Extracranial Jugular Foramen Schwannomas Treated with the Extreme Lateral Juxtacondylar Approach: Surgical Technique and Our Experience.

BACKGROUND: Schwannoma that arises in the jugular foramen (JF) represents an important challenge for neurosurgeons for its precise location, extension, and neurovascular relationship. Nowadays, different managements are proposed. In this study, we present our experience in the treatment of extracranial JF schwannomas (JFss) with the extreme lateral juxtacondylar approach (ELJA). OBJECTIVE: To present our experience in the treatment of extracranial JF schwannomas (JFss) with the ELJA. METHODS: Between January 2013 and January 2017, 12 patients with extracranial JFs underwent surgery by ELJA. All lesions were type C of the Samii classification. Indocyanine green videoangiography was used to evaluate the relationship between the internal jugular vein and the tumor and to control the presence of spasm in the vertebral artery. RESULTS: A complete exeresis was achieved in 9 patients while in 3 patients, it was subtotal. The complete regression of symptoms was obtained in 7 patients with a total resection. The remaining cases experienced a persistence of symptoms. CONCLUSION: The success of this surgery is achieved through a management that starts from the patient's position. We promote an accurate evaluation of JFs through the Samii classification: Type C tumors allow the use of ELJA that reduces surgical complications. Furthermore, we recommend the use of indocyanine green videoangiography to preserve the vessels and prevent vasospasm.

Berlin Heart EXCOR® pediatric ventricular assist device in a patient with Sotos syndrome: a case report.

INTRODUCTION: Berlin Heart EXCOR® pediatric ventricular assist device is a mechanical circulatory support device currently used in pediatric patients. Sotos syndrome is a well-described multiple anomaly syndrome characterized by overgrowth, distinctive craniofacial appearance, cardiac abnormalities, and variable learning disabilities. CASE PRESENTATION: We describe a 7-year-old female Caucasian child with classic Sotos syndrome features subjected to implantation of Berlin Heart EXCOR® pediatric biventricular assist device mechanical support. A heart transplant was carried out after a support time of 459 days. After 5 years of follow-up, our patient is clinically stable and the performance of the transplanted heart is excellent. CONCLUSION: This case confirms that Berlin Heart EXCOR® pediatric ventricular assist device can provide satisfactory and safe circulatory support for children with end-stage heart diseases, even in those with Sotos syndrome. The syndrome is not a contraindication to implantation, since the complications are the same as those observed in patients without the syndrome and the prognosis is not affected by the disease.

Endoscope-Assisted Microneurosurgery for Neurovascular Compression Syndromes: Basic Principles, Methodology, and Technical Notes.

BACKGROUND: Microscopic microvascular decompression (MVD) has a low but not negligible failure rate due to some missed conflicts, especially in case of multiple offending vessels. The reported study is aimed to assess the principles, methodology, technical notes, and effectiveness of the endoscope-assisted (EA) MVD for neurovascular compression syndromes (NVCS) in the posterior fossa. MATERIALS AND METHODS: A series of 43 patients suffering from an NVCS and undergone to an EA MVD were retrospectively reviewed. Syndromes were trigeminal neuralgia in 25 cases, hemifacial spasm in nine cases, positional vertigo in six cases, glossopharyngeal neuralgia in two cases, and spasmodic torticollis in one case. In all cases, a 0°-30° specially designed endoscope was inserted into the surgical field to find/treat those conflicts missed by the microscopic exploration. Each procedure was judged in terms of the effectiveness of the adjunct of the endoscope according to a three types classification system: Type I - improvement in the visualization of the nerve's root entry/exit zone; Type II - endoscopic detection of one or more conflicts involving the ventral aspects of the nerve and missed by the microscope; Type III - endoscope-controlled release of the neurovascular conflict otherwise difficult to treat under the only microscopic view. RESULTS: A total of 55 conflicts were found and treated. Twenty-eight procedures were classified as Type I, nine as Type II, and six as Type III. All the patients had a full recovery from their symptoms. CONCLUSIONS: In selected cases, EA MVD offers some advantages in the detection and treatment of neurovascular conflicts in the posterior fossa.

