Giant cavernous malformation of the genu of the corpus callosum

Cavernous Malformations: What They Have Taught Us.

Cavernous Malformations: A Paradigm for Progress

Cavernous malformations are benign vascular lesions that can arise throughout the central nervous system. The occurrence of a cavernous malformation within the optic nerve or chiasm, however, is extremely uncommon. The case described in this video involved a 36-yr-old woman who presented 3 mo after undergoing a left frontotemporal craniotomy for resection of an optic nerve cavernous malformation. She had initially presented to an outside hospital with vision loss, and the left optic nerve lesion was identified and resected. Although her vision had reportedly improved slightly postoperatively, she awoke 3 mo later with bilateral subjective blurriness and new visual field deficits. Magnetic resonance imaging revealed enlargement of the left optic apparatus hemorrhagic lesion, corresponding to residual cavernous malformation. Given the recurrence of hemorrhage and the associated visual symptoms, the patient underwent a redo left frontotemporal craniotomy for resection of the optic nerve and chiasmal lesion. Histopathologic evaluation revealed thick-walled vessels with focal intervening glial tissue, an absence of neoplastic cells, and hemorrhage, consistent with a cavernous malformation. The patient tolerated the procedure well. Postoperatively, she experienced immediate amelioration in her visual symptoms. She was discharged home on postoperative day 3, and her bitemporal visual field deficit continued to progressively improve through her last ophthalmologic appointment 14mo after surgery. Postoperative and subsequent surveillance neuroimaging demonstrated complete resection of the cavernous malformation without evidence of recurrence. This case demonstrates the techniques utilized to ensure complete resection of the malformation in this very eloquent region. The patient provided consent for publication.

A Guide to Percutaneous Cryoablation of Renal Masses in Difficult Anatomic Locations

No. 411 - Surviving the interventional radiology ice-bucket challenge: percutaneous cryoablation of renal masses in difficult anatomic locations

chiasmal apoplexyは1982年Maitlandによって提唱された.視交叉内に生じた出血により視野欠損を伴う頭痛で発症し,原因病変は視交叉内の血管奇形,腫瘍が多い.視神経,視交叉以外の病変の出血によるchiasmal apoplexyは,われわれが調べ得た限りでは報告はない.症例は44歳,男性.突然の頭痛,右眼視力低下,視野障害が出現し,頭部MRIで右前頭葉底部を含め多発性病変を認めた.主訴は右前頭葉底部病変による神経症状と考え,右前頭側頭開頭にて腫瘍摘出術を施行した.術中所見では右前頭葉底部に腫瘍病変を認め,右視神経近位部を分離するように血腫が存在した.腫瘍摘出とともに血腫も除去した.病理所見はcavemous angiomaに一致していた.術後には新たな神経症状は出現せず,視力・視野の明らかな改善を認めた.

The optimal management of cerebral cavernous malformations (CCMs) with epileptic seizures is still a matter of debate. The aim of our study was to examine seizure outcome in the largest published series of surgically treated patients with epilepsy due to a supratentorial CCM, and to define predictors for good surgical outcome. We retrospectively studied 168 consecutive patients with a single supratentorial CCM and symptomatic epilepsy in a multicenter study. Pre- and postoperative clinical examinations, age at epilepsy onset, age at operation, type of symptoms due to the CCM (seizures, headache, hemorrhage, focal deficits), type and frequency of epileptic seizures, and the localization and size of the CCM were assessed. Seizure outcome was determined in the first, second, and third postoperative years. The CCM was completely resected in all patients. More than two thirds of the patients were classified as seizure free in the first 3 postoperative years. Predictors for good seizure outcome were age older than 30 years at the time of surgery, mesiotemporal CCM localization, CCM size <1.5 cm, and the absence of secondarily generalized seizures. No mortality occurred in our series, but only mild postoperative neurologic deficits in 12 (7%) patients. Considering the natural history of CCMs, the favorable neurologic and seizure outcome, surgical resection of CCMs should be considered in all patients with supratentorial CCMs and concomitant epilepsy, irrespective of the presence or absence of predictors for a favorable seizure outcome.

