• Title/Summary/Keyword: vascular malformation

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Trends in the Incidence and Treatment of Cerebrovascular Diseases in Korea : Part I. Intracranial Aneurysm, Intracerebral Hemorrhage, and Arteriovenous Malformation

  • Lee, Si Un;Kim, Tackeun;Kwon, O-Ki;Bang, Jae Seung;Ban, Seung Pil;Byoun, Hyoung Soo;Oh, Chang Wan
    • Journal of Korean Neurosurgical Society
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    • v.63 no.1
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    • pp.56-68
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    • 2020
  • Objective : To analyze the incidence and treatment trends of hemorrhagic stroke (HS), according to HS subtypes, using nationwide data in Korea from January 2008 to December 2016. Methods : We used data from the national health-claim database provided by the National Health Insurance Service for 2008-2016 using the International Classification of Diseases. The crude incidence and age-standardized incidence of each disease associated with HS, which included intracranial aneurysm (IA), hypertensive intracerebral hemorrhage (ICH), and arteriovenous malformation (AVM), were calculated and additional analysis was conducted according to age and sex. Changes in trends in treatment methods and number of treatments were analyzed for each cerebrovascular disease using the Korean Classification of Diseases procedure codes. Results : In 2016, the total number of newly diagnosed adult patients with HS was 24169, showing a decrease by 7.0% since 2008; the age-standardized incidence of HS was 46.2 per 100000 person-years. The age-standardized incidence of unruptured IA (UIA) in adults was 71.4 per 100000 person-years-increased by 2.6-fold since 2008-while that of ruptured IA (RIA) was 12.6 per 100000 person-years, which had decreased at a rate of 20.3% since 2008. The number of coil embolization (CE) for UIA increased by 3.4-fold over 9 years and exceeded that of clipping since 2008. With respect to RIA, CE increased by 2.0-fold over 9 years and exceeded that of clipping from 2014. As for spontaneous ICH in adults, the age-standardized incidence was 31.3 per 100000 person-years in 2016-decreased by 34.7% since 2008-and 14.6% of patients diagnosed with ICH were treated in 2016, which was not significantly different from the proportion of patients treated since 2008. The age-standardized incidence of unruptured AVM (UAVM) was 2.0 per 100000 person-year in 2016, while that of ruptured AVM (RAVM) was 2.4 per 100000 person-years in 2016, showing a decreasing rate of 17.2% from 2008. The total number of treated patients with AVM declined since 2014. Conclusion : In Korea, age-related cerebral vascular diseases, such as RIA, ICH, and RAVM, demonstrated a declining trend in age-standardized incidence; meanwhile, UIA and UAVM demonstrated an increased trend in both crude incidence and age-standardized incidence for 9 years. The increase in the elderly population, management of hypertension, and development of diagnostic and endovascular techniques appear to have influenced this trend.

Identification of the Nonrecurrent Laryngeal Nerve during Thyroid Surgery: Variations, Associated Vascular Malformation, Adequate Surgical Technique (갑상선 수술시 발견된 비회귀성 후두신경; 유형, 동반 혈관 이상, 신경 손상 예방에 대한 고찰)

