Kadono Yoshinori, Maeno Kazushige, Kuramoto Masami, Harada Atsuko, Kishima Haruhiko
Department of Neurosurgery, Takatsuki General Hospital, Takatsuki, Osaka, Japan.
Department of Neurosurgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan.
Radiol Case Rep. 2025 Mar 21;20(6):2927-2930. doi: 10.1016/j.radcr.2025.03.005. eCollection 2025 Jun.
Perimedullary arteriovenous fistulas (PMAVFs) are rare spinal vascular lesions, particularly in pediatric patients. We present a pediatric case of a ruptured cervical PMAVF, successfully treated with endovascular embolization, along with a review of relevant literature. A 10-year-old boy with no significant medical history initially presented with neck pain and fatigue, progressing to paralysis in his upper limbs and eventually all extremities. His condition further deteriorated, leading to impaired consciousness and respiratory distress, necessitating transfer to our pediatric intensive care unit for intubation and management. Head magnetic resonance images revealed medullary edema and dilated veins in the posterior cranial fossa. At the same time, contrast-enhanced computed tomography of the neck showed an intramedullary hematoma centered at C4, with edema extending from the medulla oblongata to T3 and dilated vessels surrounding the lesion. Angiography confirmed a varix rupture of the PMAVF with feeders from the right C4 dorsal root artery and the right ascending cervical artery. Endovascular embolization was performed using 33% n‑butyl‑2-cyanoacrylate through a microcatheter placed just beyond the fistula. The shunt was successfully occluded. Postoperatively, following a tracheotomy, the patient was gradually weaned off the ventilator. With rehabilitation, he showed significant improvement in spinal cord function, including regaining the ability to transfer with a walker. Endovascular embolization is a viable treatment option for pediatric PMAVF, as demonstrated in this case, where a single-stage procedure achieved successful occlusion and significant neurological recovery.
髓周动静脉瘘(PMAVF)是一种罕见的脊柱血管病变,在儿科患者中尤为少见。我们报告一例经血管内栓塞成功治疗的小儿颈段PMAVF破裂病例,并对相关文献进行综述。一名无重大病史的10岁男孩最初表现为颈部疼痛和乏力,随后进展为上肢瘫痪,最终四肢均出现瘫痪。其病情进一步恶化,导致意识障碍和呼吸窘迫,因此被转至我们的儿科重症监护病房进行插管和治疗。头部磁共振成像显示延髓水肿和后颅窝静脉扩张。与此同时,颈部增强CT显示以C4为中心的髓内血肿,水肿从延髓延伸至T3,病变周围血管扩张。血管造影证实PMAVF的静脉曲张破裂,供血来自右侧C4背根动脉和右侧颈升动脉。通过置于瘘口远端的微导管使用33%的n-丁基-2-氰基丙烯酸酯进行血管内栓塞。分流成功闭塞。术后,气管切开后,患者逐渐脱机。经过康复治疗,他的脊髓功能有了显著改善,包括重新获得使用助行器转移的能力。如本病例所示,血管内栓塞是小儿PMAVF的一种可行治疗选择,在该病例中,单阶段手术成功实现了闭塞并使神经功能得到显著恢复。