摘要
目的探讨儿童垂体柄阻断综合征(PSIS)的临床和磁共振成像(MRI)特点及其在临床诊断中的意义。方法回顾性分析2010年7月至2013年3月首都儿科研究所附属儿童医院16例PSIS患儿的临床及MRI影像特点,所有患儿均行相关激素水平的检测及垂体增强MRI扫描。结果 15例表现为生长发育迟缓,3例伴有反复抽搐,其中1例伴步态不稳。垂体MRI增强扫描表现:垂体柄10例未见显示,6例细小;15例垂体前叶小;14例垂体后叶异位。2例合并Chiari畸形Ⅰ型。具有垂体前叶功能低下者临床表现和实验室检查:15例GH激发试验峰值均低;其中9例伴垂体性甲状腺功能减低和垂体性肾上腺皮质功能低下。1例垂体后叶功能减低。结论 PSIS临床以生长发育迟缓为主要表现,部分性或完全性垂体前叶功能低下,但多数垂体后叶功能正常。部分患儿合并其他先天发育畸形。MRI检查是无创诊断垂体柄阻断综合征的重要方法,应对该病患儿实施早期诊断和早期治疗。
Objective To evaluate the pituitary magnetic resonance imaging （MRI） and relevant clinical manifestations of pituitary stalk interruption syndrome （PSIS）. Methods The retrospective analysis was carried out in 16 patients of PSIS children from Jul.2010 to Mar.2013 in the Children＇s Hospital of Capital Institute of Pediatrics. The clinical characteristics and laboratory tests were summarized. Enhanced MRI was performed in all patients. And the pituitary imaging data were particularly analyzed. Results The morbidity had gender differences. Male patients were more than female ones. Fifteen of 16 patients presented with growih retardation and were all below the 3rd centile of the normal children of the same age and the same sex groups. Meanwhile, 3 of them had repeated seizures, one of whom had instability gait. Ten pituitary stalks in MRI with gadolinium contrast were not shown among all growth retardation patients, while five of the left had tiny stalks. Of all patients, 15 eases had pituitary dysplasia; 14 cases of all had ectopic posterior pituitary; 2 eases were eompanied with Chiari-1 malformation. All patients had clinical growth retardation and decreased peak value of growth hormone provocative test. Among them, 9 patients also presented with central hypothyroidism and hypoadrenocoritism. Only one case had posterior pituitary hypofunction. Conclusion PSIS usually presents with clinical characteristics of growth retardation, partial or complete anterior hypopituitarism. But mostly have normal posterior pituitary function. Some are accompanied by other congenital malformations. MRI-imaging is the most valuable noninvasive method. It is of great importance for early diagnosis and early treatment in PSIS children.
出处
《中国实用儿科杂志》
CSCD
北大核心
2014年第1期43-47,共5页
Chinese Journal of Practical Pediatrics
关键词
垂体柄阻断综合征
磁共振成像
垂体功能低下
pituitary stalk interruption syndrome
magnetic resonance imaging
hypopituitarism
