LOVA: the role of endoscopic third ventriculostomy and a new proposal for diagnostic criteria

Abstract

The diagnostic criteria and management of this condition continue to be a topic of debate in the literature. The term definitive symptomatic LOVA (longstanding overt ventriculomegaly in adults) is applied to patients that fulfil a series of diagnostic criteria,which are reviewed in our paper. The main subject of our study is to propose a rearrangement of the criteria for the diagnosis of this entity and adjusted treatment strategies. We present our data evaluating the role of endoscopy in this pathology. This descriptive and analytical study comprised 27 adult patients with LOVA who underwent endoscopic third ventriculostomy (ETV). Data regarding age, gender and onset of symptoms, a previously implanted shunt, clinical examination and radiological findings at the time of diagnosis, and the intracranial pressure (ICP) recordings in some cases, were carefully retrospectively reviewed. ETV success was determined based on clinical state and radiological criteria. ETV was performed in all 27 cases. Mean follow up period was 4.2 years (6 months to 8 years). 2/27 patients (7.40%) were lost to follow-up. The overall success rate for ETV was 76% (21/25 patients). ETV resulted in shunt independency in two of the three patients with a previous shunt. Based on our results, we believe that ETV must be considered as the treatment of choice for patients with symptomatic LOVA.

Longstanding overt ventriculomegaly in adults (LOVA) with patent aqueduct: surgical outcome and etiopathogenesis of a possibly distinct form of chronic hydrocephalus

“…The role of an "intracisternal" obstruction in communicant hydrocephalus with a bulging third ventricle floor has already been hypothesized by Kehler [13] in 2003 and further investigated, with promising results, by Kageyama [12] in 2016 and Al-Hakim [1] in 2019. Venous stenosis, as already theorized by other authors [9,25], could have a possible role in distal CSF blockade, but this does not explain the good results achieved in our study by ETV in patients with a patent aqueduct.…”

“…More recently, Ibanez-Botella et al suggested the presence of “communicating” and “noncommunicating” LOVA and proposed an algorithm for the treatment, also taking into account Rekate’s considerations concerning venous sinuses. According to their findings, an MR angiogram should be performed as a part of the diagnostic algorithm of LOVA, to investigate a possible venous sinus stenosis as a cause of “open-aqueduct” hydrocephalus arising from a longstanding impairment of CSF reabsorption [ 9 ].…”

“…According to their findings, an MR angiogram should be performed as a part of the diagnostic algorithm of LOVA, to investigate a possible venous sinus stenosis as a cause of "open-aqueduct" hydrocephalus arising from a longstanding impairment of CSF reabsorption [9]. Accordingly, it is reasonable to consider that there are two different forms of LOVA, as stated by Ibanez-Botella et al [9]. The first one with complete aqueductal stenosis, which shares many clinical and radiological features with late-onset idiopathic aqueductal stenosis (LIAS) [6,16], therefore making the achievement of a correct differential diagnosis and classification subtle and difficult, and the second one with at least partially patent aqueduct.…”

“…It appears that, based on the patency of the aqueduct, two different clinical entities both currently classified as LOVA, can be distinguished. The clinical and radiological features of those two groups, as well as the best treatment modalities, remain poorly investigated [ 9 ].…”

Differentiating comorbidities and predicting prognosis in idiopathic normal pressure hydrocephalus using cerebrospinal fluid biomarkers

“…LOVA presents with an enlargement of the lateral and third ventricles due to congenital cerebral aqueduct obstruction and consequences of a long-term increase of intracranial pressure, including head circumference increase and sella turcica destruction, as well as cognitive impairment, abnormal gait, and urinary incontinence in adulthood ( 8 ). As LOVA is a non-communicating hydrocephalus due to cerebral aqueduct stenosis, the effective treatment is either with endoscopic third ventriculostomy (ETV) ( 14 ), or shunt surgery if the patient has a reduced ability to absorb CSF. On the other hand, PaVM is a communicating hydrocephalus resulting from congenital occlusion or impaired circulation in the foramen magnum area.…”