• Tuberculosis and Respiratory Diseases
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• v.67 no.1
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• pp.52-58
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• 2009

Pulmonary arteriovenous malformation (PAVM) is a rare pulmonary vascular anomaly due to an abnormal communication between the pulmonary artery and vein. The most common presenting symptom is a dyspnea on exertion related to this right-to-left shunt. If left untreated, PAVM has been known to result in serious complications. Incomplete pulmonary capillary network can be the cause of cerebral abscesses and other noninfectious neurological complications, such as stroke and transient ischemic attacks due to paradoxic embolism Transcatheter embolotherapy, using coils or balloons, has replaced surgical resection as the treatment of choice for PAVM. However, the risk of device embolization has limited the use of coil embolotherapy, while the size of PAVM is huge. Recently, Amplatzer$^{(R)}$ Vascular Plug has been proposed as an alternative endovascular occlusion device for arteriovenous malformation. We report a case of 81-year-old male patient with a giant PAVM, which was successfully treated by transcatheter embolotherapy using the Amplatzer$^{(R)}$ Vascular Plug.

• Journal of Korean Neurosurgical Society
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• v.55 no.2
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• pp.89-91
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• 2014

Chronic encapsulated intracerebral hematoma (CEIH) is a rare cerebrovascular disease that behaves as a slowly expanding lesion with a gradual onset. It is well established that CEIH is associated with arteriovenous malformations; however, CEIH associated with cavernous malformation (CM) is extremely rare. We herein report a case of CEIH associated with CM, and discuss its pathogenesis. A 12-year-old female was admitted to our hospital because of a one week history of progressive headache and nausea. Brain computed tomography scan and magnetic resonance imaging showed an intracerebral hematoma surrounded by edema in the right frontal lobe. One week later, her headache and nausea worsened, and a brain computed tomography scan revealed the enlargement of hematoma. A right frontal craniotomy was performed. The capsule, mass, and hematoma were totally removed. Histological examination confirmed the diagnosis of CEIH associated with CM. Immunohistochemical analysis revealed increased expression of vascular endothelial growth factor (VEGF) and the VEGF receptor-1 in the endothelium and fibroblasts. Our findings suggest that the activated VEGF pathway might have positively contributed to development of CEIH in the present patient.

• Korean Journal of Head & Neck Oncology
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• v.32 no.1
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• pp.25-28
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• 2016

A 54-year-old female presented with a 4cm sized painless, submandibular gland area mass. Vascular lesion was suspected in computed tomography scan. Surgical excision was performed via retroauricular approach for its excellent cosmetic outcome. The vascular mass with peripheral focal calcification was clearly resected without complication such as bleeding or neural damage. Histopathological examination of the specimen revealed an arteriovenous malformation(AVM) with abnormal communications between arteries and veins without the normal intervening capillary bed. We report a successful excision of AVM in the submandibular space via retroauricular approach.

• Korean Journal of Head & Neck Oncology
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• v.32 no.1
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• pp.29-32
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• 2016

Venous malformation with phleboliths is uncommon cause of unilateral parotid swelling. The clinical and radiographic appearance of venous malformation with phleboliths may masquerade as sialolithiasis. A 49-year-old female complained about unilateral parotid swelling for 6 years. Preoperative evaluation including computed tomography and sonography showed the suspicion of venous malformation with phleboliths. Superficial parotidectomy was performed. Pathological examination confirmed that the mass was venous malformation with phleboliths combined with sialolith in the parotid gland. We present the case of unilateral parotid swelling caused by a venous malformation combined with sialolithiasis.

• BMB Reports
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• v.49 no.5
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• pp.255-262
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• 2016

The disease known as cerebral cavernous malformations mostly occurs in the central nervous system, and their typical histological presentations are multiple lumen formation and vascular leakage at the brain capillary level, resulting in disruption of the blood-brain barrier. These abnormalities result in severe neurological symptoms such as seizures, focal neurological deficits and hemorrhagic strokes. CCM research has identified 'loss of function' mutations of three ccm genes responsible for the disease and also complex regulation of multiple signaling pathways including the WNT/β-catenin pathway, TGF-β and Notch signaling by the ccm genes. Although CCM research is a relatively new and small scientific field, as CCM research has the potential to regulate systemic blood vessel permeability and angiogenesis including that of the blood-brain barrier, this field is growing rapidly. In this review, I will provide a brief overview of CCM pathogenesis and function of ccm genes based on recent progress in CCM research.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.47 no.3
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• pp.209-215
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• 2021

