Hamilton J, Blaser S, Daneman D
Division of Pediatric Endocrinology, Hospital for Sick Children, University of Toronto, Ontario, Canada.
AJNR Am J Neuroradiol. 1998 Oct;19(9):1609-15.
MR imaging findings of one or more of the following has been suggested to be a sensitive and specific indicator of hypopituitarism: small anterior pituitary gland, attenuated or absent pituitary stalk, and ectopic posterior pituitary. We hypothesized that these MR findings would be common in our group of patients with idiopathic isolated growth hormone deficiency (GHD) or multiple pituitary hormone deficiencies (MPHD) and would be a good indicator of the severity of the hypopituitarism.
MR images were obtained for 35 patients with idiopathic GHD (20 with isolated GHD and 15 with MPHD; age range, 2 to 17 years) and analyzed to define one or more of the following triad of abnormalities: 1) small/absent anterior pituitary, 2) truncated/absent pituitary stalk, and 3) ectopic posterior pituitary, as well as for any other associated anomalies. The findings were correlated with the clinical and biochemical presentation.
Pituitary abnormalities were common in both groups (80% with isolated GHD, 93% with MPHD). We found a high frequency of midline CNS malformations, including optic nerve hypoplasia (9%), Chiari type I malformations (20%), and medial deviation of the carotid arteries (37%). Breech delivery, neonatal hypoglycemia, jaundice, micropenis, or single central incisor occurred equally with both isolated GHD and MPHD. In patients whose peak growth hormone level was less than 3 microg/L (n = 19), 90% had the MR triad, compared with 390% of those with growth hormone levels 3 microg/L or greater or less than 8 microg/L (n = 13) (P <.01). Almost all (92%) of those with ectopic posterior pituitary had anterior pituitary heights less than -2 SD for age.
MR abnormalities were common in children with both isolated GHD and MPHD and were closely associated with peak growth hormone levels less than 3 microg/L. The presence of other CNS and clinical findings (eg, single central incisor and micropenis) supports the theory of an embryologic defect as the cause of the pituitary abnormalities.
以下一项或多项磁共振成像(MR)表现被认为是垂体功能减退的敏感且特异指标:垂体前叶小、垂体柄变细或缺如以及垂体后叶异位。我们推测这些MR表现在我们的特发性孤立性生长激素缺乏症(GHD)或多种垂体激素缺乏症(MPHD)患者组中会很常见,并且会是垂体功能减退严重程度的良好指标。
对35例特发性GHD患者(20例孤立性GHD和15例MPHD;年龄范围2至17岁)进行MR成像,并分析以确定以下三项异常中的一项或多项:1)垂体前叶小/缺如,2)垂体柄截断/缺如,3)垂体后叶异位,以及任何其他相关异常。将这些发现与临床和生化表现进行关联。
两组中垂体异常均很常见(孤立性GHD患者中为80%,MPHD患者中为93%)。我们发现中线中枢神经系统畸形的发生率很高,包括视神经发育不全(9%)、Chiari I型畸形(20%)以及颈动脉内侧移位(37%)。臀位分娩、新生儿低血糖、黄疸、小阴茎或单颗中切牙在孤立性GHD和MPHD患者中出现的情况相同。在生长激素峰值水平低于3μg/L的患者(n = 19)中,90%有MR三联征,而生长激素水平为3μg/L或更高但低于8μg/L的患者(n = 13)中这一比例为30%(P <.01)。几乎所有(92%)垂体后叶异位的患者垂体前叶高度低于年龄对应的-2标准差。
MR异常在孤立性GHD和MPHD儿童中很常见,并且与生长激素峰值水平低于3μg/L密切相关。其他中枢神经系统和临床发现(如单颗中切牙和小阴茎)的存在支持胚胎学缺陷是垂体异常原因的理论。