• Journal of Yeungnam Medical Science
• /
• v.35 no.2
• /
• pp.244-247
• /
• 2018

Congenital intratympanic membrane cholesteatoma (ITMC) is a rare type of congenital cholesteatoma located within the tympanic membrane. This lesion tends to increase in size over time. The development of ITMC can cause several complications such as hearing impairment, dizziness, facial palsy, and intracranial complications, similar to any other cholesteatoma. The treatment of congenital cholesteatoma requires the removal of the lesion through surgery, because disease progression induces bony destruction of the nearby tissue. Most patients presenting with this cholesteatoma type are also treated with primary surgical removal. However, we recently experienced a case of an ITMC that showed a natural transition to an external auditory canal cholesteatoma.

• Journal of Chest Surgery
• /
• v.17 no.4
• /
• pp.804-810
• /
• 1984

Two hundred twenty one cases of open heart surgery were done in the Department of Thoracic and Cardiovascular Surgery, Pusan National University Hospital from July, 1981 to October, 1984. 1.There were 154 cases [73%] of congenital anomalies and 57 cases [27%] of acquired valvular heart diseases. Among the congenital cases, 128 cases were acyanotic and 26 cases were cyanotic. Among the 57 cases of acquired valvular replacement surgery, 3 cases had open heart commissurotomy, one had Kay annuloplasty. 2.The age distribution of the congenital acyanotic anomalies ranged from 5 to 32 years with mean age of 12.8 years, the congenital cyanotic anomalies from 3 to 29 years with mean age of 14.2 years and the acquired valvular diseases from 9 to 51 years with mean age of 30 years. The difference of sex distribution was no significance. 3.Three methods for debubbling process were used in our institute, in 133 cases, the vent was inserted into the left ventricular apex, in 61 cases inserted into the left atrium through right superior pulmonary vein and in 17 cases used needle aspiration only. 4.For cardioplegia, the GIK solution was infused repeatedly from 30 to 40 minutes interval and brought excellent results for myocardial protection during open heart surgery. 5.Overall mortality was 7.6%. The mortality along with each disease is 1.56% in congenital acyanotic cases, 26.9% in congenital cyanotic cases and 12.3% in acquired valvular disease.

• Journal of Chest Surgery
• /
• v.49 no.3
• /
• pp.199-202
• /
• 2016

A 7-month-old girl with no medical history was treated with mechanical circulatory support due to myocarditis. Her cardiac contractility did not improve despite more than one week of extracorporeal membrane oxygenation treatment. Thus, we planned a heart transplant. However, a high level of cytomegalovirus was found in blood laboratory results by quantitative polymerase chain reaction. The patient's heart contractility recovered to normal range four days after ganciclovir treatment. She was discharged with slightly decreased cardiac contractility with a left ventricular ejection fraction of 45%.

• Journal of Chest Surgery
• /
• v.49 no.3
• /
• pp.207-209
• /
• 2016

A 4-month-old boy diagnosed with acute myocarditis was treated with extracorporeal membrane oxygenation (ECMO). Follow-up echocardiography eight hours after ECMO revealed intracardiac thrombosis involving all four heart chambers. Because of the high risk of systemic embolization due to a pedunculated thrombus of the aortic valve, we performed an emergency thrombectomy. After the operation, the patient had a minor neurologic sequela of left upper arm hypertonia, which had almost disappeared at the last outpatient clinic two months later. He was diagnosed with a major mutation in MTHFR (methylenetetrahydrofolate reductase), which is related to thrombosis.

• Journal of Chest Surgery
• /
• v.49 no.3
• /
• pp.203-206
• /
• 2016

Patients with venoarterial extracorporeal membrane oxygenation (ECMO) frequently suffer from pulmonary edema due to left ventricular dysfunction that accompanies left heart dilatation, which is caused by left atrial hypertension. The problem can be resolved by left atrium (LA) decompression. We performed a successful percutaneous LA decompression with an atrial septostomy and placement of an LA venting cannula in a 38-month-old child treated with venoarterial ECMO for acute myocarditis.

