PDF(Pubmed)
Abstract：
Rare presentation of pediatric angiomatosis of the paranasal sinus and skull base presenting mimicking juvenile nasopharyngeal angiofibroma (JNA). This is a 16-year-old male who presented to the emergency room with acutely worsening headaches, decreased visual acuity, subjective diplopia on lateral gaze, and a skull base mass centered in the sphenoid cavity. Endoscopic biopsy at an outside facility was aborted due to profuse bleeding. Upon transfer to a tertiary care center, contrast MR demonstrated a heterogeneously and avidly enhancing vascular mass centered around the sphenoid and skull base originating from the internal maxillary artery with significant bilateral extension into the adjacent paranasal sinuses, sella, and cavernous sinus. History of presentation and imaging was suggestive of JNA. Patient underwent preoperative embolization followed by endoscopic endonasal transphenoidal resection with a skull base trained otolaryngologist and neurosurgeon. Final pathology confirmed angiomatosis. This is only the second reported case of paranasal sinus angiomatosis in the literature. Angiomatosis has a high rate of recurrence and failure of timely diagnosis can lead to requirement of repeated surgical intervention. Re-operations are associated with increased costs, patient dissatisfaction, and poorer surgical/clinical outcomes. Because angiomatosis can mimic JNA, hemangiomas, or other vascular tumors, it is essential to maintain a broad differential diagnosis that includes angiomatosis when evaluating sinonasal tumors.
摘要：
罕见的儿童鼻旁窦和颅底血管瘤病表现为模仿青少年鼻咽血管纤维瘤（JNA）。这是一名16岁的男性，他因头痛急剧恶化而出现在急诊室，视力下降，侧注视的主观复视，和一个位于蝶骨腔中心的颅底肿块。由于大量出血，外部设施的内窥镜活检被中止。转移到三级护理中心后，对比MR显示出起源于上颌内动脉的蝶骨和颅底周围的非均匀且强烈增强的血管块，并显着双侧延伸到相邻的鼻旁窦，塞拉,和海绵窦.表现和影像学史提示JNA。患者接受了术前栓塞治疗，然后由经过训练的颅底耳鼻喉科医师和神经外科医师进行内镜经鼻蝶切除术。最终病理证实血管瘤病。这只是文献中第二例报道的鼻旁窦血管瘤病。血管瘤病复发率高，不能及时诊断可能导致需要反复手术干预。重新运营与成本增加有关，患者不满意,和较差的手术/临床结果。因为血管瘤病可以模仿JNA，血管瘤,或其他血管肿瘤，在评估鼻窦肿瘤时，必须保持广泛的鉴别诊断，包括血管瘤病。
