Goran Mrak

Treatment of giant and large fusiform middle cerebral artery aneurysms with excision and interposition radial artery graft in a 4-year-old child: case report.

BACKGROUND AND IMPORTANCE: We report an unusual case of complex giant and large fusiform aneurysms not amenable for clipping or coiling in a 4-year-old child managed with aneurysm resection and radial artery interposition graft. CLINICAL PRESENTATION: A 4-year-old child presented with repeated severe headache and vomiting. Computed tomography, magnetic resonance imaging, and magnetic resonance angiography and digital subtraction angiography showed a giant fusiform aneurysm on the right middle cerebral artery (MCA). Because of the complex shape, endovascular treatment or clip reconstruction was not possible, and a bypass procedure was planned. Right frontotemporal craniotomy and orbitotomy was performed. Two aneurysms involving the M1 segment of the MCA were found in line, 1 giant, and the other large in size. The aneurysms were resected and treated with short radial artery interposition graft, which was narrower than the proximal or distal MCA. The child recovered normally, and the bypass was patent after 1 year. CONCLUSION: Large fusiform MCA aneurysms may be difficult to treat, but there are treatment options that include a bypass procedure. Resection and short interposition radial artery graft is an excellent but rare treatment option in a very young child. This was a very successful treatment in this child. ABBREVIATIONS: ICA, internal carotid artery MCA, middle cerebral artery RA, radial artery RAG, radial artery graft STA, superficial temporal artery

Expression patterns of Wnt signaling component, secreted frizzled‑related protein 3 in astrocytoma and glioblastoma.

Secreted frizzled-related protein 3 (SFRP3) is a member of the family of soluble proteins, which modulate the Wnt signaling cascade. Novel research has identified aberrant expression of SFRPs in different types of cancer. In the present study the expression intensities and localizations of the SFRP3 protein across different histopathological grades of astrocytic brain tumors were investigated by immunohistochemistry, digital scanning and image analysis. The results demonstrated that the differences between expression levels and malignancy grades were statistically significant. Tumors were classified into four malignancy grades according to the World Health Organization guidelines. Moderate (P=0.014) and strong (P=0.028) nuclear expression levels were significantly different in pilocytic (grade I) and diffuse (grade II) astrocytomas demonstrating higher expression values, as compared with anaplastic astrocytoma (grade III) and glioblastoma (grade IV). When the sample was divided into two groups, the moderate and high cytoplasmic expression levels were observed to be significantly higher in glioblastomas than in the group comprising astrocytoma II and III. Furthermore, the results indicated that high grade tumors were associated with lower values of moderate (P=0.002) and strong (P=0.018) nuclear expression in comparison to low grade tumors. Analysis of cytoplasmic staining demonstrated that strong cytoplasmic expression was significantly higher in the astrocytoma III and IV group than in the astrocytoma I and II group (P=0.048). Furthermore, lower grade astrocytomas exhibited reduced membranous SFRP3 staining when compared with higher grade astrocytomas and this difference was statistically significant (P=0.036). The present results demonstrated that SFRP3 protein expression levels were decreased in the nucleus in higher grade astrocytoma (indicating the expected behavior of an antagonist of Wnt signaling), whereas when the SFRP3 was located in the cytoplasm an increased expression level of SFRP3 was identified in the high grade astrocytomas when compared with those of a low grade. This may suggest that SFRP3 acts as an agonist of Wnt signaling and promotes invasive behavior.

Vagus nerve stimulation in Lafora body disease.

Introduction Lafora body disease (LBD) is a rare autosomal recessive disorder characterized by progression to inexorable dementia and frequent occipital seizures, in addition to myoclonus and generalized tonic–clonic seizures (GTCSs). It belongs to the group of progressive myoclonus epilepsies (PMEs), rare inherited neurodegenerative diseases with great clinical and genetic differences, as well as poor prognosis. Since those patients have a pharmacoresistant disease, an adjunctive treatment option is vagus nerve stimulation (VNS). To date, there are four reported cases of the utility of VNS in PME — in Unverricht–Lundborg disease (ULD), myoclonic epilepsy with ragged-red fibers (MERRF), Gauchers disease, and in one case that remained unclassified. Case presentation A 19-year-old male patient had progressive myoclonus, GTCSs that often progressed to status epilepticus (SE), progressive cerebellar and extrapyramidal symptomatology, and dementia, and his disease was pharmacoresistant. We confirmed the diagnosis of LBD by genetic testing. After VNS implantation, in the one-year follow-up period, there was a complete reduction of GTCS and SE, significant regression of myoclonus, and moderate regression of cerebellar symptomatology. Conclusion To our knowledge, this is the first reported case of the utility of VNS in LBD. Vagus nerve stimulation therapy may be considered a treatment option for different clinical entities of PME. Further studies with a larger number of patients are needed.

