[Venous Anatomy of the Superior and Inferior Sagittal Sinuses].

The superior sagittal sinus(SSS)is contained within the dura, which consists of the dura propria and osteal dura at the junction of the falx cerebri, in addition to the attachment of the falx to the cranial vault. The SSS extends anteriorly from the foramen cecum and posteriorly to the torcular Herophili. The superior cerebral veins flow into the SSS, coursing under the lateral venous lacunae via bridging veins. Most of the bridging veins reach the dura and empty directly into the SSS. However, some are attached to the dural or existed in it for some distance before their sinus entrance. The venous structures of the junctional zone between the bridging vein and the SSS existed in the dura are referred to as dural venous channels. The SSS communicates with the lateral venous lacunae connecting the meningeal and diploic veins, as well as the emissary veins. These anatomical variations of the SSS are defined by the embryological processes of fusion and withdrawal of the sagittal plexus and marginal sinus.

Large Tissue Debris Causing Cerebral Embolism After Transcatheter Aortic Valve Replacement.

Cerebral vascular embolism is one of the complications of transcatheter aortic valve replacement (TAVR). Thrombolytic therapy is not expected to be effective when embolic material consists of a large tissue fragment. Instead, mechanical aspiration may be more effective therapy for acute cerebral infarction after TAVR. Here, we describe the case of an 87-year-old woman with aortic valve stenosis and heart failure who underwent TAVR using a self-expandable valve. Acute cerebral infarction with left middle cerebral artery occlusion caused by a large tissue fragment developed after the procedure.

Recurrent periventricular hemorrhage in cerebral proliferative angiopathy: Case report.

Cerebral proliferative angiopathy (CPA) is a rare vascular lesion. Bleeding from CPA is uncommon, but the risk of rebleeding is high once it bleeds. We describe a case of CPA with multiple intra- and periventricular hemorrhages during 30-year follow-up. Recurrent bleeding in these areas are common in moyamoya disease. These lesions may share the cause of bleeding: proliferation of the periventricular vessels functioning as collateral pathways. Revascularization surgery for CPA may attenuate the vascular proliferation in the vicinity of the ventricle, which may prevent rebleeding.

Nationwide survey of pediatric intracranial arteriovenous shunts in Japan: Japanese Pediatric Arteriovenous Shunts Study (JPAS).

The authors performed a nationwide study in Japan to evaluate the annual detected rate of pediatric intracranial arteriovenous (AV) shunts such as brain AV malformations (BAVMs), pial AV fistulas (PAVFs), vein of Galen aneurysmal malformations (VGAMs), and dural AV fistulas (DAVFs). These rates were revealed for the first time and showed that VGAM, DAVF, and PAVF were relatively common but that BAVMs were extremely rare in neonates and infants.

Dural Arteriovenous Fistula of the Sinus of the Lesser Sphenoid Wing Presenting with Pontine Hemorrhage.

BACKGROUND: Dural arteriovenous fistulas (DAVFs) of the sinus of the lesser sphenoid wing (SLSW) with leptomeningeal drainage are rare. We report a patient with a DAVF of the SLSW draining into the basal vein of Rosenthal (BVR) presenting with pontine hemorrhage. CASE DESCRIPTION: A 71-year-old man presented with sudden right hemisensory disturbance of the arm and leg. Brain computed tomography scan showed left pontine hemorrhage, and cerebral angiography revealed a DAVF of the left SLSW. The fistula drained solely into the left BVR, which had an anastomosis to the left lateral mesencephalic vein, which had a varix invaginated into the left pons. The diagnosis was a DAVF of the left SLSW drained into the lateral mesencephalic vein via the bridging vein of the left SLSW, the deep middle cerebral vein, and the BVR, and a varix of the lateral mesencephalic vein caused pontine hemorrhage. The fistula was occluded by clipping through frontotemporal craniotomy. The postoperative course was uneventful, and postoperative cerebral angiography confirmed disappearance of the fistula. CONCLUSIONS: A DAVF of the SLSW presenting with pontine hemorrhage is extremely rare, and DAVFs with deep leptomeningeal drainage should be included among a variety of etiologies of pontine hemorrhage.

Vein of Galen Aneurysmal Malformation in Monozygotic Twin.

