Sugawara A, Ebina K, Ohi H, Sawataishi J, Akabane M, Itoh T, Kikuchi K, Sakamoto T
Department of Neurosurgery, Nakadohri Hospital.
No Shinkei Geka. 1992 Oct;20(10):1107-12.
The pathogenesis remains unknown in the majority of patients with precocious puberty, and yet infrequently such causative cerebral lesions as hypothalamic hamartomas are associated with sexual precocity. We reported a rare case of suprasellar arachnoid cyst in an infant presenting with precocious puberty, which eventually disappeared after a cyst-peritoneal shunt. It was believed that the mass effect of the arachnoid cyst upon the hypothalamus was, at least in part, responsible for development of precocious puberty. The role of surgical decompression of the cyst was also discussed. A one-year-old girl was admitted to the hospital for evaluation of genital bleeding which had persisted on and off for two months. The height, 80cm, and the weight, 12.4kg, exceeded by far the two standard deviations from the mean level of the normal population. In addition she had the development of breast tissue as classified Tanner's Stage II, and both pubic and axillary hair. The bone age by skeletal survey of the hand was rated as 3 years. Endocrinological examination showed that serum levels of luteinizing hormone (LH), follicle-stimulating hormone (FSH) and estradiol had increased for her age, to levels equivalent to those for females at puberty. An LH-RH test revealed an excessive LH reaction. There were no definite neurological deficits. CT and MRI demonstrated the presence of a large arachnoid cyst involving the suprasellar region as well as the right middle and posterior fossa. After the patient underwent a cyst-peritoneal shunt, the cyst decreased in size and such symptoms as genital bleeding and breast growth disappeared. Serum levels of her LH and FSH also significantly decreased.(ABSTRACT TRUNCATED AT 250 WORDS)
大多数性早熟患者的发病机制尚不清楚,不过诸如下丘脑错构瘤等导致性早熟的脑损伤较为罕见。我们报告了1例患有性早熟的婴儿,其患有罕见的鞍上蛛网膜囊肿,在进行囊肿 - 腹腔分流术后囊肿最终消失。据信蛛网膜囊肿对下丘脑的占位效应至少部分导致了性早熟的发生。还讨论了囊肿手术减压的作用。一名1岁女童因持续两个月的生殖器出血入院评估。其身高80厘米,体重12.4千克,远超正常人群平均水平的两个标准差。此外,她乳房组织发育至坦纳Ⅱ期,出现了阴毛和腋毛。手部骨骼X线检查显示骨龄为3岁。内分泌检查表明,其血清促黄体生成素(LH)、促卵泡生成素(FSH)和雌二醇水平高于同龄人,达到青春期女性的水平。LH - RH试验显示LH反应过度。无明确神经功能缺损。CT和MRI显示鞍上区域以及右侧中颅窝和后颅窝存在一个大的蛛网膜囊肿。患者接受囊肿 - 腹腔分流术后,囊肿体积减小,生殖器出血和乳房发育等症状消失。其血清LH和FSH水平也显著下降。(摘要截选至250字)