Cortical aneurysms of the middle cerebral artery: A review of the literature.

BACKGROUND: Middle cerebral artery (MCA) aneurysms constitute from 18-40% of all intracranial aneurysms. They are mainly found in the proximal and bifurcation tracts and only in the 1.1-1.7% of cases they are located in the distal segment. The authors report a case of a ruptured saccular cortical MCA aneurysm with unknown etiology. CASE DESCRIPTION: A 53-year-old female was admitted with a sudden severe headache, nausea, vomiting, and a slight left hemiparesis. The computed tomography (CT) scan showed subarachnoid hemorrhage (SAH) in the left sylvian fissure and intracerebral hemorrhage (ICH) in the left posterior parietal area. The CT angiography (CTA) reconstructed with 3D imaging showed a small saccular aneurysm in the M4 segment in proximity of the angular area. A left parieto-temporal craniotomy was performed, the aneurysm was clipped and the ICH evacuated. The motor deficit was progressively recovered. At 3-month follow-up examination, the patient was asymptomatic and feeling well. CONCLUSIONS: In our opinion, surgery is the best choice for the treatment of ruptured M4 aneurysms with ICH, because it allows to evacuate the hematoma and to exclude the aneurysm from the intracranial circulation. In addition, we suggest both the use of the neuronavigation technique and of the indocyanine green videoangiography (ICGV) for the aneurismal surgery.

Stemness Marker Detection in the Periphery of Glioblastoma and Ability of Glioblastoma to Generate Glioma Stem Cells: Clinical Correlations.

BACKGROUND: Recent studies suggested glioma stem cells (GSCs) are key contributors to therapeutic resistance of glioblastoma multiforme (GBM) and are responsible for GBM recurrence. METHODS: We characterized the phenotype of cancer cells in the core and periphery of 20 GBM tumors, correlating clinical outcome to the ability to form GSCs and distinguishing survival based on Ki-67 staining. RESULTS: Similar levels of methylguanine-deoxyribonucleic acid methyltransferase were found in the core and periphery of GBM tumors, whereas Ki-67 was reduced in the periphery. Similar levels of stemness markers in the periphery and in the core of all GBM cultures were found. Only cells expressing >30% SOX2 levels were able to produce neurospheres. Immunophenotypic analysis showed higher levels of stemness markers in GSC cultures than in all GBM primary cultures. GSC in vitro production and coexpression of Ki-67 >5% negatively correlated with outcome. CONCLUSIONS: Not all GBM cultures can generate GSCs, and this capacity is linked to >30% SOX2 levels. The ability to form spheres negatively correlated to survival, and the detection of >5% Ki-67 levels may be useful to identify patients at risk of disease progression. The presence of GSC-/SOX-2-/Ki-67- cells may be regarded as a new prognostic factor. The presence of stemness markers and methylguanine-deoxyribonucleic acid methyltransferase in the periphery of GBM tumors may be the reason for treatment failure and recurrence. Development of stem cell-targeted therapies and elaboration of more aggressive treatments represent an opportunity to eliminate the GBM source and the nidus of recurrence.

Systemic sclerosis associated with colliquative necrosis in the cerebellum.

BACKGROUND: The scleroderma is a complex autoimmune collagen disorder that can affect many organs simultaneously, as it occurs in the systemic sclerosis (SS), or only the skin, as it occurs in the localized scleroderma (LS). The neurological presentation is extremely uncommon, and even more uncommon are the symptoms of the scleroderma in the cerebellum. CASE DESCRIPTION: We report the case of a 56-year-old male with cerebellar lesions mimicking a brain abscess. After surgical excision, the histopathological diagnosis deposed for an ischemic necrosis caused by a vasculopathy. All the bacteriological and viral exams were negative, whereas the rheumatologic tests were compatible with the scleroderma pattern. CONCLUSION: Up to now, the literature has described only 5 cases of scleroderma in the posterior cranial fossa. The authors report a case of SS causing colliquative necrosis in the cerebellum. Pathogenetic mechanisms, clinical aspects, and radiological features are discussed along with the pertinent literature.

