Hashimoto Hiroyuki, Maeda Akiko, Kumano Koichi, Kimoto Tatsuya, Fujisawa Yuko, Akai Takuya
Department of Pediatrics, Kanazawa Red Cross Hospital, Kanazawa, Japan.
Pediatr Int. 2014 Aug;56(4):e30-2. doi: 10.1111/ped.12387.
Fourth ventricular outlet obstruction (FVOO) is a rare cause of obstructive hydrocephalus. Although FVOO accompanied by malformative syndrome and secondary causes of obstruction are common, there are few reports of primary FVOO (PFVOO). The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a rare presenting feature of hydrocephalus. A 1-year, 8-month-old boy with a normal head circumference developed SIADH accompanied by rapid deterioration of symptoms of intracranial hypertension. PFVOO was diagnosed because magnetic resonance imaging revealed an enlarged ventricular system with a barely visible membranous obstacle at the foramen of Magendie. All symptoms were resolved by endoscopic third ventriculostomy. PFVOO should be considered as a rare form of congenital obstructive hydrocephalus, especially in patients with tetraventricular hydrocephalus. To the best of our knowledge, this is the first case of an infant with SIADH, resulting from acute deterioration of non-tumoral raised pressure hydrocephalus.
第四脑室出口梗阻（FVOO）是梗阻性脑积水的罕见病因。虽然伴有畸形综合征和继发性梗阻原因的FVOO很常见，但原发性FVOO（PFVOO）的报道却很少。抗利尿激素分泌不当综合征（SIADH）是脑积水罕见的临床表现。一名头围正常的1岁8个月男孩出现了SIADH，并伴有颅内高压症状的迅速恶化。由于磁共振成像显示脑室系统扩大，在马根迪孔处可见一个几乎看不见的膜性障碍物，因此诊断为PFVOO。通过内镜下第三脑室造瘘术，所有症状均得到缓解。PFVOO应被视为先天性梗阻性脑积水的一种罕见形式，尤其是在患有四脑室脑积水的患者中。据我们所知，这是首例因非肿瘤性压力性脑积水急性恶化导致SIADH的婴儿病例。
