• Title/Summary/Keyword: 선천성 식도 이상

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Endoscopic Balloon Dilatation in Children with Congenital and Acquired Esophageal Anomalies (소아의 선천성 및 후천성 식도 질환에서 내시경적 풍선 확장술)

  • Kwak, Ju Yuong;Park, Jae Hong
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.8 no.2
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    • pp.137-142
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    • 2005
  • Purpose: To evaluate the safety, efficacy and technical problems of the endoscopic balloon dilatation of esophageal anomalies in children. Methods: The medical records of 8 children treated by endoscopic balloon dilatation for esophageal anomalies over a 10-year period at Pusan National University Hospital were reviewed retrospectively. The balloon catheter (Maxforce TTS or CRE, Boston Scientific Co., USA) was positioned across the area of narrowing by direct visualization. The balloon was slowly inflated with normal saline to specified pressures for each balloon and maintained for 60 seconds and then deflated. After 60 seconds pause, the procedure was repeated with a larger sized balloon (increments of 1 mm for each subsequent dilation) till effective dilatation was confirmed by direct visualization without complications. Results: Three male and five female were included and their mean age was 4.2 years. A total of 27 (average of 3.2 per patient) dilatation were performed. Underlying diseases of patients are postoperative stricture of esophageal atresia in 3 cases, esophageal ring in 2 cases, achalasia, corrosive esophagitis and hypertensive LES in one case respectively. The size of initial dilating balloon was chosen on the basis of the diameter of the narrowing determined by endoscopy. The first dilation in patients with severe esophageal stricture was made with a 6 mm sized balloon. Complications observed were esophageal perforation and respiratory holding during the procedure in one case respectively. Successful outcome was seen in 6 patients (75%). Conclusion: Endoscopic balloon dilatation can provide a safe and effective mean of treating esophageal anomalies in children and should be considered the treatment of choice in the initial management of those cases.

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Audiological Findings of Congenital Ichthyosiform Dermatosis with Sensorineural Hearing Loss (난청을 동반한 선천성 어린선상 피부병의 청각학적 소견)

  • 한태희;최진택;김광현;박찬일
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1981.05a
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    • pp.40.3-41
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    • 1981
  • The ichthyosiform dermatosis is consisted of a heterogenous group of hereditary disorders. Authors report a case of 26 year old female patient who has congenital ichthyosiform dermatosis associated with sensorineural hearing loss, neovascularization of cornea, partial alopecia and abnormalities of nails and teeth. The audiological findings of this patient were as follows: 1. Bilateral severe sensorineural hearing loss. 2. SISI scores: 100% at 1, 000 Hz and 4, 000 Hz on both ear. 3. Tone decay test: 25 dB decay at 1, 000 Hz and 4, 000 Hz respectively on both ear. 4. SRTs : 85 dB on both ear. 5. Speech discrimination scores : 80% at 95 dB hearing level(MCL) on both ear. With above results, cochlear lesion is suspected in this syndrome.

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Congenital Cystic Diseases in the Neck - I. Branchial Cysts, II. Thyroglossal Duct Cysts (선천성 경부 낭종 -I. 측경새성낭종 3예, II. 갑상선설관낭종 4예-)

  • 노관택;김대성
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1972.03a
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    • pp.16.2-16
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    • 1972
  • Persistance of developmental remnants during fetal life may be attributed to congenital cysts and fistulas in the neck, which are experienced rather rarely. Recently authors have experienced three cases of progressive increased tumor mass in the lateral side of the neck and four cases of tumor mass in the suprathyroid region. We have performed surgical removal under the diagnosis of branchial cysts and thyroglossal duct cysts, respectively. The biopsy specimens were confirmed by histopathological study.

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A Rare Case of Scimitar Syndrome Accompanied with an Imperforate Anus in a Newborn (항문 막힘증을 동반한 Scimitar 증후군 1례)

  • Chun, Peter;Lee, Hyoung-Doo;Kim, Young-Don
    • Neonatal Medicine
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    • v.18 no.2
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    • pp.404-408
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    • 2011
  • Scimitar syndrome (SS) is a rare congenital anomaly characterized by hypoplasia of the right lung and partially anomalous pulmonary venous drainage to the inferior vena cava. The term scimitar derives from the shadow created by the anomalous pulmonary vein on chest X-ray that closely resembles that of a curved Turkish sword. It rarely presents as an isolated abnormality. Various cardiac and non-cardiac anomalies have been association with SS, such as right lung hypoplasia, dextroposition of the heart, hypoplasia of the right pulmonary artery, systemic arterial blood supply to the right lower lung from the infra-diaphragmatic aorta, and a secundum type of atrial septal defect. However, an imperforate anus has not been reported previously in association with SS. We describe the first case of infantile scimitar syndrome accompanied with an imperforate anus in a newborn who presented with tachypnea and right pulmonary atelectasis.