BACKGROUND: Vein of Galen aneurysmal malformation (VGAM) is a rare pediatric vascular malformation of the brain. Genetic backgrounds are not well elucidated. We report on a monozygotic twin with VGAM and his endovascular treatment, and the genetic analyses of the twins and their parents. CASE DESCRIPTION: In a monochorionic, diamniotic pregnancy of a 28-year-old healthy woman, monozygotic twins were born by emergency caesarian section because of fetal distress of the smaller twin at 25 weeks' and 4 days' gestation. Although a postnatal cranial ultrasound failed to detect VGAM in the smaller twin, mild heart failure persisted. A brain magnetic resonance (MR) examination of this twin on day 82 revealed choroidal VGAM. The twin was treated successfully by two sessions of embolization at 6 and 8 months of age. An MR examination at 1 year showed minimal residual arteriovenous shunts. He developed normally similar to the normal co-twin, with a follow-up period of 1 year and 6 months. As for the affected twin, no germline mutation or copy number variations were identified in ENG, ALK1, SMAD4, BMPR2, PTEN, RASA1, KRIT1, Marcavernin, or PDCD10 through whole-exome sequencing (WES). CONCLUSION: We have reported a rare combination of a monozygotic twin and VGAM and the successful endovascular treatment. Phenotypic discordance in monozygotic twins established early in embryogenesis could be attributable to environmental or epigenetic factors.

Neuro-Interventions for the Neonates with Brain Arteriovenous Fistulas: With Special Reference to Access Routes.

Neonatal neuro-intervention is challenging. The purpose of this article is to report the neuro-intervention for the neonates with brain arteriovenous fistulas (AVFs), with special reference to access routes. Fifteen neonates (12 boys and 3 girls) who underwent neuro-intervention within the first 14 days of life were -included. Their diagnoses included vein of Galen aneurysmal malformation (6), dural sinus malformations with arteriovenous (AV) shunts (6), pial AVF (2), and epidural AVF (1). Birth weight ranged from 1,538 g to 3,778 g (mean 2,525 g). Neuro-interventions, especially access routes, in the neonatal periods (< 1 month) were retrospectively reviewed. All neonates presented with severe cardiac failure. In total, 29 interventions (mean 1.9) were performed within 1 month. Although 12 neonates with birth weight more than 2,700 g could be treated through transfemoral arterial routes, 3 neonates with birth weight less than 2,200 g could not be treated successfully by femoral arterial routes. Interventions were performed through 19 femoral arterial, 3 femoral venous, 2 umbilical arterial, 3 umbilical venous, 3 transcardiac, and 2 direct carotid routes. Their overall outcomes were six good recovery, one moderate disability, two severe disabilities, one vegetative state, and five deaths with a mean follow-up period of 7 years 2 months. Neuro-intervention for the neonates with birth weight more than 2,700 g can be performed by femoral arterial routes using a 4F sheath. For those with birth weight less than 2,200 g, however, alternative access routes are required.

Neuroradiological Manifestations of Hereditary Hemorrhagic Telangiectasia in 139 Japanese Patients.

The purpose of this study is to report the neuroradiological manifestations of hereditary hemorrhagic telangiectasia (HHT). One hundred and thirty-nine Japanese HHT patients (73 men and 66 women, aged 2-78 years) were included in this study. Diagnosis of HHT was based on genetic analysis and/or clinical diagnosis of Curaçao. They included 68 HHT1 and 37 HHT2 patients. Essentially, all patients underwent brain magnetic resonance imaging (MRI) and pulmonary computed tomography (CT). Contrast enhanced studies of brain MRI and hepatic CT were performed in a subset of patients. Catheter cerebral angiography was performed when indicated. Their neuroradiological features were reviewed retrospectively. Various imaging abnormalities were found. Brain arteriovenous malformations (AVMs) were observed in 27/136 patients (19.9%, 21 patients with HHT1 and 1 patient with HHT2). Pulmonary arteriovenous fistulas (AVFs) were found in 73/137 patients (65.2%, 45 patients with HHT1 and 6 patients with HHT2). Cerebral infarction and brain abscess were found in 17 patients and 3 patients with pulmonary AVFs, respectively. T1 high lesions in the basal ganglia suggestive of porto-venous shunts were observed in 51/136 patients (37.5%, 9 patients with HHT1 and 28 patients with HHT2). Hepatic AVMs were observed in 61/136 patients (44.9%, 15 patients in HHT1 and 29 patients in HHT2). Brain AVMs and pulmonary AVFs were more common in HHT1 than in HHT2 (both p < 0.01), but hepatic AVMs were conversely more common in HHT2 than in HHT1 (p < 0.01). In conclusion, HHT patients present with a variety of neuroradiological manifestations, which are related to substantial causes of morbid-mortality in HHT.

