Effective Smartphone Application Use for Postoperative Management of Moyamoya Disease.

Continuous and careful management is necessary after revascularization surgery for moyamoya disease (MMD). The postoperative information has been shared in person or by telephone and emails among doctors; however, this is not always efficient. We aimed to describe the feasibility of remote diagnosis and text chats using a smartphone application on postoperative MMD management. Twenty consecutive patients who underwent combined direct and indirect revascularization for MMD were prospectively investigated in this study. In ten patients, the operator viewed postoperative images uploaded on a smartphone screen using the Join application (Allm Inc., Tokyo, Japan). The doctors shared the radiological findings and treatment plans using the group text chat function and performed postoperative management. We evaluated the intermodality agreements of radiological findings between the smartphone screen and conventional viewer. Postoperative courses were compared between the two patient groups that used or did not use the application. All postoperative images were uploaded to the cloud server and the operator viewed them remotely on the smartphone screen without restriction of location. Detected abnormal findings were cerebral hyperperfusion (CHP), CHP-related watershed shift phenomenon, fluid-attenuated inversion recovery cortical hyperintensity, high signal intensity on diffusion-weighted imaging, CHP-related crossed cerebellar diaschisis, and hypoperfusion. Radiological agreement between the modalities was good in all cases, and additional findings were not obtained on the conventional viewer. The postoperative courses of the Join group were as good as those of the control group. Remote radiological diagnosis and text chat using a smartphone application were feasible and useful for efficient and safe postoperative MMD management.

Long-term Outcomes of Combined Revascularization Surgery for Moyamoya Disease in the Elderly: A Single Institute Experience.

The opportunity to treat older patients with Moyamoya disease (MMD) is increasing. However, the surgical outcomes after combined direct and indirect revascularization for elderly patients with MMD are not fully understood, especially for those ≥60 years old. This retrospective study examined 232 consecutive hemispheres of 165 adults with MMD who underwent combined revascularization. Clinical features and surgical outcomes were compared between the elderly (≥60 years) and nonelderly group (<60 years). Thirteen (5.6%, 64.4 ± 4.0 years old) and 219 hemispheres (94.4%, 40.2 ± 10.8 years old) were included in the elderly and nonelderly group, respectively. The proportion of clinical presentations before surgery did not differ. However, the prevalence of hypertension and hyperlipidemia was significantly higher in the elderly group than in the nonelderly group. Meanwhile, hyperthyroidism was observed only in the nonelderly group. No significant intergroup differences were observed in the incidence of perioperative complications occurring within four weeks postsurgery. Notably, the elderly group was more prone to develop perioperative intracerebral hemorrhage (odds ratio (OR) 3.14, 95% confidence interval (CI) 0.45-13.5) than the nonelderly group. During a median follow-up period of 7.8 years, the incidence of stroke recurrence occurring later than four weeks postsurgery was not significantly different between the groups (hazard ratio, 1.19; 95% CI 0.133-10.6). The prevalence of independent outcomes (76.9% vs. 90.4%, P = 0.14) and mortality (7.7% vs. 1.4%, P = 0.21) did not differ significantly between the elderly and nonelderly groups, respectively. Perioperative intracerebral hemorrhage may be common in the elderly and should be considered to achieve a favorable surgical outcome.

Successful treatment with urgent revascularization and parent artery occlusion for a ruptured intratumoral aneurysm following prior meningioma surgery: illustrative case.

BACKGROUND: An intratumoral aneurysm encased within the associated intracranial tumor is rare, and hemorrhage caused by its rupture is even more rare. While urgent and adequate surgical treatment is important, the treatment can be difficult given the limited understanding of this rare condition. OBSERVATIONS: A 69-year-old man who had undergone meningioma surgery 30 years prior presented with a disturbance in consciousness. Magnetic resonance imaging revealed massive intracerebral and subarachnoid hemorrhage. A round, partially calcified mass, which was diagnosed as recurrent meningioma, was also observed. Subsequent cerebral angiography revealed that the source of the hemorrhage was an intratumoral aneurysm in the dorsal internal carotid artery (ICA) encased within the recurrent meningioma. Urgent surgical ICA trapping and high-flow graft bypass were conducted. The postoperative course was uneventful, and he was referred to another hospital for rehabilitation. LESSONS: This is the first case report of a ruptured intratumoral aneurysm being treated with urgent combined revascularization and parent artery trapping surgery. This surgical approach may be a feasible treatment option for such a challenging condition. Additionally, this case highlights the importance of diligent long-term follow-up after skull-base surgery, as minor intraoperative vascular wall injury may trigger the development and rupture of an intracerebral aneurysm.

Intracranial plasmacytoma arising from dura mater secondary to multiple myeloma and presenting with sudden lethal intracerebral hemorrhage: A case report and literature review.

