Skull Base and Cerebrovascular Laboratory, University of California, San Francisco, California, USA; Department of Neurological Surgery, University of California, San Francisco, California, USA; Department of Otolaryngology-Head and Neck Surgery, University of California, San Francisco, California, USA.
Skull Base and Cerebrovascular Laboratory, University of California, San Francisco, California, USA; Department of Neurological Surgery, University of California, San Francisco, California, USA.
World Neurosurg. 2019 Dec;132:57. doi: 10.1016/j.wneu.2019.08.172. Epub 2019 Aug 31.
Radiation-induced telangiectasia of the central nervous system has been described predominantly in children, with up to 20% of patients affected after 3-41 years of radiotherapy. We present the case of a 45-year-old male with a pontine pilocytic astrocytoma treated with standard-dose radiation for 6 weeks in 1993. He developed a 3-cm multicystic, hemorrhagic brainstem lesion but was asymptomatic. The lesion caused severe brainstem mass effect, compatible with cavernous malformation or capillary telangiectasia. It has been reported that cavernomas and capillary telangiectasias share a similar pathologic process. The patient was surgically treated with a supracerebellar infratentorial approach to diagnose the hemorrhagic component of the lesion and ensure there was no transformation of the pilocytic astrocytoma (Video 1). He was placed in a gravity-dependent supine position with the head flexed and turned to allow for natural relaxation of the cerebellum via gravity-a technique we previously described. Surgical treatment proceeded with a left suboccipital craniotomy to decompress the cyst and facilitate removal of the intraaxial lesion. We took care to avoid injuring the fourth and fifth cranial nerves and branches of the superior cerebellar artery. No further lesional tissue was seen in the resection cavity. Interestingly, the final pathologic diagnosis indicated a mix of both pilocytic astrocytoma and radiation-induced capillary telangiectasia. From the surgeon's perspective, capillary telangiectasias appear similar to cavernous malformations on gross inspection, so pathologic confirmation is essential. Postoperative imaging demonstrated total resection of the lesion. The patient was discharged on postoperative day 3 with no neurologic deficit.
中枢神经系统放射性毛细血管扩张症主要见于儿童，约 20%的患者在放疗后 3-41 年内受到影响。我们报告了 1993 年一名 45 岁男性的病例，他患有桥脑毛细胞星形细胞瘤，接受了标准剂量放疗 6 周。他出现了 3 厘米大小的多房性、出血性脑干病变，但无症状。病变引起严重的脑干肿块效应，符合海绵状血管畸形或毛细血管扩张症。据报道，海绵状血管畸形和毛细血管扩张症具有相似的病理过程。患者接受了经小脑幕下小脑上入路手术治疗，以诊断病变的出血成分，并确保毛细胞星形细胞瘤没有转化（视频 1）。患者被置于头低位仰卧位，头部弯曲并转向，通过重力使小脑自然松弛-这是我们之前描述的技术。手术治疗采用左枕下开颅术，以减压囊肿并促进颅内病变的切除。我们小心避免损伤第四和第五颅神经以及小脑上动脉的分支。切除腔中未发现更多病变组织。有趣的是，最终的病理诊断表明既有毛细胞星形细胞瘤又有放射性毛细血管扩张症。从外科医生的角度来看，毛细血管扩张症在大体检查上与海绵状血管畸形相似，因此病理确认至关重要。术后影像学显示病变完全切除。患者术后第 3 天无神经功能缺损出院。
