Rigid endoscopic surgery of brainstem cavernous malformation on the cerebral aqueduct. Case report

Cavernous angiomas (malformations) of the brain occur in 0.5% of the population. Most of them are asymptomatic, but due to their anatomical features, namely escape of blood into surrounding tissues, significant neurological symptoms can occur. The deep location of cavernous angiomas in the area of cerebral aqueduct makes surgical intervention difficult. Microsurgical approaches are the gold standard in removal of cavernous angiomas, but they are associated with certain surgical risks in the formation of the surgical corridor. Cavernous malformations in the cerebral aqueduct are a rare subtype. Due to anatomical localization and concomitant obstructive hydrocephalus ІІІ and lateral ventricles, they can be removed by endoscopic frontal transcortical transventricular approach. A 59-year-old patient was diagnosed with cavernous angioma of the brainstem (in the area of cerebral aqueduct) with hemorrhage and the formation of obstructive hydrocephalus ІІІ and lateral ventricles. The operation was performed: removal of the cavernous angioma in the area of cerebral aqueduct by endoscopic frontal transcortical transventricular approach on the right. Additionally, a triventriculocisternostomy was performed. Osteoplastic trepanation with centering at the Kocher’s point in size of 4 × 4 cm and the formation of a free bone flap was performed. The dura mater is cut in an H-shape. Approach to the anterior horn of the right lateral ventricle was performed. An intracerebral retractor was inserted into the anterior horn of the right lateral ventricle. Transforaminal approach to the tuber cinereum was performed - a triventriculocisternostomy was performed. Transforaminal approach to the cerebral aqueduct was performed and the cavernous angioma of the brainstem was removed. In the postoperative period, the patient had a slight deterioration in short-term memory, which regressed 2 weeks after surgery, an increase in oculomotor disorders, in particular persistent diplopia due to moderate paresis of the left oculomotor nerve. Three months after the operation, magnetic resonance imaging of the brain with intravenous contrast enhancement was performed. There are no signs of cavernous angioma. After the operation of frontal transcortical transventricular removal of cavernous angioma in the area of cerebral aqueduct, the compression of the latter was eliminated. Occlusive hydrocephalus regressed, the size of the ventricles decreased. Endoscopic frontal transcortical transventricular approach allows reaching the area of cerebral aqueduct in a less traumatic and minimally invasive manner. This technique is effective due to the low risk of surgical approach complications.

DIAGNOSIS AND FEATURES OF THE SURGICAL TREATMENT OF CAVERNOUS MALFORMATIONS OF THE BRAIN

The article presents an analysis on the modern aspects of diagnosis and surgical treatment of cavernous malformations of the brain. The modern approaches to surgical treatment depending on the type of clinical course of cavernous angiomas you can see in this paper.

Abstract P46: Baseline Characteristics of Patients With Cerebral Cavernous Angiomas With Symptomatic Hemorrhage in a Multisite Trial Readiness Study

Background: Patients with cerebral cavernous angiomas with symptomatic hemorrhage (CASH) have high risk of disability from recurrent bleeding. Candidate medications to prevent rebleeding in CASH lesions will require multisite clinical trials with standardized data collection. Objective: To report the prevalence and baseline cohort features in CASH patients and establish a research network infrastructure for trials. Methods: This prospective observational cohort study includes adults with radiologically verified CASH lesion within 1-year of consent. Exclusions include prior or planned surgical intervention, spinal location, or prior brain irradiation. Six sites enrolled patients into the screening and clinical assessment portion of the study starting July 2018. Patients also had the option to participate in the follow up biomarker validation at 4 sites. Baseline demographics, clinical and imaging information, and outcomes (mRS, PROMIS-29, NIHSS, and EuroQol-5D) were collected. Biomarker imaging included dynamic contrast enhanced quantitative perfusion (DCEQP) and quantitative susceptibility mapping (QSM) that correlated with symptomatic bleeding. Descriptive statistics were performed and one-sample t-test was used to compare whether mean T-scores for PROMIS-29 domains differed significantly from a reference population. Results: As of May 2020, 849 CASH patients were screened of whom 110 (13%) were eligible and enrolled; 73 also enrolled into the biomarker validation study. Average age at enrollment was 46±16 years at a mean of 4.4 months after symptom onset; 53% were female, 41% were familial, and 43% of CASH lesions were brainstem location. At enrollment, 90% of the cohort had independent functional outcome (mRS ≤ 2 and NIHSS <5). Perceived health problems affecting QoL were reported in >30% (EuroQol-5D). CASH cases had significantly worse anxiety but better depression and social satisfaction scores compared to a general population (all P<0.01). Baseline DCEQP and QSM measures did not differ significantly across sites. Conclusion: We demonstrate feasibility of multisite recruitment of CASH patients and report prevalence of baseline features that will aid in design of clinical trials and inclusion of appropriate outcome measures.

