Oh Yeon Joung, Park Hong Kyu, Yang Seung, Song Jun Ho, Hwang Il Tae
Department of Pediatrics, Hallym University College of Medicine, Seoul, Korea.
Department of Neurosurgery, Hallym University College of Medicine, Seoul, Korea.
Ann Pediatr Endocrinol Metab. 2014 Mar;19(1):20-6. doi: 10.6065/apem.2014.19.1.20. Epub 2014 Mar 31.
In the pediatric population, Rathke's cleft cysts (RCCs) are known to be an infrequent cause of headaches, visual disturbances, and pituitary dysfunction. We investigated the clinical characteristics of children in whom RCCs were incidentally discovered and evaluated whether RCCs influence the treatment response of patients with proven endocrinopathy.
A retrospective analysis was conducted in 34 patients with RCCs who were diagnosed between 2006 and 2013 at Hallym University Medical Center. Their clinical, hormonal, and imaging findings were reviewed. We evaluated the clinical outcomes of the patients with concomitant RCCs and endocrinopathy compared to matched controls.
Twenty-six of 34 patients with radiologically proven RCCs had endocrine disorders. They were 9 boys and 17 girls, with ages ranging from 4.8 to 17.4 years at the time of the diagnosis. Of these, 7 (27%) had idiopathic short stature, 7 (27%) had growth hormone deficiency (GHD), and 12 (46%) had central precocious puberty (CPP). Nineteen of 26 patients (73.1%) showed low signal intensities on T1-weighted images (T1WI) and high signal intensities on T2-weighted images. The incidence of hypointensity on T1WI was higher in the patients with RCCs accompanied by endocrinopathy than in those without endocrinopathy (P=0.033). The treatment outcomes of the patients with CPP and GHD with and without RCCs were similar.
CPP and GHD patients with a small RCC (less than 20 mm) expressing cystic magnetic resonance intensity can be managed with medical treatment, although the RCCs need to be closely monitored in radiological studies to observe their growth.
在儿科人群中,已知拉克氏裂囊肿(RCCs)是引起头痛、视觉障碍和垂体功能障碍的罕见原因。我们调查了偶然发现RCCs的儿童的临床特征,并评估RCCs是否影响已确诊内分泌病患者的治疗反应。
对2006年至2013年在韩林大学医学中心诊断的34例RCCs患者进行回顾性分析。回顾了他们的临床、激素和影像学检查结果。我们评估了合并RCCs和内分泌病的患者与匹配对照组的临床结局。
34例经放射学证实为RCCs的患者中有26例患有内分泌疾病。他们为9名男孩和17名女孩,诊断时年龄在4.8至17.4岁之间。其中,7例(27%)患有特发性身材矮小,7例(27%)患有生长激素缺乏症(GHD),12例(46%)患有中枢性性早熟(CPP)。26例患者中有19例(73.1%)在T1加权图像(T1WI)上显示低信号强度,在T2加权图像上显示高信号强度。伴有内分泌病的RCCs患者T1WI低信号强度的发生率高于无内分泌病的患者(P = 0.033)。伴有和不伴有RCCs的CPP和GHD患者的治疗结局相似。
对于患有小RCC(小于20 mm)且表现为囊性磁共振强度的CPP和GHD患者,可采用药物治疗,尽管在放射学研究中需要密切监测RCCs以观察其生长情况。