Late complication of surgically treated atlantoaxial instability: occipital bone erosion induced by protruded fixed titanium rod: a case report.

OBJECTIVE: Polyaxial screw-rod fixation of C1-C2 is a relatively new technique to treat atlantoaxial instability, and there have been few reports in the literature outlining all possible complications. The purpose of this case report is to present the occurrence and management of occipital bone erosion induced by the protruded rostral part of a posterior atlantoaxial screw-rod construct causing headache. CLINICAL FEATURES: A 70-year-old Asian man with rheumatoid arthritis initially presented to our institution with atlantoaxial instability causing progressive quadraparesis and neck pain. INTERVENTION AND OUTCOME: Posterior atlantoaxial instrumented fixation using C1 lateral mass screws in conjunction with C2 pedicle screws was performed to stabilize these segments. Postoperatively, the patient regained the ability to independently walk and had no radiographic evidence of instrumentation hardware failure and excellent sagittal alignment. However, despite a well-stabilized fusion, the patient began to complain of headache during neck extension. Follow-up imaging studies revealed left occipital bone erosion induced by a protruded titanium rod fixed with setscrews. During revision surgery, the rod protrusion was modified and the headaches diminished. CONCLUSION: This case demonstrates that occipital bone erosion after posterior atlantoaxial fixation causing headache may occur. The principal cause of bone erosion in this case was rod protrusion. Although posterior atlantoaxial fixation using the screw-rod system was selected to manage atlantoaxial instability because it has less complications than other procedures, surgeons should pay attention that the length of the rod protrusion should not exceed 2 mm.

Radical excision combined with instrumented fixation in the management of thoracic epidural angiolipoma: a case report.

INTRODUCTION: Spinal angiolipoma is a benign uncommon neoplasm composed of mature lipocytes admixed with abnormal blood vessels. They account for only 0.04% to 1.2% of all spinal tumors. We present a case of thoracic epidural angiolipoma treated by combining radical resection with instrumented spinal fixation, without any surgical complication. CASE PRESENTATION: A 32-year-old Asian woman presented with dorsal epidural angiolipoma at the upper-thoracic level. She had a seven-month history of gradually worsening weakness and numbness in her lower extremities. Imaging studies of her thoracic spine demonstrated a heterogeneously well-enhancing mass, located in her posterior epidural space without surrounding bone erosion at the upper thoracic level. We also observed compression of her thoracic cord. During surgery, a reddish-gray, highly vascularized mass was excised. Her facet joints had to be resected to expose the part migrating into the intervertebral foramen. Because there was concern regarding the stability of her thoracic spine, we performed spinal fixation using pedicle screws. Histopathological study of the surgical specimen showed a typical angiolipoma. CONCLUSION: Angiolipomas can be radically excised with good prognosis. Surgical removal is the preferred treatment for spinal angiolipoma, and the prognosis after surgical management is very good. Although outcomes remained favorable despite incomplete resections in a number of spinal angiolipoma, complete removal is preferred. We successfully achieved total resection without any surgical complication by combining radical resection with instrumented spinal fixation.

Spinal arteriovenous metameric syndrome in a neonate presenting with congestive heart failure: case report.

