• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.1
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• pp.15-18
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• 2010

Sturge-Weber syndrome is a rare nonhereditary developmental condition that is characterized by a hamartomatous vascular proliferation involving the tissue of brain and face. The clinical features are characterized by port wine nevus following one or more divisions of trigeminal nerve, ocular involvement and neurologic involvement such as epilepsy, mental retardation, and contralateral hemiplegia. Oral manifestations include unilateral blood vessel expansion of the oral mucosa, vascular hyperplasia of gingiva, pyogenic granuloma-like massive hemangiomatous proliferation of oral mucosa, macrodontia, ipsilateral macroglossia, blood vessel anomaly of maxilla or mandible and abnormal tooth eruption sequence. This case report is about 11-year-old Sturge-Weber syndrome patient presented port wine nevus on the face, venous malformation on soft plate and buccal mucosa. In this case we performed simple extraction of several deciduous teeth and periodic oral hygiene management. If a patient with Sturge-Weber syndrome has to undergo dental surgery in affected areas of the mouth, great care must be taken to prevent severe hemorrhage.

• Archives of Reconstructive Microsurgery
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• v.20 no.1
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• pp.60-63
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• 2011

Capillary malformation is common vascular malformation. In case of facial capillary malformation, patients' cosmetic and functional deficits are quite significant. The standard treatment which has been applied so far for capillary malformation is pulsed dye laser with 585nm. But in case of advanced capillary malformation, surgical interventions are inevitable. The problem of large size facial capillary malformation is how to cover the remnant defect, which occurs after resection. In this case, authors have experienced surgical treatment of large size facial capillary malformation and covered the large facial defect with free thoracodorsal artery perforator flap. The flap was thick, so facial asymmetry remained after the first surgery. But with the secondary procedure, authors have made more symmetric figures. The patient was satisfied with the result. Using free flap to replace the defect after resection due to capillary malformation is useful for these kinds of cases.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.174-182
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• 1979

This is a report on four cases of successful surgical correction of coarctation of the aorta [COA] in Department of the Thoracic & Cardiovascular Surgery, Hanyang University Hospital. The first case was a postductal type of coarctation of the aorta associated with Patent ductus arteriosus [PDA], Persistent left superior vena cava [LSVC] and richly developed collateral circulation. Blood pressure was measured to be hypertensive at the arm, but hypotensive at the legs. The coarctation of the aorta was corrected with following procedure: Partial resection of the aortic wall with diaphragmatic structure lust above and below the coarctating line of the aorta, and then the defect of the aortic wall was closed by lateral aortographic suture. PDA was closed by ligation procedure. The second case a preductal type of coarctation of the aorta associated with PDA, LSVC, ventricular septal defect [VSD] and poorly developed collateral circulation. Normal blood pressure was measured at the arm, but hypotension was observed at the legs. Correction of coarctation of the aorta was performed under the establishment of tube bypass because of poor collateral circulation. After resection of coarctating short segment, end to end anastomosis was performed without any tension. PDA was closed by division procedure. Simple suture closure of VSD was performed by open heart surgery two weeks after correction of COA. The third case was a long segment COA without any other anomaly. Blood pressure was measured to be hypertensive at the arm, but hypotensive at the legs. Vascular prosthesis was performed using Teflon graft tube after resecting coarctating long segment [6.5 cm] of the aorta. The fourth case was a long segment COA associated with aortic insufficiency and richly developed collateral circulation. Normal blood pressure was measured at the arm, but hypotension was observed at the legs. Vascular prosthesis was performed using Teflon graft tube after resecting coarctating long segment [6.0 cm] of the aorta. Both blood pressure and peripheral pulse on the arm and the legs returned to normal postoperatively in all patients.

• Journal of Genetic Medicine
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• v.18 no.2
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• pp.142-146
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• 2021

Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.

• Journal of Veterinary Science
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• v.24 no.4
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• pp.50.1-50.6
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• 2023

This paper reports the clinical findings and surgical treatment of feline right patent ductus arteriosus (RPDA) with a left aortic arch. A two-month-old female Maine Coon was referred for an investigation of regurgitation after weaning. RPDA with a left aortic arch was diagnosed based on the echocardiographic and computed tomography (CT) findings. A right-fourth intercostal thoracotomy was found to be an appropriate approach to the duct. Preoperative diagnosis is crucial and diagnostic imaging, including radiography, echocardiography, and cardiac CT examination, is essential for determining if the aortic arch is right or left.

