• Journal of Chest Surgery
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• v.48 no.1
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• pp.78-81
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• 2015

This study reports our early experience with thoracoscopic division of vascular rings. Three patients were reviewed; their ages at surgery were 25 months, 4 years, and 57 years. All patients were suffering from complete vascular rings involving combinations of the right aortic arch, left ligamentum arteriosum, Kommerell's diverticulum, and retroesophageal left subclavian artery. The median surgical time was 180.5 minutes, and the patients showed immediate recovery. Three complications, namely chylothorax, transient supraventricular tachycardia, and left vocal cord palsy, were observed. Our early experience indicates that thoracoscopic division of a vascular ring may provide early recovery and could be a promising operative choice.

• Journal of Korean Neurosurgical Society
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• v.55 no.3
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• pp.156-159
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• 2014

Coarctation and occlusion of the aorta is a rare condition that typically presents with hypertension or cardiac failure. However, neuropathy or myelopathy may be the presenting features of the condition when an intraspinal subarachnoid hemorrhage has compressed the spinal cord causing ischemia. We report two cases of middle-aged males who developed acute non-traumatic paraplegia. Undiagnosed congenital abnormalities, such as aortic coarctation and occlusion, should be considered for patients presenting with nontraumatic paraplegia in the absence of other identifiable causes. Our cases suggest that spinal cord ischemia resulting from acute spinal subarachnoid hemorrhage and can cause paraplegia, and that clinicians must carefully examine patients presenting with nontraumatic paraplegia because misdiagnosis can delay initiation of the appropriate treatment.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.86-90
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• 2003

Alagille syndrome is a autosomal dominant disorder characterized by intrahepatic bile duct paucity and resultant chronic cholestasis in combination with cardiac(mainly peripheral pulmonary stenosis), skeletal, ocular, and facial abnormalities. In addition to the pulmonary stenosis, in large series, anecdotal reports of vascular lesions have concerned the renal artery, aorta, hepatic artery, carotid artery, celiac artery or subclavian artery. Theses diffuse vascular abnormalities, which appear to be a feature of Alagille syndrome, suggest Notch signaling pathway defects affect angiogenesis. The associations of Alagille syndrome with moyamoya disease, the chronic cerebrovascular occlusive disease, were reported and suggested as additional evidence of vasculopathy of Alagille syndrome. We report another 25 month-old Alagille syndrome girl who presented with acute left hemiparesis and was diagnosed with moyamoya disease through the cerebral angiographic study.

• Journal of Chest Surgery
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• v.17 no.3
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• pp.362-370
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• 1984

The term corrected transposition of great arteries [hereafter referred to as corrected TGA] of the heart in which there is both a discordant atrio-ventricular relationship and transposition of the great vessels. Usually situs solitus is present, while the ventricles are inverted showing an l -loop. The great vessels are transposed and in the l-position so that the pulmonary artery arises from the right-sided morphological left ventricle and the anteriorly l- transposed aorta arises from the left-sided morphological right ventricle yielding an SLL pattern. In the majority of cases, associated lesions are common. The most frequent are ventricular septal defect, obstruction to the pulmonary outflow tract, tricuspid valve incompetence and atrio-ventricular conduction abnormalities. In the rare cases, no associated conditions are present and hemodynamic pathways are normal. In the report, we present one case of a 20 year-old male having corrected TGA associated with severe tricuspid valve incompetence, was corrected by tricuspid valve replacement, directly developed a supra-ventricular tachycardia but was controlled by calcium-entry blocker, verapamil, successfully.

• Journal of Korean Neurosurgical Society
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• v.50 no.3
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• pp.256-259
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• 2011

We report a rare case of Williams syndrome accompanying moyamoya disease in whom postoperative global cerebral infarction occurred unpredictably. Williams syndrome is an uncommon hereditary disorder associated with the connective tissue abnormalities and cardiovascular disease. To our knowledge, our case report is the second case of Williams syndrome accompanying moyamoya disease. A 9-year-old boy was presented with right hemiparesis after second operation for coarctation of aorta. He was diagnosed as having Williams syndrome at the age of 1 year. Brain MRI showed left cerebral cortical infarction, and angiography showed severe stenosis of bilateral internal carotid arteries and moyamoya vessels. To reduce the risk of furthermore cerebral infarction, we performed indirect anastomosis successfully. Postoperatively, the patient recovered well, but at postoperative third day, without any unusual predictive abnormal findings the patient's pupils were suddenly dilated. Brain CT showed the global cerebral infarction. Despite of vigorous treatment, the patient was not recovered and fell in brain death one week later. We suggest that in this kind of labile patient with Williams syndrome accompanying moyamoya disease, postoperative sedation should be done with more thorough strict patient monitoring than usual moyamoya patients. Also, we should decide the revascularization surgery more cautiously than usual moyamoya disease. The possibility of unpredictable postoperative ischemic complication should be kept in mind.