Objective To preliminarily discuss the operation strategy in patients with intracranial cavernous hemangioma complicated with venous malformation. Methods The clinical data and postoperative follow-up results of 13 patients with intracranial cavernous hemangioma complicated with venous malformation operated at the Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University from January 2007 to December 2014 were analyzed retrospectively. Results In the 13 patients, intracranial cavernous malformation complicated with venous malformation achieved total resection in 7 patients. They all developed severe cerebral edema after procedure. Four of them were in the supratentorial region, their prognoses were good after strengthening dehydration treatment, and 1 in the supratentorial region died, and 2 left sequelae. The postoperative condition was stable and the prognosis was good in 4 patients whose abnormal expansion of medullary veins were resected, the main drainage veins were retained and in 2 patients whose venous vascular malformations were retained. Conclusion In the operation of resection of intracranial cavernous malformation complicated with venous malformation, the resection of abnormally dilated medullary veins and retaining main draining veins at the same time of the total resection of cavernous malformation are more conducive to the rehabilitation of patients. Key words: Hemangioma, cavernous, central nervous system; Venous malformation; Microsurgery

Endoport-assisted microsurgical resection of cerebral cavernous malformations

The retrosigmoid approach is one of the main approaches used in the surgical management of pontine cavernous malformations. It definitely provides a lateral route to large central lesions but also makes possible resection of some ventral lesions as an alternative to the petrosal approaches. However, when these vascular malformations do not emerge on surface, one of the safe corridors delimited by the origin of the trigeminal nerve and the seventh-eight cranial nerve complex can be used.1-5 Baghai et al2 described the lateral pontine safe entry zone in 1982, as an alternative to approaches through the floor of the fourth ventricle when performing tumor biopsies. They advocated a small neurotomy performed right between the emergence of the trigeminal nerve and the facial-vestibulocochlear cranial nerves complex. Accurate image guidance, intraoperative cranial nerve monitoring, and comprehensive anatomical knowledge are critical for this approach.4,5 Knowing the natural history of a brainstem cavernous malformation after bleeding,6 we sought to demonstrate in this video: (1) the use of the retrosigmoid craniotomy in lateral decubitus for resection of deep-seated pontine cavernous malformations; (2) the wide opening of arachnoid membranes and dissection of the superior petrosal vein complex to improve surgical freedom and prevent use of fixed cerebellar retraction; and (3) the opening of the petrosal fissure and exposure of the lateral pontine zone for gross total resection of a cavernous malformation in a 19-yr-old female with a classical crossed brainstem syndrome. She had full neurological recovery after 3 mo of follow-up. The patient consented in full to the surgical procedure and publication of the video and manuscript.

The precise neural mechanisms of propofol anesthesia in humans are still unknown. The authors examined the acute effects of propofol on regional cerebral blood flow (rCBF) using positron emission tomography in patients with severe depression. In six severely depressed patients (mean age, 55.0 yr) scheduled for electroconvulsive therapy, anesthetic levels were monitored by electroencephalography, and rCBF was serially quantified in the awake, sedated, and anesthetized states. The authors used high-resolution positron emission tomography with 15O-labeled water and statistical parametric mapping 99 for imaging and analysis of the data. Global cerebral blood flow showed sharp decreases from the awake level during the administration of propofol, decreasing 26.8% in the sedated state and 54.4% in the anesthetized state. Moreover, a dose effect was seen in both parietal cortices and the left lateral prefrontal region with larger regions of relative decrease in rCBF at higher propofol doses. At the higher dose, the values of rCBF in the pulvinar nucleus of the thalamus, the pontine tegmentum, and the cerebellar cortex were also affected. Meanwhile, there were few changes of relative rCBF in the basal frontal lobes during both sedated and anesthetized states. As in earlier studies using normal subjects, pronounced suppression in rCBF in the brain stem reticular formation, the thalamus, and the parietal association cortex occurred even in severely depressed patients. However, previously reported decreases in rCBF in the basal frontal lobe were absent in depressed patients.

Surgical resection of cavernous malformations (CM) in the posterior thalamus, pineal region, and midbrain tectum is technically challenging owing to the presence of adjacent eloquent cortex and critical neurovascular structures. Various supracerebellar infratentorial (SCIT) approaches have been used in the surgical armamentarium targeting lesions in this region, including the median, paramedian, and extreme lateral variants. Surgical view of a posterior thalamic CM from the traditional ipsilateral vantage point may be obscured by occipital lobe and tentorium. To describe a novel surgical approach via a contralateral SCIT (cSCIT) trajectory for resecting posterior thalamic CMs. From 1997 to 2017, 75 patients underwent the SCIT approach for cerebrovascular/oncologic pathology by the senior author. Of these, 30 patients underwent the SCIT approach for CM resection, and 3 patients underwent the cSCIT approach. Historical patient data, radiographic features, surgical technique, and postoperative neurological outcomes were evaluated in each patient. All 3 patients presented with symptomatic CMs within the right posterior thalamus with radiographic evidence of hemorrhage. All surgeries were performed in the sitting position. There were no intraoperative complications. Neuroimaging demonstrated complete CM resection in all cases. There were no new or worsening neurological deficits or evidence of rebleeding/recurrence noted postoperatively. This study establishes the surgical feasibility of a contralateral SCIT approach in resection of symptomatic thalamic CMs It demonstrates the application for this procedure in extending the surgical trajectory superiorly and laterally and maximizing safe resectability of these deep CMs with gravity-assisted brain retraction.

