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儿童和年轻成人中的鞍棘:临床表现、影像学特征和垂体结局

Sellar spine in children and young adults: clinical presentation, imaging characteristics, and pituitary outcomes.

临床研究鼻科IF 1.2Q3

文献信息

中文摘要

目的: 由于现有数据有限,鞍棘的真实临床相关性仍不明确。我们旨在描述患有鞍棘的儿童和年轻成人的表现及相关的垂体功能障碍。
方法: 我们回顾性审查了在25岁以下个体中通过脑影像学检查发现鞍棘的患者。提取的变量包括初次脑影像学检查时的年龄、影像学检查的指征以及垂体功能障碍的状态和类型。
结果: 共纳入11例患者。初次发现鞍棘时的年龄范围为6至24岁。神经影像学检查的指征包括头痛(n=3)、癫痫发作(n=2)、性早熟(n=1)、严重肥胖伴骨龄提前(n=1)、疑似卒中/共济失调(n=1)、鼻息肉评估(n=1)、低促性腺激素性性腺功能减退症和继发性甲状腺功能减退症(n=1),以及作为Von Hipple Lindau筛查检测的一部分(n=1)。7例患者(63.6%)发现垂体柄偏移。2例患者(18.2%)因垂体腺形态扭曲而被误诊为垂体腺瘤。在年龄较小的儿童中(n=5;年龄≤9岁),一名患者出现性早熟,该患者显示垂体柄偏移,AP大小与蝶鞍比值为0.5。另一名有垂体柄偏移的儿童未发生性早熟。一名患者(低促性腺激素性性腺功能减退症和甲状腺功能减退症)被确定存在垂体激素缺乏,伴有垂体柄偏移,AP大小与蝶鞍比值为0.63。
结论: 鞍棘的存在可扭曲垂体解剖结构并导致诊断性误分类。部分受累患者可能发生内分泌疾病。需要进一步开展更大规模的研究,以更好地明确导致内分泌疾病的鞍棘特征。

英文摘要

OBJECTIVES: The true clinical relevance of a sellar spine remains unclear because of limited available data. We aimed to describe the presentation and associated pituitary dysfunction among children and young adults with a sellar spine.
METHODS: We retrospectively reviewed patients with a sellar spine found on brain imaging obtained in individuals less than 25 years of age. Extracted variables included age at initial brain imaging, indication for imaging, and pituitary dysfunction status and type.
RESULTS: Eleven patients were included. Age at initial identification of a sellar spine ranged from 6 to 24 years. Indications for neuroimaging included headaches (n=3), seizures (n=2), precocious puberty (n=1), severe obesity with advanced bone age (n=1), suspected stroke/ataxia (n=1), nasal polyp evaluation (n=1), hypogonadotropic hypogonadism and secondary hypothyroidism (n=1), and as part of screening testing for Von Hipple Lindau (n=1). Pituitary stalk deviation was found in 7 patients (63.6 %). Pituitary gland distorted morphology leading to a misdiagnosis of pituitary adenoma found in 2 patients (18.2 %). Among younger children (n=5; age ≤9 years), precocious puberty occurred in one patient who demonstrated pituitary stalk deviation with an AP size-to-sella ratio of 0.5. Another child with stalk deviation did not develop precocious puberty. Pituitary hormone deficiency was identified in one patient (hypogonadotropic hypogonadism and hypothyroidism) with stalk deviation and AP size-to-sella ratio of 0.63.
CONCLUSIONS: The presence of a sellar spine can distort pituitary anatomy and result in diagnostic misclassification. Endocrinopathies may develop in some affected patients. Further, larger studies are warranted to better define the characteristics of sellar spine leading to endocrinopathies.