Parasagittal meningiomas: Our surgical experience and the reconstruction technique of the superior sagittal sinus.

BACKGROUND: The radical resection of parasagittal meningiomas without complications and recurrences is the goal of the neurosurgeon. Nowadays, different managements are proposed. This study describes our surgical technique during the lesional excision and the reconstruction of the superior sagittal sinus (SSS). METHODS: The total removal (Simpson I and II) of parasagittal meningiomas (WHO grade I and II) was obtained in 75 patients from September 2000 to January 2010. The indocyanine green videoangiography was used before the dural opening and, when necessary, to identify and preserve the cortical veins. The surgery of the SSS was performed in accordance with Sindou's classification, and its reconstruction was achieved through the use of a patch of galea capitis. RESULTS: We had no cases of recurrence and thrombotic occlusion of the SSS in 5 years after the reconstruction. No complications were observed in 65 patients, and no cases of mortality were reported. Neurological focal deficits were observed in 5 patients. A brain swelling and a venous infarction were observed in 1 patient. Only one case of thrombotic occlusion was observed. A cerebrospinal fluid leak was observed in 2 patients, and a systemic complication was found in 1 patient. CONCLUSION: Several factors contribute to the success of the parasagittal meningioma surgery. We consider the preservation of the cortical veins to be important, and, when possible, we recommend the reconstruction of the anterior third of the SSS. Our experience has led us to believe that until now surgery is a winning choice if practiced by expert hands.

Surgical management of falcine meningiomas: Experience of 95 patients.

Falcine meningiomas, defined as a meningiomas arising from the falx not involving the superior sagittal sinus, account for 9% of all intracranial meningiomas. We analyzed 95 patients with falcine meningiomas who underwent surgical removal of their lesion at our institution between 2001 and 2014. Surgical management of these patients, focusing on anatomical and clinical features is described. Thus, based on our series, a surgical algorithm, classifying the falcine meningioma into four types, according to location at the falx, and using an ipsilateral interhemispheric approach in supine or prone position, is described. The median length of follow-up was 7.1years (range 1.6-12.3years). Approximately one-third of all patients was asymptomatic, headaches occurred in 27 patients, seizures in 14 cases, and lower-extremity weakness in 9 cases. In this series, the middle third of the falx was the most frequently involved site (55,78%), while the anterior third (26,31%) and the posterior type (17,89%) were less common. The transitional and meningothelial types occurred in 69 of patients and a high grade in only two patients. Compared with previous series in literature, there was no mortality and Gross Total Resection was obtained in 83 (87,5%) cases. Three of 95 patients experienced new or worsened neurological deficits after surgery while other complications were relatively in only 6 cases. This study presents our good results about removal of the tumor while preserving major cortical veins and the sinus using advanced microsurgical tools.

Dorsal extradural meningioma: Case report and literature review.

BACKGROUND: Extradural spinal mass lesions are most commonly metastatic tumors. Extradural meningiomas are rare, accounting for approximately 2.5-3.5% of spinal meningiomas; intraoperatively, they are easily mistaken for malignant tumors, especially in the en plaque variety, resulting in inadequate surgical treatment. CASE DESCRIPTION: Our case is one of the first to describe a patient with two purely extradural meningiomas, one each between D3-D4 and between D5-D6 vertebral levels. Surgical resection was radical, and pathologically both lesions were meningothelialmeningiomas. CONCLUSIONS: Reviewing the literature, we discuss the pathogenesis, treatment strategies, and long-term behavior of these uncommon lesions.

Dural repair using autologous fat: Our experience and review of the literature.

