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孤立性生长激素缺乏症伴巨大动静脉畸形。

Isolated growth hormone deficiency associated with a giant arteriovenous varix.

作者信息

Martin N A, Macagba-Crain C L, Geffner M, Peacock W

机构信息

Department of Surgery, University of California-Los Angeles School of Medicine.

出版信息

Neurosurgery. 1990 Aug;27(2):295-9. doi: 10.1097/00006123-199008000-00021.

DOI:10.1097/00006123-199008000-00021
PMID:2385347
Abstract

A 6.5-year-old girl with short stature (height age, 3 years; bone age, 1.75 years) had isolated growth hormone deficiency. Preoperative computed tomography and magnetic resonance imaging demonstrated a large, well-demarcated, homogeneous mass above the dorsum sellae with a density consistent with flowing blood. Vertebral angiography showed a giant intracranial varix caused by an arteriovenous fistula that originated at the apex of the basilar artery. At operation, an aneurysm clip was placed at the origin of the fistula, and occlusion of the fistula was confirmed by intraoperative digital subtraction angiography. There were no permanent neurological sequelae. Despite shrinkage of the varix, demonstrated by follow-up computed tomography, growth hormone deficiency persisted postoperatively. Biosynthetic growth hormone therapy was initiated 6.5 months after surgery and resulted in a height increment of 8.2 cm after 9 months of treatment.

摘要

一名6.5岁身材矮小的女孩(身高年龄3岁;骨龄1.75岁)患有单纯性生长激素缺乏症。术前计算机断层扫描和磁共振成像显示蝶鞍背上方有一个大的、边界清晰的均匀肿块,其密度与流动血液一致。椎动脉造影显示由起源于基底动脉顶端的动静脉瘘引起的巨大颅内静脉曲张。手术中,在瘘口起源处放置了动脉瘤夹,并通过术中数字减影血管造影证实瘘口闭塞。无永久性神经后遗症。尽管随访计算机断层扫描显示静脉曲张缩小,但术后生长激素缺乏症仍持续存在。术后6.5个月开始生物合成生长激素治疗,治疗9个月后身高增加了8.2厘米。

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