• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1363-1366
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• 2006

Aneurysmal dilatation of the ductus arteriosis has been considered a rare but potentially fatal abnormality. The mechanism of ductal aneurysmal formation remains uncertain. Plain chest radiography has proven helpful in the diagnosis of ductus arteriosus aneurysm (DAA), before the application of transthoracic echocardiography. The transthoracic echocardiography is an important tool for the diagnosis and follow-up of DAA. We present a case of congenital ductus arteriosus aneurysm in a newborn, that was an incidental discovery. The diagnosis was made by echocardiography, three-dimensional surface rendering computed tomography (CT), and spontaneous regression after four weeks of follow-up.

• Journal of Chest Surgery
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• v.40 no.4 s.273
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• pp.280-287
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• 2007

Background: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. Material and Method: Between Feb. f 984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male, Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90%, (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was peformed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with hi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs): 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. Result: The postoperative median NYHA functional class $(3{\rightarrow}1)$ and the mean cardio-thoracic ratio $(0.65{\rightarrow}0.59)$ were decreased significantly (p<0.001, p=0.014). The mean oxygen saturation $(86.6{\rightarrow}94.1%)$, and median TR grade $(4{\rightarrow}1)$ were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.80 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%, the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. Conclusion: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.

• Journal of the korean academy of Pediatric Dentistry
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• v.41 no.1
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• pp.72-79
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• 2014

Cleft lip and palate(CLP) is one of the most common craniofacial deformities that requires systemic management involving a multidisciplinary team approach. Although there has been great improvement in the field of cleft surgery, surgical approach alone cannot resolve the various problems in treating cleft lip and palate. Hence the need for presurgical treatment was appreciated and especially, the concept of presurgical nasoalveolar molding was applied to treat unilateral and bilateral cleft lip and palate patients. Presurgical nasoalveolar molding(PNAM) of unilateral cleft mainly aims to recover nasal symmetry while the objectives of pre-surgical nasoalveolar molding in the bilateral cleft are to elongate the columella, to erect the tip of nose, and to reposition the forward displaced premaxillary region. This report covers the case of fraternal twins diagnosed with bilateral cleft. Retraction of the premaxillary region and nasoalveolar molding were conducted for 70 days until cheiloplasty, using elastic bands and nasoalveolar molding appliances. After cheiloplasty, there had been improvements in the length of columella and the position of forward-displaced premaxilla for both patients compared to their initial states. The esthetics was also satisfactory for both the surgery and the parents. In order to maximize the efficacy of the appliance, three components should be in balance; patients' adaptation to the appliance, parents' cooperation and proper selection and careful adjustment of the appliance by the dentist.

• The Journal of Korean Physical Therapy
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• v.12 no.2
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• pp.255-265
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• 2000

The term 'myositis ossificans' encompasses four categories of clinicopathological disorders. The first, myositis ossificans progressive(fibrodysplasia ossificans progressive), is a rare genetic disease characterized by progressive heterotopic ossification involving skeletal muscle, tendon, ligaments, and fascia, with congenital malformation of the great toes, and usually microdactyly, monophalangism, and mal formed proximal phalanges. with valgus deformity of metatarsophalangeal joint. The ossification begins shortly after birth and may contribute to the patient's death. The second, heterotopic ossificans, can occur in patients with neuromuscular and chronic diseases such as paraplegia, poliomyelitis, polymyositis, bum, tetanus, and infection. But the lesions in these cases often lack the typical histologic features of myositis ossificans. The third, myositis ossificans traumatica, is the most common; it develops in response to soft tissue trauma such as a single severe injury, minor repetitive injures, fracture, joint dislocation, stab wound, or surgical incision. The forth, nontraumatic myositis ossificans, also designated :pseudomalignant osseous tumors of extraskeletal soft tissues' and 'psedomalignant myositis ossificans', occurs in persons repeated small mechanical injures or nonmechanical soft tissue injuries due to local ischemia, inflammation. or other factors cannot be ruled out in such cases.

• Tuberculosis and Respiratory Diseases
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• v.65 no.2
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• pp.110-115
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• 2008

Background: Congenital cystic adenomatoid malformation of the lung (CCAM) is a rare congenital developmental anomaly of the lower respiratory tract. Most cases are diagnosed within the first 2 years of life, so adult presentation of CCAM is rare. We describe here six adult cases of CCAM and the patients underwent surgical resection, and all these patients were seen during a five and a half year period. The purpose of this study was to analyze the clinical, radiological and histological characteristics of adult patients with CCAM. Methods: Through medical records analysis, we retrospectively reviewed the clinical characteristics, the chest pictures (X-ray and CT) and the histological characteristics. Results: Four patients were women and the mean age at diagnosis was 23.5 years (range: 18~39 years). The major clinical presentations were lower respiratory tract infection, hemoptysis and pneumothorax. According to the chest CT scan, 5 patients had multiseptated cystic lesions with air fluid levels and one patient had multiple cavitary lesions with air fluid levels, and these lesions were surrounded by poorly defined opacities at the right upper lobe. All the patients were treated with surgical resection. 5 patients underwent open lobectomy and one patient underwent VATS lobectomy. On the pathological examination, 3 were found to be CCAM type I and 3 patients were CCAM type II, according to Stocker's classification. There was no associated malignancy on the histological studies of the surgical specimens. Conclusion: As CCAM can cause various respiratory complications and malignant changes, and the risks associated with surgery are extremely low, those patients who are suspected of having or who are diagnosed with CCAM should go through surgical treatment for making the correct diagnosis and administering appropriate treatment.

