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马凡综合征患者脑脊液漏致垂体假性肿瘤。

Pituitary pseudo-adenoma from cerebrospinal fluid leak in patient with Marfan syndrome.

机构信息

Department of Internal Medicine, Beaumont Hospital Royal Oak, Royal Oak, Michigan, USA

Department of Internal Medicine, Beaumont Hospital Royal Oak, Royal Oak, Michigan, USA.

出版信息

BMJ Case Rep. 2024 Aug 25;17(8):e260189. doi: 10.1136/bcr-2024-260189.

DOI:10.1136/bcr-2024-260189
PMID:39182923
Abstract

Intracranial hypotension may result in pituitary gland enlargement due to compensatory hyperaemia and venous engorgement. Spontaneous intracranial hypotension (SIH) is frequently associated with connective tissue disorders predisposing patients to dural weakening including dural ectasia and meningeal diverticula. Symptoms of SIH typically include postural headache, dizziness and tinnitus. We present a case of a female in her 20s with Marfan syndrome and a history of pituitary adenoma, who reported intractable postural headaches. Hormonal workup revealed no abnormalities, whereas brain MRI showed sequelae of intracranial hypotension. Further MRI studies revealed thoracic and lumbar meningeal diverticula with significant dural sac ectasia at the L4-S2 level. Myelogram confirmed numerous lumbar spine diverticula with cerebrospinal fluid leak at the L5 and S1 right nerve roots. The patient underwent blood patch administrations at the level of the leak with improvement of symptoms.

摘要

颅内低血压可导致垂体腺增大,这是由于代偿性充血和静脉充血所致。自发性颅内低血压(SIH)常与结缔组织疾病有关,这些疾病使患者易发生导致硬脑膜薄弱的病变,包括硬脑膜扩张和脑膜憩室。SIH 的症状通常包括体位性头痛、头晕和耳鸣。我们报告了一例 20 多岁的女性,患有马凡综合征和垂体腺瘤病史,她报告了难治性体位性头痛。激素检查未见异常,而脑部 MRI 显示颅内低血压后遗症。进一步的 MRI 研究显示胸腰椎脑膜憩室,L4-S2 水平硬脊膜囊显著扩张。脊髓造影证实 L5 和 S1 右侧神经根存在多个腰椎憩室,伴有脑脊液漏。患者在漏口水平进行了血液贴补治疗,症状得到改善。

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BMJ Case Rep. 2024 Aug 25;17(8):e260189. doi: 10.1136/bcr-2024-260189.
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