• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.737-744
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• 2005

Purpose : Infants and children with cardiovascular diseases often present with respiratory symptoms. However, missed or delayed evaluation for potential airway problem may complicate overall prognosis. The aim of this study is to determine the clinical characteristics of these patients and explore the cause of airway problem. Methods : We reviewed the medical records of 64 patients(M : F=33:31, mean age : $6.3{\pm}7.5$ months) whose airway problems were proven by computed tomography or bronchoscopy in perioperative periods at the Asan Medical Center from January 1997 to June 2004. Patients were divided into two groups based on the duration of ventilator care : ${\leq}7$ days(group 1 : 23 cases, M : F=10 : 13) and >7 days(group 2 : 41 cases, M : F=23 : 18). Results : The patients in group 2 significantly developed more post-operative respiratory symptoms than group 1(P<0.001) and had more airway problems including extrinsic obstruction, intrinsic anomaly, and combined problem than group 1 although not significantly different(P=0.082). Among underlying diseases, the most common diseases were vascular anomaly(26.2 percent) and aortic arch anomaly(26.2 percent) in group 1 and pulmonary atresia with ventricular septal defect(22.4 percent) in group 2. The most frequent respiratory symptoms were recurrent wheezing pre-operatively and failure of ventilator weaning post-operatively. The major types of airway anomaly were tracheomalacia and tracheal stenosis(in each case 18.2 percent). Nineteen patients with persistent airway problems underwent aortopexy or other vascular correction. Of the 19 patients, 13(68.4 percent) were improved, but 2 failed in weaning ventilator and 4 died of non-airway problems. Conclusion : Early evaluation and treatment for potential airway problems may affect natural or surgical prognosis in patients with cardiovascular diseases presenting with respiratory symptoms.

• Korean Journal of Bronchoesophagology
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• v.11 no.1
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• pp.32-36
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• 2005

This study aimed to report a case of infant who presented with a prenatal ultrasound diagnosis of agnathia, polyhydramnios followed by a review of previous studies. As widely acknowledge, agnathia is a complex lethal malformation characterized by absent mandible, microstomia, microglossia and ear anomaly which are secondary results to a defect of the first branchial auh. Newborn infants with agnathia often suffer from airway obstruction, causing fatal respiratory failure. The most difficult part of treating those newborns is to keep the airway patent. Therefore, as early airway management planning as possible is the most important part. Airway management was achieved with tracheotomy through an ex utero intrapartum treatment procedure(EXIT). The case of this infant, reporting here, was underwent tracheotomy with preservation of uteroplacental blood flow and gas exchange.

• Journal of Dental Anesthesia and Pain Medicine
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• v.20 no.1
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• pp.49-53
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• 2020

Cri-du-chat syndrome (CdCS) is caused by the deletion of the short arm of chromosome 5. Most patients with CdCS develop intellectual disabilities. Therefore, they have poor oral hygiene and a high caries index. However, treating such patients is not an easy task, because of the difficulty in communication. General anesthesia may be a useful option in adult patients with CdCS and intellectual disability. General anesthesia should be administered very carefully, owing to the presence of comorbid diseases, which may need airway management. Infants with CdCS need general anesthesia if they have a concomitant cardiac anomaly. Intubation is reportedly difficult for such patients was, owing to the structural and functional abnormalities in the larynx and vocal cords. The purpose of this study was to report a case of difficult intubation while inducing general anesthesia in a patient with CdCS during dental treatment, due to a narrow larynx and trachea.

• Journal of The Korean Dental Society of Anesthesiology
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• v.12 no.1
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• pp.33-37
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• 2012

Dental treatment under general anesthesia is considered a good way for behavioral control in disabled patients. General anesthesia for disabled patients sometimes requires difficult airway management. In this case, intra-oral camera is great helpful to intubation. Originally, the intra-oral camera is designed to facilitate diagnosis dental disease. The intra-oral camera is very efficient on intubation. Laryngeal photo taken by Intra-oral camera can be good guide for difficult intubation. In this report, an 11-year-old boy with chromosomal anomaly received dental care under general anesthesia. And he had a potentially difficult airway. We achieved nasotracheal intubation successfully with intra-oral camera.

