• Journal of Chest Surgery
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• v.18 no.2
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• pp.327-333
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• 1985

Pulmonary arteriovenous fistula is a congenital malformation of the pulmonary vasculature in which there is a persistence of one or more sizable communications that bypass the pulmonary capillary bed that thus directs unoxygenated pulmonary arterial blood directly into the pulmonary venous system. The developmental cause of pulmonary arteriovenous fistula is unknown, but it is postulated that the abnormal channels represent persistent vessels of the primitive splanchnic capillary bed that do not resorb during growth and maturation. We have experienced a case of pulmonary arteriovenous fistula. The 45 years old woman admitted to Kosin Medical College Hospital via emergency room because of massive hemoptysis, and was diagnosed pulmonary arteriovenous fistula by twice postoperative histopathologic examination. The postoperative course was uneventful and she was discharged on the 16th, second postoperative day in a healthy condition.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.335-340
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• 1986

Pulmonary arteriovenous fistula is unusually congenital malformation consisting of an abnormal connection between a pulmonary artery and vein. It may appear as an isolated anomaly or with hereditary hemorrhagic telangiectasis. In the classic form, symptoms and signs are caused by the abnormal right to left shunt. since severe clinical symptoms such as paradoxical embolism, infection, and spontaneous rupture with massive bleeding may occur, surgical treatment is often indicated. Recently we have experienced a surgical treatment of pulmonary arteriovenous fistula in 54 years old housewife. She was admitted in May 1986 with recurrent hemoptysis for 1 month. On admission, the PaO2 was 65.2 mmHg without specific findings. Pulmonary arteriovenous fistula was confirmed by preoperative pulmonary arteriography. A well circumscribed cystic mass was noted in visceral subpleural and inferior lingular segment of left upper lobe. Left upper lobectomy was performed with good results. Histologically angiomatous dilatation of abnormal vessels embedded in lung parenchyma was noted. Postoperative PaO2 was 90 mmHg. Postoperative results were good.

• Journal of Chest Surgery
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• v.18 no.4
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• pp.806-811
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• 1985

Pulmonary arteriovenous fistula is a rare congenital vascular malformation in the lung, resulting from erroneous capillary development, with incomplete formation or disintegration of the vascular septa that would normally divide the primitive connection between the venous and arterial plexus. The pathogenesis of its symptom is that unoxygenated and desaturated arterial blood enter into the pulmonary venous system directly. Recently we have experienced a case of multiple pulmonary arteriovenous fistula in a 15 year old male patient, who presented the symptom of cyanosis and dyspnea on exertion. The operation revealed well circumscribed and multilobulated aneurysmal lesion in left lower lobe with its subpleural and posterolateral basal location, and another aneurysmal lesion in inferior lingular segment of left lung. There was no abnormal connection between the fistula and systemic circulation. The left lower lobectomy was performed along with local extirpation of the inferior lingular segment of left lung. Both lesions showed angiomatous dilatation of the various sized vessels embedded in the parenchyma microscopically. Postoperative clinical course disclosed much improvement in symptoms and in the value of blood gas analysis. The patient was discharged without any complication.

• Journal of Chest Surgery
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• v.21 no.4
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• pp.711-715
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• 1988

Pulmonary arteriovenous fistula is a congenital vascular malformation originated from abnormalities of capillary development. Fistulas may develop only in the lung or may be associated with similar abnormalities of the skin, mucous membrane, and the other organs. It may occur with hereditary hemorrhagic telangietasia[Rendu-Osier-Weber syndrome]. Recently we have experienced a case of the pulmonary arteriovenous fistula associated with Schwannoma in 20 year old male man. Tennis ball sized bright reddish mass which was composed of variable sized vascular channels filled with blood clots was located in the just subpleural portion of left lower lobe. Left lower lobectomy was done. Microscopic findings showed variable sized numerous blood vessels embedding in the parenchyma. There was a thumb-tip sized brown nodular mass which was turned out to be Schwannoma at the left 7th thoracic paravertebral area.

• 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.

