EFORE recent diagnostic procedures were introduced for the diagnosis of hydrocephalus, one of the most accepted indicators for progressive hydrocephalus in infants was head circumference. However, formulating a diagnosis of arrested hydrocephalus on the basis of this indicator alone would be questionable. Whittle and colleagues 41 used continuous ICP monitoring to as-sess 46 children and adolescents in whom a clinical diagnosis of arrested hydrocephalus had been made. They found that 80% of patients who had previously been treated had a nonfunctioning shunt and that 63% of patients who had received no previous treatment had episodic or persistent increased ICP. When serial psychometric testing demonstrated a fall in cognitive functioning, ICP monitoring revealed abnormal ICPs in 88% of patients. The results of this study may indicate that CSF dynamics change over time, shifting hydrocephalus between active and inactive states; however, the factors affecting such changes remain uncertain.In 1996, we reported on the specific clinical features of infantile hydrocephalus that continues into adulthood, Object. Long-standing overt ventriculomegaly in adults (LOVA) is a unique form of hydrocephalus that develops during childhood and manifests symptoms during adulthood. The aim of the present study was to analyze the specific pathophysiological characteristics of LOVA.Methods. The specific diagnostic criteria for LOVA include severe ventriculomegaly in adults that is associated with macrocephalus measuring more than two standard deviations in head circumference and/or neuroradiological evidence of a significantly expanded or destroyed sella turcica. Twenty patients who fulfilled these criteria, 14 males and six females, were retrospectively studied. These patients' ages at diagnosis ranged from 15 to 61 years (mean 39.4 years). All had symptoms and/or signs indicating that hydrocephalus first occurred at birth or during infancy in the absence of any known underlying disease. The authors performed a pathophysiological study that included specific variations of magnetic resonance (MR) imaging, such as fluid-attenuated inversion recovery and cardiac-gated cine-mode imaging; intracranial pressure (ICP) monitoring; three-dimensional computerized tomography (CT) scanning; and other techniques.Hydrocephalus was caused by aqueductal stenosis in all patients. Severe ventriculomegaly involving the lateral and third ventricles was associated with a marked expansion or destruction of the sella turcica in 17 cases. Cardiac-gated cine-MR imaging did not reveal any significant movements of cerebrospinal fluid in the aqueduct. Three-dimensional CT ventriculography confirmed that the expanded third ventricle protruded into the sella and, sometimes, extended a diverticulum. Fourteen patients revealed symptoms and signs that indicated increased ICP with prominent pressure waves. Dementia or mental retardation was seen in 11 patients, gait disturbance in 12, and urinary incontinence in eight; all three of these symptoms were observed in sev...

Our minimally invasive preferential regimen clarified the precise indication for neuroendoscopic procedures, and the majority of our patients with dilated ventricles and no evidence of tumor markers were treated satisfactorily with effective neuroendoscopic procedures as the initial procedure, avoiding unnecessary craniotomy and radiotherapy and promising excellent therapeutic outcomes. The treatment for malignant pineal region tumors remains a subject for further study.

Neuroendoscopic surgery was used to treat patients with various forms of hydrocephalus with specific pathophysiology, including long-standing overt ventriculomegaly in adulthood (LOVA), isolated unilateral hydrocephalus (IUH), isolated IV ventricle (IFV), disproportionately large IV ventricle (DLFV), isolated rhombencephalic ventricle (IRV), isolated quarto-ventriculomegaly (IQV), dorsal sac in holoprosencephaly (DS), and loculated ventricle (LV). A total of 26 operative procedures were performed, with neuroendoscopic surgery in 22 patients, 12 with unique forms of noncommunicating hydrocephalus and 10 with various types of postshunt isolated compartment. These procedures included III ventriculostomy, aqueductal plasty by both rostral and caudal approaches, foraminal plasty in the foramen of Monro/foramen of Magendie, septostomy, IV ventriculostomy, fenestration of septation in the loculated ventricle, fenestration of arachnoid cyst or cystic tumor obstructing a ventricle with or without tumor removal, and dorsal sac ventriculostomy. The characteristics of the cerebrospinal fluid (CSF) dynamics in the individual specific pathophysiologies were delineated by cardiac-gated cine-mode magnetic resonance imaging (MRI) before and after the endoscopic procedure. The consequent success rate (success = restoration of communication of the CSF pathway in the individual patients) was 19/22 (86.4%). The progression of ventricular dilatation was stopped in 17 of 19 patients (89.5%) in whom the endoscopic procedure was successful (radiologically arrested hydrocephalus). Improvement in the clinical symptoms and signs (clinically arrested hydrocephalus) was obtained in 15 of the patients (68.2% of all patients: 5 with LOVA, 3 with IQV, 5 with IUH and 2 with LV). Seven patients (2 LOVA, 2 IFV, 1 DS, 1 DFLV and 1 IRV) underwent a shunt procedure after the neuroendoscopic procedure(s). The postoperative changes of ventriculomegaly were complicated, reflecting the differences in the brain parenchymal compliance and postoperatively corrected CSF flow dynamics in the major CSF pathway.

Although arachnoid cysts (ACs) are associated with chronic subdural hematomas (CSDHs), especially in young patients, the detailed features of CSDHs associated with ACs remain poorly understood. The objective of this study was to clarify the relationship between the location of CSDHs and ACs and the significance of ACs in young patients with CSDHs. We retrospectively assessed 605 consecutive patients 7 years of age and older who were diagnosed with a CSDH between 2002 and 2014. Twelve patients (2%) had ACs, and 10 of the 12 patients were 7–40 years of age. Patients with ACs as a complication of CSDHs were significantly younger than those without ACs (p < 0.05). Three different relationships between the location of CSDHs and ACs were found: a CSDH contacting an AC, an ipsilateral CSDH apart from an AC, and a CSDH contralateral to an AC. In 21 patients with CSDHs who were 7–40 years of age, 10 (47.6%) had ACs (AC group) and 7 (33.3%) had no associated illnesses (non-AC group). All 10 young patients with ACs showed ipsilateral CSDHs including a CSDH apart from an AC. All 17 patients in both the AC and non-AC groups showed headache but no paresis at admission. The pathogenesis of CSDHs associated with ACs may be different among the three types of locations. The clinical characteristics of patients with a combination of a CSDH and an AC including headache as a major symptom may be attributed to young age in the majority of patients with ACs.

It is emphasized that postnatal prognosis is not simply a function of the form of the diagnosis but is also dependent on the progression of hydrocephalus and the degree to which that process affects neuronal development. Early decompressive procedures, conventionally performed after but, hopefully, performed before birth, are indicated to obtain the optimal postnatal prognosis of fetuses with hydrocephalus diagnosed at PCCH Stage II.