Adan L, Bussières L, Dinand V, Zerah M, Pierre-Kahn A, Brauner R
Department of Paediatric Endocrinology, Hôpital Necker-Enfants Malades, Paris, France.
Eur J Pediatr. 2000 May;159(5):348-55. doi: 10.1007/s004310051285.
A suprasellar arachnoid cyst may cause disorders of growth, puberty and hypothalamic-pituitary function, due to the proximity of the cyst to the hypothalamic-pituitary area. A total of 30 patients (17 boys) with cyst diagnosed at 4.3 +/- 1 years were routinely evaluated at 5.4 +/- 1 years; 24 of them had one or multiple cyst derivations. Some 23 cases had an abnormal height, weight or puberty: short (< -2SD, 5 cases) or tall ( > 2SD, 10 cases) stature, overweight (body mass index, BMI, > 2SD, 6 cases), central precocious puberty (10 cases) and/or no progression of pubertal development (3 cases). The growth hormone (GH) peaks after pharmacological stimulation test were low (< 10 MICROg/L) in 16 patients, confirmed by a second evaluation in 8/11 of them. The plasma free thyroxine was low in five patients, prolactin was high in two and the cortisol and concomitant plasma and urinary osmolalities were normal. BMI was correlated negatively with the GH peaks (r = -0.37, P < 0.01) and positively with the plasma leptin concentrations (r = 0.55, P < 0.01). The plasma fasting insulin concentrations were also correlated negatively with the GH peaks (r = -0.55, P < 0.02) and positively with the plasma insulin-like growth factor I concentrations (r = 0.64, P < 0.002). The adult height (12 cases) was at 4SD in 1 and < -2SD in 4 patients, two of whom had precocious puberty untreated with gonadotropin releasing hormone (GnRH) analogue, and two had untreated GH deficiency. The adult height of those treated was normal. One girl had primary amenorrhoea and two boys had low plasma testosterone, despite a normal gonadotropin response to a GnRH test.
Suprasellar arachnoid cysts may cause deficiencies of growth hormone and thyrotropin, stimulation of the hypothalamic-pituitary-gonadal axis, tall stature and/or overweight. These last two disorders may be due to hyperinsulinism, itself due to suprasellar arachnoid cyst.
鞍上蛛网膜囊肿可能导致生长、青春期及下丘脑 - 垂体功能障碍,因为囊肿紧邻下丘脑 - 垂体区域。共有30例囊肿患者(17名男孩)在4.3±1岁时被诊断,在5.4±1岁时接受常规评估;其中24例有一个或多个囊肿衍生。约23例患者有身高、体重或青春期异常:身材矮小(<-2SD,5例)或高大(>2SD,10例),超重(体重指数,BMI,>2SD,6例),中枢性性早熟(10例)和/或青春期发育无进展(3例)。16例患者经药物刺激试验后的生长激素(GH)峰值较低(<10μg/L),其中8/11经二次评估得到证实。5例患者血浆游离甲状腺素较低,2例催乳素较高,皮质醇以及血浆和尿渗透压正常。BMI与GH峰值呈负相关(r = -0.37,P < 0.01),与血浆瘦素浓度呈正相关(r = 0.55,P < 0.01)。空腹血浆胰岛素浓度也与GH峰值呈负相关(r = -0.55,P < 0.02),与血浆胰岛素样生长因子I浓度呈正相关(r = 0.64,P < 0.002)。12例患者的成人身高,1例在4SD,4例<-2SD,其中2例患有未用促性腺激素释放激素(GnRH)类似物治疗的性早熟,2例患有未治疗的生长激素缺乏症。接受治疗者的成人身高正常。一名女孩有原发性闭经,两名男孩血浆睾酮水平低,尽管对GnRH试验的促性腺激素反应正常。
鞍上蛛网膜囊肿可能导致生长激素和促甲状腺激素缺乏,刺激下丘脑 - 垂体 - 性腺轴,身材高大和/或超重。后两种病症可能是由于高胰岛素血症,其本身是由鞍上蛛网膜囊肿引起的。