• Journal of Chest Surgery
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• v.27 no.8
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• pp.700-704
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• 1994

Congenital coronary arteriovenous fistula is relatively rare congenital heart disease which has formation of fistula between coronary artery and cardiac ventricle, atrium, or pulmonary artery, but that can be repaired with simple surgical procedure. We experienced three cases of congenital coronary arteriovenous fistula, which were repaired surgically under cardiopulmonary bypass, so we report these cases with review of the literatures.

• Investigative Magnetic Resonance Imaging
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• v.25 no.3
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• pp.193-196
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• 2021

Congenital absence of the bilateral internal carotid arteries (ICA) is a very rare occurrence. Recognition of this rare anomaly is important, when considering intracranial endovascular interventions in the event of thromboembolic events with revascularization, transsphenoidal surgery, and the surveillance and detection of associated cerebral aneurysms. We report a case of a 25-year-old man who presented with headache since 2 years ago, and was incidentally discovered to have a congenital bilateral absence of ICAs.

• Journal of Korean Neurosurgical Society
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• v.67 no.3
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• pp.270-279
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• 2024

Prompt medical attention is crucial for congenital intracranial vascular malformations in children and newborns due to potential severe outcomes. Imaging is pivotal for accurate identification, given the diverse risks and treatment strategies. This article aims to enhance the identification and understanding of congenital intracranial vascular abnormalities including arteriovenous malformation, arteriovenous fistula, cavernous malformation, capillary telangiectasia, developmental venous anomaly, and sinus pericranii in pediatric patients.

• Clinical and Experimental Pediatrics
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• v.57 no.2
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• pp.85-90
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• 2014

Purpose: This study aimed to assess the characteristics of thyroid nodules among infants diagnosed with congenital hypothyroidism. Methods: A retrospective study of 660 infants (374 males, 286 females) diagnosed with congenital hypothyroidism was carried out at the Pediatric Endocrine Clinic in Soonchunhyang University Hospital, Korea, between May 2003 and February 2013. The average age at diagnosis was $1.16{\pm}1.68$ months. Results: Of the 28 patients (4.2%) with thyroid nodules, 17 (2.6%) had cystic thyroid nodules and 11 (1.6%) had solid thyroid nodules. There were no significant differences in gender or age between congenital hypothyroidism patients who hadthyroid nodules and those who did not. All nodules were asymptomatic. The average age at diagnosis of congenital hypothyroidism with nodules was $1.42{\pm}1.39$ months. All detected nodules measured less than 1 cm in diameter. Twenty-two of the 28 infants (78.6%) had only one nodule, while multiple nodules were found in 6 infants (21.4%). Of the 28 infants diagnosed with nodules, 16 underwent thyroid ultrasonography during follow-up and 8 of them (50%) showed no signs of nodules at thyroid ultrasonography. Conclusion: The prevalence of thyroid nodules in infants with congenital hypothyroidism was 4.2%. Most thyroid nodules were small in size and benign, disappearing during follow-up observation. We therefore conclude that thyroid nodules in infants with congenital hypothyroidism can simply be observed and do not require direct treatment.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.446-450
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• 2006

Congenital central hypoventilation syndrome (CCHS) or Ondine's curse is a very rare sleep disorder that is the result of a congenital failure of the autonomic control of ventilation caused by insensitivity of the chemoreceptor to hypercapnea during sleep. Gastrointestinal motility disorders, particularly a congenital megacolon (Hirschsprung disease) is often combined with CCHS. This combination can be explained by a defect in the migration of neuronal cells from the neural crest (neurocristopathy) during the intrauterine period. A diagnosis of CCHS is made by confirming the failure of adequate ventilation in response to hypercapnea and hypoxia during sleep and the exclusion of other diseases. Young infants frequently show atypical clinical courses, and their conditions are frequently complicated with the long-term sequela of hypoxemic episodes. Therefore, a high index of suspicion and active treatment with mechanical ventilation are important for reducing recurrent hypoxemic episodes in the neonatal period. This paper reports the follow up of a case of CCHS in a neonate who showed frequent intractable apnea and cyanosis and was given artificial mechanical ventilation during sleep.