Objectives: Vascular malformation (VM) of the tongue can cause true macroglossia in children. Reduction glossectomy provides primary relief when sclerotherapy has failed or is not possible. In this study, we evaluated the surgical role in functional outcome of reduction glossectomy performed for VM of the tongue. Patients and Methods: We evaluated the functional and surgical outcomes of seven children who were treated at a tertiary care centre in Southern India between 2013 and 2018. Results: Six children underwent median glossectomy, while one child underwent lateral glossectomy. Functional assessment was performed at least 2 years after the date of surgery. At the time of assessment, speech was comprehensible for three children and was occasionally unintelligible in four children. Taste and swallowing were normal in all seven children. Six children exhibited a minimal residual lesion after surgery, of which only one was symptomatic. Residual lesions were managed with sclerotherapy (n=3), observation (n=2), or repeat surgery (n=1). Conclusion: Reduction glossectomy in children with macroglossia secondary to VMs has acceptable outcomes in terms of cosmesis and speech, with no gastronomic restriction.

• Journal of the Korean Society of Radiology
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• v.84 no.2
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• pp.504-511
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• 2023

Hemolymphangioma or hemangiolymphangioma is a rare venolymphatic vascular malformation composed of proliferations or networks of vascular spaces including the lymphatics, capillaries, veins, or arteries. The small bowel is a rare location for hemolymphangioma, and the small bowel mesentery is an even rarer site. Herein, we report a surgically confirmed large complex hemolymphangioma in the small bowel mesentery in a 55-year-old male.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.152-156
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• 2016

Megalencephaly-capillary malformation-polymicrogyria syndrome (MCAP), previously known as macrocephaly-cutis marmorata telangiectatica congenita and macrocephaly-capillary malformation syndrome, is a rare multiple-malformation syndrome that is characterized by progressive megalencephaly, capillary malformations of the midline face and body, or distal limb anomalies such as syndactyly. Herein, we report a female infant case that satisfies the recently proposed criteria of MCAP and describe the distinctive neuroradiological and morphological features. We have also reviewed recently published reports and the diagnostic criteria proposed by various authors in order to facilitate the clinical diagnosis of these children in pediatric neurology clinics.

• Journal of Korean Neurosurgical Society
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• v.67 no.4
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• pp.458-466
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• 2024

Objective : Several clinical studies have explored the feasibility and efficacy of radiosurgical treatment for mesial temporal lobe epilepsy, but the long-term safety of this treatment has not been fully characterized. This study aims to report and describe radiation-induced cavernous malformation as a delayed complication of radiosurgery in epilepsy patients. Methods : The series includes 20 patients with mesial temporal lobe epilepsy who underwent Gamma Knife radiosurgery (GKRS). The majority received a prescribed isodose of 24 Gy as an adjuvant treatment after anterior temporal lobectomy. Results : In this series, we identified radiation-induced cavernous malformation in three patients, resulting in a cumulative incidence of 18.4% (95% confidence interval, 6.3% to 47.0%) at an 8-year follow-up. These late sequelae of vascular malformation occurred between 6.9 and 7.6 years after GKRS, manifesting later than other delayed radiation-induced changes, such as radiation necrosis. Neurological symptoms attributed to intracranial hypertension were present in those three cases involving cavernous malformation. Of these, two cases, which initially exhibited an insufficient response to radiosurgery, ultimately demonstrated seizure remission following the successful microsurgical resection of the cavernous malformation. Conclusion : All things considered, the development of radiation-induced cavernous malformation is not uncommon in this population and should be acknowledged as a potential long-term complication. Microsurgical resection of cavernous malformation can be preferentially considered in cases where the initial seizure outcome after GKRS is unsatisfactory.

• Clinical and Experimental Pediatrics
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• v.60 no.1
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• pp.1-9
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• 2017

Malformations of cortical development are rare congenital anomalies of the cerebral cortex, wherein patients present with intractable epilepsy and various degrees of developmental delay. Cases show a spectrum of anomalous cortical formations with diverse anatomic and morphological abnormalities, a variety of genetic causes, and different clinical presentations. Brain magnetic resonance imaging has been of great help in determining the exact morphologies of cortical malformations. The hypothetical mechanisms of malformation include interruptions during the formation of cerebral cortex in the form of viral infection, genetic causes, and vascular events. Recent remarkable developments in genetic analysis methods have improved our understanding of these pathological mechanisms. The present review will discuss normal cortical development, the current proposed malformation classifications, and the diagnostic approach for malformations of cortical development.