• 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 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.

• 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.

• Korean Journal of Bronchoesophagology
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• v.11 no.2 s.22
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• pp.32-35
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• 2005

Laryngotracheoesophageal clefts are rare congenital anmalies of the upper aerodigestive tract. They range in severity from small soft tissue defects in the interarytenoid region to complete clefts of the larynx, trachea, and esophagus. Minor clefts with mild symptoms can be managed conservatively. But, major clefts requires prompt surgical managemet fur the best result. We report a case of a 1 month old male with laryngotracheoesophageal cleft. This cleft was corrected by thoracotomy and anterior laryngofissure approach.

• 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.

• 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.

• Journal of Chest Surgery
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• v.30 no.8
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• pp.843-846
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• 1997

Congenital bronchial atresia is one of the rare bronchopulmonary anomalies which is thought to be caused by a vascular insult of uncertain timing during fefal development. It is defined as an anomaly which does not have communication between a segmental or lobar bronchus a'nd the main airway. Because of the collateral ventilation, almost all of these cases show hyperlucency of the involved segment or lobe in chest roentgenogram. We report an extremely rare case of congenita bronchial atresia with collapse of the right upper lobe which was treated by surgical resection.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.28 no.1
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• pp.20-24
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• 2017

Laryngomalacia is the most common congenital anomaly that causes inspiratory stridor and airway obstruction in the newborn. Symptoms begin to appear after weeks of age, become worse at 4-8 months, improve between 8-12 months, and usually heal naturally at 12-18 months. Despite these common natural processes, the symptoms of the disease can be very diverse and, in severe cases, require surgical treatment. The diagnosis can be made by suspicion of clinical symptoms and direct observation of the larynx with the spontaneous breathing of the child. Typical laryngeal features include omega-shaped epiglottis, retroflexed epiglottis, short aryepiglottic fold, poor visualization of the vocal folds, and edema of the posterior glottis, including inspiratory supra-arytenoid tissue prolapse. In this review, we discuss the classification and treatment based on symptoms and laryngoscopic findings in patients with laryngomalacia.

• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.660-664
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• 2008

The term tracheal bronchus refers to an abnormal bronchus that comes directly off of the lateral wall of the trachea (above the carina) and supplies ventilation to the upper lobe. Tracheal bronchi occur almost exclusively on the right trachea and are associated with other congenital anomalies. In addition, tracheai bronchus may be related to other inflammatory conditions with persistent wheezing, such as recurrent pneumonia, chronic bronchitis and bronchiectasis, which is a result of the relatively poor local drainage of the involved bronchi. An infant with recurrent wheezing is likely to be a challenge for a clinician in the evaluation of the etiology of airway obstruction and in the differential diagnosis of wheezy breathing. The authors report a case of an 8-month-old female infant with a ventricular septal defect, who presented with stridor and recurrent respiratory infection and finally was finally diagnosed with a tracheal bronchus using computed tomography and a bronchoscopy. Therefore, tracheal bronchus should be included in the differential diagnosis of any child who presents with chronic or recurrent respiratory tract symptoms such as coughing, wheezing, stridor and recurrent respiratory infection, particularly in children with other congenital deformities.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1993.05a
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• pp.108-108
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• 1993

Cystic hygroma is a congenital anomaly of the lymphatic system for which the exact pathogenetic mechanism remains to be elucidated. It is filled with lymphatic fluid and commonly present in the cervical area in either single isolated or multiple form. It is usually innocuous without any pain but may be life-threatening when it is large enough to compromise the airway or interfere with swallowing. Since spontaneous regressions rarely occur, it requires surgical removal. However, this may be rendered difficult in multiple forms and in those with extensive infiltration into the surrounding tissue. This is a presentation of 10 pediatric patients with cystic hygroma who have been successfully treated by sclerotherapy using bleomycin at the authors' department.