Suresh Neeraj V, Shah Viraj N, Matichak David, Ghiam Michael K, Pasick Luke J, Abecassis Isaac J, Saad Ali G, Morcos Jacques, Sargi Zoukaa, Bhatia Rita
Department of Otolaryngology-Head and Neck Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.
Department of Neurological Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.
Radiol Case Rep. 2022 Jul 29;17(10):3527-3534. doi: 10.1016/j.radcr.2022.07.031. eCollection 2022 Oct.
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.
鼻窦和颅底小儿血管瘤病的罕见表现，酷似青少年鼻咽血管纤维瘤（JNA）。这是一名16岁男性，因头痛急剧加重、视力下降、侧视时主观复视而就诊于急诊室，颅底肿物位于蝶窦。在外院进行内镜活检时因大出血而中止。转至三级医疗中心后，增强磁共振成像显示一个不均匀且明显强化的血管性肿物，以蝶窦和颅底为中心，起源于上颌内动脉，双侧显著延伸至相邻鼻窦、蝶鞍和海绵窦。临床表现和影像学表现提示为JNA。患者接受了术前栓塞，随后由一名擅长颅底手术的耳鼻喉科医生和神经外科医生进行了内镜经鼻蝶窦切除术。最终病理确诊为血管瘤病。这是文献中报道的第二例鼻窦血管瘤病。血管瘤病复发率高，未能及时诊断会导致需要反复进行手术干预。再次手术会增加费用、引起患者不满，并导致更差的手术/临床结果。由于血管瘤病可酷似JNA、血管瘤或其他血管肿瘤，在评估鼻窦肿瘤时，保持包括血管瘤病在内的广泛鉴别诊断至关重要。