• Journal of Chest Surgery
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• v.23 no.1
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• pp.201-204
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• 1990

The double aortic arch is the commonest anomaly among the vascular rings are relatively rare congenital vascular anomalies. This anomaly is malformation of the aortic arch system may, by compression of the trachea and esophagus, cause respiratory distress and dysphagia. We experienced one case of double aortic arch with right sided descending aorta with predominant right anterior arch treated surgically at Kyung Hee University Medical Center. 1-year-old male patient with acute airway obstruction due to combination of double aortic arch and right descending aorta. The diagnosis was made by simple X-ray & confirmed by barium esophagogram & aortogram. The operative approach was through left thoracotomy & underwent division of the left aortic arch & division of ligamentum arteriosum & suspension of divided proximal end of anterior arch to anterior thoracic wall. The postoperative courses was uneventful and doing well on the 3 years.

• Journal of Chest Surgery
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• v.47 no.4
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• pp.389-393
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• 2014

Retroesophageal aortic arch, in which the aortic arch crosses the midline behind the esophagus to the contralateral side, is a rare form of vascular anomaly. The complete form may cause symptoms by compressing the esophagus or the trachea and need a surgical intervention. We report a rare case of a hypoplastic left heart syndrome variant with the left retroesophageal circumflex aortic arch in which the left aortic arch, retroesophageal circumflex aorta, and the right descending aorta with the aberrant right subclavian artery encircle the esophagus completely, thus causing central bronchial compression. Bilateral pulmonary artery banding and subsequent modified Norwood procedure with extensive mobilization and creation of the neo-aorta were performed. As a result of the successful translocation of the aorta, the airway compression was relieved. The patient underwent the second-stage operation and is doing well currently.

• Tuberculosis and Respiratory Diseases
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• v.45 no.3
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• pp.583-586
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• 1998

Tracheal bronchus is an uncommon anomaly of the airway in which an ectopic bronchus arises from the trachea superior to its bifurcation. It is usually asymptomatic and no intervention is needed. However in the cases complicated with recurrent pneumonia, bronchiectasis or abscess, surgical excision may be needed. We report 5 cases of tracheal bronchus with or without complication or combined anomaly.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.3 no.2
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• pp.75-79
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• 2007

Background: Down's syndrome, or trisomy 21, is the commonest congenital chromosome anomaly. With improvement in medical care, these patients increasingly reach adulthood in spite of their physical maldevelopment and mental retardation. And, the number of those who required general anesthesia for dental treatment is increasing. Methods: We reviewed the 26 cases of 22 patients with Down's syndrome who underwent outpatient general anesthesia for dental treatment at the clinic for the disabled in Seoul National University Dental Hospital. Results: The mean age was 22 years. They all had severe mental retardation and some had congenital heart anomaly, epilepsy, hypothyroidism, acute leukemia, autism, cleft palate, and chronic renal failure. For anesthesia induction, 4 cases was needed physical restriction, but others showed good or moderate cooperation. Drugs used for anesthesia induction was thiopental (17 cases) and sevoflurane (9 cases). All patients received nasotracheal intubation and 3 cases needed difficult airway management. Mean total anesthetic time was $166{\pm}60$ min and staying time at PACU was $92{\pm}48$ min. There was no death or long term hospitalization because of severe complications. Conclusion: If general anesthesia is needed, pertinent diagnostic tests and workup about anomaly, and appropriate anesthetic planning are essential for safety.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.38
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• pp.8.1-8.4
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• 2016

Congenital alveolar synechiae is a rare anomaly mostly presenting in association with cleft palate. Owing to reduced mouth opening, feeding difficulties, and compromised airway in extreme cases along with presentation in early neonatal period, these patients present unique challenges to the surgeon as well as the anesthetist. Here, we discuss the surgical and anesthetic management of this entity in a 12-month-old female child.

• Journal of Genetic Medicine
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• v.1 no.1
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• pp.1-3
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• 1997

Nager syndrome is a rare malformation complex characterized by facial, limb, and skeletal morphogenesis. The mode of inheritance has not been definitely established. Major karyotypic abnormalities were seldom associated with this syndrome. We report on an infant with Nager acrofacial dysostosis that was associated with 45,X monosomy. This baby was born to a 36-year-old multigravid woman after 37 weeks of gestation and with maternal hydramnios. The baby girl died of airway obstruction due to retruded tongue 3 hours after birth. Phenotypically, this this patient had mandibulofacial dysostosis, radioulnar synostosis, hypoplasia and aplasia of thumbs, peripheral edema and apparently normal genital organs. We confirmed that major chromosomal anomaly including 45,X monosomy could be associated with Nager syndrome, although its pathogenetic significance remains unanswered.