• Journal of the Korean Society of Radiology
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• v.81 no.6
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• pp.1523-1528
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• 2020

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiac anomaly that can be difficult to detect and often remains undiagnosed. PAPVR is diagnosed using non-invasive imaging techniques such as echocardiography, CT, and MRI. Image data are reviewed on a 2-dimensional (D) monitor, which may not facilitate a good understanding of the complex 3D heart structure. In recent years, 3D printing technology, which allows the creation of physical cardiac models using source image datasets obtained from cardiac CT or MRI, has been increasingly used in the medical field. We report a case involving a 3D-printed model of PAPVR with a biatrial connection. This model demonstrated separate drainages of the right upper and middle pulmonary veins into the lower superior vena cava (SVC) and the junction between the SVC and the right atrium, respectively, with biatrial communication through the right middle pulmonary vein.

• Archives of Reconstructive Microsurgery
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• v.21 no.1
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• pp.8-13
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• 2012

Purpose: Purpose: The free deep inferior epigastric artery perforator (DIEP) flap is a popular option for autologous breast reconstruction. However, the anatomy of the deep inferior epigastric artery(DIEA) may vary from one individual to another. Unexpected vascular anomaly can confuse the surgeon and affects on the safety of the free DIEP flap. Materials and Methods: Thirty five consecutive patients who underwent free DIEP/TRAM flap for immediate breast reconstruction between Mar. 2010 and Oct. 2010 were enrolled in this study. Computed tomography angiography (CT angiography) of abdomen was evaluated part of our standard preoperative assessment: atypical patterns of DIEA/DIEP were evaluated by preoperative CT angiography and compared with intraoperative finding. Results: Atypical patterns of DIEA/DIEP which may affect preoperative planning were noted as the following: Circummusclar/subfascial DIEA (n=1), DIEA running underneath rectus muscle (n=8), septocutaneous perforator (n=3), peritoneo-cutaneous perforator (n=1), a large branch going into peritoneum (n=1), and very early division and muscle penetration of DIEA (n=1). Conclusion: Atypical DIEA/DIEP that might change the operation plan is not rare, so the individualized planning based on the preoperative CT angiography is recommended. Preoperative CT angiography could help to select reliable and easy-to-dissect perforator in free DIEP/TRAM breast reconstruction.

• Journal of Korean Neurosurgical Society
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• v.30 no.1
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• pp.5-11
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• 2001

Objectives and Importance : The present study investigated the levels of basic fibroblast growth factor(bFGF) in the CSF of patients with moyamoya disease and its clinical significance. Methods : The levels of bFGF in CSF, taken from 26 hemispheres of 14 moyamoya patients and 20 patients without vascular anomaly(control group), were measured by an enzyme-linked immunosorbent assay. We analyzed the correlation between the level of bFGF and the clinical factors such as age, onset pattern, development of neovascularization, and cerebral circulation. Results : The CSF of moyamoya patients contained a high concentration of bFGF to a significant extent. The bFGF level was apparently elevated in the patients in whom neovascularization from indirect revascularization, such as modified encephalo-duro-arterio-synangiosis(EDAS) was well developed. A linear correlation between the values of bFGF and clinical progression was noted. Conclusions : The elevation of bFGF in moyamoya disease seems to be specific. Clinically, the bFGF level may be considered a useful indicator to predict the efficacy of indirect revascularization.

• Journal of Korean Neurosurgical Society
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• v.61 no.4
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• pp.485-493
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• 2018

Objective : Cerebral varices (CVs) without an arteriovenous shunt, so called nonfistulous CVs, are very rare, and their etiology and natural course are not well understood. The aim of this study is to evaluate the clinical outcomes of nonfistulous CVs by the analysis of 39 cases. Methods : From 2000 to 2015, 22 patients with 39 nonfistulous CVs (${\geq}5mm$) were found by searching the medical and radiologic records of our institute. Clinical data and radiological data including numbers, sizes and locations of CVs and associated anomalies were retrospectively collected and analyzed. Previously reported cases in literature were reviewed as well. Results : The mean age of the patients was 21 years (range, 0-78 years). On average, $1.8{\pm}1.2CVs$ were found per patient. CVs were categorized as either fusiform or saccular depending on their shapes. Two patients had saccular type CVs, seventeen patients had fusiform types, and three patients had both fusiform and saccular CVs. Eight patients had associated compromise of the vein of Galen and the straight sinus. Four of those patients had sinus pericranii, as well. Five patients had CVs that were distal draining veins of large developmental venous anomalies. One patient had associated migration anomaly, and two patients had Sturge-Weber syndrome. Six patients with an isolated cerebral varix were observed. Of the 39 CVs in 22 patients, 20 lesions in 14 patients were followed up in outpatient clinics with imaging studies. The average follow-up duration was 6.6 years. During this period, no neurological events occurred, and all the lesions were managed conservatively. Conclusion : Nonfistulous CVs seemed to be asymptomatic in most cases and remained clinically silent. Hence, we suggest conservative management.