BACKGROUND: Various materials have been proposed to obliterate dead spaces and to reconstruct dural defects during a neurosurgical approach. This study describes our technique of using the abdominal autologous fat graft and evaluates the complications and characteristics related to the use of this tissue during cranial procedures. METHODS: Autologous fat grafts were used in 296 patients with basicranial and convexity extraaxial tumors from April 2005 to January 2015. The adipose tissue was removed from the paraumbilical abdominal region and was transformed into a thin foil. When possible, a watertight suture was made between the dural or bone edge with a fat graft. We always used fibrin glue to reinforce the dural closure. RESULTS: Complications occurred between 2 days and 1 year following procedure. Cerebrospinal fluid leaks were found in 11 cases. No case of mortality, pseudomeningoceles, fistula, infections, bacterial meningitides, or lipoid meningitides was reported. No patient required removal of the graft. No adhesion was observed between the brain and the autologous fat. Other fat-related complications observed were 2 cases of fat necrosis in the abdomen and 2 cases of abdominal hemorrhage. CONCLUSION: The technique of harvesting and applying fat grafts is fairly simple, although it must be performed meticulously to be effective. Our experience has led us to believe that the use of fat grafts presents low morbidity and mortality. However, a neurosurgeon should never forget the possible late or early complications related to the use of fat grafts.

Angiolymphoid hyperplasia with eosinophilia and entrapment of the ulnar nerve.

BACKGROUND: The angiolymphoid hyperplasia with eosinophilia (ALHE) is a sporadic vasoproliferative lesion of uncertain etiology involving the skin and the subcutaneous tissue. Occasionally, it involves also the large arteries compressing the near nerves. ALHE is commonly confused with Kimura's disease because of their clinical and histological similarities. CASE DESCRIPTION: We report a case of a 52-year-old female suffering from a 6-month pain and paresthesias in the fourth and fifth finger of the right hand. The angiography showed a pseudoaneurysm in the proximal third of the right ulnar artery. A complete surgical excision of the vascular lesion was undertaken. The lesion forced the right ulnar nerve. The histopathological diagnosis deposed for ALHE. CONCLUSION: Up to now, literature has described 8 cases of ALHE involving the arteries, and only one case originating from the ulnar nerve. The authors report a case of a female with ALHE involving the ulnar artery that compressed the ulnar nerve. Clinical aspects, radiological features, surgical treatment, and operative findings are discussed reviewing the pertinent literature.

Intracranial aneurysms and optic glioma--an unusual combination: a case report.

BACKGROUND: The coexistence of glial high grade tumors (glioblastoma, anaplastic astrocytoma) and cerebral aneurysms is common but the association with optic glioma is rare. The treatment of these associated lesions is problematic. CASE PRESENTATION: A 36-year-old white woman presented to our institution with recurrent attacks of headache. Her preoperative radiological studies revealed a lesion of her left optic nerve with extension to the optic chiasma, an aneurysmatic dilatation of the left carotid bifurcation, a second aneurysm on the left of her middle cerebral artery, and a third one on her right anterior cerebral artery in the A2 tract. A left pterional approach was used to remove the tumor and clip the three aneurysmatic dilatations in a single stage. CONCLUSIONS: We report this unusual case of optic glioma associated to multiples aneurysms and review the pertinent literature. An adequate knowledge of this association is mandatory to plan the correct approach to avoid complications.

Cavernous hemangioma of the dura mater mimicking meningioma.

BACKGROUND: Cavernomas are benign lesions that most commonly occur intra-parenchymally, but occasionally they have been described as arising from the dura mater. Extra-axial cavernous angiomas (or hemangiomas) account for 0.4-2% of all intracranial vascular malformations, and they usually occur in the middle cranial fossa, associated with the cavernous sinus. Other possible localizations (e.g. tentorium, convexity, anterior cranial fossa, cerebellopontine angle, Meckel's cave, sella turcica and internal auditory meatus) are rare, and they account only for 0.2-0.5%. CASE DESCRIPTION: We report a case of a 30-year-old female presenting with a 2 years history of headache unresponsive to drug therapy. The magnetic resonance imaging showed a dural-based lesion in the left frontal region; the lesion size was: 1.5 cm × 3.5 cm. The appearance suggested a convexity meningioma. A left frontal craniotomy was performed, and the histopathological diagnosis deposed for a cavernous hemangioma of the dura mater. The follow-up at 1-year was good without any neurologic deficit. CONCLUSIONS: Dural-based cavernous hemangiomas of the convexity are uncommon lesions. Up to now, only 13 cases have been described in the literature. The authors have discussed clinical aspects, radiological features, surgical treatment, and operative findings.

