Bizzarri Carla, Marini Romana, Ubertini Graziamaria, Cappa Marco
Unit of Endocrinology and Diabetes, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Clin Pediatr Endocrinol. 2012 Oct;21(4):75-80. doi: 10.1297/cpe.21.75. Epub 2012 Oct 30.
Rathke cleft cysts are remnants of the Rathke pouch. Most of them are asymptomatic, but sometimes they can grow enough to cause compression of structures within and/or close to the sella, thus eliciting symptoms such as visual disturbance, pituitary defects, and headache. Asymptomatic cysts can safely be followed up with serial imaging, while the standard treatment for symptomatic lesions is surgical removal. We describe a 14-yr-old boy, admitted for anorexia, fatigue, weight loss, recurrent headache and vomiting. Magnetic resonance imaging showed an intra- and suprasellar cystic lesion, which was surgically removed. Histology was consistent with Rathke's cleft cyst. Diabetes insipidus and multiple anterior pituitary defects (GH, ACTH and TSH) were found preoperatively, and substitutive therapy was started. No additional hormonal defect appeared after surgery. After 4 yr of follow up, pituitary function was retested, and there were no confirmed GH or ACTH defects, allowing a partial withdrawal of replacement therapy. Our report confirms that pituitary defects, in patients with a Rathke cleft cyst, may recover even year after surgery. Thus, retesting of pituitary axes is indicated during long-term follow up.
拉克氏裂囊肿是拉克氏囊的残余物。它们大多无症状，但有时会生长到足以压迫蝶鞍内和/或附近的结构，从而引发视觉障碍、垂体缺陷和头痛等症状。无症状囊肿可通过系列影像学检查安全随访，而有症状病变的标准治疗方法是手术切除。我们描述了一名14岁男孩，因厌食、疲劳、体重减轻、反复头痛和呕吐入院。磁共振成像显示鞍内和鞍上囊性病变，已通过手术切除。组织学检查结果与拉克氏裂囊肿一致。术前发现尿崩症和多种垂体前叶缺陷（生长激素、促肾上腺皮质激素和促甲状腺激素），并开始替代治疗。术后未出现其他激素缺陷。经过4年随访，重新检测垂体功能，未确认存在生长激素或促肾上腺皮质激素缺陷，因此可以部分停用替代治疗。我们的报告证实，拉克氏裂囊肿患者的垂体缺陷甚至在术后数年也可能恢复。因此，在长期随访期间需要重新检测垂体轴。
