• Journal of Cerebrovascular and Endovascular Neurosurgery
• /
• v.26 no.2
• /
• pp.119-129
• /
• 2024

Knowledge of the venous anatomy is essential for appropriately treating dural arteriovenous fistulas (AVFs). It is challenging to determine the overall venous structure despite performing selective angiography for dural AVFs with feeder from multiple selected arteries. This is because only a part of the veins can be observed through the shunt in the selected artery. Therefore, after performing selective angiography of all vessels to understand the approximate venous anatomy, the venous anatomy can be easily understood by closely examining the source image of computed tomographic angiography or magnetic resonance angiography. Through this, it is possible to specify the vein that is to be blocked (target embolization), thereby avoiding extensive blocking of the vein and avoiding various complications. In the case of dural AVF with feeder from single selected artery, if the multiplanar reconstruction image of the three-dimensional rotational computed tomography obtained by performing angiography is analyzed thoroughly, a shunted pouch can be identified. If embolization is performed by targeting this area, unnecessary sinus total packing can be avoided.

• Neonatal Medicine
• /
• v.25 no.3
• /
• pp.126-130
• /
• 2018

Parkes Weber syndrome is a rare congenital vascular anomaly, related to the RAS p21 protein activator 1 (RASA1) gene. It is characterized by capillary cutaneous malformations, bony and soft tissue hyperplasia, and multiple arteriovenous fistulas throughout the affected upper or lower extremity. These arteriovenous fistulas can be associated with life-threatening complications such as bleeding, thrombosis, and high output heart failure. In this report, we present a neonate who had a disproportionately hypertrophied left upper limb with port-wine stain, dystrophy of the left humerus, and hypertrophy of the left clavicle on X-ray, and arteriovenous malformation and massive dilatation of the left subclavian artery on magnetic resonance angiography. Exome sequencing analysis revealed a novel heterozygous splicing mutation (c.1776+2T>A) in the RASA1 gene. To the best of our knowledge, this report is the first case of RASA1-related Parkes Weber syndrome in Korea.

• Investigative Magnetic Resonance Imaging
• /
• v.19 no.4
• /
• pp.241-247
• /
• 2015

Arteriovenous malformation (AVM) of the pancreas is extremely rare, although it may be increasingly diagnosed due to the widespread use of cross-sectional imaging of the abdomen. Early diagnosis of this disease is important to prevent delay of treatment and resulting fatal complications. We report a rare case of pancreatic AVM in a 48-year-old man who presented with severe chronic anemia and early gastric cancer, which made diagnosis challenging. Imaging findings, including ultrasound, computed tomography, and magnetic resonance imaging, are shown, as well as the pathologic features.

• Journal of Chest Surgery
• /
• v.34 no.12
• /
• pp.952-955
• /
• 2001

Pulmonary arteriovenous malformations(PAVM) are uncommon diseases. Brain abscesse could be a rare and devastating comlplication of PAVM. Central nervous complaints may be the first manifestations of PAVM. We report a case of PAVM presented by brain abscess which was treated by craniotomy.

• Journal of Korean Neurosurgical Society
• /
• v.66 no.2
• /
• pp.199-204
• /
• 2023

It is critical to identify the ruptured cerebral arteriovenous malformations (AVMs) for secondary prevention. However, there are rare cases unidentified on the radiological evaluation. We report on a patient with the delayed appearance of radiologically occult AVM as a probable cause of the previous intracerebral hemorrhage (ICH). An 18-year-old male patient presented with a right temporal ICH. The preoperative radiological examination did not reveal any causative lesions. Because of the intraoperative findings suggesting an AVM, however, only hematoma was evacuated. Disappointedly, there were no abnormal findings on postoperative and follow-up radiographic examinations. Eleven years later, the patient presented with an epileptic seizure, and an AVM was identified in the right temporal lobe where ICH had occurred before. The patient underwent partial glue embolization followed by total surgical resection of the AVM and anterior temporal lobe. Based on the literature review published in the era of magnetic resonance imaging, common clinical presentation of radiologically occult AVMs included headache and seizure. Most of them were confirmed by pathologic examination after surgery. In cases of the ICH of unknown etiology in young patients, long-term follow-up should be considered.

