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.

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.

OBJECTIVEThis nationwide study was organized to evaluate the clinical characteristics, especially 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) in Japan.METHODSParticipation invitation emails for the study were sent to all members of the Japanese Society for Neuroendovascular Therapy and the Japanese Society for Pediatric Neurosurgery, totaling 4123 physicians. Excel files were attached to the emails to collect clinical data on patients aged 5 years old or younger with intracranial AV shunts at each hospital in the 5-year period from 2012 to 2016.RESULTSRepresentative physicians from 209 hospitals returned Excel files containing the patient data. Thirty-four hospitals reported on 72 patients with intracranial AV shunts. Among this cohort, 24 patients (33%) had BAVMs, 22 (31%) had PAVFs, 16 (22%) had VGAMs, and 10 (14%) had DAVFs. Male predominance (70%) was observed among all types of AV shunts. Most PAVFs (73%), VGAMs (88%), and DAVFs (100%) were diagnosed in patients younger than 1 year, especially neonates, whereas most BAVMs (92%) were diagnosed in children older than 1 year. In the patients older than 1 year, BAVMs were relatively common, and VGAMs and DAVFs were rare.CONCLUSIONSThe annual detected rates of BAVMs, PAVFs, VGAMs, and DAVFs in patients 5 years old or younger were 0.0775, 0.0710, 0.0517, and 0.0323 per 100,000 persons, respectively. In neonates and infants, VGAM, DAVF, and PAVF were relatively common, but BAVMs were extremely rare.

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 screwrod 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 Journal of Chiropractic Medicine (2014) 13, 278-281 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.

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Background: Spinal arteriovenous metameric syndrome (SAMS), also known as Cobb syndrome is a rare metameric developmental disorder presenting as an extradural-intradural vascular malformation that involves bone, muscle, skin, spinal cord, and nerve roots. Case Report: An 11-year-old girl presented with history of progressive paraparesis, lower extremity sensory loss, along with bowel and bladder incontinence who was diagnosed with Cobb syndrome. She underwent endovascular embolization of one arterial feeder and two metameric components. Post embolization patient experienced further decline in muscle strength. Physiotherapy regimen was implemented for 4-5 days per week for period of two months. The patient was discharged with a home exercise programme. Result: At the time of discharge, muscle power was improved. Patient was able to walk with the help of walker. Conclusion: Spinal metameric arteriovenous syndrome is a complex nonhereditary genetic vascular disorder associated with variety of neurologic deficits. Physiotherapy management will play a major role in minimizing disability hence improve clinical outcome.

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.