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In this article we provide an update on the recent developments in understanding the diagnosis and treatment of spinal cerebrospinal fluid(CSF)leaks. Patients with spinal CSF leak show an increasingly broad spectrum of clinical presentations including intractable whiplash-associated disorder(WAD)and orthostatic dysregulation in childhood, in addition to classical spontaneous intracranial hypotension(SIH). A simple understanding of the condition defined by the presence of low CSF pressure is no longer sufficient or accurate. Fat-suppressed T2-weighted magnetic resonance images and continuous epidural fluid infusion as a diagnostic therapy ensure accurate diagnosis. Accurate localization of the leak followed by targeted epidural blood patch(EBP)significantly improves the outcome of patients with spinal CSF leak. In addition to the known sites of CSF leakage, a newly identified CSF leak site, at the craniovertebral junction dural entry point of the vertebral artery(VA), is described.Intraventricular tumors often cause hydrocephalus because their location in the ventricles affect cerebrospinal fluid circulation. Even small tumors can lead to acute hydrocephalus when they block the cerebrospinal fluid flow. They may also be found as large tumors occurring in large spaces, such as in lateral ventricles. Since various histological tumors occur in ventricles, it is important to consider the treatment strategy according to the expected histological type before treating hydrocephalus in the early stage. In addition, it is beneficial to predict and evaluate the site and size of the tumor, the cause of hydrocephalus, and the effect of postoperative chemotherapy and radiation therapy. Some tumors are sensitive to chemotherapy and radiation therapy, so there is an advantage in performing a biopsy at the same time as hydrocephalus treatment. Ventricular drainage and ventricular peritoneal shunts for patients with high intracranial pressure are at risk of developing ascending hernias, so we should be careful with the procedure.Intraventricular hemorrhage(IVH)in patients with intracerebral hemorrhage is an independent risk factor. IVH can cause acute hydrocephalus by impairing cerebrospinal fluid dynamics. However, the pathological mechanism remains clear. In addition to the conventional concept of ventricular system obstruction by hematoma clots, secondary effects of heme and iron originating from hemoglobin might contribute to ventricular enlargement. The toxicity of accumulated hematoma in the ventricles might also influence the hydrocephalus and cause poor outcomes. An external ventricular drainage should be inserted promptly to control the intracranial pressure. Moreover, the hematoma should be removed as soon as possible to minimize the toxicity of the hematoma. The use of thrombolytic agents significantly reduces both the duration of ventricular drainage and mortality compared to external ventricular drainage alone. However, the functional outcome is not improved in patients with IVH. As another surgical option, endoscopy may be useful to evacuate the hematoma immediately. Finally, more evidence is essential for establishing the effectiveness of endoscopic techniques.Hydrocephalus is one of the major complications secondly occurring with or after subarachnoid hemorrhage(SAH). Hydrocephalus can be classified into acute(≦ 48 h), subacute(3-14 days), or chronic phases(≧ 2 weeks)in which a cerebrospinal fluid(CSF)shunt is required as the curative treatment at the rate of approximately 30%. On the mechanism of hydrocephalus development, it is generally accepted that an inflammatory reaction and the ensuing fibrosis process impede continuous CSF flow outward to the sinus, terminally from arachnoid granulation, leptomeninges, or ventricle walls, in addition to the mechanical blockage due to SAH clots. Several factors associated with the severity of SAH, such as a high Hunt and Hess grade, intraventricular hemorrhage, and a ruptured aneurysm in the posterior circulation also predict the development of shunt-dependent hydrocephalus. The predictive scoring system is available. Effective treatment of hydrocephalus still involves the use of CSF shunts. No superiority between ventriculoperitoneal and lumboperitoneal shunts has been established. Even during oral, single antiplatelet treatment after coil embolization for ruptured aneurysms, curative shunt operation is possible with low frequency of hemorrhagic complications. Hydrocephalus should be diagnosed early and treated appropriately to improve the functional outcome of the patients after SAH.The purpose of treatment hydrocephalus is to maintain intracranial pressure, as well as to create a favorable psychomotor developmental environment, particularly in pediatric cases. Various complications associated with shunt surgery require long-term care. When a shunt is performed for neonates and infants with hydrocephalus, proper management is required thereafter during whole their life. The most common complication that can occur as a result of shunt surgery is obstruction of the ventricular catheter. Ventricular catheter obstruction can be avoided by placing that the tip of the catheter is placed in an appropriate position. A further measure would include preventing the ventricular catheter from being pulled out as the skull expands. The next common complication is obstruction of the peritoneal catheter. The risk of occlusion can be reduced by making that the tip of the peritoneal catheter is open-ended as opposed to having side slits. Isolated ventricles and slit ventricle syndrome are peculiar shunt-rs could result in death. Shunt surgery for the treatment of pediatric hydrocephalus is merely the