• Clinical and Experimental Pediatrics
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• 제53권6호
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• pp.680-687
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• 2010

Patients with congenital heart diseases (CHD) are confronted with early- and late-onset complications, such as conduction disorders, arrhythmias, myocardial dysfunction, altered coronary flow, and ischemia, throughout their lifetime despite successful hemodynamic and/or anatomical correction. Rhythm disturbance is a well-known and increasingly frequent cause of morbidity and mortality in patients with CHD. Predisposing factors to rhythm disturbances include underlying cardiac defects, hemodynamic changes as part of the natural history, surgical repair and related scarring, and residual hemodynamic abnormalities. Acquired factors such as aging, hypertension, diabetes, obesity, and others may also contribute to arrhythmogenesis in CHD. The first step in evaluating arrhythmias in CHD is to understand the complex anatomy and to find predisposing factors and hemodynamic abnormalities. A practical stepwise approach can lead to diagnosis and prompt appropriate interventions. Electrophysiological assessment and management should be done with integrated care of the underlying heart defects and hemodynamic abnormalities. Catheter ablation and arrhythmia surgery have been increasingly applied, showing increasing success rates with technological advancement despite complicated arrhythmia circuits in complex anatomy and the difficulty of access. Correction of residual hemodynamic abnormalities may be critical in the treatment of arrhythmia in patients with CHD.

• Journal of Korean Neurosurgical Society
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• 제44권3호
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• pp.156-158
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• 2008

Intraorbital hemorrhage is a rare clinical condition caused by orbital trauma, surgery around the orbit, intraorbital vascular abnormalities, and neoplasm. It was reported to occur spontaneously without any known causes and in association with orbital pseudotumor in a very few cases. A 59-year-old, female patient admitted with sudden onset of severe exophthalmos and pain on the left eye. Orbital CT and MR imaging suggested hemorrhage in the upper part of retrobulbar area of the left orbit. Cerebral angiography was taken to rule out any possible vascular abnormalities. On the left carotid cerebral angiography, the run-off of the distal ophthalmic artery was not seen and the engorgement of the supraophthalmic artery was noted. Systemic administration of corticosteroid did not improve the clinical status and craniectomy was done and retrobulbar hematoma was removed, and the clinical symptoms and signs were improved. Authors report a case of spontaneous intraorbital hemorrhage with the clinical features similar to those of orbital pseudotumor, requiring surgical decompression.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제37권
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• pp.42.1-42.5
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• 2015

In the field of oral-maxillofacial surgery, vascular malformations present in various forms. Abnormalities in the size of the tongue by vascular malformations can cause mandibular prognathism and skeletal deformity. The risk in surgical treatment for patients with vascular malformation is high, due to bleeding from vascular lesions. We report a rare case of macroglossia that was treated by partial glossectomy, resulting in an improvement in the swallowing and mastication functions in the patient. A 25-year-old male patient with severe open-bite and mandibular prognathism presented to our department for the management of macroglossia. The patient had a difficulty in food intake because of the large tongue. Orthognathic surgery was not indicated because the patient had severe jaw bone destruction and alveolar bone resorption. Therefore, the patient underwent partial glossectomy under general anesthesia. There was severe hemorrhaging during the surgery, but the bleeding was controlled by local procedures.

• Korean Journal of Radiology
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• 제25권8호
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• pp.726-741
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• 2024

Recent advancements in Alzheimer's disease treatment have focused on the elimination of amyloid-beta (Aβ) plaque, a hallmark of the disease. Monoclonal antibodies such as lecanemab and donanemab can alter disease progression by binding to different forms of Aβ aggregates. However, these treatments raise concerns about adverse effects, particularly amyloid-related imaging abnormalities (ARIA). Careful assessment of safety, especially regarding ARIA, is crucial. ARIA results from treatment-related disruption of vascular integrity and increased vascular permeability, leading to the leakage of proteinaceous fluid (ARIA-E) and heme products (ARIA-H). ARIA-E indicates treatment-induced edema or sulcal effusion, while ARIA-H indicates treatment-induced microhemorrhage or superficial siderosis. The minimum recommended magnetic resonance imaging sequences for ARIA assessment are T2-FLAIR, T2^* gradient echo (GRE), and diffusion-weighted imaging (DWI). T2-FLAIR and T2^* GRE are necessary to detect ARIA-E and ARIA-H, respectively. DWI plays a role in differentiating ARIA-E from acute to subacute infarcts. Physicians, including radiologists, must be familiar with the imaging features of ARIA, the appropriate imaging protocol for the ARIA workup, and the reporting of findings in clinical practice. This review aims to describe the clinical and imaging features of ARIA and suggest points for the timely detection and monitoring of ARIA in clinical practice.