Autosomal dominant cerebral cavernous malformations (CCM) are leaky vascular lesions that can cause epileptic seizures and stroke-like symptoms. Germline mutations in either CCM1, CCM2 or CCM3 are found in the majority of patients with multiple CCMs or a positive family history. Recently, the first copy number neutral inversion in CCM2 has been identified by whole genome sequencing in an apparently mutation-negative CCM family. We here asked the question whether further structural genomic rearrangements can be detected within NGS gene panel data of unsolved CCM cases. Hybrid capture NGS data of eight index patients without a pathogenic single nucleotide, indel or copy number variant were analyzed using two bioinformatics pipelines. In a 58-year-old male with multiple CCMs in his brain and spinal cord, we identified a 294 kb insertion within the coding sequence of CCM2. Fine mapping of the breakpoints, molecular cytogenetic studies, and multiplex ligation-dependent probe amplification verified that the structural variation was an inverted unbalanced insertion that originated from 1p12-p11.2. As this rearrangement disrupts exon 6 of CCM2 on 7p13, it was classified as pathogenic. Our study demonstrates that efforts to detect structural variations in known disease genes increase the diagnostic sensitivity of genetic analyses for well-defined Mendelian disorders.

In this surgical video, we present a 57-year-old man with neck pain, dizziness, and imbalance. MRI showed a heterogeneously enhancing mass lesion within the posterior medulla at the level of the foramen magnum. Because the patient was symptomatic from this cavernous malformation, the decision was made to proceed with surgical resection. The patient underwent a midline suboccipital craniotomy with C1 laminectomy for surgical resection of the cavernous malformation in the medulla oblongata, with concurrent monitoring of motor and somatosensory evoked potentials.The surgery and postoperative course were uneventful. The postoperative MRI showed gross-total resection of the mass with histopathology indicating a cavernous malformation. The patient continues to do well without recurrence at 7 years of follow-up. In this video, we demonstrate important microsurgical steps for the resection of this challenging and rare vascular malformation.The video can be found here: https://youtu.be/gbGleLowzxo.

Cerebral cavernous malformations (CCMs) are a cluster of abnormal vessels of the brain. CCMs have a low risk for intracerebral hemorrhage and appear increasingly often as incidental findings on MRI. Zabramski classification has been used to describe the radiological features of CCMs. How the Zabramski classification associates with the clinical symptoms at presentation and clinical course of the disease is not well established. The authors reviewed the medical records of conservatively managed and surgically treated CCM patients over a 20-year period treated in Kuopio and Tampere University Hospitals. Both clinical and radiological data were analyzed. The modified Rankin Scale (mRS) was used to score the symptoms. Altogether, 775 CCM patients were identified. In both cohorts, most patients were asymptomatic (53% in the Kuopio University Hospital [KUH] cohort and 64% in the Tampere University Hospital [TAUH] cohort). Most CCMs were located supratentorially (77%, 661/858), and most patients presented with epileptic seizures. Of the infratentorial CCMs, most were located in the cerebellum (61%, 121/198). The most common symptoms caused by these CCMs were double vision, sensory deficits, and impaired balance. The median mRS score for symptoms in all CCMs was 0, and if only symptomatic CCMs were accounted for, it was 2. Most CCMs were Zabramski type II, and these CCMs were relatively asymptomatic, similar to Zabramski type III CCMs. Zabramski type I CCMs were mostly symptomatic, whereas Zabramski type IV CCMs were completely asymptomatic. Of patients with type I CCMs, approximately 15% developed new symptoms within 1 year, and in the long-term follow-up 26% had new symptoms. In radiological follow-up, 81% of the type I CCMs regressed to type II or III. Of type II or III CCMs, 2.3% changed over time to type I. Age was associated with the Zabramski classification (p < 0.001 in the TAUH cohort and p = 0.034 in the KUH cohort). Most CCMs are asymptomatic, incidental findings, in particular, nearly all Zabramski type IV CCMs. If symptoms are present, they are mainly associated with Zabramski type I CCMs and occasionally with Zabramski type II or III CCMs. Although most Zabramski type I CCMs will regress to type II or III and remain asymptomatic, over long-term follow-up, up to one-quarter develop new symptoms.