• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.38 no.3
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• pp.171-176
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• 2012

Congenital syngnathia refers to the fusion of bony tissues, a rare disorder with only 41 cases reported in the international literature from 1936 to 2009. The occurrence of syngnathia without any other associated systemic disease or congenital anomaly is extremely rare. This report presents a case of congenital syngnathia with unilateral maxillomandibular bony adhesion without any other oral or maxillofacial anomaly. No recommended protocol for surgery exists due to the rarity of the disorder. There is a very low survival rate for the few patients who have forgone surgical management. This case describes a 74-year-old female patient who was suffering from limitation of mouth opening and was subsequently diagnosed with congenital syngnathia. The surgical staff performed separation surgery and reconstructed the malformed oral vestibule and cheek using the radial forearm free flap operation.

• Journal of Chest Surgery
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• v.22 no.6
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• pp.1025-1035
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• 1989

Since we first performed open heart surgery on December 30, 1986, 126 cases were operated on up to August 31, 1989. Among the 126 cases, 65 cases were congenital heart disease of which 63 were acyanotic disease, and 61 cases were acquired heart disease, most of which were valvular heart disease. The age distribution of congenital heart disease was from 1 years 2 months to 48 years, and males had a slightly higher incidence. The age of acquired heart disease was from a minimum of 15 years to a maximum of 68 years, and the male to female ratio was 1;1.5. Midsternotomy was performed in all cases, and the aortic cannula was inserted through ascending aorta and the venous cannula inserted into the SVC and IVC through the right atrium. Vent was inserted through the right superior pulmonary vein. Cardioplegia solution was used in all cases; it was composed of sodium bicarbonate 3.5 ampule, KCL 14 mEq, 2% lidocaine 2.5 ml, 20 % albumin 50 ml and heparin 1000 units mixed to 950 ml with Hartman solution, and was made to 4oC and infused 10 ml per Kg every 20 minutes. The congenital heart disease had a variety of VSD in 32 cases, ASD 23 cases, PS 6 cases, PDA 2 cases, and one case each of Ebsteins anomaly and tricuspid atresia. The operations performed for acquired heart disease were 4 cases of OMC, 33 cases of MVR, and 5 cases of AVR, and 1 case of AVR with CABG. DVR was perfomed in 13 cases, and triple valve replacement was done in 1 case. Other than these, excision of LA myxoma was 2 cases, and repair of traumatic VSD and removal of a pulmonary embolism were one case each. The surgical mortality was 5 cases[4%], all of which occurred in valve replacement cases. Follow-up study revealed 2 late deaths. One died after a traffic accident and one died due to sepsis after he had received a gastrectomy for ulcer bleeding. The remaining patients were in good condition.

• Journal of Chest Surgery
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• v.33 no.8
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• pp.684-687
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• 2000

Congenital bronchobiliary fistual is a rare disease with unclear etiology. An abnormal tract communicates the tracheobronchial junction to a hepatic segment, usually the left lobe. Billous sputum, a positive HIDA(o-Dimethyliminodiacetic acid) scan, and a trification at the level of the carina lead to the diagnosis, which can be confirmed by bronchoscopic contrast injection. We experienced a case of congenital bronchobiliary fistual in a 27-day-old girl. Our case is reported with literature reviews.

• Journal of Chest Surgery
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• v.22 no.3
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• pp.514-517
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• 1989

Congenital esophageal stenosis is a rare disease in childhood. By virtue of its complex embryological development, the esophagus is the site of many congenital abnormalities. Congenital Esophageal stenosis is one tenth as rare as tracheoesophageal fistula with esophageal atresia and is very rare in the cervical esophagus, which mostly occurred below mid-esophagus. Congenital esophageal web may be caused by the resorption failure of the epithelium following the vacuolization stage in embryonic development in the esophagus. Recently, we experienced 1 cases of congenital esophageal web, as the symptoms of life-long dysphagia. According to her history of dysphagia, radiologic and clinical findings, her esophageal stenosis was considered as congenital. For dilatation and relief of dysphagia, she underwent the Heineke-Mikulicz type of esophagoplasty. The results of surgical treatment were relatively good without any clinical events. So we reported it with its literature review.

• Child Health Nursing Research
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• v.18 no.3
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• pp.150-156
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• 2012

Purpose: The main purposes of this study were to assess maternal-fetal attachment (MFA) of the expectant mothers of a fetus with a prenatal diagnosis of congenital heart disease (CHD) and to identify factors associated with MFA. Methods: The methodology was a cross sectional survey study using a self-administered questionnaire. Thirty pregnant women carrying a fetus with a prenatal diagnosis of CHD and 30 pregnant women with a normal fetus were enrolled in this study. The MFA Scale and PPS (The Prenatal Psychosocial Profile) were used to collect data. Data were analyzed using SPSS 20.0 Window version. Descriptive statistics, $X^2$-test and t-test were used to compare the two groups. The factors associated with MFA were identified by multiple regression analysis. Results: There was no significant difference between the two groups in MFA and social support from spouse was the only variable showing a significant difference. The model from the multiple regression analysis explained 33.8% of MFA for both groups. Conclusion: MFA of expectant mothers with a prenatal diagnosis of CHD and of mothers with a normal fetus were not significantly different. It is important that health care providers encourage expectant fathers to support the expectant mothers to increase MFA.

