Schmitt S, Wichmann W, Martin E, Zachmann M, Schoenle E J
Department of Paediatrics, University of Zurich, Switzerland.
Eur J Pediatr. 1993 May;152(5):399-401. doi: 10.1007/BF01955896.
In up to 25% of cases of children with central diabetes insipidus no organic cause can be documented. We present three boys (age 2.2, 2.3 and 6 years at diagnosis) with acute onset central diabetes insipidus, in whom the only pathological finding using MRI was a thickened central part of the pituitary stalk (> 2.5 mm). Recent reports demonstrate similar MRI findings in adults with Langerhans cell histiocytosis (LCH), sarcoidosis, or tuberculosis, and in children with proven LCH and diabetes insipidus. In those adults with LCH, the pituitary stalk lesion has been histologically verified as a sequela of LCH. In contrast, in two of our three patients pituitary stalk thickening preceded the typical peripheral lesions of LCH by several months, whereas in the third patient there is as yet no evidence of systemic disease. We conclude that thickening of the central part of the pituitary stalk might represent the first manifestations of LCH clinically presenting with diabetes insipidus. MRI investigation of the pituitary stalk in children with unexplained central diabetes insipidus and accurate follow up in patients with thickening of the pituitary stalk in necessary to avoid missing other manifestations of a systemic disease.
在高达25%的中枢性尿崩症儿童病例中，无法找到器质性病因。我们报告了三名男孩（诊断时年龄分别为2.2岁、2.3岁和6岁），他们患有急性起病的中枢性尿崩症，MRI检查仅发现垂体柄中央部分增厚（>2.5毫米）。最近的报告显示，成人朗格汉斯细胞组织细胞增多症（LCH）、结节病或结核病患者以及确诊为LCH和尿崩症的儿童也有类似的MRI表现。在那些LCH成人患者中，垂体柄病变经组织学证实为LCH的后遗症。相比之下，在我们的三名患者中，有两名患者的垂体柄增厚比LCH典型的外周病变早几个月出现，而第三名患者尚无全身疾病的证据。我们得出结论，垂体柄中央部分增厚可能是临床上以尿崩症为表现的LCH的首发症状。对于不明原因的中枢性尿崩症儿童，对垂体柄进行MRI检查，并对垂体柄增厚的患者进行准确随访，对于避免漏诊全身疾病的其他表现很有必要。
