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自发性脑脊液鼻漏为生长激素分泌型垂体腺瘤的首发表现。

Spontaneous cerebrospinal fluid rhinorrhea as the initial presentation of growth hormone-secreting pituitary adenoma.

机构信息

Department of Endocrinology, Endocrinology and Metabolism Institute, Cleveland, OH, USA.

出版信息

Am J Otolaryngol. 2011 Sep-Oct;32(5):433-7. doi: 10.1016/j.amjoto.2010.07.012. Epub 2010 Oct 2.

Abstract

Patients with acromegaly usually present with characteristic clinical features or comorbidities associated with excess insulinlike growth factor 1 (IGF-1)/growth hormone (GH) or may come to medical attention secondary to mass effects causing visual field distortions. Herein, we report a case of spontaneous cerebrospinal fluid (CSF) rhinorrhea as the presenting symptom of acromegaly. A 68-year-old man presented to an outside facility with a 2-day history of headache associated with nausea, vomiting, dizziness, and clear nasal discharge and underwent 2 attempted repairs of a sphenoid sinus CSF leak. Examination on admission to our hospital was significant for fluctuating level of consciousness. Subsequently, subtle coarse facial features were appreciated. Pituitary function testing showed thyrotropin and gonadotropin deficiencies along with an elevated age- and sex-matched IGF-1 of 285 (normal level, 59-225 ng/mL). Nadir GH during oral glucose tolerance test was 5.5 ng/mL and confirmed the diagnosis of acromegaly. Magnetic resonance imaging showed pneumocephalus, an enlarged sella with an elongated pituitary stalk, and partial erosion of the anterior wall of the sphenoid sinus. A distinct adenoma could not be identified. An endoscopic, transnasal, transsphenoidal exploration and biopsy with multilayered skull base reconstruction were performed. Histologic examination of the biopsy contents was consistent with a GH-producing adenoma. Postoperatively, the patient's fluctuating level of consciousness improved and returned to baseline after his successful skull base repair. During the follow-up period, he had an IGF-1 of 713 ng/mL and started treatment with a somatostatin analogue. To our knowledge, this is the first reported case of a GH-producing pituitary adenoma presenting with spontaneous CSF rhinorrhea. Pituitary adenomas should be considered in the differential diagnosis of patients presenting with spontaneous CSF rhinorrhea with abnormal sellar image, and these patients should undergo a thorough hormonal evaluation.

摘要

患者患有肢端肥大症时通常会表现出与胰岛素样生长因子 1(IGF-1)/生长激素(GH)过量相关的特征性临床特征或合并症,或者由于引起视野扭曲的肿块效应而引起医疗关注。在此,我们报告了一例以自发性脑脊液(CSF)鼻漏为首发症状的肢端肥大症病例。一名 68 岁男性因头痛伴恶心、呕吐、头晕和清亮的鼻漏就诊于一家外部医疗机构,此前已尝试修复蝶窦 CSF 漏 2 次。入院时的检查发现意识水平波动。随后,观察到细微的粗糙面容特征。垂体功能检查显示促甲状腺激素和促性腺激素缺乏,同时 IGF-1 升高,与年龄和性别匹配的 IGF-1 为 285(正常水平为 59-225ng/mL)。口服葡萄糖耐量试验时的 GH 最低点为 5.5ng/mL,确诊为肢端肥大症。磁共振成像显示气颅、鞍扩大伴垂体柄延长和蝶窦前壁部分侵蚀。未能识别出明显的腺瘤。进行了内镜、经鼻、经蝶窦探查和活检,并进行了多层颅底重建。活检内容的组织学检查与 GH 分泌性腺瘤一致。手术后,患者意识水平波动改善,成功修复颅底后恢复基线水平。在随访期间,他的 IGF-1 为 713ng/mL,并开始使用生长抑素类似物治疗。据我们所知,这是首例报道的 GH 分泌性垂体腺瘤表现为自发性 CSF 鼻漏的病例。对于表现出异常鞍图像和自发性 CSF 鼻漏的患者,应考虑垂体腺瘤作为鉴别诊断,这些患者应进行彻底的激素评估。

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