Li Zi-Fu, Li Qiang, Xu Yi, Hong Bo, Huang Qing-Hai, Liu Jian-Min
Department of Neurosurgery, Changhai Hospital, Second Military Medical University, Shanghai, China.
Department of Neurosurgery, Changhai Hospital, Second Military Medical University, Shanghai, China.
World Neurosurg. 2017 Nov;107:1043.e7-1043.e13. doi: 10.1016/j.wneu.2017.06.080. Epub 2017 Jun 20.
To present 2 cases of Parkes Weber syndrome (PWS) with spinal arteriovenous malformation (AVM) and discuss the radiologic features and clinical treatment with literature review.
Clinical data on 2 patients with PWS with spinal AVM was acquired in a prospective follow-up investigation. Clinical manifestations, radiographic features, procedural results, and follow-up outcome were collected and reviewed together with a literature review.
The first patient presented with limb weakness and urinary dysfunction and the second with repetitive subarachnoid hemorrhage followed by paraplegia. Limb hypertrophy, skin ulceration, and extensive microfistulas in the affected limb were observed in both patients. Spinal AVM was confirmed by digital subtraction angiography and endovascular embolization was performed. The first patient experienced limb amputation at 6-year follow-up as a result of chronic ulceration and the second did not have neurologic improvement. After literature review, 15 cases (male/female ratio, 5:10; mean age, 22±10.4 years) were included. The presentations comprised subarachnoid hemorrhage in 6, radicular pain in 5, myelopathy in 4, and asymptomatic in 1. Embolization was performed in 9 cases, solitary surgery in 2, and combined therapy in 4. Among 10 cases with known follow-up results, 6 achieved neurologic recovery after surgery and 1 died after solitary surgery.
Awareness of the association between spinal AVM and PWS is essential for radiographic screening of spinal lesions with myelopathy or intracranial subarachnoid hemorrhage. Clinical therapeutic strategy should be multidisciplinary and individualized on the basis of vasculature and lesion behavior.
介绍2例伴有脊髓动静脉畸形（AVM）的帕克斯·韦伯综合征（PWS）病例，并结合文献复习讨论其影像学特征及临床治疗方法。
通过前瞻性随访研究获取2例患有脊髓AVM的PWS患者的临床资料。收集并复习患者的临床表现、影像学特征、手术结果及随访结果，并进行文献复习。
首例患者表现为肢体无力和排尿功能障碍，第二例患者表现为反复蛛网膜下腔出血，随后出现截瘫。2例患者均观察到患肢肢体肥大、皮肤溃疡及广泛的微瘘。通过数字减影血管造影确诊为脊髓AVM，并进行了血管内栓塞治疗。首例患者在6年随访时因慢性溃疡行肢体截肢，第二例患者神经功能未改善。文献复习后，纳入15例病例（男/女比例为5:10；平均年龄22±10.4岁）。临床表现包括蛛网膜下腔出血6例、神经根性疼痛5例、脊髓病4例、无症状1例。9例行栓塞治疗，2例行单纯手术，4例行联合治疗。在10例有已知随访结果的病例中，6例术后神经功能恢复，1例单纯手术后死亡。
认识脊髓AVM与PWS之间的关联对于对患有脊髓病或颅内蛛网膜下腔出血的脊髓病变进行影像学筛查至关重要。临床治疗策略应基于血管系统和病变行为进行多学科个体化制定。