• The korean journal of orthodontics
• /
• v.23 no.3 s.42
• /
• pp.319-326
• /
• 1993

The purpose of this study was to evaluate the frequency of congenital missing teeth and supernumerary teeth in cleft patients. The subjects were divided into bilateral cleft lip and palate(BCLP), unilateral cleft lip and palate(UCLP) and cleft palate alone(CP alone) groups. 97 cleft patients(BCLP 15, UCLP 70, CP alone 12) between 6-20 years old were evaluated. Panorama film, Orthodontic chart and initial intraoral photogram were employed for this research. The obtained results were as follows. 1. The incidence of congenital missing teeth in total cleft samples was $57.7\%$, and the incidence of supernumerary teeth was $26.8\%$. Each incidence was higher than non-cleft. 2. The incidence of congenital missing teeth was the highest in BCLP and the lowest in CP alone. 3. The number of congenital missing teeth per perso was usually one, and the frequency was higher in the maxillary lateral incisors$(67.8\%)$, and maxillary second premolar$(14.9\%)$ than other teeth. 4. Most of tooth number anomalies in cleft patients were found in maxilla, especially adjacent region to the cleft site.

• Clinical and Experimental Pediatrics
• /
• v.54 no.5
• /
• pp.183-191
• /
• 2011

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened Widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.10 no.2
• /
• pp.211-214
• /
• 2007

An lumbar hernia is a rare clinical disease entity thatarises from the superior or inferior lumbar triangle and presents as a reducible protruding mass in the flank region between twelfth rib and the iliac crest. There are two classifications one classification is a congenital or acquired hernia according to the occurrence timing and the other classification is a superior (Grynfeltt-Lesshaft hernia) or inferior (Petit's hernia) lumbar hernia according to the anatomical location. Approximately 10% of all lumbar hernias are congenital and the vast majority of them are unilateral. We report here a case of a congenital lumbar hernia through the superior lumbar triangle of Grynfeltt-Lesshaft without any other congenital anomalies or complications in a four-month-old male. It was treated successfully with a surgical repair.

• Child Health Nursing Research
• /
• v.7 no.4
• /
• pp.451-460
• /
• 2001

Congenital heart disease is now estimated to be the most prevalent chronic illness in children. The overall purpose of study is to enhance our understanding of mother's perception of family stress, perceived social support, and coping who has a child newly diagnosed with congenital heart disease. In this investigation, the relationship between family stress, perceived social support, and coping within the context of a acute, non life- threatening chronic illness in the situation of newly diagnosed as Rolland's typology of chronic illness. The study employed data from a subset of a large longitudinal study, children's chronic illness: parents and family adaptation conducted by M. McCubbin (5 R29 NR02563) which was funded by the NIH. The subject for this study were 92 mothers who have a child under age 12 who was newly diagnosed with congenital heart disease within the last 3-4 months. Results form correlational and regression analysis revealed that perceived social support operated as a resiliency factor between family stress and coping of mothers. Child and family characteristics appeared to be important predictors of perceived social support and mother's coping. Therefore, the findings provide an incremental contribution to the explanation of effects for perceived social support and may challenge resiliency model in previous literature. Further, these findings suggest that perceived social support and coping are both influencing in the resiliency of relatively high risk groups of families who has a child with congenital heart disease.

• Journal of Chest Surgery
• /
• v.11 no.4
• /
• pp.523-528
• /
• 1978

Recent advances in the surgical treatment of congenital disorders of the heart have necessitated an accurate preoperative diagnosis. Right heart catheterization has become widely accepted as a research tool and diagnostic test to detect the heart diseases, especially in the congenital heart anomalies. Right heart catheterizations were carried out in 50 cases of congenital heart diseases at Department of Thoracic and Cardiovascular Surgery, , Kyungpook National University Hospital, during the period of June, 1975 through September 1978. In age distribution, 27cases were below 10 years of age, 18 cases between 11 and 20, and 5 cases above 20 male to female ratio was 2.8:1. The distribution of congenital heart diseases was VSD [42%], TOF [36%], PDA [10%], ASD [8%], and PS[4%]. Of these, 44 cases [88%], were compatible with the clinical impressions that were made preliminarily before cardiac catheterization, and all the cases except 1 case of VSD was correlated well with the postoperative diagnosis. The right heart catheterization is considered to be reliable and accurate toll in the preoperative diagnosis of congenital heart diseases. These procedures caused complications such as left side hemiplegia [lcase], occlusion of the femoral artery [lcase], and transient ventricular tachycardia [1case], and so the complication rate of right heart catheterization was 6% [3 cases]. None of patients who have undergone right heart catheterization was died.