BACKGROUND: Intracranial and central nervous system's involvement with multiple myeloma (MM) is a clinically rare manifestation. Furthermore, the development of intracranial plasmacytoma without bone involvement is much rarer. Herein, we report the case of massive intracerebral hemorrhage form intracranial plasmacytoma that arose from the dura mater without bone involvement. CASE DESCRIPTION: A 71-year-old woman, who had been diagnosed as MM and treated 2 years prior, developed sudden lethal intracerebral hemorrhage from the intracranial plasmacytoma. Massive hemorrhage was observed after a rapid tumor growth in the middle fossa. Immediate hematoma evacuation and tumor resection allowed the patient to avoid severe neurological deficits and lethal conditions. CONCLUSION: A close follow-up by neuroimaging studies is essential in cases of intracranial plasmacytoma in MM patients and early intervention with surgical resection or radiotherapy should be considered.

[Embolization of Arteriovenous Fistulae of the Maxillary Artery after Le Fort I Osteotomy:A Case Report].

BACKGROUND: Le Fort I osteotomy, one of the most common techniques applied to correct jaw deformities, is generally considered operatively safe. However, a few articles reported that this procedure can lead to formation of arteriovenous fistulae(AVF)involving the maxillary artery infrequently. CASE REPORT: A man in his 40s with a jaw deformity underwent Le Fort I osteotomy. Two days later, he noticed pulsating tinnitus in his right ear. Angiography revealed AVF between the proximal part of the maxillary artery(first segment)and the pterygoid plexus. On further evaluation, AVF were embolized with n-butyl-2-cyanoacrylate(NBCA). Obliteration of AVF was confirmed on the final angiography. The tinnitus resolved shortly after the embolization. CONCLUSION: Embolization with NBCA is a promising treatment for maxillary AVF after Le Fort I osteotomy.

Mollaret Meningitis with a High Level of Cytokines in the Cerebrospinal Fluid Successfully Treated by Indomethacin.

A rare case of Mollaret meningitis characterized by four recurrent episodes of aseptic meningitis during a three-year period is reported. The patient showed a high fever and severe headache accompanied by a high level of cerebrospinal fluid (CSF) cytokines, such as interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α). The symptoms and high CSF cytokines were resolved immediately after introducing indomethacin treatment. Reactivation of the latent virus is considered to be the cause of this rare disease, and indomethacin is believed to inhibit the periodic abnormal generation of eicosanoid in the brain, resulting in a reduction in the fever and subsequent inflammation.

[Efficacy of Stent-Assisted Coil Embolization for a Dissecting Aneurysm of the Cervical Internal Carotid Artery Caused by a Systemic Vascular Disease: A Case Report].

Systemic vascular diseases such as fibromuscular dysplasia, Ehlers-Danlos syndrome, Marfan syndrome, and Behçet's disease are known to cause spontaneous dissecting aneurysms of the cervical internal carotid artery. These diseases are generally associated with vascular fragility; therefore, invasive treatments are avoided in many cases of dissecting aneurysms, and a conservative approach is used for the primary disease. Surgical or intravascular treatment may be chosen when aneurysms are progressive or are associated with a high risk of hemorrhage; however, there is no consensus on which treatment is better. We report a case of a dissecting aneurysm of the cervical internal carotid artery in a patient with suspected Behçet's disease, which was treated using stent-assisted coil embolization. A man in his 40's, with suspected Behçet's disease, presented with an enlarged dissecting aneurysm of the right cervical internal carotid artery. The lesion was present for approximately 10 years. We performed stent-assisted coil embolization for the lesion. Post-surgery, no aneurysms were detected with carotid artery echography. Our case report suggests that stent-assisted coil embolization is a promising treatment for dissecting aneurysms of the cervical internal carotid artery. In addition, the procedure demonstrates the utility of carotid artery echograms for examining recanalization after stent-assisted coil embolization.

Regression of dural arteriovenous fistulae after venous flow reconstructive surgery in a case with hemangiopericytoma at the confluence of sinuses.

BACKGROUND: The mechanism by which acquired dural arteriovenous fistula (dAVF) develops is still unclear. Few cases have been reported with both dAVF and intracranial tumors, and in these few cases the authors have proposed that induced venous hypertension may lead to the pathogenesis of dAVF. We experienced a case of intrasinusoidal hemangiopericytoma (HPC) with dAVF development. In addition to its rare pathology and tumor location, this case showed regression of dAVF immediately after tumor removal. CASE REPORT: The patient was a 23-year-old man who developed progressively worse headaches and papilledema. The HPC was located entirely inside the confluence of the sinuses (CoS) and resulted in venous sinus occlusion. Cerebral angiography demonstrated a dAVF located in the straight sinus, upstream of the occluded CoS, which was fed by the dural branch of the posterior cerebral artery. After the endovascular embolization of the tumor feeders, subsequent surgery included venous reconstruction in addition to tumor excision. Although the dAVF was not treated with an endovascular procedure or surgery, postoperative angiography revealed complete disappearance of the dAVF. CONCLUSION: We conclude that venous reconstructive surgery greatly contributed to the immediate regression of the dAVF. When planning the treatment strategy for such cases, it should be remembered that acquired dAVF may regress due to the normalization of venous hypertension.

