CSF dynamics in long-standing overt ventriculomegaly in adults

Abstract

OBJECTIVE Long-standing overt ventriculomegaly in adults (LOVA) is a form of chronic hydrocephalus and its pathophysiology and treatment remain debated. An analysis of CSF dynamics in this condition has rarely been reported. The aim of this study was to analyze hydrodynamic characteristics of patients with suspected LOVA to discuss its pathophysiological mechanisms and the importance of CSF dynamics analysis for diagnosis and treatment of these patients. METHODS This retrospective cohort study, conducted between May 2018 and October 2022, included adult patients aged > 18 years investigated in a department of neurosurgery through a lumbar infusion study for suspicion of LOVA (n = 23). These patients were then compared with a control cohort explored for suspicion of idiopathic normal pressure hydrocephalus (iNPH; n = 30). Clinical symptoms, radiological findings, and hydrodynamic parameters were analyzed. The authors specifically compared two hydrodynamic parameters: resistance to CSF outflow, or Rout, which relies on CSF resorption, and pressure-volume index (PVI), which reflect overall craniospinal compliance. The lumbar infusion study was considered pathological (confirming the diagnosis of chronic hydrocephalus) when at least one of these two parameters was altered. RESULTS Rout was significantly less frequently increased (cutoff ≥ 12 mm Hg/ml/min) in patients with LOVA (52%) than in those with iNPH (97%; p < 0.001). In contrast, PVI was impaired (cutoff ≤ 25 ml) in both cohorts, i.e., in 61% of patients with LOVA and in 83% of patients with iNPH. Overall, the rate of pathological lumbar infusion study in LOVA (87%) was not statistically different than in iNPH (100%). However, PVI was the only impaired parameter most frequently found in those with LOVA (35%) compared with those with iNPH (3%; p = 0.002). CONCLUSIONS This study suggests that there is a differential CSF dynamics pattern when comparing patients with LOVA versus those with iNPH. A higher proportion of patients with LOVA showed isolated compliance impairment. These findings highlight the utility of CSF dynamics analysis for the evaluation of patients with suspected chronic obstructive hydrocephalus such as LOVA. Future research with larger case series may help define diagnosis and treatment algorithms of chronic obstructive hydrocephalus based on CSF dynamics analysis, in addition to clinical and radiological criteria.

Structural neuroimaging markers of normal pressure hydrocephalus versus Alzheimer’s dementia and Parkinson’s disease, and hydrocephalus versus atrophy in chronic TBI—a narrative review

“…In cases where LOVA occurs with an open aqueduct, where it may be differentiated from Late Onset Idiopathic Aqueductal Stenosis (LIAS), it becomes more crucial to distinguish it from NPH due to their clinical and symptomatic overlap ( 157 ). ICP monitoring in LOVA patients was shown to correlate with patient conditions pre- and post-surgery ( 158 ), and CSF dynamics was recommended to differentiate them from NPH patients ( 159 ). A noninvasive and accurate diagnostic score consisting of clinical features like age, presence/absence of the Hakim triad, headache, nausea/vomit, and neuroradiological features (evaluated on MRI) like the head circumference, EI-x, 3 V width, DESH, sellar bone distortion with the bulging of the 3 V floor was proposed recently by Palandri et al ( 157 ) which was shown to classify probable NPH patients from LOVA and LIAS patients with a high AUC of 0.97, sensitivity of 95.1%, and specificity of 90.6%.…”

Long-Standing Overt Ventriculomegaly in Adults (LOVA) With Absent Septum Pellucidum and Spontaneous Ventriculostomy: Report of a Rare Case

“…First described by Oi et al in 2000, LOVA is a unique form of chronic hydrocephalus with presumable infant onset hydrocephalus that manifests symptoms during adulthood, characterized by macrocephaly measuring more than two standard deviations in head circumference with severe ventriculomegaly and/or evidence of destruction or expansion of sella turcica [ 8 ]. It has been proposed that macrocephaly and supratentorial ventricular dilation develop due to the partial or complete obstruction of the cerebral aqueduct prior to the fusion of the cranial fontanelles, and various compensatory mechanisms such as alternative circulation pathways restore the CSF flow so that only in adulthood the symptoms of hydrocephalus develop when the compensatory mechanisms exhaust [ 2 ]. Typical symptoms include gait and visual disturbances, urinary incontinence, and cognitive decline [ 2 , 8 ].…”

“…It has been proposed that macrocephaly and supratentorial ventricular dilation develop due to the partial or complete obstruction of the cerebral aqueduct prior to the fusion of the cranial fontanelles, and various compensatory mechanisms such as alternative circulation pathways restore the CSF flow so that only in adulthood the symptoms of hydrocephalus develop when the compensatory mechanisms exhaust [ 2 ]. Typical symptoms include gait and visual disturbances, urinary incontinence, and cognitive decline [ 2 , 8 ]. Similar to previous studies, we observed symptoms of headache, visual disturbance, gait ataxia, and mild cognitive deficit in our patient.…”

“…Macrocephaly is defined as a head circumference > 98th percentile in adulthood (53.8 cm for males and 52.9 cm for females) [ 2 ]. Upon general inspection, macrocephaly was apparent with a head circumference of 61.8 cm.…”

“…Long-standing overt ventriculomegaly in adults (LOVA) is a type of chronic hydrocephalus characterized by macrocephaly with massive enlargement of the third and lateral ventricles that is presumably of infant onset with paradoxical mild clinical presentations in adulthood [ 1 ]. Due to the poorly understood pathophysiology and several overlapping clinical and radiological diagnostic criteria with other chronic cerebrospinal fluid (CSF) disorders, LOVA frequently remains undiagnosed and maltreated even in the centers of excellence [ 2 ].…”