• Journal of Chest Surgery
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• v.47 no.4
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• pp.389-393
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• 2014

Retroesophageal aortic arch, in which the aortic arch crosses the midline behind the esophagus to the contralateral side, is a rare form of vascular anomaly. The complete form may cause symptoms by compressing the esophagus or the trachea and need a surgical intervention. We report a rare case of a hypoplastic left heart syndrome variant with the left retroesophageal circumflex aortic arch in which the left aortic arch, retroesophageal circumflex aorta, and the right descending aorta with the aberrant right subclavian artery encircle the esophagus completely, thus causing central bronchial compression. Bilateral pulmonary artery banding and subsequent modified Norwood procedure with extensive mobilization and creation of the neo-aorta were performed. As a result of the successful translocation of the aorta, the airway compression was relieved. The patient underwent the second-stage operation and is doing well currently.

• 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.11 no.3
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• pp.342-347
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• 1978

A 8 year old male was admitted to the Department of Thoracic Surgery, Korea University Hospital on June 22, 1978. The chief complaints were cyanosis and exertional dyspnea since at birth. EKG shows BVH and dextrocardia, phonocardiogram revealed the accentuation of second heart sound in aortic area. Echocardiogram from the left ventricle to the base of the heart, there is a discontinuity between the ventricular septum and the anterior aortic margin with a large aortic root & aortic overriding. His cardiac catheterization data and cardiac angiogram shows situs inversus totalis, dextrocardia, right aortic arch, large ventricular septal defect etc., and finally diagnosed Truncus Arteriosus. Edwards type IV with retrograde aortogram and selective bronchial angiogram. This is the first operative case reported as Rastelli operation for Truncus Arteriosus type IV in the literatures in Korea. Authors have experienced I case of Truncus Arteriosus, Edward type IV and Rastelli operation with Dacron Arterial Conduit Graft under cardiopulmonary bypass on July 3, 1978. The procedures were as follows; 2] Cardiopulmonary bypass: Origin of bronchial arteries excised from descending aorta bilaterally; defects in aorta closed. 2] Horizontal incision made high in right ventricle. 2] Ventricular septal defect [Kirklin type I+II] closed with Teflon patch. 4] Bifurcated dacron arterial graft with pericardial monocusp sutured to the bilateral pulmonary arteries. [Diameter 9 mm: Length 7 cm]. 5] Proximal end of the conduit graft anastomosed to right ventricle. [Diameter 19 mm: Length 5 cm]..Total perfusion time was 220 min. The result of operation was poor due to anastomotic leakage and increased pulmonary vascular resistance resulting acute right heart failure. The patient was died on the operation table. Literatures were briefly reviewed.

• Journal of Korean Neurosurgical Society
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• v.46 no.6
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• pp.568-571
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• 2009

Accessory middle cerebral artery (MCA) is an infrequent vascular anomaly of the brain. Cerebral aneurysms associated with this anomalous artery are also very rare. To our knowledge, there have only been ten previous reports of an aneurysm associated with accessory MCA. The authors present two patients with accessory MCA-related aneurysms. A 38-year-old male and a 59-year-old female both presented with sudden-onset severe headache. In both patients, computed tomography (CT) scan revealed subarachnoid hemorrhage. A subsequent angiogram demonstrated an accessory MCA arising from the anterior cerebral artery (ACA) and a saccular aneurysm at the anterior communicating artery (ACoA) complex associated with an accessory MCA. Surgical clipping allowed for complete exclusion of the aneurysm from the arterial circulation. Based on our review of the ten cases of aneurysms associated with accessory MCA documented in the literature, we suggest that accessory MCA-related aneurysms can be classified according to whether the accessory MCA originates from the proximal A1 segment or from the ACoA complex. We also emphasize the importance of precise interpretation of preoperative angiograms and intraoperative precaution in determining the presence of this anomalous artery prior to temporary clip placement.

• Journal of Korean Neurosurgical Society
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• v.61 no.1
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• pp.28-34
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• 2018

Objective : Duplication of the vertebral artery (VA) is a rare vascular variant. This paper describes the anatomy and embryological development of duplicated VAs and reviews the clinical significance. Methods : Computed tomography (CT) angiography was performed in 3386 patients (1880 females, 1506 males) between March 2014 and November 2015. We defined duplication of the VA as a condition in which the VA has two origins that fused at different levels of the neck. Results : Ten of the 3386 patients (0.295%) who received CT angiography had a dual origin of the VA; three on the left side, and seven on the right side. In all seven with right dual origin of the VA, both limbs of the VA origin originated from the right subclavian artery. In all three patients with left dual origin of the VA, both limbs of the VA originated from the left subclavian artery and aortic arch. In all 10 patients, the medial limb of the duplicated VA was located posteriorly and medially to the common carotid artery (CCA) and anteriorly and laterally to the vertebral transverse foramen. In two patients, the medial limb of the duplicated VA was located in close proximity to the CCA. In another two patients, the medial limb of the duplicated VA was located in close proximity to the CCA, carotid bifurcation, and proximal internal carotid artery. Conclusion : Although duplication of the VA is asymptomatic in most patients, clinicians should consider this anomaly during diagnosis and treatment.