Secondary Esophageal Reconstruction for Esophageal Atresia (식도 폐쇄증 환자에서 치험한 2차 식도 재건술)

  • Sa, Young-Jo;Park, Jae-Kil;Jeon, Hae-Myung;Moon, Young-Kyu;Sim, Sung-Bo;Lee, Sun-Hee
    • Journal of Chest Surgery
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    • v.41 no.5
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    • pp.671-674
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    • 2008
  • Jejunum and fasciocutaneous and myocutaneous flaps are theoretically recommended as esophageal substitutes in reconstruction of the esophagus after several occurrences of failed reconstruction. However, other esophageal substitutes should also be considered. Secondary esophageal reconstruction was successfully performed on a 24-year-old woman who had a history of cervical, thoracic, and abdominal operations and esophageal stricture secondary to several failed reconstructions for esophageal atresia and tracheo-esophageal fistula 21 years prior. The esophageal reconstruction was done subcutaneously by end-to-side anastomosis at the left cervical area using a deformed stomach graft. The patient was discharged with the ability to consume a regular diet after the operation.

Two Cases of Fourth Branchial Anomaly: Pyriform Sinus Fistula Treated by Chemical Cauterization (제 4 새낭 기형 2 례: 화학소작에 의한 이상동누공 치험)

  • 김광현;신진성;성명훈
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1993.05a
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    • pp.109-109
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    • 1993
  • Fourth branchial pouch anomaly is an extremely rare condition involving the pharyngeal apparatus with only about 30 cases having been reported in the last 20 years. It almost invariably occurs on the left side and typically presents with repeated episodes of neck swelling, recurrent thyroiditis, or repeated bouts of retropharyngeal abscess. This is a presentation of 2 patient with pyriform sinus fistula, presumably of fourth branchial origin, which, were successfully closed with 10ole trichloroacetic acid chemical cautery. Details of the cases along with the summary of embryology and fourth branchial anomaly are given.

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Surgical Treatment of the Congenital Esophageal Atresia (선천성 식도 폐쇄증의 외과적 치료)

  • 최필조;전희재;이용훈;조광조;성시찬;우종수
    • Journal of Chest Surgery
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    • v.32 no.6
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    • pp.567-572
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    • 1999
  • Background: Surgical correction of the full spectrum of esophageal atresia with tracheoesophageal fistula has improved over the years, but the mortality and morbidity assoiated with repair of these anomalies still remains high. Material and Method: We retrospectively analyzes 27 surgically treated patients with esophageal atresia and tracheoesophageal fistula at Dong-A University Hospital between January 1992 and March 1997. Result: There were 21 male and 6 female patients. Mean birth weight was 2.62$\pm$.385 kg(2.0~3.4 kg). Twenty- four(88.9%) had esophageal atresia with distal tracheoesophageal fistula, and 3(11.1%) had pure esophageal atresia. Four(14.8%) infants were allocated to Waterston risk group A, 18(66.7%) to group B, and 5(18.5%) to group C. In eighteen(66.7%) infants with associated anomalies, cardiovascular anomalies were the most common. Three had a gap length of 3.5 cm or greater(ultra-long gap) between esophageal segments, 7 had 2.0 to 3.5 cm(long gap), 8 had 1.0 to 2.0 cm(medium gap), and 9 had 1 cm or less(short gap) gap length. Among 27 neonates, 3 cases underwent staged operation, late colon interposition was done in 2, and all other 24 cases underwent primary esophageal anastomosis. Oerative mortality was 2/27(7.4%). Causes of death included acute renal failure(n=1), empyema from anastomotic leak(n=1), necrotizing enterocolitis(n=1), sepsis(n=1), insulin-dependent diabetus mellitus(n=1 . There were 4 anastomosis- related complications including stricture in 3, leakage in 1. Mortality was related to the gap length(p<.05). Conclusion: Although the complication rate associated with surgical repair of these anomalies is high, this does not always implicate the operative mortality. The overall survival can be improved by effective treatment for combined anomalies and intensive postoperatve care.

