Abstract:
Suprasellar arachnoid cysts represent a rare occurrence in the pediatric population and usually cause symptoms related to mass effect and can occasionally cause endocrine dysfunctions. The association between SAC and the syndrome of inappropriate antidiuretic hormone (SIADH) in the pediatric population has rarely been described previously. In most cases, SIADH is temporary and resolves by treating the underlying cause. The first-line treatment consists of fluid restriction in asymptomatic children. Oral urea and demeclocycline are other effective treatment options. Vaptans are a new class of medication for the management of SIADH. These agents are a nonpeptide vasopressin V2 receptor antagonist that selectively antagonizes the antidiuretic effect of AVP, resulting in excretion of diluted urine or \"aquaresis.\" Their efficacy has been shown in adult patients with euvolemic or hypervolemic hyponatremia. However, evidence is lacking in pediatric patients with SIADH. We report the case of a 9-year-old female child with a SAC, who underwent endoscopic fenestration at the age of 2 years. After surgery she developed chronic hyponatremia due to SIADH. Hyponatremia was refractory to treatment with fluid restriction, oral sodium, and urea. In order to normalize serum sodium levels, tolvaptan treatment was started on a compassionate-use basis; 24-48 h later serum sodium levels returned to normal. To date, tolvaptan has been used regularly for 6 years with no side effects occurring during the treatment period. This is the first case of a child with chronic SIADH secondary to SAC successfully treated with tolvaptan. Further studies are needed to demonstrate its usefulness on a broader case series.
摘要:
鞍上蛛网膜囊肿在儿科人群中很少发生,通常会引起与肿块效应相关的症状,偶尔会引起内分泌功能障碍。先前很少描述SAC与儿科人群中抗利尿激素不当综合征(SIADH)之间的关联。在大多数情况下,SIADH是暂时的,可以通过治疗根本原因来解决。一线治疗包括无症状儿童的液体限制。口服尿素和去氯环素是其他有效的治疗选择。Vaptans是用于管理SIADH的一类新药物。这些药物是选择性拮抗AVP的抗利尿作用的非肽血管加压素V2受体拮抗剂,导致排泄稀释的尿液或“水便”。“他们的疗效已被证明在成人患者中具有正常血容量或高血容量低钠血症。然而,SIADH儿科患者缺乏证据。我们报告了一个9岁的女性儿童患有SAC的病例,在2岁时接受内窥镜开窗术的患者。手术后,由于SIADH,她出现了慢性低钠血症。低钠血症对液体限制治疗难以治疗,口服钠,和尿素。为了使血清钠水平正常化,托伐普坦治疗是在同情使用的基础上开始的;24-48小时后,血清钠水平恢复正常。迄今为止,托伐普坦已定期使用6年,在治疗期间无副作用发生。这是用托伐普坦成功治疗的SAC继发的慢性SIADH儿童的第一例。需要进一步的研究来证明其在更广泛的案例系列中的有用性。