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先天性甲状腺半侧缺如

Congenital thyroid hemiagenesis.

作者信息

McHenry C R, Walfish P G, Rosen I B, Lawrence A M, Paloyan E

机构信息

Department of Surgery, Case Western Reserve University School of Medicine, MetroHealth Medical Center, Cleveland, Ohio 44109-1998, USA.

出版信息

Am Surg. 1995 Jul;61(7):634-8; discussion 638-9.

PMID:7793746
Abstract

Failure of embryologic development of a lobe of the thyroid gland is a rare anomaly. In order to characterize this unusual entity, we report our experience in seven patients with thyroid hemiagenesis involving the left lobe in five and the right lobe in two patients. The diagnosis was made as a result of evaluation and treatment of a thyroid nodule (4), diffuse thyroid enlargement with thyrotoxicosis (2), and a simple goiter in a patient with a prior history of radiation treatment for facial acne. In five patients thyroid scintigraphy demonstrated unilateral absence of function, four of whom had an ultrasound exam of the neck that revealed a corresponding absence of thyroid tissue. In one patient the diagnosis was made incidentally on a screening ultrasound exam of the neck. Thyroid hemiagenesis was unsuspected preoperatively in one patient with Graves' disease because of marked hyperplasia of a single thyroid lobe and isthmus. Thyroid hemiagenesis was confirmed in four patients who underwent thyroidectomy. The presence of a thyroid isthmus was established in six patients. Postoperatively, all patients were treated with thyroid hormone. Associated thyroid pathology included adenoma (1), follicular carcinoma (1), colloid nodule (2), Graves' disease (2), and a simple goiter (1). In conclusion, the diagnosis of thyroid hemiagenesis should be considered in any patient with unilateral absence of function on thyroid scintigraphy and confirmed by ultrasonography. Recognition of this rare congenital anomaly is important to avoid unnecessary contralateral neck exploration with its potential morbidity and to insure that all patients receive careful follow-up and appropriate thyroid hormone therapy when necessary.

摘要

甲状腺叶胚胎发育失败是一种罕见的异常情况。为了描述这一特殊实体,我们报告了7例甲状腺半侧缺如患者的情况,其中5例为左叶缺如,2例为右叶缺如。诊断是在对甲状腺结节(4例)、伴有甲状腺毒症的弥漫性甲状腺肿大(2例)以及一名既往有面部痤疮放射治疗史的单纯性甲状腺肿患者进行评估和治疗时做出的。5例患者的甲状腺闪烁显像显示单侧无功能,其中4例颈部超声检查显示相应部位无甲状腺组织。1例患者是在颈部筛查超声检查时偶然确诊的。1例患有格雷夫斯病的患者术前未怀疑有甲状腺半侧缺如,因为单个甲状腺叶和峡部明显增生。4例接受甲状腺切除术的患者确诊为甲状腺半侧缺如。6例患者确定存在甲状腺峡部。术后,所有患者均接受甲状腺激素治疗。相关的甲状腺病理包括腺瘤(1例)、滤泡癌(1例)、胶样结节(2例)、格雷夫斯病(2例)和单纯性甲状腺肿(1例)。总之,对于任何甲状腺闪烁显像显示单侧无功能的患者,均应考虑甲状腺半侧缺如的诊断,并通过超声检查加以证实。认识到这种罕见的先天性异常对于避免不必要的对侧颈部探查及其潜在的发病率很重要,并且要确保所有患者在必要时接受仔细的随访和适当的甲状腺激素治疗。

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