• Investigative Magnetic Resonance Imaging
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• v.23 no.3
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• pp.276-278
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• 2019

The cisterna chyli, a dilated lymphatic sac in the retrocrural space, is usually located to the right of the aorta. We report a case of a left-sided cisterna chyli, which was incidentally detected on the radiologic examinations of a preoperative workup for cholangiocarcinoma. Computed tomography (CT) and magnetic resonance (MR) images revealed a cisterna chyli measuring 2.5 cm in length in the left retrocrural space. The dilated lumbar lymphatics joined with the cisterna chyli, which was continuous with the left-sided thoracic duct. To the best of our knowledge, this is the second antemortem case of a left-sided cisterna chyli in literature. The cisterna chyli can mimic retrocrural lymphadenopathy, solid tumor with cystic degeneration, abscess or hematoma. The left-sided cisterna chyli should be referred to as a structure so as to be cautious in surgical approach.

• Journal of Chest Surgery
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• v.42 no.5
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• pp.639-644
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• 2009

Characterized by unique phenotypic features such as aortic aneurysm/dissection, hypertelorism, bifid uvula/cleft palate and generalized tortuosity in the arterial system, Loeys-Dietz syndrome is a newly described aggressive connective tissue disorder associated with mutation in the gene encoding transforming growth factor-$\beta$ receptor type I or type II. Some phenotypic manifestations of Loeys-Dietz syndrome overlap with those of Marfan syndrome or Ehlers-Danlos syndrome type IV. However, due to its more malignant pathophysiologic nature, physicians should be alert to Loeys-Dietz syndrome. High suspicion, early diagnosis, preventive surgery and serial imaging assessments are warranted for optimal management of Loeys-Dietz syndrome. We present here a case of a young patient with Loeys-Dietz syndrome who had aortic rupture, bifid uvula and hypertelorism. We also present a review of the medical literature.

• Journal of Chest Surgery
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• v.16 no.2
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• pp.170-174
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• 1983

Aortic arch syndrome is an unusual disease entity characterized by the narrowing or obliteration of major branches of the arch of the aorta regardless of etiology. We have experienced 2 cases. One of them was 22 years old office girl with 3 months history of headache, intermittent syncope and weakness and claudication on left arm especially during her physical exercise. On physical examination, pulseless on left antecubital and radial artery and blood pressure on left arm was inable to check and coldness with weakness were noted on the same side. Aortic angiography reealed 34% narrowing of left subclavian artery as that of right. But both common carotid artery and both axillary arterial patency were relatively good. Through right supraclavicular and left axillary incision, bypass graft with Gore-tex prosthesis (I.D. 6mm, Length 25 cm) was implanted from right subclavian artery on 2cm distal to origin of right common carotid arery to left axillary artery distal to axillary fossa. End to side anastomosis with preservation of left subclavian artery was done. Postoperative state was stable with blood pressure of 110/70 mmHg on left arm and palpable antecubital and radial pulsation. Another one was 41 year old male patient with 8 months history of pain and numbness on right upper arm and shoulder. On admission, right arm blood pressure was 110/80 mmHg, left arm was 160/110 mmHg, but other physical findings had no abnormalities. Angiography revealed segmental narrowing of right axillary artery on the beginning with 2 cm in length. Operative treatment with right wupraclavicular and right axillary incision, bypass graft with great saphenous vein (Length; 15 cm) from right subclavian artery between scalenus anticus and medius to axillary artery at distal end of axillary fossa was done. The authors report two cases of Aortic arch syndrome treated with bypass graft using Autograft or Gore-tex with good result.

• Tuberculosis and Respiratory Diseases
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• v.39 no.6
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• pp.548-553
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• 1992

The Unilateral absence of a pulmonary artery (UAPA) is an uncommon congenital anomaly. Approximately 160 cases have been reported in the literature since Frantzel's first report in 1968. Most of the patients with UAPA are asymptomatic but some patients may suffer from recurrent respiratory infections, hemoptysis, or pulmonary hypertension. The diagnosis could be suspected from the chest roentgenogram and lung scan, and definitely confirmed by pulmonary angiography. We experienced a case of UAPA in a 39-year-old male with the recurrent hemoptysis. Chest X-ray revealed that the left lung volume was moderately decreased and the heart and mediastinum were displaced to the left side. Lung perfusion scan showed that the left lung was not perfused. Pulmonary angiography revealed the absence of the left main pulmonary artery. Aortic arch and descending aorta on aortogram were right sided. Blood supply to the left lung was originated from numerous systemic collaterals from intercostal and brachiocephalic origin. No other intrinsic or internal abnormalities of the cardiac chambers were noted.

• 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.