• Journal of Cerebrovascular and Endovascular Neurosurgery
• /
• v.26 no.1
• /
• pp.79-84
• /
• 2024

Intracranial non-galenic pial arteriovenous fistula (PAVF) is an extremely rare vascular malformation, where one or more pial arteries feeds directly into a cortical vein without any intervening nidus. Though occasionally they can be asymptomatic, neurological symptoms such as headache, seizure, or focal neurological deficit are more common presenting features. Life threatening or fatal hemorrhage is not uncommon, hence needed to be treated more often than not. Spontaneous occlusion of PAVF is reported only four times before. We report a 49-year-old gentleman, who was diagnosed to have a PAVF, possibly secondary to trauma. He presented 5 months and 22 days from initial digital subtraction angiography (DSA) for treatment, and follow-up angiogram showed complete obliteration. He denied any significant event, medication or alternate treatment during this period. His clinical symptoms were stable as well. We postulate iodinated contrast medium induced vasculopathy as a possible cause, which has been described for other vascular pathologies, but never for PAVF.

• Journal of Cerebrovascular and Endovascular Neurosurgery
• /
• v.26 no.1
• /
• pp.71-78
• /
• 2024

Dural arteriovenous fistula (DAVF) is a rare condition affecting approximately 1.5% of 1,000,000 individuals annually. It frequently occurs in the transsigmoid and cavernous sinuses. An isolated sigmoid sinus is extremely rare and is treated by performing transfemoral transvenous embolization along the opposite transverse sinus. A 69-year-old woman presented with asymptomatic Borden type III/Cognard type III DAVF involving an isolated sigmoid sinus. She underwent a staged operation in which a navigation system was used to expose the sigmoid sinus in the operating room before transferring the patient to the angio suite for transvenous embolization. Various modalities have been used to treat DAVF, including surgical disconnection, transarterial embolization, transvenous embolization, and stereotactic radiosurgery. However, treating DAVF cases where the affected sinus is isolated can be challenging because an easily accessible surgical route may not be available. In this case, direct sinus cannulation and transvenous embolization were the most effective treatments.

• Journal of Korean Neurosurgical Society
• /
• v.39 no.5
• /
• pp.374-377
• /
• 2006

Cerebellar hemorrhage in adults is a well-described condition, but rarely occurs in children. Such hemorrhages in children are commonly results from arteriovenous malformations, trauma, infection or hematological abnormalities; a neoplastic origin is rare. We report a case of cerebellar hemorrhage as the initial manifestation of cerebellar glioblastoma in a child with review of literature.

• Tuberculosis and Respiratory Diseases
• /
• v.66 no.4
• /
• pp.314-318
• /
• 2009

Hereditary hemorrhagic telangiectasia (HHT, also called Osler-Weber-Rendu Disease) is a rare systemic fibrovascular dysplasia characterized by recurrent epistaxis, cutaneous telangiectasia, and visceral arteriovenous malformations (AVMs). HHT is an autosomal dominant disease with a prevalence of 1 in 5,000~8,000. Recurrent epistaxis is often the first and most common manifestation, and about 30% of patients reveal pulmonary AVM. Presently, we report a familial case of HHT. A 61-year-old male with asymptomatic multiple pulmonary AVMs was successfully treated with embolization. His older brother who presented with recurrent epistaxis and multiple telangiectasias was treated with laser ablation. Their pedigree revealed a family history of recurrent epistaxis.

• Radiation Oncology Journal
• /
• v.13 no.1
• /
• pp.87-94
• /
• 1995

Since March 1992, total 200 patients who visited our hospital as functional or organic lesions of central nervous system were treated by gamma knife stereotactic radiosurgery for 27 months. Thirty-nine patients of total cases was diagnosed as cerebral arteriovenous malformation. The rate of magnification on X-ray film was reduced by cutting fixation adaptor from 1.0 to below 1.45 times. In order to treat the deep- and lateral-seated cerebral arteriovenous malformation, we slightly modified the angiographic indicator, the commercial Leksell system, by cutting each inner sides about 5mm, We performed the more distinction of the scales by adapting 0.5mm or 1mm copper filter to angiographic indicator. The center point of indicator(X=100mm, Y=100mm, Z=100mm) is corrected by adjusting scales of X-, Y-, Z-axis to each inner 100 and outer 100 point within 1-2mm by repeated exposure of X-ray on films in trial-and-errors. We have developed the 'GKANGIO' programed as the Fortran-77 in Microvax - 3100, which can save treatment planning time and perform accurate pretreatment planning using the theoretical target metrix center. The theoretical description of the simplified method is presented for the reduction of experimental and numerical errors in treatment planning of radiosurgery.