Clinicopathologic Assay of 15 Tumor Resections in a Family with Neurofibromatosis Type 2

The objective of this study is the management of multiple family members with multiple neurofibromatosis type 2 (NF2) related tumors of the skull base that can be challenging, on purely technical, decision-making, and ethical levels. These issues are addressed in this manuscript based on an experience treating an unique large family with NF2. A retrospective chart review was performed, reviewing clinical, radiological, surgical, and pathological data. A unique family of 17 siblings, whose father was the proband as a sporadic mutation is reported. Over a 4-month period, five of eight affected siblings underwent 12 procedures for resection of 15 different NF2-related tumors. This single family experience of NF2-related skull base tumors underscores the importance of preservation of function and quality of life as the major determinants of treatment success.

Endovascular treatment of giant-dissecting posterior cerebral artery aneurysm in an infant

Intracranial aneurysms are extremely rare in the infant age group, with less than 200 cases described in the literature [1]. The pathogenesis most commonly includes connective tissue disorders, trauma, infection, or spontaneous arterial dissection [2, 3]. Clinical presentation is mainly due to ischemia, subarachnoid, or intracerebral hemorrhage, as well as mass effect of giant aneurysms, with their occurrence being more common in children than in adults. Asymptomatic, incidental aneurysms in children appear in up to 35% of the cases according to a report by Kakarla et al., while other authors report significantly lower percentages [4, 5]. We performed a literature review and discovered that there are no reports on incidentally found aneurysms in infants. Herein, we present a case of a 4-month-old male infant with an asymptomatic, posterior cerebral artery aneurysm. The patient was born in the 38th week of gestation, as a result of premature rupture of membranes. Due to immediate feeding difficulties and somnolence, later ascribed to an upper respiratory tract infection, a wide variety of diagnostic procedures was performed including cranial ultrasound. A choroid plexus cyst was found on ultrasound, requiring regular, monthly controls. At 2 months, a new anechogenic mass was found in the right temporal horn region. A computed tomography (CT) scan confirmed a hyperdense lesion, measuring 2 cm in its greatest diameter, with contrast opacification. Magnetic resonance angiogram (MRA) verified a vascular lesion (Figs. 1 and 2). Afterwards, digital subtraction angiography (DSA) was performed for definitive diagnosis before treatment. A 4F introducer sheath was placed for femoral access and 4F diagnostic catheter was used for selective catheterization. Large, dissecting, right posterior cerebral artery (PCA) aneurysmwas found, located between the P1 and P2 segments, measuring 15 mm in diameter. After verification of distal PCA filling via collateral vessels from the left PCA and left middle cerebral artery (MCA), the same catheter was then used as a Bguiding^ catheter, being placed in proximal cervical segment of the left vertebral artery. A 1.7F microcatheter was passed in coaxial fashion, and placed inside the aneurysmal neck and subsequently aneurysm and parent vessel occlusion were performed by placing four detachable coils. Control angiography showed obliteration of the parent vessel and aneurysm. The patient was discharged home on the fourth postoperative day. Follow-up magnetic resonance imaging (MRI) and MRA were performed 4 months after the procedure. Distal parts of the P3 segment have been revascularized via leptomeningeal collaterals. An ischemic lesion, 3 mm in diameter was found in the lower dorsal part of the thalamus, medially to the crus posterius of the internal capsule. At follow-up, there were no new infarcts on MRI, angiography showed parent vessel and aneurysm sac occlusion, and the patient was neurologically intact. This case of an incidentally found intracranial aneurysm in an infant provides a unique insight into the treatment of asymptomatic aneurysms in this age group. Although it is impossible to predict further dynamics of the clinical course had the aneurysm not been treated, the uneventful recovery of the patient demonstrates that endovascular coiling is an effective treatment method. Appropriate timing of the procedure, * Jakob Nemir jakob.nemir@kbc-zagreb.hr

Middle cerebral artery fusiform aneurysm presented with stroke and delayed subarachnoid hemorrhage trapping, thrombectomy, and bypass

Background: Ischemic stroke is a well-described but less frequent consequence of ruptured or unruptured intracranial aneurysms. To date, the optimal form of treatment for patients with a thrombosed cerebral aneurysm has not yet been well-defined. Case Description: Here, we report a case of a 68-year-old female patient presenting with cerebral stroke. Five days poststroke multislice computed tomography (MSCT) and MSCT angiography were performed for the evaluation of clinical deterioration, showing a left M2 middle cerebral artery (MCA) bifurcation aneurysm and subarachnoid hemorrhage. Having in mind the high mortality and morbidity rates after a re-rupture, as well as the digital subtraction angiography features of the aneurysm, urgent surgery was performed consisting of aneurysm trapping and superficial temporal artery (STA) to M3 MCA segment end-to-side anastomosis. The surgery and early postoperative period proceeded uneventfully and the patient gradually recovered from the previously diagnosed expressive dysphasia and cranial and extremity motor deficit. Conclusion: Our case describes a complex aneurysm treatment that consisted of aneurysm trapping, thrombus removal and an STA-M3 MCA branch bypass creation for the protection of the patent M3 insular MCA branch and prevention of further ischemia. This procedure rewarded us with an excellent clinical result.