Syringobulbia associated with posterior fossa meningioma: a review of the literature.

PURPOSE: Syringomyelia can be defined as a degenerative, progressive and chronic spinal cord disease. Its association with tumors of the posterior cranial fossa (PCF) is a rare condition. METHODS: The authors report a rare case of syringobulbia consequent to a meningioma originating from PCF in a 17-year-old female, discussing the pathogenetic mechanism of development and the resolution of the syrinx cavity after surgical procedure. RESULTS: The postoperative period was uneventful without complications. At 6-month follow-up, MRI revealed complete tumor removal with resolution of the syrinx cavity. CONCLUSIONS: In cases of syringomyelia and tonsillar herniation associated with PCF meningioma, the tumor resection allows to eliminate the mass effect and increases the size of the posterior fossa with the progressive ascent of the cerebellar tonsils and the consequent reduction of their downward movement with systolic pulsation. The re-establishment of a normal anatomical condition led to the gradual disappearance of syrinx and hydrocephalus.

Giant aneurysm of distal posterior inferior cerebellar artery: a case report and review of the literature.

INTRODUCTION: Aneurysms in the vertebrobasilar system are rare and in the distal segment of the posterior inferior cerebellar artery they are even less frequent. Giant aneurysms are also rare in the posterior cranial fossa. Giant aneurysms of the distal posterior inferior cerebellar artery generally can have mainly compressive effects on the adjacent structures and they can be mistaken for tumors. CASE PRESENTATION: We report the case of a 74-year-old Italian woman who presented with a complaint of dizziness. Her dizziness was found to be a result of aneurysmal dilatation arising from the distal segment of the right posterior inferior cerebellar artery. A mid-line suboccipital craniotomy was performed, and the aneurysm was clipped without post-operative deficits and with improvement in the patient's dizziness. In our present report, we also review the literature and discuss our case with regard to the clinical and radiological features and surgical procedure performed. CONCLUSION: To the best of our knowledge, few cases of this type of aneurysm have been described in the literature. Our patient had a good outcome after surgical treatment.

Cavernous hemangioma of the frontal bone: a case report.

INTRODUCTION: Cavernous hemangiomas are rare benign bone tumors and those at the level of the cranial bones are even rarer. CASE PRESENTATION: A 50-year-old woman of Italian ethnicity presented with a frontal mass. A computed tomography scan showed an osteolytic lesion and a magnetic resonance imaging scan revealed a hypointense lesion on the T1-weighted image and a hyperintense lesion on the T2-weighted image. We performed a tailored craniectomy and cranioplasty. Histological examination revealed a cavernous hemangioma. CONCLUSIONS: These benign tumors do not have classic radiographic features and so can be misinterpreted as lesions like multiple myeloma or osteosarcoma. Consequently, the diagnosis is most often made during surgical resection.

Endoscope-assisted microneurosurgery for intracranial aneurysms.

