• Tuberculosis and Respiratory Diseases
• /
• v.45 no.4
• /
• pp.896-901
• /
• 1998

Pulmonary arteriovenous malformation(PAVM) is an uncommon congenital anomaly. As pulmonary arteriovenous malformation is a direct communication between the branches of pulmonary artery and vein which originated from the malformation of capillary development, major disturbances in gas exchange can result. This malformation results in the several symptoms such as dyspnea, hemopyssis, cyanosis, and severe neurologic complaints. However, the most of patients are usually asymptomatic. Selective pulmonary angiography is well known the helpful diagnostic method. Recently, therapeutic embolization has been advocated as the treatment of choice for pulmonary arteriovenous malformations. We report a case of multiple pulmonary arteriovenous malformation, which was detected on the simple chest X-ray and successfully treated with coil embolization in a 19-year-old asymptomatic woman.

• Journal of Chest Surgery
• /
• v.19 no.2
• /
• pp.352-357
• /
• 1986

Congenital cystic adenomatoid malformation [CCAM] of the lung is a rare but often fatal congenital lung bud anomaly that causes acute respiratory distress in the newborn infants. In those who survive infancy and early childhood, its clinical manifestations are not unusual to detect. Recently we experienced two children with CCAM; one with coughing and high fever, another without any symptom On case I, the patient was a 9 year-old female, and was suffered from coughing and high fever since 15 days before. Medical treatment was failed and then, exploratory thoracotomy was performed under the impression of loculated empyema. Finally, a right lower lobectomy was done with a satisfactory postoperative course. On case II, the patient was a 12 year-old female, and admitted for evaluation of known large pulmonary cyst which was checked at the outpatient clinic. Preoperative evaluations gave no more informations, so obliteration of cysts was performed with open biopsy. Two patients with CCAM are described in Korean Univ. Medical Center.

• Clinical and Experimental Pediatrics
• /
• v.53 no.6
• /
• pp.718-721
• /
• 2010

Rubinstein-Taybi syndrome (RTS) is a congenital disorder characterized by typical facial features, broad thumbs and toes, with mental retardation. Additionally, tumors, keloids and various congenital anomalies including congenital heart defects have been reported in RTS patients. In about 50% of the patients, mutations in the $CREB$ $binding$ $protein$ ($CREBBP$) have been found, which are understood to be associated with cell growth and proliferation. Here, we describe a typical RTS patient with Arnold-Chiari malformation. A mutation in the CREBBP gene, c.4944_4945insC, was identified by mutational analysis.

• Journal of Chest Surgery
• /
• v.22 no.1
• /
• pp.95-105
• /
• 1989

The records of 248 patients over 16 years of age who had undergone a surgical correction of a congenital cardiovascular malformation during the period of 10 years from August, 1978 to July, 1988 were reviewed. During this period, the incidence of congenital cardiovascular malformation in adult was 18.2% of 1376 total heart disease operated on and 25.5% of 986 congenital heart defects. Among them, there were 200 patients in acyanotic group and 48 patients in cyanotic group. Male versus female ratio was 1:1.28. The oldest patient was 59 years old female who had atrial septal defect. The mean age was 24.4 years old. The distribution of the lesions showed a large preponderance of atrial septal defects [37.19o] followed in frequency by ventricular septal defects [918.1%], patent ductus arteriosus [17.3%], tetralogy of Fallot [16.1%], and a variety of other complex malformations[3.2%]. In the pediatric age group, relative frequency was different from that of this adult group, showing ventricular septal defects, tetralogy of Fallot, patent ductus arteriosus and atrial septal defects in order of incidence. The hospital mortality and late mortality were 6.0% and 1.7% respectively. The causes of hospital death were low cardiac output in 10 patients, arrhythmia in 2, air embolism in 1, sepsis in 1 and respiratory failure in l. Clinical improvement upto NYHA functional class I or II postoperatively has been achieved and sustained in all patients following repair except the patients of late death and receiving reoperation. This result confirms that congenital heart defects in the adults can be corrected with a good outcome and an aggressive operative approach seems justified.

