Spennato Pietro, Chiaramonte Carmela, Cicala Domenico, Donofrio Vittoria, Barbarisi Manlio, Nastro Anna, Mirone Giuseppe, Trischitta Vincenzo, Cinalli Giuseppe
Departments of 1 Paediatric Neurosurgery and.
Division of Neurosurgery, Department of Neurosciences and Reproductive and Odontostomatological Sciences, School of Medicine and Surgery "Federico II," Naples; and.
Neurosurg Focus. 2016 Nov;41(5):E9. doi: 10.3171/2016.8.FOCUS16269.
OBJECTIVE Intraventricular choroid plexus cysts are unusual causes of acute hydrocephalus in children. Radiological diagnosis of intraventricular choroid plexus cysts is difficult because they have very thin walls and fluid contents similar to CSF and can go undetected on routine CT studies. METHODS This study reports the authors' experience with 5 patients affected by intraventricular cysts originating from the choroid plexus. All patients experienced acute presentation with rapid neurological deterioration, sometimes associated with hypothalamic dysfunction, and required urgent surgery. In 2 cases the symptoms were intermittent, with spontaneous remission and sudden clinical deteriorations, reflecting an intermittent obstruction of the CSF pathway. RESULTS Radiological diagnosis was difficult in these cases because a nonenhanced CT scan revealed only triventricular hydrocephalus, with slight lateral ventricle asymmetry in all cases. MRI with driven-equilibrium sequences and CT ventriculography (in 1 case) allowed the authors to accurately diagnose the intraventricular cysts that typically occupied the posterior part of the third ventricle, occluding the aqueduct and at least 1 foramen of Monro. The patients were managed by urgent implantation of an external ventricular drain in 1 case (followed by endoscopic surgery, after completing a diagnostic workup) and by urgent endoscopic surgery in 4 cases. Endoscopic surgery allowed the shrinkage and near-complete removal of the cysts in all cases. Use of neuronavigation and a laser were indispensable. All procedures were uneventful, resulting in restoration of normal neurological conditions. Long-term follow-up (> 2 years) was available for 2 patients, and no complications or recurrences occurred. CONCLUSIONS This case series emphasizes the necessity of an accurate and precise identification of the possible causes of triventricular hydrocephalus. Endoscopic surgery can be considered the ideal treatment of choroid plexus cysts in children.
目的 脑室内脉络丛囊肿是儿童急性脑积水的罕见病因。脑室内脉络丛囊肿的影像学诊断较为困难，因为其壁非常薄且囊液成分与脑脊液相似，在常规CT检查中可能无法被发现。方法 本研究报告了作者对5例起源于脉络丛的脑室内囊肿患者的治疗经验。所有患者均急性起病，伴有迅速的神经功能恶化，有时伴有下丘脑功能障碍，需要紧急手术。其中2例症状呈间歇性，有自发缓解和突然的临床恶化，提示脑脊液通路间歇性梗阻。结果 这些病例的影像学诊断困难，因为平扫CT仅显示三脑室脑积水，所有病例均有轻度侧脑室不对称。采用驱动平衡序列的MRI及1例患者的CT脑室造影使作者能够准确诊断通常占据第三脑室后部、阻塞导水管和至少1个孟氏孔的脑室内囊肿。1例患者紧急植入了外引流管（完成诊断性检查后行内镜手术），4例患者行紧急内镜手术。内镜手术在所有病例中均使囊肿缩小并近乎完全切除。神经导航和激光的使用必不可少。所有手术均顺利，神经功能恢复正常。2例患者获得了超过2年的长期随访，未发生并发症或复发。结论 本病例系列强调了准确识别三脑室脑积水可能病因的必要性。内镜手术可被视为儿童脉络丛囊肿的理想治疗方法。
