Gonzales-Portillo G, Tomita T
Division of Pediatric Neurosurgery, Children's Memorial Hospital and Northwestern University Medical School, Chicago, Illinois 60614, USA.
Neurosurgery. 1998 Apr;42(4):917-21; discussion 921-2. doi: 10.1097/00006123-199804000-00131.
Childhood craniopharyngiomas may present with variable endocrine dysfunctions. However, hyponatremia secondary to the syndrome of inappropriate secretion of antidiuretic hormone has never been reported. We describe three children with craniopharyngioma who presented with hyponatremia.
Three children had hyponatremia at presentation, two of whom had experienced generalized seizures. Urine sodium levels measured in two patients were abnormally high. Neuroimaging tests, including computed tomography and magnetic resonance imaging, showed a large partially calcified and partially cystic craniopharyngioma in the sellar/suprasellar location.
Intake of fluids was restricted for each patient, with improvement of hyponatremia, and each patient subsequently underwent a successful tumor resection. Postoperatively, all patients developed panhypopituitarism, including diabetes insipidus, and needed multiple hormonal replacement therapy.
Endocrine dysfunctions at diagnosis are commonly associated with childhood craniopharyngiomas, but the association of the syndrome of inappropriate secretion of antidiuretic hormone with craniopharyngioma has never been reported. Craniopharyngiomas should be included as a possibility in making the differential diagnosis of the syndrome of inappropriate secretion of antidiuretic hormone in children.
儿童颅咽管瘤可能伴有多种内分泌功能障碍。然而,抗利尿激素分泌不当综合征继发的低钠血症此前从未有过报道。我们描述了3例患有颅咽管瘤并出现低钠血症的儿童。
3例儿童就诊时均有低钠血症,其中2例曾发生全身性癫痫发作。对2例患者测定的尿钠水平异常升高。包括计算机断层扫描和磁共振成像在内的神经影像学检查显示,鞍区/鞍上区有一个大的部分钙化、部分囊性的颅咽管瘤。
对每位患者限制液体摄入,低钠血症得到改善,随后每位患者均成功进行了肿瘤切除。术后,所有患者均出现全垂体功能减退,包括尿崩症,需要多种激素替代治疗。
诊断时的内分泌功能障碍通常与儿童颅咽管瘤相关,但抗利尿激素分泌不当综合征与颅咽管瘤的关联此前从未有过报道。在对儿童抗利尿激素分泌不当综合征进行鉴别诊断时,应考虑颅咽管瘤的可能性。