• Journal of Chest Surgery
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• v.13 no.4
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• pp.414-417
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• 1980

During the period from 1958 to July 31, 1980, there were 1764 Cardiovascular Surgical cases consisting of 1166 congenital and 598 acquired lesions in the Department of Cardiothoracic Surgery, Seoul National University Hospital. For 1764 cases 1162 open heart surgery and 602 conventional operations were performed. Among 1166, congenital lesions 266 adult cases were found. Adult cases occupy 15.1% of the total cases consisting of 24.5% of acyanotic and 19.9% of cyanotic group. Over all operative mortality of adult cases was 6.8% compared with 12.4% of total cases. Left over congenital cardiovascular anomalies in Korea can be safely operated at the present time. The oldest operated patient among adult congenital cardiovascular anomalies was 57 years old female atrial septal defect case. For 1162 cases open heart surgery bubble oxygenators were utilized except 2 membrane oxygenators. Various kinds of bubble oxygenators have been used but recently Shiley**** oxygenators are being used exclusively with satisfactory results.

• Korean Journal of Bronchoesophagology
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• v.13 no.2
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• pp.65-67
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• 2007

Congenital epiglottic cyst is rare cause of stridor in neonate and if managed inadequately, disaster such as death can occur. Diagnosis of congenital epiglottic cyst includes imaging studies and endoscopy. Fiberoptic or rigid endoscopic examination excludes other causes of stridor in neonate. Complete excision of cyst is treatment of choice under suspension laryngoscopic guidance. Here, we report a case of congenital epiglottic cyst and concomitant laryngomalacia presenting with seizure and respiratory difficulty in neonate with a review of literature.

• Journal of Chest Surgery
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• v.24 no.9
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• pp.870-880
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• 1991

One hundred eleven cases of open heart surgery had been performed at the department of thoracic and cardiovascular surgery in College of Medicine Hallym Univ. from November 28, 1984 to August, 1991. There were 68 cases of congenital heart disease and 43 cases of acquired heart disease. Among the 68 cases of congenital heart diseases, 65 cases of acyanotic group and 3 cases of cyanotic group were noted. The majority of acquired heart diseases were valvular heart disease. The congenital heart disease consisted of 33 ASD, 27 VSD, 6 PS, 3 TOF. The sex ratio of congenital and acquired heart diseases was represented as 1.4: 1 and 1; 1.4 respectively. The age distribution of congenital heart diseases ranged from 11 months to 50 years old and that of acquired heart diseases from 15 to 61 years old. The overall operation mortality was 1 case[0.9%].

• Journal of Chest Surgery
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• v.47 no.4
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• pp.394-397
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• 2014

Numerous technical modifications and various complications of the Senning procedure have been described in the literature. We describe the excellent clinical status and anatomic result of a 33-year-old patient who underwent a modified Senning operation using the left atrial appendage for reconstruction more than 30 years prior to presentation.

• Journal of Chest Surgery
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• v.27 no.11
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• pp.914-921
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• 1994

From May 1977 to November 1993, 1, 500 cases of open heart surgery were performed under the cardiopulmonary bypass. In 1500 cases of open heart surgery 975 cases[65%] were congenital heart disease and 525 cases[35%] were acquired heart disease. In 975 cases of congenital heart disease, there were 812 cases[83%] of acyanotic congenital heart anomaly and 163 cases of cyanotic congenital anomaly. In 525 cases of acquired heart disease, most cases were valvular heart disease in which 557 valves were implanted. The each operative mortality of congenital and acquired disease was 7.7% and 7.4% respectively and then overall mortality rate was 7.6%. There has been increasing incidence of open heart surgery in infants in recent years. After 1990, 28 cases of infant open heart surgery below the 12month were performed. Most lowest weight was 2, 200g[15 days]. Operative mortality of open heart surgery in infant with weight below the 10kg was very high as a 25.7%.