• Journal of Trauma and Injury
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• 제37권2호
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• pp.166-169
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• 2024

Stab wounds, particularly those affecting multiple body regions, present considerable challenges in trauma care. This report describes a case of sustained self-inflicted stab injuries to the abdomen and thighs of a 23-year-old male patient. Although the patient's vital signs were stable and bleeding was minimal from thigh wounds without overt signs of vascular injury, the patient experienced a sudden, profound hemorrhage from the right thigh, leading to cardiac arrest. Successful resuscitation was followed by surgical repair of a right superficial femoral arterial injury accompanying a resuscitative endovascular balloon of the aorta. Subsequent lower extremity computed tomography angiography revealed no additional vascular abnormalities. The patient was discharged in stable condition on the 12th postoperative day. This case underscores the unpredictability of stab wound trajectories and the potential for hidden vascular injuries, even in the absence of immediate life-threatening signs. It also emphasizes the critical role of advanced imaging modalities, such as computed tomography angiography, in identifying concealed injuries, and the importance of strategic intraoperative techniques, including resuscitative endovascular balloon occlusion of the aorta, in achieving favorable patient outcomes.

• Journal of Genetic Medicine
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• 제10권1호
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• pp.47-51
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• 2013

Loeys-Dietz syndrome (LDS) is an autosomal dominant disorder caused by heterozygous mutations in the genes encoding transforming growth factor-${\beta}$ receptor type 1 or 2. It is typically characterized by a triad of hypertelorism, cleft palate or bifid uvula, and arterial tortuosity with aneurysm or dissection. Characteristic vascular abnormalities such as tortuosity, aneurysms, dissections, and stenosis are the most severe complications of LDS and can occur in the neurovascular system. We report a 5-year-old boy who presented with headaches and neurovascular abnormalities and was diagnosed with LDS with a novel mutation of the TGFBR1 gene. It is the first Korean report of neurovascular abnormalities in LDS.

• 한국임상수의학회지
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• 제18권3호
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• pp.293-296
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• 2001

Hepatic encephalophthy was diagnosed with serum chemistry, abdominal radiography and ultrasonography in a 2.6kg, 4 year-old maltese dog showing signs of hypersalivation, involutary spasm of facial muscles, ataxia, behavior abnormalities like dullness, sleep disorder, restlessness. In serum chemistry, the level of alanine transferase and aspartate trasferase was mildly elevated, ammonia was severely increased. On abdominal radio-graphs, the size of liver was mildly decreased. In ultrasonographic examination, diffuse lesion with hyperechoic change and decreased vasculature were seen in the hepatic region. But vascular abnormalities of liver were not observed. Drug and dietary therapy were undertaken and severities of clinical sign were alleviated.

• Journal of Yeungnam Medical Science
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• 제35권2호
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• pp.232-235
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• 2018

Fabry disease (FD) is an X-linked, recessively inherited, rare, progressive, disorder of glycosphingolipid metabolism affecting multiple organs resulting in organ dysfunction. It is rare to find only one FD affected subject with a de novo mutation. Here we report a case of a 41-year-old Asian male diagnosed with de novo FD. Comprehensive ophthalmological evaluation was performed using slit lamp, color fundus photography, optical coherence tomography, fluorescein angiography, and indocyanine green angiography. On slit lamp examination, cornea verticillata and slightly tortuous, and aneurysmal dilatation of inferior bulbar conjunctival vessels were observed. Other imaging modalities showed unremarkable findings. Cornea verticillata and inferior bulbar conjunctival vascular abnormalities may be detected earlier than other ocular abnormalities in de novo FDs like hereditary FDs.

• BMB Reports
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• 제49권5호
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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 Chest Surgery
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• 제26권4호
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• pp.316-319
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• 1993

Although Behcet`s disease, characterized by oro-genital ulceration and ocular abnormalities, is rare and unfamiliar disease to thoracic surgeon, one must remember that it can cause serious complications on the vascular system such as thrombophlebitis,thrombotic obstuction and aneurysm.We report a case of multiple arterial aneurysms with Behcet`s disease. This 31 year-old female had five times surgical interventions during short period[19 months]. 6 aneurysms were developed in this case, 3 were spontaneous aneurysms, 2 were anastomotic aneurysms and 1 was false aneurysm due to arterial puncture.