• Journal of Chest Surgery
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• v.24 no.3
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• pp.310-321
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• 1991

Between April 1986 and September 1990, 34 patients with a single or dominant right ventricle underwent modified Fontan procedure for definite palliation in Seoul National University Children`s Hospital. Their age at operation ranged from 8 months to 14 years [Mean 5.5 years]. The ventricular chamber was solitary and of indeterminate trabecular pattern in 6 patients. 28 patients had posteriorly located rudimentary chamber, all of which were trabecular pouches having no communication with outlet septum. The patterns of atrioventricular connection were common inlet[9], double inlet [11], left atrioventricular valve atresia [12] and right atrioventricular valve atresia with L-loop [2]. Pulmonary outflow tracts were atretic in 7 patients and stenotic in 26 patients. Major associated anomalies included anomalous systemic venous drainage [15], dextrocardia [12] and total anomalous pulmonary venous connection[3]. Shunt operations were previously performed in 13 patients and pulmonary artery banding and atrial septectomy in 1 patients. Surgery included intraatrial baffling in 26 patients, bidirectional cavopulmonary shunt in 13 patients, atrioventricular valve obliteration in 3 patients and atrioventricular valve replacement in 3 patients. Central venous pressure measured postoperatively at intensive care unit ranged from 18cm H2O to 28cm H2O [mean 23.2cm H2O]. Hospital mortality was 35.3% [12/34], all died out of low output syndrome. Suspected causes of low output syndrome include ventricular dysfunction [8], hypoplastic or tortuous pulmonary artery [2] and elevated pulmonary vascular resistance [2]. 19 patients had 31 major complications including low output syndrome [18], arrhythmia [4], acute renal failure [3] and respiratory failure [3]. Mortality rate was significantly higher in the groups receiving intraatrial baffling and AV valve replacement respectively [p<0.05]. 20 patients were followed up postoperatively with the mean follow-up period 15.0$\pm$11.6 months. There were no late death and follow-up catheterization was performed in 10 patients. Mean right atrial pressure was 15.4$\pm$6.8mmHg and ventricular contraction was reasonable in all but one case. Thus, Fontan principle can be applied successfully to all the patients with complex cardiac anomaly of single ventricle variety and better results can be anticipated with judicious selection of patient and improvement of postoperative care.

• Neonatal Medicine
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• v.15 no.2
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• pp.176-182
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• 2008

A congenital portosystemic shunt is a very rare portosystemic vascular anomaly which leads to jaundice, hypoglycemia, hyperammonemia, liver cirrhosis, hepatic coma, and pulmonary hypertension. Anatomically, portosystemic shunts are divided into intra- and extrahepatic shunts. Congenital intrahepatic portosystemic shunts are rare anomalies, and the early diagnosis is important to prevent hepatic encephalopathy and hypoglycemia. We report a case of an infant with symptoms of heart failure due to a congenital intrahepatic portosystemic shunt and a ventricular septal defect (VSD), which were treated successfully with four coil embolizations and open heart surgery for the VSD.

• Investigative Magnetic Resonance Imaging
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• v.17 no.1
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• pp.55-58
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• 2013

Persistent proatlantal artery (PPA) is a rare embryologically remnant carotico-vertebrobasilar anastomoses. There are two types of PPA according to embryological considerations, origin and anatomic course. Type I PPA usually originate from internal carotid artery and not traversing transverse foramen. Type II PPA traverses from external carotid artery to C1 transverse foramen. The PPA is usually found incidentally without clinical symptoms, but can be related to several clinically significant vascular lesions, such as hypoplastic vertebral artery, intracranial arteriovenous malformation and in a case of carotid endarterectomy or external carotid artery embolization. So, thorough understanding of this anomaly is needed and we report a case of type II PPA diagnosed by MR angiography.

• Journal of Yeungnam Medical Science
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• v.32 no.2
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• pp.118-121
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• 2015

Cardiogenic embolic stroke accounts for approximately 20% of ischemic strokes and the likelihood of its recurrence is high. Paradoxical embolism may be an important cause of cardioembolic stroke, which can be evaluated through multiple diagnostic modalities including transesophageal echocardiography (TTE) or transcranial Doppler. A persistent left superior vena cava (PLSVC) is a rare congenital vascular anomaly, which mainly drains to the right atrium via the coronary sinus. Although rare, PLSVC draining into the left heart predisposes the patient to paradoxical embolism through a right-to-left shunt. We report on a 78-year-old female patient with an ischemic stroke associated with PLSVC draining into the left atrium through the pulmonary vein, which was investigated via TTE with an agitated saline test and computed tomography.

• Journal of Chest Surgery
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• v.39 no.2 s.259
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• pp.171-175
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• 2006

Minimally invasive thoracic surgery has been one of the most important surgical advances recently. Congenital cystic adenomatoid malformation of the lung is a relatively rare anomaly and is clearly associated with various congenital anomalies such as pectus excavatum, cardiac and pulmonary vascular lesions. We have experienced a case that was treated with minimal invasive methods for congenital cystic adenomatoid malformation involving in the right lower lobe and pectus excavatum in a 5-year-old boy. We simultaneously performed thoracoscopic right lower lobectomy and Nuss procedure of pectus excavatum using a substernal steel bar. Therefore, a minimally invasive surgical treatment for this diseases is feasible and cosmetically excellent.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.1
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• pp.80-84
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• 1999

Dieulafoy's disease, a vascular anomaly mainly in the upper stomach, is a rare but potentially life-threatening cause of upper gastrointestinal bleeding. Pathogenesis is still controversial, but the most accepted theory is that a persistent caliber vessel in the submucosa is exposed by a small mucosal erosion leading to massive bleeding. The bleeding site is usually within 6 cm of the esophagogastric junction in the cardia or fundus of the stomach. The treatment of choice is therapeutic endoscopy or surgery. The age of patients reported is mainly between 50 and 70 years, and patients of pediatric age are extremely rare. We are reporting a 5-year-old male patient who had Dieulafoy's disease which was diagnosed by emergency upper gastrointestinal endoscopy. Endoscopic finding was a nodular lesion with an adherent clot on the lessor curvature of the stomach 2 cm below the esophagogastric junction. Epinephrine and $Beriplast^{(R)}$ was injected in the lesion. On the second day after endoscopic sclerotherapy, the patient had recurred massive hematemesis and accompanying shock. So we performed gastrotomy and ligation. After the operation, he showed an improved general condition and was discharged at the 12th hospital day.