Gaunt Trevor, Aboelmagd Sharief, Spohr Hilmar, Saada Janak
Norwich Radiology Academy, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK.
BJR Case Rep. 2016 Jul 28;2(3):20160016. doi: 10.1259/bjrcr.20160016. eCollection 2016.
Studies have established a complex age-related variation of the position of the cerebellar tonsils relative to the foramen magnum (FM). Chiari malformation type 1 (CM1) is generally defined by a protrusion >5 mm from the FM and may be an asymptomatic incidental finding. Symptoms include headache, nausea and neurological disturbances, including trigeminal neuralgia. Moreover, tonsils are often peg shaped and associated with syringohydromyelia. Symptomatic CM1 may be managed with decompression of the posterior cranial fossa, but spontaneous regression in adults has been reported occasionally. Theories include restoration of normal cerebrospinal fluid dynamics around the FM after rupture of subarachnoid adhesions or the syrinx itself during transient episodes of raised intracranial pressure. Supratentorial neurosurgery has also been implicated. We present a 58-year-old female diagnosed with CM1 and no associated syringohydromyelia following MRI investigation of trigeminal neuralgia. Managed medically, she re-presented 6 years later with new neurological symptoms. A subsequent MR study of the posterior cranial fossa showed resolution of the CM1, with only residual tonsillar ectopia. At no point was intracranial intervention performed, nor were there any events that might favour CM1 regression. This case demonstrates spontaneous resolution of CM1 without surgical intervention.
研究已经证实,小脑扁桃体相对于枕骨大孔(FM)的位置存在复杂的年龄相关变化。1型Chiari畸形(CM1)通常定义为扁桃体从枕骨大孔突出超过5毫米,可能是无症状的偶然发现。症状包括头痛、恶心和神经功能障碍,包括三叉神经痛。此外,扁桃体常呈钉状,并与脊髓空洞症相关。有症状的CM1可通过后颅窝减压治疗,但成人偶尔也有自发缓解的报道。理论包括蛛网膜粘连或脊髓空洞症本身在颅内压短暂升高期间破裂后,枕骨大孔周围正常脑脊液动力学的恢复。幕上神经外科手术也与之有关。我们报告一例58岁女性,因三叉神经痛进行MRI检查后被诊断为CM1,且无相关脊髓空洞症。经药物治疗后,她6年后再次出现新的神经症状。随后对后颅窝进行的MR研究显示CM1消失,仅残留扁桃体异位。在任何时候都未进行颅内干预,也没有任何可能促使CM1缓解的事件。该病例证明了CM1无需手术干预即可自发缓解。