The correct interpretation of the modern neuroradiologic techniques, including CSF flow MR imaging, may help in identifying a "real" Dandy-Walker malformation. Among therapeutical strategies, single shunting (ventriculo-peritoneal or cyst-peritoneal shunts) appears effective in the control of both ventricle and cyst size. Endoscopic third ventriculostomy may be considered an acceptable alternative, especially in older children, with the aim to reduce the shunt-related problems. Prognosis and intellectual outcome mostly depend on the presence of associated malformations, the degree of vermian malformation and the adequate control of hydrocephalus.
The correct interpretation of the modern neuroradiologic techniques may help in selecting adequate treatment between the two main options (third ventriculostomy or shunting). In the last decades, endoscopic third ventriculostomy has become the first-line treatment of aqueductal stenosis; however, some issues, such as the cause of failures in well selected patients, long-term outcome in infant treated with ETV, and effect of persistent ventriculomegaly on neuropsychological developmental, remain unanswered.
Endoscopic third ventriculostomy should be considered as an alternative procedure to ventriculo-peritoneal shunting and external ventricular draining for the emergency control of severe hydrocephalus caused by posterior fossa tumors, since it can quickly eliminate symptoms, and hence, can delay surgery scheduling if required. Even though ETV does not prevent postoperative hydrocephalus in all cases, it does protect against acute postoperative hydrocephalus due to cerebellar swelling. In addition, it eliminates the risks of cerebrospinal fluid (CSF) infection related to external drainage and minimizes the risk of overdrainage because it provides more physiological CSF drainage than the other procedures. Since postoperative hydrocephalus is very often physically obstructive, ETV should always be considered a possible treatment procedure.
Endoscopic placement of a stent in the aqueduct is more effective in preventing the repeated occlusion of the aqueduct than aqueductoplasty alone and should be indicated as the initial treatment in each case of compatible anatomy.
The high ICP observed in a group of patients in the early postoperative days is probably related to the slow permeation of the subarachnoid spaces by the cerebrospinal fluid flowing out of the third ventriculostomy. Management of intracranial hypertension after ETV remains a matter of controversy. The role of the lumbar puncture in the faster normalization of the ICP is examined in this article. By increasing the compliance and the buffering capacities of the spinal subarachnoid spaces, it probably decreases the cerebrospinal fluid outflow resistance from the ventricular system, facilitating the decrease of the ventricular volume and allowing faster permeation of the intracranial subarachnoid spaces. High postoperative ICP can account for persistent symptoms of intracranial hypertension and ventricular dilatation on computed tomographic scans after third ventriculostomy. A cycle of one to three lumbar punctures should always be performed in patients who remain symptomatic and who show increasing ventricular dilatation after ETV, before ETV is assumed to have failed and an extracranial cerebrospinal fluid shunt is implanted.
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