• Annals of Clinical Neurophysiology
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• v.12 no.1
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• pp.27-31
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• 2010

Ullrich disease is a rare congenital muscular dystrophy, which is clinically characterized by generalized muscular weakness, distal joint hyperextensibility, proximal joint contractures, protuberant calcanei and high-arched palate. The disease is caused by collagen VI deficiency in interstitum and/or sarcolemma of skeletal muscles, for which mutations either in COL6A1, COL6A2 or COL6A3 are responsible. We report a girl who presented with symptoms typical of Ullrich disease, in whom the diagnosis was confirmed by immunohistochemistry and molecular genetic study.

• Journal of Korean Academy of Nursing
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• v.34 no.7
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• pp.1298-1306
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• 2004

Purpose: The purpose of this study was to explore the relationships of family strain, perceived social support, family hardiness, and family adaptation and identify the family resiliency factors for the adaptation of families who have a child with congenital heart disease. Method: The sample consisted of 90 families who had a child diagnosed with congenital heart disease and completed surgical treatment. Data was collected from parents using a questionnaire. Results: Results from path analyses revealed that family strain had a direct effect on both perceived social support and family hardiness, and an indirect effect on family adaptation. Also, the findings revealed that perceived social support had a direct effect on both family hardiness and family adaptation, and family hardiness had a direct effect on family adaptation. Thus, these results indicated that perceived social support and family hardiness had a mediating effect on family strain. Conclusion: Findings provide the evidence for the theoretical and empirical significance of perceived social support and family hardiness as family resiliency factors for family adaptation. Clinical implications of these findings might be discussed in terms of family-centered nursing interventions for the families who have a child with congenital heart disease based on an understanding of family resiliency for adaptation.

• The Korean Journal of Rehabilitation Nursing
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• v.4 no.1
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• pp.73-83
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• 2001

The aims of this study were to identify the mothers educational needs when they have children who have had open heart surgery in congenital heart disease, to identify the children's characteristics with this problem, and also to provide the basic information on the development of the educational program for the mothers. The subjects of the study were 101 mothers of children and their children hospitalized with congenital heart disease in pediatric wards of G. University Hospital in Inchon and S. Hospital in Puch on from June to November 2000. The study used a Likert-type questionnaire with 39 questions which was based on a previous questionnaire developed by Lee Mi-ryun(1989) for adult open heart surgery patients. The reliability of the questionnaire was Cronbach' ${\alpha}$=.9375. The data were collected directly from mothers of the patients in wards. The t-test, ANOVA was adopted for the data analysis. The key results of the study are as follows : 1) The average age of the children was 32.90 months. The majority of the children are male and weighted 3.0-3.5Kg at birth. Most of the children had VSD(29.7%), and 10.9% of children had a family history of heart disease. 2) An average score of 4.62 out of a maximum of 5.00 was recorded for the educational needs of the children's mother. The highest score of 4.69 was for home care after discharge followed by a score of 4.67 for diagnosis and prognosis on congenital heart disease. And pre-post operation care scored 4.51. The mean scores of single-question items of educational need were, in order, 4.81 for immunization after operation, 4.80 for recovery process, and 4.77 for prognosis of the disease. The score of 4.18 for measurement of intake and output was the lowest. 3) The analysis on the children's mothers educational needs by social-demographic characteristics such as sex, age of patient and mother, economical status, educational status, number of child, hospitalization times, and hospitalization cause showed no significant difference statistically, but there was a significant difference between mothers with jobs and with out jobs.

• Neonatal Medicine
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• v.25 no.4
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• pp.137-143
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• 2018

Purpose: This study was performed to determine the clinical features of full-term infants with hypoxemia detected by pulse oximetry and to establish the diagnosis of critical congenital heart disease (CCHD). Methods: We retrospectively reviewed the medical records of neonates who had been admitted to the neonatal intensive care unit within 2 weeks of birth at Korea University Ansan Hospital between January 2013 and October 2017 (n=450). We classified these neonates based on the presence of hypoxemia at admission and investigated neonatal characteristics, initial symptoms, echocardiographic findings, and final diagnosis associated with hypoxemic diseases. Results: Of 450 term infants, 265 infants (58.9%) were identified hypoxemia by pulse oximetry at admission. The most common symptoms of them were cyanosis and tachypnea. Among them, 80.1% of infants (214/265) were diagnosed with respiratory tract disease and 8.3% of infants (22/265) had congenital heart disease. Thirteen infants (13/265, 4.9%) had CCHD and were treated with urgent surgery or transcatheter intervention within 28 days of birth. Majority of infants with respiratory tract disorder were transferred from hospital immediately after birth, but 46.1% of infants (6/13) with CCHD remained asymptomatic after birth and were admitted after 48 hours after birth. In addition, other hypoxemic illnesses were identified as neonatal infectious and neurological diseases. Conclusion: This study showed the importance of assessment in neonates with hypoxemia, including those diagnosed with CCHD. The possibility of CCHD should be considered in the differential diagnosis in neonates demonstrating hypoxemia after 48 hours of birth. A larger prospective study is needed to assess the effectiveness and outcomes of pulse oximetry for neonatal screening in Korea.

• Neonatal Medicine
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• v.15 no.2
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• pp.190-195
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• 2008

Cholelithiasis is rarely recognized in children, especially in infants. Hemolytic disorders, long-term total parenteral nutrition (TPN), congenital anomalies of the biliary tree leading to stasis of bile flow, congenital IgA-deficiency, furosemide treatment, and prolonged fasting have been reported as predisposing factors for cholelithiasis in childhood. Hemolytic disease of the newborn due to anti-E has rarely been reported as a risk factor for cholelithiasis. We report a case of gallbladder stones in a neonate associated with anti-E antibody hemolytic disease.