BACKGROUND: Spinal arteriovenous metameric syndrome (SAMS) is a combination of more than two separate vascular malformations in the same embryonic metameres. This syndrome, also known as Cobb syndrome, is rare, especially in the neonate. CASE DESCRIPTION: A neonatal girl with a birthmark in the occipital and posterior nuchal regions presented with severe heart failure on the day of birth. The large arteriovenous fistulas in the left hypoglossal canal and in the posterior nuchal region were embolized with detachable coils on the postnatal days 5 and 18, which improved heart failure markedly. The associated intramuscular arteriovenous malformation in the posterior neck was left untreated because large arteriovenous fistulas had been occluded. She grew up without any neurological deficits and developed with normal milestones until the latest follow-up of 8 years old. CONCLUSION: To our knowledge, this is the first case with SAMS in a neonate presenting with congestive heart failure. Presence of a birthmark in a neonate presenting with congestive heart failure may suggest the possible underlying high-flow vascular malformations in the same metamere.

Transcardiac cerebral angiography in a child.

The authors present a case in which transfemoral venous, transcardiac cerebral angiography was performed. In this 5-year-old girl with PHACE syndrome, both transfemoral and transbrachial arterial routes could not be used due to aortic interruption and aneurysmal dilation and small looping of the proximal portion of bilateral subclavian arteries. A 5-F balloon-tipped double-lumen catheter was advanced to the right atrium of the heart from the femoral vein. The catheter was then advanced to the left atrium through the patent foramen ovale and was further advanced to the left ventricle and then to the ascending aorta. The balloon catheter was exchanged for a 4-F catheter. Bilateral common carotid angiography was performed without difficulty. This transcardiac approach is useful in the unusual situation in which both femoral and brachial arterial routes are not available.

[Spontaneous cerebrospinal fluid rhinorrhea associated with long-standing overt ventriculomegaly in adults (LOVA)].

OBJECTIVE: Spontaneous cerebrospinal fluid rhinorrhea associated with aqueductal stenosis is rare. CSF diversion is reported to be a failure in the majority of cases. The combination of the repair of the skull base and CSF diversion is reported to be successful. We describe a case successfully treated by intradural repair with ventricular drainage followed by endoscopic third ventriculostomy. CLINICAL PRESENTATION: A 28-year-old woman presented with rhinorrhea, and occasional attacks of headache, vomiting, and unconsciousness for two years. She had been diagnosed as arrested hydrocephalus for 10 years. Magnetic resonance imaging revealed triventriculomegaly with ballooning of the floor of the third ventricle, tonsilar herniation, right anterior horn herniation into the cribriform plate, and bilateral temporal lobe herniation into the temporal base. INTERVENTION: A ventricular drain was inserted followed by dissection of the herniated brain and repair of the enlarged cribriform foramen with periosteal flap. Make sure that the bacterial culture negative, endoscopic third ventriculostomy has been performed. There is no recurrence of hydrocephalus and rhinorrhea for two years. CONCLUSION: Direct communication between the lateral ventricle and the nasal/paranasal sinus is a rare complication of aqueductal stenosis and LOVA. Surgical repair of the skull base followed by cerebrospinal fluid diversion with endoscopic third ventriculostomy was a safe and reliable method.

[Transsphenoidal surgery for pituitary tumors from microsurgery to the endoscopic surgery: single surgeon's experience].

We reviewed results of the surgical outcome of pituitary tumors treated via the transsphenoidal approach between January, 1994 and January, 2010 at our institution. This data included 100 patients (124 procedures) treated through the sublabial transsphenoidal approach and 45 patients (54 procedures) treated through the endoscopic endonasal (bilateral nostrils) transsphenoidal approach performed by a single surgeon. The extent of tumor removal was significantly improved with endoscopic surgery; adjuvant gamma knife radiosurgery was needed for 65% of patients undergoing microsurgery vs. 30% for patients who had endoscopic surgery (p<0.0001). Patients who underwent endoscopic surgery had less intraoperative blood loss (mean volume: 100 mL for microsurgery patients vs. 30 mL for endoscopic surgery patients, p<0.0001), less pain, and less need for postoperative hormone replacement therapy (19% for microsurgery patients vs. 6% for endoscopic surgery patients; p<0.05). CSF leakage and meningitis were experienced in one microsurgery patient (1%) and one endoscopic surgery patient (2.2%). Endoscopic surgery is a reasonable alternative to microsurgery and our experience supports the concept that an otolaryngologist/neurosurgeon team skilled in endoscopic techniques and pituitary surgery can safely make the transition from microsurgery to endoscopic surgery.