• Archives of Plastic Surgery
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• v.32 no.5
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• pp.648-652
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• 2005

In 1940, Kasabach and Merritt first described the association of a large vascular tumor and thrombocytopenia and termed this Kasabach-Merritt(KM) syndrome. It is characterized by a rapidly enlarging vascular anomaly and consumptive coagulopathy with thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of D-dimer and fibrin split product, with or without microangiopathic hemolytic anemia. This is a potentially life-threatening condition with mortality rates from 20 to 30% as a result of severe sepsis, coagulopathy, or invasion of vital organs. Treatment modalities are corticosteroids, interferon alfa-2a or 2b, chemotherapy(vincristine, cyclophosphamide, etc.), aspirin, dipyridamole, com- pression, radiation therapy, embolization of feeding vessels and surgical excision. A standard treatment regimen for KM syndrome has not been established and most reports on definitive management of these complex vascular lesions have been anecdotal, involving small numbers of patients. The authors have successfully treated a patient of KM syndrome with actively bleeding huge hemangioma by surgical excision. They present it with the review of articles.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.261-266
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• 2002

Vascular ring, originating from abnormal regression of the aortic arch during fetal life, can cause prolonged and recurrent respiratory symptoms and dysphagia when the diagnosis is delayed. We report a 4 month old girl with vascular ring, who had been treated for persistent respiratory symptoms including stridor, wheezing, and dyspnea soon after birth. Initially her respiratory symptoms were thought to be due to bronchiolitis, for which respiratory syncytial virus was confirmed by immunofluorescent staining. Her clinical course was again complicated with tracheitis and pneumonia due to Haemophilus influenzae type b. The possibility of anatomical anomaly was investigated when it was felt to be difficult to insert a suction catheter deep down through a endotracheal tube which was placed for adequate ventilatory management. A three-dimensional chest CT revealed a vascular ring consisting of a double aortic arch. For 5 months following surgery, her respiratory symptoms have slowly been improving. She developed another episode of pneumonia which was milder than the one which occurred before the surgery.

• 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 the Korean Society of Radiology
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• v.83 no.4
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• pp.846-860
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• 2022

Persistent left superior vena cava (PLSVC) is a rare congenital, thoracic, and vascular anomaly. Although PLSVCs generally do not have a hemodynamic effect, several types of PLSVC and some cardiac anomalies may manifest with clinical symptoms. The presence of PLSVC can render catheterization via left subclavian access difficult when placing a pacemaker or central venous catheter. As such, recognizing a PLSVC that is typically incidentally discovered can prevent complications such as vascular injury. Differentiating vessels found in a similar location as PLSVC is necessary when performing thoracic vascular procedures. This pictorial essay explains the multi-detector CT findings of a PLSVC, and provides a summary of other blood vessels that require differentiation during thoracic vascular procedures.

• Journal of the Korean Society of Radiology
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• v.81 no.6
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• pp.1511-1516
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• 2020

The anomalous retro-psoas iliac artery is an extremely rare congenital iliolumbar vascular anomaly. A 51-year-old woman presented to our emergency department with worsening right lower extremity pain and weakness for 3 months. CT angiography of the right lower extremity showed no evidence of stenosis in the lower extremity arteries and the incidental finding of an anomalous right retro-psoas iliac artery. Herein, we report a rare case of anomalous retro-psoas iliac artery. Surgeons and clinicians need to be aware of this rare congenital anomaly to avoid severe complications during pelvic or orthopedic surgery.

• Journal of Chest Surgery
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• v.38 no.9 s.254
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• pp.633-636
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• 2005

Sixteen-day-old baby with severe Ebstein anomaly underwent emergency operation to relieve progressive hypoxia and congestive heart failure. Operative findings showed huge right atrium and atrialized right ventricle (aRV) with very small functional RV by distal displacemcent of tricuspid valve mechanism. We elected to perform modified Starness operation because biventricular repair was deemed unattainable. After pulmonary and tricuspid valves were primarily closed, aRV was obliterated with multiple sutures from RV apex to the base. Then a PTFE (Gore-Tex, USA) vascular graft was interposed between innominate artery and main pulmonary artery for systemic to pulmonary shunt. The patient was discharged uneventfully, and received bi-directional cavopulmonary shunt 6 months later.

• Journal of Chest Surgery
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• v.37 no.3
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• pp.282-285
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• 2004

Aberrant right subclavian artery (ARSA) is an anomaly with a reported incidence of 0.5% to 2%. Most patients with an ARSA remain asymptomatic; however about to% of adult patients have compressive symptoms. A case is reported of a 64-year old female patient who had a few years of history of dysphagia and recurrent pneumonia. Angiography was performed, which demonstrated an ARSA with common origin of the right and left carotid arteries. Surgical correction was performed via right thoracotomy. The proximal aberrant artery was mobilized behind the esophagus. The distal, right subclavian artery was exposed, transected, and transposed with reimplantation into the aortic root by graft bypass.

• Tuberculosis and Respiratory Diseases
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• v.76 no.6
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• pp.295-298
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• 2014

Pulmonary systemic arterialization to normal basal lung without sequestration is a rare congenital anomaly. In this rare abnormality, arterialization of the left lower lobe is the most common type. In general, surgical treatments have been performed. Recently, for reducing the complications and risks of surgery, embolization is mainly attempted by using coils. We report a case of 22-year-old male patient with a 10 mm anomalous arterial supply to his normal lung, which is being successfully treated by transcatheter embolization when using the Amplatzer Vascular Plug that has been adapted for the treatment of high-flows and large artery occlusions.

• Journal of Veterinary Clinics
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• v.35 no.1
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• pp.26-29
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• 2018

A 6-month-old, female poodle presented with a three-month history of persistent regurgitation immediately after eating. On physical examination, the patient was emaciated and dehydrated. Thoracic radiography showed ventral displacement of the trachea and increased radiopacity in the mediastinum, cranial to the heart base. A severely dilated esophagus was identified cranial to the heart on esophagram. Computed tomography (CT) revealed the esophagus was filled with gas, fluid and a little of contrast and dilated from caudo-cervical to cranio-thoracic part. The esophageal diameter was markedly decreased at the heart base. In addition, the trachea was displaced to the left-ventral side of the right aortic trunk and an aberrant left subclavian artery originating from the aorta was identified. There was no evidence of abdominal vascular anomaly. Based on diagnostic imaging, persistent right aortic arch (PRAA) with an aberrant left subclavian artery was diagnosed. The patient did not undergo surgery and died at 15 days after diagnosis. This report describes imaging diagnosis, including CT and radiography in a weaned dog with regurgitation due to esophageal obstruction by PRAA. When PRAA is suspected and conventional radiography or contrast study is insufficient for diagnosis, CT may be helpful for diagnosing PRAA.

• Journal of the Korean Society of Radiology
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• v.82 no.4
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• pp.948-952
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• 2021

The absence of the common carotid artery (CCA) and the common origin of the left external carotid artery (ECA) and the right CCA are rare anomalies of the cervical vascular system. We report here a case involving the coexistence of these vascular anomalies with an aberrant right subclavian artery, which is a common congenital anomaly in the aortic arch, and review the embryologic mechanism and clinical importance of this case.