• Neonatal Medicine
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• v.15 no.1
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• pp.61-66
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• 2008

Purpose : The purpose of this study was to describe the epidemiologic features of febrile illnesses in newborns and to predict the risk of serious infections in this population. Methods : A retrospective study was conducted on 123 full-term infants <30 days of age with an axillary temperature >38$^{\circ}C$ who were hospitalized between 2000 and 2006. Neonates with prenatal risk factors, congenital anomalies, antibiotic administration prior to admission to the hospital, or suspected hospital-acquired infections were excluded. We evaluated the symptoms, physical examination findings, laboratory data, and clinical course between the high- and low-risk groups for serious infections. Results : The high-risk group included 30 infants with the following diagnoses in order of frequency: aseptic meningitis, urinary tract infection, bacterial meningitis, infectious enteritis, sepsis concomitant with disseminated intravascular coagulopathy, bacteremia, pneumonia, cellulitis, and omphalitis. Leukocytosis and thrombocytopenia were statistically different between the two groups. Factors, such as moaning signs, seizures, body temperature, and pulse rate were statistically significant. Conclusion : Unlike previous studies, we included newborns with clinical bacterial infections and aseptic meningitis as the high-risk group. Leukocytosis, thrombocytopenia, moaning signs, seizures, and changes in vital signs were considered useful predictors for identifying febrile neonates at high-risk for serious infections in spite of a difference in the definition of serious infection.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.196-202
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• 1999

Purpose : When the mature kidney fails to reach its norml location in the renal fossa, the condition is known as ectopic kidney. Presenting symtoms can be various and it generally depend on the associated anomaly. Beside urologic anomalies such as hydronephrosis and vesicoureteral reflux, various anomalous vascular net work, skeletal anomaly or genital anomaly can be observed in this condition. Methods : Sixteen children with ectopic kidney was studied retrospectively to analyse initial presentation, accompanied anomaly and prognosis. Results : 56% of the children were accompanied with other urologic anomalies such as true incontinence and vesicoureteral reflux that required surgical treatment. 31% of children were either diagnosed incidentally during evaluation of other non-urologic disease or during follow-up evaluation of abnormal antenatal renal sonogram. Conclusion : Ectopic kidney can be often misdiagnosed as tumorous condition or as a surgical condition depend on the abnormal location of the kidney. Careful evaluation using abdominal sonogram, DMSA, VCUG and abdominal CT scan should be performed in order to search for associated anomalous condition and for proper management.

• Journal of Music and Human Behavior
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• v.2 no.2
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• pp.15-31
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• 2005

Terminal pediatric patient include congenital metabolic abnormalities, chromosome aberrations, congenital anomalies, neuromuscular diseases and other incurable conditions as well as malignant tumors. One third of these diseases are cancers, and two thirds of cancers are lymphadenoma and leukemia. Terminal pediatric patient may feel fear, anger and frustration against treatment process in hospitals, lose control of themselves and stay in helplessness due to restrictions within controlled hospital environment. This study examined the relationship between hospitalized childrens behavior and music using Contextual Support Model of Music Therapya theory stating therapeutic music environments possess three elements of Structure, Autonomy support and Involvement, and increase childrens active engagement. Focused on these three therapeutic elements, this study analyzed music therapy cases of terminally ill children to examine the relationship between childrens behavior and music as environmental supportive medium in the environment. This study is on a single case case subject, a 10-year-old girl with acute lymphocytic leukemia. Nine sessions of music therapy activities were conducted and analyzed using qualitative method. Focusing on three therapeutic elements of Contextual Support Model, analysis of sessions was made on the basis of activities specifically designed for this study. Main music therapy activities included singing while playing musical instruments, singing while listening to music through CD player, making up songs, and searching for song lines. The findings of this study are as follows: a) in terms of Structure, music therapy suggested a direction for effective musical activities for terminally ill children by providing environments where child can sing, play musical instruments and make up songs; b) in terms of Autonomy support, music therapy encouraged childs voluntary participation by giving her chances to make choice while singing or playing musical instruments; c) in terms of Involvement, child could gain positive approval of the therapist and conduct musical activities in cooperation with the therapist. Music therapy serves as supportive medium in the environment, develops spontaneity and self-reliance in emotionally depressed children, and promotes voluntary attitudes in the restricted hospital environment. Based on the positive effects of music therapy, more studies need to be conducted with an approach to more various terminal pediatric patients.

• 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.

• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.845-850
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• 2006

Purpose : To assess the incidence of neonatal hearing loss in a neonatal intensive care unit and the relative importance of risk factors for hearing imparement in a neonatal intensive care unit which the Joint Committee on Infant Hearing(JCIH) had recommended. Methods : One thousand, two hundred and one newborns admitted to the Good Moonhwa Intensive Care Unit from May 2003 to December 2005 were assesed using the automated auditory brainstem response(AABR). The screening was performed on those aged more than 36 weeks and weighing more than 2,200 g. We divided the infants into two groups, 'pass' and 'refer'. The 'refer' group were retested one month later, and if classified as 'refer' during the retest, were referred to a hearing impairment clinic. Results : From the 1,201 neonates, 1,187(98.8 percent) passed the test and 14(1.2 percent) failed. 293(24.4 percent) of the 1,201 neonates had a risk factor for hearing impairment; 282(96.2 percent) passed the test and 11(3.8 percent) failed. The group with risk factors were shown to have a higher incidence of hearing loss(P<0.001). The neonates in the refer group were shown to have a higher incidence of ototoxic drugs(P<0.001), low birth weight(<1,500 g)(P<0.001) and craniofacial anomalies(P=0.007). On the other hand, there were no statistical differences between the pass and refer groups in congenital infection, hyperbilirubinemia, bacterial meningitis, low Apgar scores, prolonged mechanical ventilation and syndromes known to include hearing loss. Conclusion : In order to identify hearing-impaired infants within an appropriate period, neonatal hearing screening tests and identification of the risk factors for neonatal hearing loss are important.