  • Lee Jan-Dee;Yun Ji-Sup;Lim Chi-Young;Nam Kee-Hyun;Chang Hang-Seok;Chung Woong-Youn;Park Cheong-Soo
    • Korean Journal of Head & Neck Oncology
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    • v.22 no.1
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    • pp.3-7
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    • 2006
  • Purpose: The nonrecurrent laryngeal nerve(NRLN) is a rare anomaly that is associated with the developmentally aberrant subclavian artery. Although rare on the right side and exceptional on the left, an aberrant nonrecurrent pathway for RLN represents a major surgical risk. Three course variations of right NRLN can be distinguished: descending(type I) , horizontal(type II), ascending(type III). This study is performed to characterize the variations of NRLN, associated vascular anomaly, and proper surgical methods for preventing nerve damage. Materials and Methods: Between January 1998 and March 2006 3,381 thyroidectomy were performed at our institution, and during these operations a nonrecurrent laryngeal nerve was observed in 13 cases (0.38%). There were 1 men and 12 women with a median age of 48 years(range 28-57). All of them are identified on the right side. Results: In all cases, there were no clinical symptoms observed preoperatively. The nerve anomaly was diagnosed preoperatively in only one case. There were type I variations of right RLN in 2 cases and type II variations in 11 cases. The retroesophageal aberrant right subcalvian artery; no innominate(brachiocephalic) artery was found and the right common carotic artery was arising directly from the aortic arch, was seen in 12 cases. A vocal cord palaysis caused by NRLN damage during operation was observed in one patient(7.6%) , where the nerve was close to the superior thyroid artery. No other complications were noted. Conclusion: It can be possible to predict NRLN from signs associated with the vascular anomaly; clinical symptoms or imaging studies. When an vascular anomaly is not detected preoperatively, overlooking possibility of NRLN may lead to severe operative morbidity. Hence, It is most important to identify all the thyroid structures carefully during thyroid surgery and to be aware of the possibility of anatomic variations of RLN.

Endovascular Treatments Performed Collaboratively by the Society of Korean Endovascular Neurosurgeons Members : A Nationwide Multicenter Survey

  • Kim, Tae Gon;Kwon, Oki;Shin, Yong Sam;Sung, Jae Hoon;Koh, Jun Seok;Kim, Bum-Tae
    • Journal of Korean Neurosurgical Society
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    • v.62 no.5
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    • pp.502-518
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    • 2019
  • Objective : Since less invasive endovascular treatment was introduced to South Korea in 1994, a considerable proportion of endovascular treatments have been performed by neuroradiology doctors, and endovascular treatments by vascular neurosurgeons have recently increased. However, few specific statistics are known regarding how many endovascular treatments are performed by neurosurgeons. Thus, authors compared endovascular treatments collaboratively performed by vascular neurosurgeons with all cases throughout South Korea from 2013 to 2017 to elucidate the role of neurosurgeons in the field of endovascular treatment in South Korea. Methods : The Society of Korean Endovascular Neurosurgeons (SKEN) has issued annual reports every year since 2014. These reports cover statistics on endovascular treatments collaboratively or individually performed by SKEN members from 2013 to 2017. The data was requested and collected from vascular neurosurgeons in various hospitals. The study involved 77 hospitals in its first year, and 100 in its last. National statistics on endovascular treatment from all over South Korea were obtained from the Healthcare Bigdata Hub website of the Health Insurance Review & Assessment Service based on the Electronic Data Interchange (EDI) codes (in the case of intra-arterial (IA) thrombolysis, however, statistics were based on a combination of the EDI and I63 codes, a cerebral infarction disease code) from 2013 to 2017. These two data sets were directly compared and the ratios were obtained. Results : Regionally, during the entire study period, endovascular treatments by SKEN members were most common in Gyeonggido, followed by Seoul and Busan. Among the endovascular treatments, conventional cerebral angiography was the most common, followed by cerebral aneurysmal coiling, endovascular treatments for ischemic stroke, and finally endovascular treatments for vascular malformation and tumor embolization. The number of endovascular treatments performed by SKEN members increased every year. Conclusion : The SKEN members have been responsible for the major role of endovascular treatments in South Korea for the recent 5 years. This was achieved through the perseverance of senior members who started out in the midst of hardship, the establishment of standards for the training/certification of endovascular neurosurgery, and the enthusiasm of current SKEN members who followed. To provide better treatment to patients, we will have to make further progress in SKEN.