[Intradural arachnoid cyst associated with syringomyelia: a case report].

An intradural arachnoid cyst is a relatively rare condition, occurring within the spinal subarachnoid space. We present the even-more rare case of an intradural arachnoid cyst associated with syringomyelia at the same spinal level. The patient was a 66-year-old man who presented with bilateral leg numbness and gait disturbance. Magnetic resonance imaging (MRI) revealed an intradural arachnoid cyst located dorsal to, and compressing, the thoracic spinal cord at the level of the 7th thoracic vertebra (Th 7). In addition, syringomyelia existed at the level of Th 8, slightly caudal to the intradural arachnoid cyst. We dissected the cyst but did not perform any surgical procedures for the syringomyelia. Post-operative MRI showed that the cyst had disappeared and the syringomyelia had spontaneously shrunk. The patient was discharged with improvement in his numbness and gait disturbance. There are a few case reports of intradural arachnoid cysts associated with syringomyelia, but recent evidence suggests that its occurrence is more common than previously thought. A combination of these two diseases is thought to be caused by blockage of cerebrospinal fluid (CSF) flow, which is also thought to cause adhesive arachnoiditis. For this reason, resection of the arachnoid cyst could improve the CSF flow and contribute to the shrinkage of syringomyelia. Furthermore, early treatment may correlate with improvement in radiological findings and neurological symptoms.

Ivy sign, misery perfusion, and asymptomatic moyamoya disease: FLAIR imaging and (15)O-gas positron emission tomography.

BACKGROUND: The prevalence of ivy sign on fluid-attenuated inversion recovery (FLAIR) imaging in patients with asymptomatic moyamoya disease is unclear. The aim of this study was to clarify the incidence of ivy sign in these patients, as well as the correlation between MRI and (15)O gas PET findings. METHODS: A retrospective analysis including 16 consecutive patients with asymptomatic moyamoya disease enrolled between 2001 and 2010 in a single center. FLAIR imaging at the initial visit was categorized as ivy sign present, negative, or equivocal. Hemodynamic and metabolic parameters were quantified in 11 of 16 patients by (15)O-gas positron emission tomography, and the relationship between ivy sign and (15)O-gas PET parameters was analyzed. Cerebrovascular events within the follow-up period (54 ± 28 months) were also examined. RESULTS: Five of 16 asymptomatic moyamoya patients (31.3 %) had positive ivy sign (6/30 hemispheres, 20 %). In (15)O-gas PET examinations, 18 % of 22 hemispheres had elevated oxygen extraction fraction values that were significantly associated with positive ivy sign. Of these 16 asymptomatic moyamoya patients, six patients (37.5 %) underwent combined surgical revascularization. In this series, no patients experienced ischemic stroke, but one had intraventricular bleeding 1 year after surgery. CONCLUSIONS: Ivy sign on FLAIR imaging is still not rare in patients with moyamoya disease, even when asymptomatic. Although optimal management is still under debate, ivy sign may be an indicator of misery perfusion, and patients with asymptomatic moyamoya disease and ivy sign on FLAIR imaging will benefit from more careful follow-up.

Pituitary apoplexy manifesting as massive intracerebral hemorrhage. Case report.

A 68-year-old man presented with severe conscious disturbance caused by pituitary apoplexy resulting in massive intracerebral hemorrhage (ICH). He had been periodically followed up for asymptomatic pituitary adenoma at another hospital for 8 years. Neuroimaging examination revealed pituitary apoplexy and massive ICH located in the left frontal lobe, and the ICH was directly connected to the intratumoral hemorrhage. The diagnosis was massive ICH from pituitary apoplexy. The patient underwent emergent evacuation of hematoma and removal of the pituitary adenoma via bi-frontal craniotomy. Postoperatively, he continued to exhibit deep consciousness disturbance and died 1 month after the operation. Pituitary apoplexy is usually characterized by intra-tumoral hemorrhage. The treatment strategy for asymptomatic pituitary adenoma is still controversial. This case shows that we should always consider the risk of pituitary apoplexy manifesting as ICH which may cause a fatal outcome.

[A successful treatment for a case of idiopathic syringomyelia with syrinx located in the anterior horn].