• Biomedical Science Letters
• /
• v.10 no.4
• /
• pp.397-401
• /
• 2004

We have examined congenital malformation in developing chick embryo caused by endocrine disruptor, bisphenol A (BPA). We injected BPA into the air sac of developing egg on day 4 of incubation. BPA-treated group with a concentration of 10 ㎍/egg showed a little decrease on their body length compared to the untreated group. But the group treated with 50㎍/egg revealed severe malformation in eyeballs and bills. The group treated with 100㎍/egg could not continue their development after few days of incubation. These results indicate that BPA clearly inhibits the normal development in chick and it should be toxic to the developing fetus at early stage and in various tissues. The study should contribute to the understanding of toxic effect of BPA in developing human fetus when exposed to the BPA.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.11 no.1
• /
• pp.56-59
• /
• 2008

Extrahepatic portosystemic shunts, known as Abernethy malformations, were first reported by John Abernethy in 1793. They are classified into two types: Type I refers to a congenital absence of the portal vein and Type II refers to a shunt involving a side-to-side anastomosis with reduced portal blood flow into the liver parenchyma. This malformation is so rare that less than 100 cases have been reported in the medical literature. We report the case of a 13-month-old boy who had a congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This case was complicated with an atrial septal defect and a large hyperplastic nodule in the liver. The patient was diagnosed with a Type II Abernethy malformation. We planned on surgical occlusion of the extrahepatic portocaval shunt. However, six months later, the patient had a sudden onset of a fever of unknown origin and developed hepatic encephalopathy. Although he underwent a liver transplantation, he died of acute hepatic failure.

• 대한생식의학회:학술대회논문집
• /
• 2006.06a
• /
• pp.104-106
• /
• 2006

• 순환기질환의공학회:학술대회논문집
• /
• 2003.11a
• /
• pp.4-5
• /
• 2003

• Journal of Chest Surgery
• /
• v.23 no.2
• /
• pp.407-415
• /
• 1990

A total of 69 patients were treated for congenital lung cysts at Seoul National University Hospital during the period between 1960 and Aug, 1989. They were 37 patients of bronchogenic cyst, 18 patients of pulmonary sequestration, 7 patients of congenital cystic adenomatoid malformation [C.C.A.M.], and 7 patients of congenital lobar emphysema. In cases of bronchogenic cyst, involved age was variable from 7 months to 53 years and most cases were asymptomatic. They were located 14 cases in the right lung, 6 cases in the left lung and 17 cases in the mediastinum. Their sizes were also variable from 2.5 to 12 cm and most of them except 3 cases were solitary cysts. There were 6 cases that had bronchial communication. In cases of pulmonary sequestration, aged from 3 months to 29years, all were intrapulmonary type. 15 cases of them were found in the left lower field and 3 cases in the right lower field. Anomalous vessels were confirmed in 16 cases by aortography and/or surgical exploration. In cases of congenital cystic adenomatoid malformation, aged from 7 days to 6 years, 3 cases of infants were admitted due to severe dyspnea and cyanosis but the remaining 4 cases were admitted due to large cyst or abscess on chest roentgenogram. 3 cases were located in the right lung and 4 cases in the left lung. In cases of congenital lobar emphysema, aged from 14 days to 11 years, 6 cases were located in the right lung and one case was located in the left lung. All of them were male and had shown some degree of dyspnea from birth There were 2 cases of mortality, one in C.C.A.M and one in congenital lobar emphysema. But the remaining cases were well treated and recovered with the surgical resections.

• Journal of Chest Surgery
• /
• v.49 no.4
• /
• pp.295-297
• /
• 2016

Aorto-ventricular tunnel is a rare congenital malformation, and aorto-right ventricular tunnel (ARVT) is an even less common entity. Here, we report the case of a 3-month-old female who underwent successful surgical closure of ARVT. The origin of the right coronary artery was proximal to the ostium of the tunnel.