A Case of Pulmonary Arteriovenous Fistula with Family History (가족력이 동반된 폐동정맥루 1예)

  • Kuwon, Eun-Sang;Park, Won-Koung;Ryu, Jeong-Seon;Kwak, Seung-Min;Lee, Hong-Lyeol;Jo, Cheul-Ho;Rho, Hyung-Keun
    • Tuberculosis and Respiratory Diseases
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    • v.45 no.3
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    • pp.649-653
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    • 1998
  • Pulmonary arteriovenous fistula is a congenital vascular malformation in lung resulting from abnormal capillary development and the exact etiology is not well known but it may occur with or without hereditary hemorrhagic telangiectasia. Hereditary hemorrhagic telangiectasia (HHT) or Rendu-osler-Weber disease is an autosomal dominant vascular disorder which associates epistaxis, mucocutaneous and visceral telangicetases, and recurrent hemorrhage with chronic anemia and visceral shuntings. Recently we experienced a case of the pulmonary arteriovenous fistula in a 23 year-old woman with a family history of this disease, which was confirmed by pulmonary angiography.

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A Case of Hereditary Hemorrhagic Telangiectasia Diagnosed through Spontaneous Hemothorax (자발성 혈흉으로 내원하여 진단된 유전출혈모세혈관확장증 1예)

  • Kim, Chong-Whan;Park, Il-Hwan;Kwon, Woo-Cheol;Kim, Young-Joo;Jung, Soon-Hee;Lee, Shun-Nyung;Lee, Seok-Jeong;Lee, Ji-Ho;Jung, Sae-Hyun;Jung, Ye-Ryung;Kim, Sang-Ha
    • Tuberculosis and Respiratory Diseases
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    • v.72 no.1
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    • pp.50-54
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    • 2012
  • Hereditary hemorrhagic telangiectasia (HHT, Osler-Weber-Rendu disease) is a rare autosomal dominant disease characterized by heterogenous multisystemic dysplasia of the vascular tissue. Prevalence of HHT is 1 in 5,000~8,000. HHT commonly presents with recurrent epistaxis, but may have more serious consequences if visceral vascular beds are involved. Approximately 30~50% of HHT cases also present with pulmonary arteriovenous malformation (PAVM). Spontaneous hemothorax is less common, and PAVM is one of the causes leading to hemothorax. Our case involved an 18-year-old female who had suddenly developed right chest pain. The reason for chest pain was due to right spontaneous hemothorax accompanied by PAVM in the right middle lobe. The patient was additionally diagnosed with HHT upon examination of her family history, specifically through her mother's symptom that included recurrent epistaxis and mucosal telangiectasia.

Curative transvenous embolization for congenital multi-hole pial arteriovenous fistula

  • Lane Fry;Aaron Brake;Catherine Lei;Frank A. De Stefano;Adip G. Bhargav;Jeremy Peterson;Koji Ebersole
    • Journal of Cerebrovascular and Endovascular Neurosurgery
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    • v.26 no.1
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    • pp.85-96
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    • 2024
  • Objective: Congenital intracranial pial arteriovenous fistula (PAVF) is a rare cerebral vascular pathology characterized by a direct shunt between one or more pial feeding arteries and a cortical draining vein. Transarterial endovascular embolization (TAE) is widely considered first line therapy. Curative TAE may not be achievable in the multi-hole variant due to the potential to harbor innumerable small feeding arteries. Transvenous embolization (TVE) may be considered to target the final common outlet of the lesion. Here, we present a series of four patients with complex multi-hole congenital PAVF treated with staged TAE followed by TVE. Methods: A retrospective review was conducted on patients who underwent treatment for congenital, multi-hole PAVFs treated by a combined TAE/TVE approach at our institution since 2013. Results: We identified four patients with multi-hole PAVF treated by a combined TAE/TVE. Median age was 5.2 (0-14.7) years. Median follow-up of 8 (1-15) months by catheter angiography and 38 (23-53) months by MRI/MRA was obtained. TVE achieved complete occlusion in three patients that proved durable on radiographic follow-up and demonstrated excellent clinical outcomes with a modified Rankin Score (mRS) of 0 or 1. Complete occlusion of the draining vein was not achieved by TVE in one case. This patient is graded as pediatric mRS=5 three years post-procedure. Conclusions: With thorough technical considerations, our series indicates that TVE of multi-hole PAVF that are refractory to TAE is feasible and effective in arresting the consequences of chronic, high-flow AV shunting produced by this pathology.