beginning, as neurosurgeons must be responsible for management until the child reaches adulthood.Certain congenital or developmental anomalies of adult-onset hydrocephalus often share clinical symptoms with idiopathic normal pressure hydrocephalus(iNPH). These anomalies include long-standing overt ventriculomegaly in adults(LOVA), persistent Break's pouch cyst(PBC)and panventriculomegaly(PaVM). Certain patients with adult-onset hydrocephalus might have congenital or secondary etiologies, such as late-onset idiopathic aqueductal stenosis(LIAS), and syndrome of hydrocephalus in young and middle-aged adults(SHYMA). Some of these conditions have unknown etiologies, and the definitions of LOVA differ between Japan and overseas. Adult-onset hydrocephalus usually presents with chronic onset, with younger patients tending to have headaches and older patients tending to have iNPH symptoms. In cases where the third ventricle floor bulging, endoscopic third ventriculostomy(ETV)is often performed. However, the arachnoid membrane of the pontine cistern might be strong, and the brain stem might be shifted to the clivus due to the enlarged fourth ventricle. For treating such patients, experienced hands might be needed. check details At present, it is necessary to carefully examine the cerebrospinal fluid dynamics in each patient and select the best test and treatment.Since hydrocephalus is usually an exacerbating disease, we need to arrest the hydrocephalus by surgical treatments such as ventricular access devices, V-P shunts, and endoscopic third ventriculostomy(ETV)with or without choroid plexus cauterization. For a long time, V-P shunt has been the GOLD standard treatment for pediatric hydrocephalus. In recent years, although there are more and more reports on the usefulness of ETV ± CPC, its results are not completely superior to V-P shunt, and therefore, V-P shunt is expected to remain the gold standard treatment for pediatric hydrocephalus in the near future. Therefore, overcoming complications, such as shunt dysfunction and shunt infection, will continue to be important in V-P shunt. A recent clinical trial has shown that antibiotic-impregnated catheters are effective in preventing shunt infections, which is why the incidence of shunt infection is expected to decrease in the future. For pediatric hydrocephalus, it is important to establish and maintain a regular follow-up system, because shunt malfunction may occur even in the chronic postoperative period.Endoscopic third ventriculostomy(ETV)is a basic procedure for the surgical treatment of hydrocephalus. It buffers pulsatile pressure by creating an alternative route for the flow of cerebrospinal fluid and reduces trans-mantle pulsatile stress, thereby increasing compliance of the brain parenchyma. Blunt perforation of the third ventricular floor is done while avoiding injury to the foramen of Monro, the hypothalamus, the pituitary stalk, and some cisternal vessels. A major complication of ETV is arterial bleeding caused by injury to the basilar artery. Surgeons should wait with irrigation and opening the root into the ventricle to control the intra-ventricular pressure until packing the third ventricle with hematoma. Since ETV may close by gliosis or scarring of the inter-peduncular cistern, regular physical examinations and MRI should follow the procedure.The type of shunt surgery with the best outcome and lowest complication rate has not been established. Ventriculo-atrial(V-A)shunts have almost been abandoned as the first choice of surgery. However, the V-A shunt has many advantages; the operative site is narrower than in other types of shunt surgery, and intra-atrial pressure is lower than intra-abdominal pressure, which may guarantee constant cerebrospinal fluid flow and less malfunction. Since this procedure has been neglected, devices of V-A shunt are scarce. Furthermore, the technique to insert an atrial catheter into the jugular vein is not commonly performed by general neurosurgeons. Herein, we introduce a simple method of V-A shunt for general neurosurgeons.The SINPHONI-2 study(a group of Japanese prospective multicenter cohort studies on the treatment of idiopathic normal pressure hydrocephalus[iNPH])showed the safety and efficacy of lumboperitoneal(L-P)shunt surgery for iNPH. A total of 660 probable iNPH patients underwent L-P shunt surgery at our NPH center between April 2009 and March 2020(age 77.3 ± 6.2 years). Our surgical technique includes 1)general anesthesia, 2)use of the original drape, 3)upward insertion of the spinal tube through L2/3 via a paramedian puncture in patients with highly deformed lumbar spines, 4)posterior placement of a Codman Hakim programmable valve with SiphonguardTM, 5)inclination of the table at a 35° angle without position change and re-sterilization, 6)laparotomy via rectal muscle splitting, and 7)oblique maneuvering the peritoneal tube from the upper lateral to the lower medial sector. During the first year after surgery, postoperative complications occurred in 14 of 172 patients (8.1%), including four patients with chronic subdural hematoma requiring evacuation (2.3%), three with spinal tube occlusion (1.7%), three with migration of the spinal tube, two with lower-limb numbness (1.2%), and two with abdominal tube occlusion. Our L-P shunt procedure seems to be generally acceptable considering the low number of complications.Revision surgeries after cerebrospinal shunt placement are sometimes unavoidable mainly because of the mechanical limitations of current shunt devices despite their technological improvements. This article discusses evidence on existing shunt devices, basic knowledge on their structure and function, and possible future issues.
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