• Archives of Craniofacial Surgery
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• v.22 no.3
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• pp.141-147
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• 2021

Background: Arteriovenous malformation (AVM) which is a high-blood-flow lesion with connections between arteries and veins without an intervening capillary bed, is difficult to manage. The ear is the second most common site of extracranial AVM. However, studies regarding the management of this condition remain lacking. The purpose of this study was to share managing experiences in our center and to investigate the treatment effect through a retrospective analysis of cases. Methods: Among 265 patients with AVM treated in our vascular anomalies center between January 2008 and January 2021, 10 patients with auricular AVM were included in the study to investigate the lesion distribution, clinical stage, and treatment methods by performing a retrospective evaluation. Results: Among 10 patients, five patients had AVMs distributed in the upper half of the ear, one patient in the lower half of the ear, and four patients in whole ear, respectively. Seven patients had Schobinger stage II, and three had stage III. One patient received surgical treatment only, four patients received sclerotherapy only, and five patients received both surgical treatment and sclerotherapy. The posttreatment status was checked as controlled in two patients, improved in seven patients, persistent in one patient. There were no worsening patients. Conclusion: Auricular AVM is a disease that is difficult to manage by one specific department, thus requiring a collaborative management effort from multidisciplinary team.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.4
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• pp.680-684
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• 2004

The hemangioma, a benign proliferation of blood vessel, is the most common tumor of infancy and childhood. In many instances, the lesion probably represents a hamartoma or malformation rather than a true neoplasm. In the oral cavity, common sites are lips, followed by tongue, buccal mucosa and palate. Clinical characteristics appear as a flat or raised reddish-blue lesion and are generally solitary. They are classified on the basis of their histological appearance into capillary, mixed, cavernous or a sclerosing variety. A 6-year-old male of this case was referred to the Department of Pediatric Dentistry, Chonnam National University with a chief complaint of swelling lesion on gingiva. The strawberry appearance mass was detected by clinical examination on attached gingiva at the upper left primary lateral incisor and canine. Surgical excision and biopsy were carried out for histological examination and the lesion was diagnosed with a capillary hemangioma. The risk of recurrence after this therapy is rare, and there is no malignant transformation. Despite their benign origins and behaviour, hemangiomas in the region of oral cavity are always clinically important to the dental profession because of bleeding tendency.

• 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.

• Journal of Korean Neurosurgical Society
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• v.67 no.3
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• pp.280-288
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• 2024

Brain arteriovenous malformations (bAVMs) are aberrant arteriovenous shunts through a vascular nidus with no intervening capillary beds. They are one of the commonest causes of spontaneous intracranial haemorrhage in children and may be associated with significant morbidity and mortality in cases of rupture. Treatment strategies include microsurgical resection, endovascular embolisation, stereotactic radiosurgery, multimodality treatment with a combination thereof, and particularly in high-grade bAVMs, conservative management. Clinicians involved in treating bAVMs need to have familiarity with the natural history pertaining to bAVMs in terms of risk of rupture, risk factors elevating rupture risk as well as understanding the clinical manifestations of bAVMs. This invited review serves to provide a synthesis on natural history and clinical presentation of bAVMs with particular focus in children to inform decision-making pertaining to management.

• Archives of Plastic Surgery
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• v.44 no.4
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• pp.301-307
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• 2017

Background Many difficulties exist in establishing a treatment plan for slow-flow vascular malformation (SFVM). In particular, little research has been conducted on the surgical treatment of SFVMs. Thus, we investigated what proportion of SFVM patients were candidates for surgical treatment in clinical practice and how useful surgical treatment was in those patients. Methods This study included 109 SFVM patients who received care at the authors' clinic from 2007 to 2015. We classified the patients as operable or non-operable, and analyzed whether the operability and the extent of the excision varied according to the subtype and location of the SFVM. Additionally, we investigated complications and self-assessed satisfaction scores. Results Of the 109 SFVM patients, 59 (54%) were operable, while 50 (46%) were non-operable. Total excision could be performed in 44% of the operable SFVM patients. Lymphatic malformations were frequently non-operable, while capillary malformations were relatively operable (P=0.042). Total excision of venous malformations could generally be performed, while lymphatic malformations and combined vascular malformations generally could only undergo partial excision (P=0.048). Complications occurred in 11% of the SFVM patients who underwent surgery; these were minor complications, except for 1 case. The average overall satisfaction score was 4.19 out of 5. Conclusions Based on many years of experience, we found that approximately half (54%) of SFVM patients were able to undergo surgery, and around half (44%) of those patients were able to fully recover after a total excision. Among the patients who underwent surgical treatment, high satisfaction was found overall and relatively few complications were reported.

• Archives of Plastic Surgery
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• v.42 no.5
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• pp.552-558
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• 2015

Background The Klippel-Trenaunay syndrome (KTS) is characterized by three clinical features, namely cutaneous capillary malformations, venous malformations, and soft tissue and/or bony hypertrophy of the extremities. The varied manifestations are attributed to the unpredictable clinical nature and prognosis of the syndrome. To elucidate the clinical characteristics of this disease, we reviewed a relatively large number of KTS patients who presented to our vascular anomalies center. Methods We conducted a retrospective study with 19 patients who were diagnosed with KTS and treated in our vascular anomalies clinic between 2003 and 2014, and examined their demographic characteristics, their clinical features, and the treatments administered. Results The sex distribution was balanced, with 9 (47%) males and 10 (53%) females. The mean follow-up period was 4.1 years (range, 7 months-9 years). Most of the patients received conservative treatments such as medication or physiotherapy. Compression therapies such as wearing of elastic garments/bandages were also administered, and surgical interventions were considered only when the patients became excessively symptomatic. Other treatments included laser therapy and sclerotherapy, and all the treatments were adjusted according to each case, tailored to the conditions of the individual patients. Conclusions KTS is an extremely rare, multifactorial disorder that induces widely varied symptoms. Because of this unique feature, plastic surgeons, when not careful, tend to attach a one-sided importance to typical symptoms such as limb hypertrophy or capillary malformation and thus overlook other symptoms and clinical features. KTS can be suspected in all infants who show capillary malformations or limb hypertrophy and require a multi-disciplinary approach for comprehensive management.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.78 no.4
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• pp.408-411
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• 2015

Pulmonary arteriovenous malformations (AVMs) are caused by abnormal vascular communications between the pulmonary arteries and pulmonary veins, which lead to the blood bypassing the normal pulmonary capillary beds. Pulmonary AVMs result in right-to-left shunts, resulting in hypoxemia, cyanosis, and dyspnea. Clinical signs and symptoms vary depending on the size, number, and flow of the AVMs. Transcatheter embolization is the treatment of choice for pulmonary AVMs. However, this method can fail if the AVM is large or has multiple complex feeding arteries. Surgical resection is necessary in those kind of cases. Here, we report the case of a patient with a 6-cm pulmonary AVM with multiple feeding arteries that was successfully treated by repeated coil embolization without surgery.

• 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.