BACKGROUND: The endovascular techniques has widely changed the treatment of intracranial aneurysms. However surgery still represent the best therapeutic option in case of broad-based and complex lesions. The combined use of endoscopic and microsurgical techniques (EAM) may improve surgical results. OBJECTIVE: The purpose of our study is to evaluate the advantages and limits of EAM for intracranial aneurysms. METHODS: Between January 2002 and December 2012, 173 patients, harboring 206 aneurysms were surgically treated in our department with the EAM technique. One hundred and fifty-seven aneurysms were located in the anterior circulation and 49 were in the posterior circulation. Standard tailored approaches, based on skull base surgery principles, were chosen. The use of the endoscope included three steps: initial inspection, true operative time, and final inspection. For each procedure, an intraoperative video and an evaluation schedule were prepared, to report surgeons' opinions about the technique itself. In the first cases, we always used the endoscope during surgical procedures in order to get an adequate surgical training. Afterwards we became aware in selecting cases in which to apply the endoscopy, as we started to become familiar with its advantages and limits. RESULTS: After clipping, all patients were undergone postoperative cerebral angiography. No surgical mortality related to EAM were observed. Complications directly related to endoscopic procedures were rare. CONCLUSION: Our retrospective study suggests that endoscopic efficacy for aneurysms is only scarcely influenced by the preoperative clinical condition (Hunt-Hess grade), surgical timing, presence of blood in the cisterns (Fisher grade) and/or hydrocephalus. However the most important factors contributing to the efficacy of EAM are determined by the anatomical locations and sizes of the lesions. Furthermore, the advantages are especially evident using dedicated scopes and holders, after an adequate surgical training to increase the learning curve.

Posterior cranial fossa tumors: Results and prognostic factors in a consecutive series of 14 operated patients by occipital transtentorial approach.

BACKGROUND: The objective of our study was to determine the safety and usefulness of performing surgery via occipital transtentorial approach to treat posterior cranial fossa tumors, which is well known as an approach to the pineal region (Poppen's approach). METHODS: Fourteen patients with posterior cranial fossa tumors were successfully treated using occipital transtentorial approach between 2007 and 2012. The lesions included five meningiomas, three astrocytomas, two metastases, two hemangioblastomas, one cavernoma, and one dysgerminoma. RESULTS: Lesions were <3 cm in 12 cases and ≥3 cm in two cases. Average Karnofsky Performance Status (KPS) scoring at admission was 88.5. Eleven patients scored ≥70 and seven patients <70. Average age was 43.1 years. All patients underwent surgical treatment by the same surgical team. All tumors were completely removed surgically without any injury to the venous complex and the adjoining structures. There was no incidence of mortality or morbidity in all patients, and all functional outcomes were good to excellent postoperatively. Postoperative computed tomography (CT) imaging revealed that none of the patients had suffered brain damage or infarction around the cerebellum, brainstem, or occipital lobe. CONCLUSIONS: We found that the use of occipital transtentorial approach is strongly supported by the successful removal of posterior cranial fossa tumors without serious complications. Open microneurosurgery is probably still the most effective therapy in improving survival and KPS in patients with posterior cranial fossa tumors, given that the proper surgical technique is used and complications do not occur. This case study has strongly suggested that this approach is very useful, safe, and accurate for removing the tumors of posterior fossa and evaluating the surrounding anatomy, as well as for determining operative strategy.

Trigeminal neuralgia and persistent trigeminal artery.

We report a case of trigeminal neuralgia caused by persistent trigeminal artery (PTA) associated with asymptomatic left temporal cavernoma. Our patient presented unstable blood hypertension and the pain of typical trigeminal neuralgia over the second and third divisions of the nerve in the right side of the face. The attacks were often precipitated during physical exertion. MRI and Angio-MRI revealed the persistent carotid basilar anastomosis and occasionally left parietal cavernoma. After drug treatment of blood hypertension, spontaneous recovery of neuralgia was observed and we planned surgical treatment of left temporal cavernoma.

Spontaneous intracranial extradural haematoma associated with frontal sinusitis and orbital involvement.

Intracranial extradural hematoma is usually traumatic. Rarely, it can occur spontaneously associated with coagulative disorders (spontaneous or iatrogenic), dural vascular malformation, cranio-facial tumors and infections. In these cases, spontaneous extradural hematoma (SEH) is a serious event that needs to be recognized and managed in time to avoid fatal outcome. The authors report a case of a 12-year-old young girl with a 3-year history of right frontal sinusitis treated urgently for a right frontal extradural hematoma involving the orbit. Diagnosis and management of this case is discussed reviewing the pertinent literature.