• Journal of Veterinary Clinics
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• v.26 no.2
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• pp.123-129
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• 2009

Patent ductus arteriosus(PDA) is an abnormal shunt between the descending aorta and pulmonary artery through the incompletely closed ductus arteriosus and is the most common congenital heart defect in dogs. Recent human genetic studies found that a the gene mutation in transcription factor AP-2 beta(TFAP2B) was responsible for syndromic cases of PDA. Mutations in the TFAP2B gene are associated with certain congenital cardiac defects in humans that include PDA. In this study, we isolated the entire coding exons of canine TFAP2B gene for genetic screening in dogs with PDA. Analysis of the deduced amino acid sequence suggested that the canine TFAP2B are phylogenetically closer to the human TFAP2B(100% identity in amino acid sequence) than mouse and rat. In cTFAP2B gene screening, one single c.936+203G>A base change was found in affected Maltese dogs with PDA. However, further screening found the same base change in one unaffected control dog, suggesting this base change might be polymorphism. No other base changes were found in other dog breeds enrolled in this study. Because the base change was located in the intronic region and found in an unaffected control dog, TFAP2B might not be responsible for familial PDA in Malteses and sporadic cases of other dog breeds, although the gene promoter region should be investigated before reaching to this conclusion. A future study that may take this study further would be to collect more samples and to screen TFAP2B in various breeds of dogs with PDA and other various congenital heart defects.

• Korean Journal of Cleft Lip And Palate
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• v.4 no.1
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• pp.45-53
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• 2001

Congenital craniofacial disorders represent approximately 20% of all birth defects, One of these disorders is syngnathia, Congenital fusion of the maxilla and mandible is rare and can present in a wide range of severity from single mucosal band(synechiae) to complete bony fusion(syngnathia), Syngnathia, congenital bony fusion of the mandible and maxilla, is even less common than synechiae, with only 25 cases reported in the literature, Most of them have presented as an incomplete, unilateral fusion, We report a case of unilateral bony fusion of the maxilla, mandible, and zygomatic arch, Details of operative management and follow-up data are presented with review of literature.

• Archives of Craniofacial Surgery
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• v.9 no.2
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• pp.90-92
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• 2008

Purpose: Congenital upper eyelid defect is rare anomaly whether it is compared with syndromic anomaly or not. It has many clinical manifestation in the extent, location. Many operation procedures such as simple closure, semicircular rotation flap, Cutler Beard procedure, lower lid rotation flap, etc can be used to reconstruct eyelid defects. We intend to introduce a simple, congenital eyelid defect which was not compared with syndromical anomaly, ophthalmic complication. Methods: Our experience is a case of 19 years old female who had a upper eyelid coloboma without any other anomalies. we could not find any skeletal deformity in orbital CT scan. she had no ophthalmic problem. we reconstructed the defect with bilateral marginal flap after deepithelization of supramarginal area and tarsal reposition. Results: There were no visible deformity of lid lining. postoperative scar was favorable. satisfactory results were obtained in cosmetic and functional aspects. Conclusion: Upper lid coloboma without other anomalies is rare. we obtained satisfactory outcome as treated this rare case with marginal flap advancement.

• Journal of Korean Academy of Nursing
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• v.44 no.2
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• pp.228-236
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• 2014

Purpose: Common conditions, such as dehydration or respiratory infection can aggravate hypoxia and are associated with interstage mortality in infants who have undergone palliative surgery for congenital heart diseases. This study was done to evaluate the efficacy of a home monitoring program (HMP) in decreasing infant mortality. Methods: Since its inception in May 2010, all infants who have undergone palliative surgery have been enrolled in HMP. This study was a prospective observational study and infant outcomes during HMP were compared with those of previous comparison groups. Parents were trained to measure oxygen saturation, body weight and feeding volume and to contact the hospital through the hotline for emergency situations. Telephone counseling was conducted by clinical nurse specialists every week post discharge. Results: Forty-one infants were enrolled in HMP. Nine hundred telephone counseling sessions were conducted. Seventy-three infants required telephone triage with the most common conditions being gastrointestinal (50.7%) and respiratory symptoms (32.9%). With HMP intervention, interstage mortality decreased from 18.6% (8/43) to 9.8% (4/41) (${\chi}^2$=1.15, p=.283). Conclusion: Results indicate that active measures and treatments using the HMP decrease mortality rates, however further investigation is required to identify various factors that contribute to hemodynamic complications during the interstage period.

• Archives of Plastic Surgery
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• v.37 no.6
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• pp.763-768
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• 2010

Purpose: Reconstruction of soft tissue defect using tissue expander can provide better flap which is more similar to surrounding tissue in color, skin texture and hair compared to other methods. Many pediatric patients need reconstruction of soft tissue defect because of giant congenital nevi, congenital or acquired malformations and burn scars. Reconstruction using tissue expander is adequate to minimize dysmorphism in these patients. We intended to assess outcomes of using tissue expander in pediatric patients by retrospective study. Methods: Total cases were 168 of pediatric patients who received soft tissue reconstruction using tissue expander by the same surgeon from February, 1982 to May, 2009. All patients who received soft tissue reconstruction were under 10 years old. Mean age was 4.3 years old, the youngest 13 months, the oldest 8 years. Eightynine cases were male and 79 cases were female. Most common cause was giant hairy nevi (67 cases, 39.9%), secondary cause was burn scar/scar contracture (61 cases, 36.3%). Trunk (38 cases, 22.6%) was most common anatomical location. Results: Soft tissue defects were successfully covered using tissue expander in 149 cases (88.7%) without major complications. There was infection on 8 cases (4.7%) and we treated by adequate antibiotics in these cases. There were tissue expander folding or valve displacement on 5 cases (3%). Conclusion: Usage of tissue expander is useful on pediatric patients because tissue expansion is rapid on children and there are less secondary contractures on operation site than full thickness skin graft. Because of psychological stress due to tissue expander, operation should be performed before school age.

