University of Texas Southwestern Medical School, Dallas, Texas, USA.
Neurosurg Focus. 2011 Jul;31(1):E3. doi: 10.3171/2011.5.FOCUS1178.
Rathke cleft cysts (RCC) are benign sellar lesions most often found in adults, and more infrequently in children. They are generally asymptomatic but sometimes require surgical treatment through a transsphenoidal corridor. The purpose of this study was to compare adult versus pediatric cases of RCC.
The authors retrospectively reviewed presenting symptoms, MR imaging findings, laboratory study results, and pathological findings in 147 adult and 14 pediatric patients who underwent surgery for treatment of RCCs at the University of Californial at San Francisco between 1996 and 2008.
In both the adult and pediatric groups, most patients were female (78% of adults, 79% of pediatric patients, p = 0.9). Headache was the most common symptom in both groups (reported by 50% of pediatric patients and 33% of adults, p = 0.2). Preoperative hypopituitarism occurred in 41% of adults and 45% of pediatric patients (p = 0.8). Growth delay, a uniquely pediatric finding, was a presenting sign in 29% of pediatric patients. Visual complaints were a presenting symptom in 16% of adult and 7% of pediatric patients (p = 0.4). There was no difference between median cyst size in adults versus pediatric patients (1.2 cm in both, p = 0.7). Temporary or permanent postoperative diabetes insipidus occurred in 12% of adults and 21% of pediatric patients (p = 0.4). Kaplan-Meier analysis revealed an 8% RCC recurrence rate at 2 years for each group (p = 0.5).
The incidence of RCCs is much lower in the pediatric population; however, symptoms, imaging findings, and outcomes are similar, suggesting that pediatric RCCs arise from growth of remnants of the embryonic Rathke pouch earlier in life than adult RCCs but do not differ in any other way. It is important to consider RCCs in the differential diagnosis when pediatric patients present with visual impairment, unexplained headache, or hypopituitarism including growth delay. Although the average RCC size was similar in our pediatric and adult patient groups, the smaller size of the pituitary gland in pediatric patients suggests an increased relative RCC size.
Rathke 裂隙囊肿（RCC）是最常见于成年人的良性鞍内病变，在儿童中则较为少见。它们通常是无症状的，但有时需要通过经蝶窦入路进行手术治疗。本研究的目的是比较成人和儿童 RCC 病例。
作者回顾性分析了 1996 年至 2008 年期间在加利福尼亚大学旧金山分校接受手术治疗 RCC 的 147 例成人和 14 例儿童患者的临床表现、磁共振成像（MRI）表现、实验室研究结果和病理发现。
在成人和儿童组中，大多数患者为女性（成人组占 78%，儿童组占 79%，p=0.9）。头痛是两组中最常见的症状（儿童组报告 50%，成人组报告 33%，p=0.2）。术前垂体功能减退症发生在成人组中占 41%，儿童组中占 45%（p=0.8）。生长迟缓是一种独特的儿童表现，在 29%的儿童患者中为首发症状。成人和儿童患者中，视力障碍分别占 16%和 7%（p=0.4）。成人和儿童患者的囊肿中位大小无差异（分别为 1.2cm，p=0.7）。成人组中有 12%和儿童组中有 21%的患者发生暂时性或永久性术后尿崩症（p=0.4）。Kaplan-Meier 分析显示，两组患者的 RCC 复发率在 2 年内均为 8%（p=0.5）。
RCC 在儿童人群中的发病率较低；然而，症状、影像学表现和结局相似，表明儿童 RCC 比成人 RCC 更早地从胚胎 Rathke 囊的残留物生长而来，但在其他方面没有不同。当儿童患者出现视力障碍、不明原因头痛或垂体功能减退症（包括生长迟缓）时，应考虑 RCC 作为鉴别诊断。尽管我们的儿童和成人患者组的 RCC 平均大小相似，但儿童患者的垂体较小，表明相对 RCC 大小增加。
