Pang Chang Hwan, Lee Soo Eon, Kim Chi Heon, Chung Chun Kee
Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Korea.
Korean J Spine. 2015 Sep;12(3):150-2. doi: 10.14245/kjs.2015.12.3.150. Epub 2015 Sep 30.
The Chiari malformation is an infrequently detected congenital anomaly characterized by the downward displacement of the cerebellum with a tonsillar herniation below the foramen magnum that may be accompanied by either syringomyelia or hydrocephalus. Surgery, such as foramen magnum decompression, is indicated for a symptomatic Chiari malformation, although an incidental lesion may be followed-up without further treatment. Infrequently, increased intracranial pressure emerges due to hyperthyroidism. A nineteen-year-old girl visited our outpatient clinic presented with a headache, nausea and vomiting. A brain and spinal magnetic resonance image study (MRI) indicated that the patient had a Chiari I malformation without syringomyelia or hydrocephalus. An enlarged thyroid gland was detected on a physical examination, and serum markers indicated Graves' disease. The patient started anti-hyperthyroid medical treatment. Subsequently, the headache disappeared after the medical treatment of hyperthyroidism without surgical intervention for the Chiari malformation. A symptomatic Chiari malformation is indicated for surgery, but a surgeon should investigate other potential causes of the symptoms of the Chiari malformation to avoid unnecessary surgery.
Chiari畸形是一种罕见的先天性异常,其特征是小脑向下移位,扁桃体疝入枕骨大孔以下,可能伴有脊髓空洞症或脑积水。对于有症状的Chiari畸形,需进行手术,如枕骨大孔减压术,不过偶然发现的病变可进行随访而无需进一步治疗。颅内压升高偶尔会因甲状腺功能亢进症而出现。一名19岁女孩到我们门诊就诊,表现为头痛、恶心和呕吐。脑部和脊髓磁共振成像(MRI)检查表明该患者患有Chiari I型畸形,无脊髓空洞症或脑积水。体格检查发现甲状腺肿大,血清标志物提示为格雷夫斯病。患者开始接受抗甲状腺药物治疗。随后,在未对Chiari畸形进行手术干预的情况下,甲状腺功能亢进症经药物治疗后头痛消失。有症状的Chiari畸形需进行手术,但外科医生应调查Chiari畸形症状的其他潜在原因,以避免不必要的手术。