• Tuberculosis and Respiratory Diseases
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• v.56 no.1
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• pp.97-102
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• 2004

Anomalous systemic arterial supply to the lung is a rare congenital anomaly. The lung supplied by the anomalus systemic artery has a normal bronchial tree, which is usually in the basal segment of the lung, especially in the left lung. Most of patients are asymptomatic, but the main clinical symptoms of this disease are hemoptysis and exertional dyspnea. CT is useful for the diagnosis and showed a retrocardiac nodular shadow connected to the descending aorta branching into the basal segments of the relatively normal lower lobe. Surgery is indicated for all patients. Here we report a case of anomalous systemic arterial supply to normal basal segments of left lower lobe in a patient with hemoptysis with a review of the relevant literature.

• Journal of the korean academy of Pediatric Dentistry
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• v.48 no.1
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• pp.122-128
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• 2021

Short root anomaly (SRA) is a rare dental condition with abnormally short and blunt root morphology. It mostly affects maxillary central incisors symmetrically and only has been observed in permanent teeth. A 9-year-old girl was referred from a local dental clinic for short root development in mixed dentition with no symptoms. Radiographic and intraoral examinations revealed SRA on upper and lower incisors and mandibular first molars along with other dental anomalies such as enamel hypoplasia and dens invaginatus. During long - term follow - up for 5 years, her mixed dentition has changed to permanent dentition and generalized SRA was observed in all permanent teeth. Cephalometric radiograph also revealed the calcification between the anterior and posterior clinoid processes described as a sella turcica bridge which was reported associating with dental anomalies. Early diagnosis of SRA is emphasized for successful management and prevention of root resorption and tooth loss. This report aimed to present a rare case of generalized SRA along with other dental anomalies and sella turcica bridging in a female patient through long - term follow - up.

• Advances in pediatric surgery
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• v.17 no.2
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• pp.162-169
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• 2011

Branchial cleft anomalies are the second most common head and neck congenital lesions seen in children. Amongst the branchial cleft malformations, second cleft lesions account for 95 % of the branchial anomalies. This article analyzes all the cases of second branchial cleft anomalies operated on at Seoul National University Hospital from September 1995 to February 2011. We analyzed sex, age, symptom and sign, accompanied anomaly, diagnosis, treatment, pathologic report and outcome via retrospective review of medical records. In this series, we had 61 patients (27 female and 34 male). The mean age at the time of operation was 38 months. 31 lesions were on the right, 20 were on the left and 10 were bilateral. The most frequent chief complaints at presentation were non-tender mass and cervical opening without any discharge. According to anatomic type, 29 patients had branchial cleft sinuses, 14 had cysts, 14 had fistulas and 4 had skin tags. Complete excision was attempted if possible and antibiotics challenged when infection was suspected. Complete excision was achieved in 96.7 % of cases. Incision and drainage was done in 2 cases due to severe inflammation, and both recurred. Postoperative complications included wound infection in 2 cases. Microscopic examonation revealed squamous epithelium in 90.2 % and squamous metaplasia in one case in the branchial cleft cyst wall. In summary, second branchial anomaly is found more frequently on right side of neck. Fistulas are diagnosed earlier than cystic forms. Most cases could be diagnosed by physical examination. The definitive treatment is complete excision and sufficient antibiotics coverage for cases with inflammation. After drainage of infected lesions, follow up excision after 1 year might be beneficial for preventing recurrence.

• Journal of Chest Surgery
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• v.36 no.3
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• pp.189-193
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• 2003

The patient was a 30-day-old female infant with symptoms of severe dyspnea and cyanosis, when she was admitted to the ER. The echocardiography revealed DORV with subpulmonary VSD, and the diagnosis of Taussig-Bing anomaly was made. Two days after admission, an urgent operation was performed. The operation consisted of intraventricular tunnel repair and arterial switch operation. She was discharged, and after checking her chest X-ray through OPD, there was no interval change of cardiomegaly. She was then re-admitted, and the angiography revealed coactation of aorta. We performed a resection and end-to-end anastomosis of aorta. She is currently in good condition 11 months postoperatively.