Idiopathic syringomyelia not associated with Chiari malformation, tumor, nor tetherd cord syndrome has not been frequently reported. So the clinical significance and optimum treatment strategy for this disease remains unclear. We report a 37-year-old man who was diagnosed as having idiopathic syringomyelia and was treated successfully using implantation of a syringo-subarachnoid shunt. The patient suffered gradual worsening of sensory disturbance in the upper extremities, and Magnetic resonance imaging demonstrated a large syrinx located in the cervical spinal cord (C2-3), located mainly at the left anterior horn. We performed syringo-subarachnoid shunt for syringomyelia under partial hemilaminectomy. During the surgery, the spinal cord was markedly rotated and dorsal root entry had shifted to the midline because of the large syrinx. In order to perform myelotomy at the dorsal root entry zone, we needed additional drilling to the medial portion of the lamina. After this procedure, we were able to perform implantation of the syringo-subarachnoid shunt safely. After the operation, the syrinx collapsed immediately and no neurological deficit was observed. The patient was discharged 2 weeks after the operation. Idiopathic syringomyelia in which the syrinx has deviated to the anterior horn is rare. In those cases, we consider that sufficient hemilaminectomy for the midline should be performed.

[A case of metastatic choroid plexus tumor from cholangiocellular carcinoma].

Metastatic intraventricular tumor located in the choroid plexus is very rare. Only a few cases have been reported in the past. According to past reports, these tumors originated from lung, colon, and so on, but not from the bile duct. This is the first case report of choroid plexus metastasis from cholangiocellular carcinoma. A 57-year-old woman who had a history of cholagiocellular carcinoma, demonstrated intraventricular tumor. Although sufficient examination was performed, the tumor was difficult to diagnose as being a metastatic tumor or a choroid plexus carcinoma. Because of this, we performed endoscopic biopsy of the intraventricular tumor. However intraoperative findings were not helpful in distinguishing metastatic tumor and choroid plexus carcinoma. Postoperatively, histological examination was performed. However it was still difficult to differentiate this rare tumor from choroid plexus carcinoma by only hematoxylin and eosin stain. For further examination, Ber EP-4 stain was performed. Ber EP-4 showed strongly positive which indicates metastatic tumor. This method led us to make an appropriate diagnosis of this extremely rare tumor. We considered that in order to diagnose this rare tumor, appropriate histopathological examination, including immunohistopathological examination should be performed.

[Case of a large intra and extra medullary abscess of the spinal cord due to dermal sinus].

Congenital dermal sinuses, resulting from abnormal neurulation, are relatively uncommon and cause the patients to contract intraspinal infection. Sometimes, this intraspinal infection develops intramedurally abscess with severe neurological deficits. Therefore, a lack of awareness about this problem can result in fatal neurological sequelae. We report the case of a 1-year-old boy who had a lumbo-sacral dimple at birth without further evaluation. He presented repeated fever and rapidly progressive paraplegia. MRI showed a huge intraspinal and intramedullary abscess with dermoid, which was thought to be the result of a dermal sinus. Subsequently, he was transferred to our hospital and received immediate surgery. The patient underwent irrigation of purulent material in the intraspinal abscess including on intramedullary lesion, removal of dermoid tumor, and the resection of the dermal sinus. After that, he was treated with 8 weeks of antibiotic therapy. The patient remained paraplegia but made improvement. This patient illustrates the importance of the recognition and evaluation of skin markers. Prophylactic surgery is indicated to prevent dangerous and recurrent infections of the central nervous system.

[Case of symptomatic subacute in-stent thrombosis after carotid angioplasty and stenting for severe carotid stenosis].

We report a case of symptomatic subacute in-stent thrombosis after carotid artery angioplasty and stenting (CAS). The patient was a 72-year-old man who had severe asymptomatic right carotid artery stenosis. He received CAS with distal protection, and gained 100% opening of the right carotid artery. The administration of dual antiplatelet therapy (Aspirin 100 mg/day+Cilostazol 100 mg/day), which had been given since two weeks before the procedure, was continued afterwords. On the seventh day after the stent placement, the patient showed sudden onset of left hemiparesis and conjugated deviation of both eyes to the right side and followed by falling into a comatose state. Emergency angiography showed near occlusion of the right carotid artery, suggesting subacute in-stent thrombosis. In conjunction with the intravenous administration of tissue plasminogen activator (1300,000 IU), we performed additional stent placement on the stented portion of the ICA and gained full recanalization of the ICA about three hours after the onset of the symptoms. The patient showed rapid recovery and returned home with slight clumsiness of his right hand. Symptomatic subacute in-stent thrombosis after CAS is a rather rare complication. We discuss on the possible cause of this and stress the necessity of an additional emergency stenting to gain rapid recanalization.