Generalized Lymphangiomatosis: A Case Report (전신성 림프관종증: 증례 보고)

  • Cha, Jang-Gyu;Park, Jai-Soung;Paik, Sang-Hyun;Kim, Hee-Kyung
    • Investigative Magnetic Resonance Imaging
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    • v.13 no.2
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    • pp.190-194
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    • 2009
  • Generalized lymphangiomatosis is a rare congenital malformation of the lymphatics. CT and MR scan have been used to evaluate lymphangiomas, which appear as large multicystic fluid-filled masses. CT and MR Imaging findings are often helpful in distinguishing lymphangiomas from various vascular disorders. We report the findings of CT, MRI and bone scan in a patient with generalized cystic lymphangiomatosis. Whole body 3.0-T MR scan using STIR sequence with a larger FOV could detect the additional lesions that were not seen at other imaging modalities. We believe that whole body 3.0 T MR imaging is a good modality to evaluate the extent of the disease and following up the patients with the generalized cystic lymphangiomatosis.

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RASA1-Related Parkes Weber Syndrome in a Neonate

  • Koh, Hong Ryul;Lee, Yeon Kyung;Ko, Sun Young;Shin, Son Moon;Han, Byoung-Hee
    • Neonatal Medicine
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    • v.25 no.3
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    • pp.126-130
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    • 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.

A Case of Hepatocellular Carcinoma after Hepatic Artery Ligation (간동맥 결찰 환자에서 발생한 간세포암 1례)

  • Suh, Jeong-Il;Kim, Joon-Hwan;Lee, Dong-Joon;Kim, Ki-Yoon;Kang, Ho-Jung;Park, Chan-Won;Lee, Heon-Ju
    • Journal of Yeungnam Medical Science
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    • v.13 no.1
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    • pp.146-151
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    • 1996
  • Majority of hepatocellular carcinoma is evolved from a well differentiated cancerous condition such as hyperplastic lesions eg. adenomatous hyperplasia in cirrhotic liver or de no vo carcinogenesis and prolifenation along with dedifferentiation. Adenomatous hyperplasia is may be seen in severe acute hepatic injury, like submassive hepatic necrosis, or in chronic liver diseases, particularly liver cirrhosis and it has recently attracted much interest from both clinicians and pathologists because it is regarded as a precursor lesion of hepatocellular carcinoma. Hepatic adenomatous hyperplasia resembling focal nodular hyperplasia might have developed from localized vascular changes associated with chronic liver disease, pre-existing arterial malformation and early stage of angiogenesis in hepatocarcinogenesis. We present a patient who developed hepatocellular carcinoma after hepatic artery ligation.

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Double Outlet of Right Ventricle in Criss-Cross Heart -Surgical Experience of One Case (십자형심장에 동반된 양대혈관우심실기시증 -수술치험 1례)

  • Kim, Dae-Yeon;Jo, Seong-Rae;Park, Seong-Dal;Jeong, Hyeon-Gi
    • Journal of Chest Surgery
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    • v.30 no.12
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    • pp.1242-1246
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    • 1997
  • Criss-cross heart which is a cardiac malformation caused by abnormal rotation of the ventricles early in embryonic development, is rare but a double outlet of right ventricle in priss-cross heart is very rare. We experienced a case of criss-cross heart which is situs solidus, concordant atrioventricular connection and double outlet of rig t ventricle with remote ventricular septal defect of perimembranous inlet type. A 4-years old female was diagnosed as a double outlet of right ventricle in criss-cross heart after echocardiography, cardiac catheterization and cardiac angiography. The surgical correction was a intraventricular reconstruction of left ventricular outflow with 314 circle of 20 mm Hemashield vascular graft from the ventricular septal defect to the aorta. The patient had a temporary atrioventricular block but was recovered uneventfully, and a postoperative echocardiogram showed no left ventricular outflow obstruction, no intracardiac shunt.

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