• Journal of Chest Surgery
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• v.30 no.1
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• pp.108-111
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• 1997

Congenital unilateral agenesis of pulmonary artery is a rare anomaly and it usually occurs in association with other cardiac anomaly such as tetralogy of Fallot. Since most patients affected by this defect without associated congenital cardiac anomaly or pulmonary Infection are asymptomatic, the clinical diagnosis of this anomal is first recognized by a characteristic pattern in chest roentgenogram taken as a routine checking; the findings on chest film consists of cardiac and mediastinal displacement, absence of the pulmonary arterial shadow, smaller hemithorax, and elevationof the hemidiaphragm, all on the affected side. We experienced rlght pulmonary artery agenesis in a 48 year-old male, who complained of massive hemoptysis, and it was diagnosed by digital subtraction pulmonary arteriogram and perfusin scan, and treated by right middle and lower lobe bi-lobectomy, and we report this case with the review of relevant literatures.

• Journal of Chest Surgery
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• v.35 no.4
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• pp.303-306
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• 2002

Patients with severe Ebstein's anomaly showing in the neonatal period, represent progressive cardiac enlargement with pulmonary hypoplasia and functional pulmonary atresia with patent ductus alteriosus-dependent pulmonary circulation. Biventricular repair in these patients had been mostly unsuccessful except for Starnes' procedure that converts the anatomy to single ventricle physiology for Fontan procedure. A 4-days old male was admitted with the diagnosis of severe Ebstein's anomaly with anatomic pulmonary atresia and severe cardiac enlargement. He successfully underwent biventricular repair with vertical plication method of atrialized right ventricle, tricupid annuloplasty, transannular right ventricular outflow tract reconstrulltion, atrial septal defect patch closure with fenestration, and right atrial reduction angioplasty Postoperatively, cardiothoracic ratio was significantly reduced and mild tricuspid regurgitation was remnant in echocardiography. The patient is currently 10 months old and is fully active without restrictions.

• Advances in pediatric surgery
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• v.2 no.2
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• pp.119-128
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• 1996

The survey on branchial anomalies was conducted by Korean Association of Pediatric Surgeons. A total of 173 cases were reported, which were managed by 36 members and cooperators during the three years from January 1, 1993 through December 31, 1995. The following results were obtained by retrospective analysis of the 173 cases of branchial anomalies. The presenting symptoms were cervical mass in 101 cases, pit with or without discharge in 71, cervical abscess in 47 and respiratory difficulty in 3. The average age of the patients with cervical abscess was 52 months. Seventy(79%) of 89 patients with branchial anomalies and a cystic mass had their first clinical manifestations by 1 year of age, while 40(51%) of 78 patients with only a branchial cyst had their first clinical manifestation in first year of life. Radiologic studies were carried out in 77 patients (43%). The preferred diagnostic modalities were ultrasonography(47 patients), simple neck radiogram(19) and CT scan(17). Preoperative diagnosis was correctly made in 156(91%) of 173 patients. Seventeen patients were incorrectly diagnosed as thyroglossal duct cyst in 5 patients, cystic hygroma in 4, dermoid cyst in 3, and lymphadenopathy in 3. There were no remarkable difference in sex and laterality of presentation but bilateral lesions were found in 9(5%) patients and unusual locations of the anomalies were the manubrium, left subclavicular area, median cervial area, preauricular and parotid area. There were 78(45%) patients with cyst, 52(30%) patients with sinus, 35(20%) patients with fistula and 8(5%) patient with skin tag. Embryological classification was possible in only 64(37%) patients. The 2nd branchial anomaly was present in 50(78%), the 1st branchial anomaly in 10(18%), and the 3rd or 4th branchial anomaly in 4(6%). Histopathological study of the lining epithelium(N=134) is recorded that 45% were lined with squamous epithelium, 17% with respiratory epithelium, 6% with. squamous and respiratory epithelium, 14% with inflammatory change. Lymphoid tissue was common(62%) in the wall of the lesions. Twelve(7%) of 158 patients had postoperative complications including wound complication, recurrence and facial nerve palsy.