• Journal of Chest Surgery
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• v.16 no.3
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• pp.362-367
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• 1983

Pulmonary arteriovenous fistula is a congenital malformation resulting from errant capillary development, with incomplete formation or disintegration of the vascular septa that normally divide the primitive connections between the venous and arterial plexuses. It generally occurs as part of the disorder known as hereditary hemorrhagic telangiectasia [Rendu-Osler-Weber disease]. The hereditary lesion is transmitted as a simple non-sex-linked dominant trait. It may be single or multiple, too small to see on plain chest films or large and easily recognized. One third of the lesions are multiple on plain chest film. The pathogenesis of its symptoms is that unoxygenated, desaturated arterial blood enters into the pulmonary venous system, directly. Recently we have experienced a case of the pulmonary arteriovenous fistula in 26 years old male soldier, which was confirmed by pulmonary angiography preoperatively. 2 thumb-tip sized, well circumscribed cystic masses filled with bright red colored blood were seen in subpleural and anterolateral portion of the right upper lobe. Right upper Iobectomy was performed due to close approximation of the fistula with pulmonary vein. Microscopically, it shows angiomatous dilatation of the abnormal vessels embedding in the parenchyma. Postoperative physiologic studies show nearly normal arterial oxygen saturation, hemoglobin and RBC count. There was good, uneventful postoperative course.

• 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.

• Journal of Korean Neurosurgical Society
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• v.63 no.4
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• pp.415-426
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• 2020

Arteriovenous malformations (AVMs) are congenital anomalies of the cerebrovascular system. AVM harbors 2.2% annual hemorrhage risk in unruptured cases and 4.5% annual hemorrhage risk of previously ruptured cases. Stereotactic radiosurgery (SRS) have been shown excellent treatment outcomes for patients with small- to moderated sized AVM which can be achieved in 80-90% complete obliteration rate with a 2-3 years latency period. The most important factors are associated with obliteration after SRS is the radiation dose to the AVM. In our institutional clinical practice, now 22 Gy (50% isodose line) dose of radiation has been used for treatment of cerebral AVM in single-session radiosurgery. However, dose-volume relationship can be unfavorable for large AVMs when treated in a single-session radiosurgery, resulting high complication rates for effective dose. Thus, various strategies should be considered to treat large AVM. The role of pre-SRS embolization is permanent volume reduction of the nidus and treat high-risk lesion such as AVM-related aneurysm and high-flow arteriovenous shunt. Various staging technique of radiosurgery including volume-staged radiosurgery, hypofractionated radiotherapy and dose-staged radiosurgery are possible option for large AVM. The incidence of post-radiosurgery complication is varied, the incidence rate of radiological post-radiosurgical complication has been reported 30-40% and symptomatic complication rate was reported from 8.1% to 11.8%. In the future, novel therapy which incorporate endovascular treatment using liquid embolic material and new radiosurgical technique such as gene or cytokine-targeted radio-sensitization should be needed.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.18 no.4
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• pp.741-745
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• 1996

AVMs is not a neoplasm, but a congenital developmental anomalies.$^{5)}$ In oral and maxillofacial area, the high recurrence rate and more facial disfigurement give a difficult problem to surgen and patient.$^{8)}$ Conventional treatment modality nowadays is presurgical embolization and surgical resection. In treatment planning, we consider the possibility of complete surgical resection and the esthetics of soft tissue reconstruction. But, two things are very difficult to achieve. We present a patient with AVMs in left upper lip, he had presurgical embolization with Ivalon and Bucrylate, and conservative surgical procedure. So we present this possible treatment modality.

• Journal of the Korean Society of Radiology
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• v.82 no.4
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• pp.1018-1023
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• 2021

A meandering pulmonary vein (MPV) is a rare pulmonary vascular anomaly characterized by an abnormal course of the pulmonary vein draining into the left atrium. We report the case of a 55-year-old female who was diagnosed with a right MPV on pulmonary angiography. Enhanced chest CT revealed a vascular structure with an abnormal course that drained into the right superior pulmonary vein in the right upper lobe, which resembled a pulmonary arteriovenous malformation (AVM). Pulmonary angiography performed to discriminate between an MPV and pulmonary AVM showed no feeding artery, normal parenchymal staining, and drainage to the left atrium via the culprit vessel. Thus, the patient was diagnosed with MPV. When an MPV is difficult to differentiate from other vascular anomalies on enhanced chest CT, pulmonary angiography can be helpful. By recognizing the angiographic findings of an MPV, unnecessary treatment can be prevented.