Gazioglu Nurperi, Canaz Huseyin, Camlar Mahmut, Tanrıöver Necmettin, Kocer Naci, Islak Civan, Evliyaoglu Olcay, Ercan Oya
Department of Neurosurgery, Istanbul Bilim University, Istanbul, Turkey.
Department of Neurosurgery, Tepecik Research and Training Hospital, University of Health Sciences, Izmir, Turkey.
Childs Nerv Syst. 2019 Nov;35(11):2127-2132. doi: 10.1007/s00381-018-4013-5. Epub 2018 Nov 28.
Pituitary adenomas are rare in childhood in contrast with adults. Adrenocorticotropic hormone (ACTH)-secreting adenomas account for Cushing's disease (CD) which is the most common form of ACTH-dependent Cushing's syndrome (CS). Treatment strategies are generally based on data of adult CD patients, although some difficulties and differences exist in pediatric patients. The aim of this study is to share our experience of 10 children and adolescents with CD.
Medical records, images, and operative notes of 10 consecutive children and adolescents who underwent transsphenoidal surgery for CD between 1999 and 2014 in Cerrahpasa Faculty of Medicine were retrospectively reviewed. Mean age at operation was 14.8 ± 4.2 years (range 5-18). The mean length of symptoms was 24.2 months. The mean follow-up period was 11 years (range 4 to 19 years).
Mean preoperative cortisol level was 23.435 μg/dl (range 8.81-59.8 μg/dl). Mean preoperative ACTH level was 57.358 μg/dl (range 28.9-139.9 μg/dl). MR images localized microadenoma in three patients (30%), macroadenoma in four patients (40%) in our series. Transsphenoidal microsurgery and endoscopic transsphenoidal surgery were performed in 8 and 2 patients respectively. Remission was provided in 8 patients (80%). Five patients (50%) met remission criteria after initial operations. Three patients (30%) underwent additional operations to meet remission criteria.
Transsphenoidal surgery remains the mainstay therapy for CD in pediatric patients as well as adults. It is an effective treatment option with low rate of complications. Both endoscopic and microscopic approaches provide safe access to sella and satisfactory surgical results.
与成人相比，垂体腺瘤在儿童期较为罕见。分泌促肾上腺皮质激素（ACTH）的腺瘤可导致库欣病（CD），这是ACTH依赖性库欣综合征（CS）最常见的形式。尽管儿科患者存在一些困难和差异，但治疗策略通常基于成年CD患者的数据。本研究的目的是分享我们对10例儿童和青少年CD患者的治疗经验。
回顾性分析了1999年至2014年在切拉帕萨医学院接受经蝶窦手术治疗CD的10例连续儿童和青少年的病历、影像资料及手术记录。手术时的平均年龄为14.8±4.2岁（范围5 - 18岁）。症状的平均持续时间为24.2个月。平均随访期为11年（范围4至19年）。
术前平均皮质醇水平为23.435μg/dl（范围8.81 - 59.8μg/dl）。术前平均ACTH水平为57.358μg/dl（范围28.9 - 139.9μg/dl）。在我们的系列研究中，磁共振成像（MR）显示3例患者（30%）为微腺瘤，4例患者（40%）为大腺瘤。分别有8例和2例患者接受了经蝶窦显微手术和内镜经蝶窦手术。8例患者（80%）实现缓解。5例患者（50%）在初次手术后达到缓解标准。3例患者（30%）接受了额外手术以达到缓解标准。
经蝶窦手术仍然是儿科和成年CD患者的主要治疗方法。它是一种有效的治疗选择，并发症发生率低。内镜和显微手术方法都能安全进入蝶鞍并取得满意的手术效果。
