool spots of varying sizes and ages surround the optic nerve. A branch arterial occlusion is present along the supertemporal arcade with retinal whitening between the major artery and vein. Multiple occlusions are seen in arterial and venous trees with disruption of the blood columns.
全身性红斑狼疮患者发生Purtscher-like视网膜病变，该患者还有微血管脑病和视网膜病变。视神经周围出现多个大小不等、新旧不一的棉絮斑。颞上血管弓附近有分支动脉阻塞，颞上动脉和静脉间视网膜变白。动脉、静脉分支多发阻塞，可见分段血柱。
Physical
The most common retinal findings in Purtscher retinopathy are cotton-wool spots, Purtscher flecken around the optic nerve, and intraretinal hemorrhages.
Less common reported findings include serous detachment of the macula, preretinal hemorrhages, dilated vessels, and optic disc edema.
Confluence of cotton-wool spots in the central macula may simulate the cherry-red spot that is seen in central retinal artery occlusion.
Retinal microinfarcts that are observed in patients with fat embolization are usually smaller in size and located in the peripheral, not central, retina.
Pigment migration and optic atrophy have been reported as late findings in the disease.
体格检查：
Purtscher视网膜病变最常见体征：棉絮斑，视神经周围的Purtscher 斑，和视网膜层间出血。
报道发现的少见体征有黄斑浆液性脱离、视网膜前出血、血管扩张、视盘水肿。
黄斑区的棉絮斑融合时，类似视网膜中央动脉阻塞所见的樱桃红斑。
脂肪栓塞所致视网膜微小梗塞，通常范围较小，位于周边部视网膜。
疾病后期，有报道发现色素移行和视神经萎缩。
Causes
Traumatic chest compression and blunt head trauma are common causes.
•\tChest trauma that is associated with Purtscher retinopathy ranges from mild to severe; the degree is not necessarily indicative of the risk of developing retinopathy.
•\tCompressive chest injuries often are seen with unrestrained drivers in motor vehicle accidents (MVAs).
病因：
胸部挤压伤和头部钝伤是常见的原因。
可能伴发远达性视网膜病变的胸部创伤，轻重不等，外伤严重程度与是否发生视网膜病变并无必然关系。
胸部挤压伤多见于机动车事故(MVAs)中未采取安全措施的司机。
Mild Purtscher retinopathy in a patient after blunt chest compression as an unrestrained driver in a motor vehicle accident. This patient presented with visual loss in the left eye and had unilateral disease. The fundus photograph shows a large cotton-wool spot along the inferotemporal arcade and more subtle microvascular injury between the disc and fovea in the central macula. Whitening of the central fovea is present. Visual acuity was 20/200 but returned to 20/30 after 6 weeks.
一起机动车交通事故中，无安全措施司机胸部钝挫伤后，发生轻度远达性视网膜病变，病人左眼视力下降。眼底照相示，沿颞下血管弓排列的大片棉絮斑，视盘和中心凹间的黄斑区有大量微血管损伤。中心凹发白，受伤时视力20/200，伤后六周20/30。
Patients with known vasculitic disease (eg, systemic lupus erythematosus, scleroderma, dermatomyositis) are at risk for developing a Purtscher-like retinopathy with microvascular occlusion.
Childbirth and the uncommon complication of amniotic fluid embolism are known risk factors. There have been several case reports of Purtscher retinopathy in the setting of preeclampsia without other known risk factors.[8]
Acute pancreatitis is a known risk factor. The risk from acute pancreatitis may be due to fat embolization or complement activation with secondary leukocyte aggregation and occlusive vasculopathy.
血管炎患者(如系统性红斑狼疮、硬皮病、皮肌炎)，有因微血管病变导致Purtscher-like视网膜病变的高危风险。
分娩和罕见的羊水栓塞也是已知的危险因素。发生于子痫前期、且无其他已知的危险因素的远达性视网膜病变，已有几例报道。
急性胰腺炎是一项已知危险因素，可能与脂肪栓塞、或补体激活及继发白细胞聚集、闭塞性血管病变有关。