Burr hole microsurgery in treatment of patients with intracranial lesions: Experience of 44 clinical cases

Background: Modern technical capabilities have made minimally invasive surgery increasingly popular. Small incisions can reduce surgical duration and the degree of tissue trauma, which reduces the risk of complications. Burr hole microsurgery is a relatively new minimally invasive technique used in neurosurgery. The objective of this study was to assess the feasibility and outcomes of using burr hole microsurgery for the management of intracranial lesions. Methods: Forty-four adults were treated with burr hole microsurgery. Patients were divided into groups according to the presence of (1) brain tumors (n = 20); (2) congenital brain cysts (n = 16); (3) cavernous angiomas (n = 3); and (4) neurovascular conflicts of the 5th cranial nerve (n = 5). All surgical interventions were performed using the “MARI” device. Results: The transcortical approach was used to remove 16 brain tumors, and 2 brain tumors were biopsied. In the two tumor biopsy cases, the parasagittal interhemispheric route was used. Gross total resection was achieved in 10 cases (62.5%) when tumor size reached up to 4 cm, subtotal resection was achieved in four cases (25%) in large tumors, and partial resection in two cases (12.5%). In patients with congenital cysts, cavernous angiomas, trigeminal neuralgia, and symptomatic regression were noted the postoperative period. The surgical duration was 30–180 min (median, 75 min). A hemorrhagic complication was observed in one case. Significant postoperative complications and mortality were not observed. Conclusion: Burr hole microsurgery can treat different intracranial lesions effectively. Despite a smaller craniotomy diameter of 11–14 mm compared with keyhole approaches, surgery was successful.

Subclinical imaging changes in cerebral cavernous angiomas during prospective surveillance

OBJECTIVEThe purpose of this study was to systematically assess asymptomatic changes (ACs), including subclinical hemorrhage, growth, or new lesion formation (NLF) during longitudinal follow-up of cerebral cavernous angiomas (CAs), and to correlate these with symptomatic hemorrhage (SH) during the same period and with clinical features of the disease.METHODSOne hundred ninety-two patients were included in this study, among 327 consecutive patients with CA, prospectively identified between September 2009 and February 2019. Included patients had undergone clinical and MRI follow-up, in conjunction with institutional review board–approved biomarker studies, and harbored ≥ 1 CA with a maximum diameter of ≥ 5 mm on T2-weighted MRI. Rates of AC and SH per lesion-year and patient-year were assessed using prospectively articulated criteria. In multifocal/familial cases, rates of NLF were also assessed.RESULTSThere were no differences in demographic or disease features among cases included or excluded in the study cohort, except for a higher proportion of included patients with CCM3 mutation. Follow-up was 411 patient-years (2503 lesion-years). The rate of AC was higher than the rate of SH (12.9% vs 7.5% per patient-year, and 2.1% vs 1.2% per lesion-year, both p = 0.02). Patients presenting with a prior history of SH had a higher rate of AC than those with other forms of presentation (19.7% and 8.2% per patient-year, respectively; p = 0.003). A higher rate of NLF on T2-weighted MRI (p = 0.03) was observed in patients with prior SH. Three of 6 solitary/sporadic and 2 of 28 multifocal/familial patients underwent resection of the lesion after AC.CONCLUSIONSRates of AC are greater than SH during prospective follow-up of CAs, and greater in cases with prior SH. AC may be a more sensitive biomarker of lesional activity, and a more efficient surrogate outcome in clinical trials than SH. Patients experiencing an AC are more likely to undergo a surgical intervention when CAs are solitary/sporadic than when they are multifocal/familial.

Neurovascular Lesions in Pediatric Epilepsy

Introduction: Neurovascular lesions are rare and understudied in the pediatric population. Their initial presentation can range from seizures to focal neurologic deficits, as well as headaches. The goal of this study was to examine the clinical presentation and natural history of neurovascular lesions in children with epilepsy. Methods: We reviewed all pediatric epilepsy patients with neurovascular lesions diagnosed between 2006 and 2018 at the University of Alberta and the Stollery Children’s Hospital, Edmonton, Canada. Initial clinical presentation and brain imaging, as well as long-term epilepsy and postsurgical outcome, were assessed. Results: Of the 14 patients, 10 patients had an initial presentation of focal seizures with impaired awareness, whereas 2 patients presented with headache, 1 presented with visual field defects as well as chronic headaches, and 1 with decreased level of consciousness. Seven patients had cavernous angiomas, 6 had arteriovenous malformation, and 1 patient had an arteriovenous fistula. Notably, all patients with cavernous angiomas and 4 of 6 patients with arteriovenous malformations presented with seizures. Among 9 of the 14 who underwent neurovascular corrective surgery, all 9 patients required long-term antiepileptic treatment of at least 1 antiepileptic drug for seizure control after the operation. Conclusion: In this novel case series, we describe focal seizures as the initial presentation of pediatric neurovascular lesions. This clinical presentation appears to be independent of the type of neurovascular lesion. Furthermore, unlike our pediatric surgical patients with epilepsy due to other causes, seizure freedom following neurovascular surgery is limited, and patients require long-term antiepileptic treatment.