Treatment of Giant and Large Fusiform MCA Aneurysms With Excision and Interposition Radial Artery Graft in a Four Year Old Child.

BACKGROUND AND IMPORTANCE: We report an unusual case of complex giant and large fusiform aneurysms not amenable for clipping or coiling in a 4-year-old child managed with aneurysm resection and RA interposition graft. CLINICAL PRESENTATION: A 4-year-old child presented with repeated severe headache and vomiting. Computed tomography, magnetic resonance imaging and magnetic resonance angiography and digital subtraction angiography showed a giant fusiform aneurysm on the right MCA. Because of the complex shape, endovascular treatment or clip reconstruction was not possible, and a bypass procedure was planned. Right frontotemporal craniotomy and orbitotomy was performed. Two aneurysms involving the M1 segment of the MCA were found in line, one giant, and the other large in size. The aneurysms were resected and treated with short radial artery interposition graft, which was narrower than the proximal or distal MCA. The child recovered normally, and the bypass was patent after 1 year. CONCLUSION: Large fusiform MCA aneurysms may be difficult to treat but there are treatment options that include a bypass procedure. Resection and short interposition radial artery graft is an excellent but rare treatment option in a very young child. This was very successful treatment in this child.

Tentorial alignment and its relationship to cisternal dimensions of the pineal region: MRI anatomical study with surgical implications using the new clivotentorial method

OBJECTIVES Tentorial alignment and dimensions of posterior fossa cisterns are measurements whose variability can decrease surgical freedom if not taken into account when choosing the approach to the pineal region. The aim is to provide quantitative anatomical information regarding these dimensions, and to discuss their relevance in two most commonly used approaches to this region: the occipital transtentorial and supracerebellar-infratentorial approach. PATIENTS AND METHODS A retrospective study of midsagittal T1-weighted MRI images of 410 randomly selected healthy subjects was performed. The clivus-tentorium (C-T) angle was measured to assess tentorial alignment. The following distances were used as craniocaudal cisternal measurements: quadrigeminal cistern = superior colliculi - inferior part of the splenium of corpus callosum (SC-ISCC), and superior cerebellar cistern = vermis - inferior part of the splenium of corpus callosum (VER-ISCC). RESULTS Median C-T angle value was 19 ± 7°, the quadrigeminal cistern height 6.7 ± 1.6 cm, and the superior cerebellar cistern height 10.4 ± 2.6 cm. The C-T angle was negatively correlated with the SC-ISCC distance (r = -0.271; p <  0.001) and the VER-ISCC distance (r = -0.052, p >  0.001). The SC-ISCC distance was positively correlated with the VER-ISCC distance (r = 0.282; p < 0.001). CONCLUSION Our new method of measuring tentorial alignment provides a simple and effective aid in preoperative planning. For the first time, we present data on craniocaudal dimensions of posterior fossa cisterns, their relationship with tentorial alignment, and discuss their relevance in SCIT and OT approaches.

Intraoperative Eptifibatide Administration During Urgent Arterial Bypass in Neurosurgery

BACKGROUND In some cases when risk of occlusion of a blood vessel is greater than risk of bleeding when patients undergo urgent or unplanned bypass during neurosurgery, the use of eptifibatide may be an option. We describe 2 patients who underwent arterial bypass in whom eptifibatide was used successfully intraoperatively during neurosurgery for prevention of bypass occlusion. CASE DESCRIPTION The first patient presented with a right middle cerebral artery (MCA) aneurysm with subocclusive stenosis of the M1 branch. After right-sided osteoplastic frontotemporal craniotomy, the MCA bifurcation was exposed with a bifurcational 6-mm aneurysm with a wide neck. Prebifurcation stenosis was found, with yellow calcification of the vessel wall, and postbifurcation calcification was found on the upper M2 branch. Superficial temporal artery-MCA bypass and occlusion of the MCA aneurysm was done. Before the bypass, continuous intravenous infusion of eptifibatide 1 μg/kg/minute was administered. The patient recovered normally without hemorrhage or neurologic deficit. The second patient presented with a left-sided lateral sphenoid wing meningioma. Left-sided frontotemporal craniotomy was performed, and the tumor was completely removed from the arachnoid layer. The temporal M3 branch was invaded by the meningioma. As there was no flow through the invaded segment of the aforementioned artery, termino-terminal M3 arterial anastomosis was done. Continuous intravenous infusion of eptifibatide 1 μg/kg/minute was administered. Indocyanine green angiography showed normal flow through the anastomosis, and the patient recovered normally. CONCLUSIONS Future studies are needed to test the safety and potential efficacy of eptifibatide in intraoperative settings.

Hydrocephalus caused by H3N2 type A influenza virus or cerebellopontine angle schwannoma

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