• Journal of the Korean Society of Radiology
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• v.82 no.3
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• pp.749-755
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• 2021

Congenital defects of the pericardium, which are generally asymptomatic, are rare disorders characterized by complete or partial absence of the pericardium. Here, we report a rare case of a 19-year-old male who was incidentally diagnosed with congenital absence of the left pericardium during examination for symptoms of pneumothorax. Chest radiography and CT revealed a collapsed left lung without any evidence of trauma, no unusual findings of free air spaces along the right side of the ascending aorta, heart shifted toward the left side of the thorax, and a shallow chest. Subsequent thoracoscopy confirmed the absence of the left pericardium and displacement of the heart toward the left thoracic cavity. We further discuss the correlation between radiologic images and surgical findings of a congenital pericardial defect associated with spontaneous pneumothorax.

• Clinical and Experimental Pediatrics
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• v.56 no.3
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• pp.125-129
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• 2013

Purpose: The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. Methods: This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. Results: Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was $14.5{\pm}5.4$ years (range, 2 to 22 years). The follow-up duration was $28.9{\pm}20.4$ months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1), cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years). Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia. During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery. Conclusion: The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.

• Journal of Chest Surgery
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• v.29 no.5
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• pp.564-568
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• 1996

Pseudoaneurysm of the ascending aorta following cardiac surgery is very unusual and it is poten- tially fatal. We report here a fourteen year-old female patient with pseudoaneurysm of the ascending aorta following a repair of a congenital ventricular septal defect at other hospital 50 months ago. Although she had a mild superficial wound infection postoperatively, she enjoyed uneventful. life until she visited our hospital for a generalized weakness and exertional dyspnea which developed a month ago. Chest CT and echocardiogram showed partially calcified pseudoaneurysm of the ascending aorta. Two aortic defects were located on the anterolateral ascending aortic wall wkere it was suspected as a previous sites of aortic and cardioplegic cannulation. The internal wall of the pseudoaneurysm was covered with neoendothelium and intervened by septal tissue. Two defects on he aortic wall were oval in shape and about 1.5cm in the greatest diameter The defects were trimmed to make a one large de- fect and it was reconstructed with patch designed from 22mm collagen impregnated double velour Dacron graft. The postoperative course was uneventful.

• Journal of Chest Surgery
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• v.16 no.1
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• pp.75-82
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• 1983

This report is concerned to our experience of 10 cases of open heart surgery under the extracorporeal circulation at the Department of Thoracic and Cardiovascular Surgery, Capital Armed Forces General Hospital during the period between May, 1982 and February, 1983. 1. Six cases were male and two cases were female. Age was varied from 21 years to 50 years and mean age was 34 years. 2. The cases included 2 Ventricular Septal Defects, 1 Atrial Septal Defect, I Tetralogy of Fallot and 6 acquired valvular heart diseases. 3. The surgical managements were 3 primary repairs for Ventricular Septal Defects and Atrial Septal Defect, I total correction for Tetralogy of Fallot and 6 mitral valve replacements with bovine xenograft by Ionescu-Shiley combining 3 Tricuspid annuloplasties [ De Vega method ] and 1 deauricularization of left atrial appendage for acquired valvular heart diseases. 4. The average cardiopulmonary bypass time was 37 minutes for acyanotic congenital heart diseases and 92 minutes for cyanotic heart disease and acquired valvular heart diseases. And the average aortic cross clamping time was 19 minutes for the former and 70 minutes for the latter. 5. Postoperatively, there were 1 hemolytic anemia, 1 congestive heart failure, 1 hemolytic jaundice and 1 thermal burn as complications, but there was no operative mortality. 6. All patients received valve replacement were recommended anticoagulation with Persantin and Aspirin.

• Journal of Korean Neurosurgical Society
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• v.58 no.6
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• pp.528-533
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• 2015

Objective : We sought to examine anatomic variations of the atlas and the clinical significance of these variations. Methods : We retrospectively reviewed 1029 cervical 3-dimensional (3D) CT images. Cervical 3D CT was performed between November 2011 and August 2014. Arcuate foramina were classified as partial or complete and left and/or right. Occipitalization of the atlas was classified in accordance with criteria specified by Mudaliar et al. Posterior arch defects of the atlas were classified in accordance with criteria specified by Currarino et al. Results : One hundred and eight vertebrae (108/1029, 10.5%) showed an arcuate foramen. Bilateral arcuate foramina were present in 41 of these vertebrae and the remaining 67 arcuate foramina were unilateral (right 31, left 36). Right-side arcuate foramina were partial on 18 sides and complete on 54 sides. Left-side arcuate foramina were partial on 24 sides and complete on 53 sides. One case of atlas assimilation was found. Twelve patients (12/1029, 1.17%) had a defect of the atlantal posterior arch. Nine of these patients (9/1029, 0.87%) had a type A posterior arch defect. We also identified one type B, one type D, and one type E defect. Conclusion : Preoperative diagnosis of occipitalization of the atlas and arcuate foramina using 3D CT is of paramount importance in avoiding neurovascular injury during surgery. It is important to be aware of posterior arch defects of the atlas because they may be misdiagnosed as a fracture.