• Journal of Chest Surgery
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• v.31 no.2
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• pp.216-219
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• 1998

Ebstein's anomaly is a complex malformation that can be treated by various surgical techniques, either repair or replacement of the abnormal tricuspid valve, with variable results. The essence of the malformation is the downward displacement of the septal and posterior leaflets into the ventricle, resulting in the formation of an atrialized portion of the right ventricle. The aim of surgical repair is to correct the tricuspid valve dysfunction and to plicate the atrialized portion of the right ventricle A 12-months old female was admitted with the diagnosis of Carpentier type A of Ebstein's anomaly with severe tricuspid regurgitation. She successfully underwent operation with vertical plication of right ventricle and reimplantation of tricuspid leaflets. Postoperatively cardiac size was significantly reduced and tricuspid regurgitation was trivial in echocardiography. She was diacharged the 14th postoperative day.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.60-66
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• 1997

Even though the urinary stones are rare in children, careful observation and monitoring are necessary because the incidence has been increasing. This study is aimed to document the characteristics of urinary stones in children including the symptoms, diagnosis, with or without urinary tract infection, other urinary tract anomaly and treatment. 45 patients under the age of 15 years with urinary stones hospitalized during Jan. 1986 to Jun. 1996 at Severance Hospital were reviewed retrospectively. Patients' mean age was 6.5 years and sex ratio (male : female) was 5.4:1. The most common symptom was gross hematuria. Stones accompanied with urinary tract infection was 46.7%, and stones associated with urinary tract anomaly was 35.6%. Extracorporeal shock wave lithotripsy, percutaneous nephrolithotorny, hydration and diuretics were the treatment modality used. Urinary stone were found to be a significant cause of urinary tract obstruction in children, requiring prompt diagnosis and treatment. Although KUB, IVP, and ultrasonography were commonly used to make the diagnosis, many cases were detected only by ultrasonographic study.

• Advances in pediatric surgery
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• v.11 no.2
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• pp.150-156
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• 2005

Failure of the urachus to regress completely results in anomalies that may be classified as patent urachus, urachal sinus, urachal cyst and bladder diverticula. The presenting symptoms of children with urachal anomalies are variable and uniform guidelines for diagnosis and treatment are lacking. The purpose of this study was to analyze our experience and develop conclusions regarding the presentation, diagnosis and treatment of urachal anomalies. We retrospectively analyzed the records of 32 patients who were admitted for urachal anomalies from March 1995 to February 2005. The age distribution of these patients at presentation ranged from 1 day to 14 years old (median age 1 month). There were 20 boys and 12 girls. The 32 cases comprised 13 cases of urachal sinus (40.6 %), 10 urachal cyst (31.3 %), and 9 patent urchus (28.1 %). In 30 patients ultrasonography was used for diagnosis and 2 patients with patent urachus were explored without using a diagnostic method. Twenty-three patients were confirmed by ultrasonography alone and 7 patients were examined using additional modalities, namely, computed tomography for 2 patients with an urachal cyst, magnetic resonance imaging for 1 patient with an urachal cyst, and fistulography for 3 patients with an urachal sinus. The presenting symptoms were umbilical discharge (14 patients), umbilical granuloma (8), abdominal pain and fever (3), fever (3), abdominal pain (2), and a low abdominal mass (2). Excision was performed in 29 patients, and 3 patients were conservatively managed. Urachal anomalies in children most frequently presented in neonates, and the most common complaint was umbilical discharge with infection. Urachal anomalies can be diagnosed by a physical examination and an appropriate radiographic test. Ultrasound was the most useful diagnostic method. Complete surgical excision of an urachal anomaly is recommended to avoid recurrence, and the rare development of carcinoma.