Ribeiro Pedro, Nogueira João, Cambango Maura, Oliveira Leandro, Coimbra Frederica
Neurosurgery, Hospital de Braga, Braga, PRT.
Cureus. 2025 Apr 8;17(4):e81898. doi: 10.7759/cureus.81898. eCollection 2025 Apr.
Chiari type I malformation (CM-1) is a condition that is often asymptomatic, though when symptoms occur, they commonly involve occipital headaches. Acute respiratory insufficiency as the presenting symptom requiring emergent surgical intervention is extremely rare. We present the case of a 51-year-old female who developed sudden-onset respiratory insufficiency due to CM-1 and syringomyelia affecting the brainstem, necessitating urgent surgical intervention. The patient, with a history of mild tetraparesis, arrived at the emergency department with an acute loss of consciousness, severe respiratory acidosis, and acidemia. Imaging revealed tonsillar herniation at the foramen magnum, significant compression of the bulbomedullary junction, and an extensive syringomyelic cavity extending from the cervical to the dorsal spine. The patient underwent a comprehensive evaluation, with no alternative causes found for the clinical presentation. An emergent posterior fossa decompressive craniectomy and C1 laminectomy were performed, leading to a satisfactory clinical recovery. This case highlights the importance of prompt surgical intervention in rare presentations of CM-1, which can lead to significant improvement in symptoms and favorable outcomes.
Chiari I型畸形（CM-1）通常无症状，但出现症状时，常见症状为枕部头痛。以急性呼吸功能不全为首发症状且需要紧急手术干预的情况极为罕见。我们报告一例51岁女性病例，该患者因CM-1和影响脑干的脊髓空洞症而突发呼吸功能不全，需要紧急手术干预。该患者有轻度四肢轻瘫病史，因急性意识丧失、严重呼吸性酸中毒和酸血症被送至急诊科。影像学检查显示枕骨大孔处扁桃体疝、延髓球部交界处明显受压，以及从颈椎至胸椎广泛的脊髓空洞腔。患者接受了全面评估，未发现导致临床表现的其他原因。紧急实施了后颅窝减压颅骨切除术和C1椎板切除术，临床恢复情况良好。该病例强调了在CM-1罕见表现中及时进行手术干预的重要性，这可显著改善症状并取得良好预后。