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식도 천공의 외과적 치료

  • 이재익
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 2003.09a
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    • pp.108-108
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    • 2003
  • 식도 천공의 치료는 최근 치료법의 발전에도 불구하고 여전히 난해한 문제이다. 이 환자들은 천공의 원인이나 위치, 심한 정도가 다양한 이질적인 군이며, 반수 이상이 이미 식도에 기존의 질환을 가지고 있어 문제를 더욱 복잡하게 한다. 따라서 절대적이고 획일적인 치료방법은 아직 확립되어 있지 않으며 많은 다양한 방법들이 제시되고 있다. 2002년 11월부터 2003년 7월까지 총 8명의 환자가 식도 천공으로 동아대학교병원 흥부외과에서 치료를 받았으며, 원인별로는 의인성(iatrogenic) 손상이 6례, 자발성(spontaneous) 손상이 2례였고 부위별로는 경부가 3례, 흥부가 5례였다. 의인성 손상에는 내시경 검사중 정상 경부 식도에 발생한 1례, 부식성 식도협착 환자의 풍선 확장술과 스텐트 삽입시 흉부 식도에 발생한 경우가 각각 1례씩, 외상성 경추손상의 수술시 정상 경부 식도에 발생한 경우가 2례, 선천성 식도폐쇄증(esophageal atresia)의 술후 문합부 누출이 생긴 경우가 1례 있었으며, 자발성 손상에는 하부식도에 발생한 기압성(barogenic) 손상 1례와 상흉부 식도암 천공 1례가 있었다. 경부 식도 천공 3례는 모두 경부 배액(drainage)과 식이용 장루술(feeding jejunostomy)을 시행하였고, 부식성 식도협착이 있던 환자 2례는 식도절제술과 흉부내 식도-위 문합을 시행하였으며, Boerhaave씨 증후군 환자는 1차 봉합술, 술후 문합부 유출이 있었던 환자와 식도암 천공이 있었던 환자는 식도절제 및 경부 식도루(esophagostomy), 배액용 위루(gastrostomy), 식이용 장루술을 시행하였다. 모든 환자는 패혈증 등의 심각한 합병증으로의 진행 없이 회복되었다. 현재 위장관의 연속성이 유지 혹은 복원된 환자는 경구식이 중이며 그 외의 환자들은 장루를 통해 영양을 공급하며 식도재건술을 기다리고 있다. 식도 천공은 최근 항생제의 사용, 과영양요법(hyperalimentation), 술후 환자관리의 개선 등으로 치료에 많은 발전이 있었으나, 치료 방침에 있어서는 여전히 논란이 있으며 높은 사망률을 보이는 난제로 남아있다. 비록 일반적인 지침이 도움이 되겠지만, 치료 방침은 환자 개개인의 상황에 따라 적절하게 선택해야 하며, 임상경과에 따라 언제라도 방침을 변경할 수 있는 유연함이 필요하리라 생각한다.

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Surgical Treatment of Bronchoesophageal Fistula in Adult (성인 식도 기관지루의 외과적 치료)

  • 곽영태;김동원
    • Journal of Chest Surgery
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    • v.29 no.1
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    • pp.67-72
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    • 1996
  • Bronchoesophageal fistula is a rare clinical entity whether congenital or acquired in adult. We experinced 8 cases of bronchoesophageal fistula and performed surgical correction from 1991 to 1994. Of the 8 patients, 5 patients were male and three were female aging from 21 to 61 years(mean 44.12$\pm$14.62 years). Seven of 8 patients had congenital bronchesophageal fistula and the other one had acquired bronchoesophageal fistula. According to the classification of Braimbridge and Keith, 4 cases were belonged to type I and 3 cases were type II . The diagnosis was confirmed by esophagogram in six patients, by bronchoscopy and bronchogram in two patients, and in one patient, the fistula was discovered i cidentally during operation. All patients received astulectomy and concomitant procedures were applied as follows ; 4 diverticulectomy, 4 right lower lobectomy, 1 bilobectomy, 1 left lower lobectomy and 1 wedge resection of left lower lobe. All but one patient were discharged without any complication and have been in good condition.

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A Clinical Study of Congenital Duodenal Obstruction (선천성 십이지장 폐쇄증의 임상적 고찰)

  • Huh, Young-Soo;Suh, Bo-Yang;Kwun, Koing-Bo
    • Journal of Yeungnam Medical Science
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    • v.7 no.2
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    • pp.39-48
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    • 1990
  • Congenital duodenal obstruction in the newborn infant may be due to a variety of causes. Duodenal obstruction often presents with bilious vomiting and upper abdominal distention. Diagnosis is usually established on plain x-ray of the abdomen by the classic finding of the double-bubble. In the period July 1986 to June 1990, 16 patients with congenital duodenal obstruction were operated and the following results were obtained. 1. Sixteen patients were comprised of 11 males and 5 females, the ratio of male and female was 2.2:1. 2. Thirteen patients(81%) had been admitted to our hospital during one month of life. 3. Congenital duodenal obstruction was in 16 cases; malrotation in eight(50%), annular pancreas in six(38%), type 1 atresia in one(6%), and wind-sock anomaly in one(6%). 4. There were two premature patients and six patients of small for gestational age. 5. Overall, bilious vomiting, occurring in three fourths, was the single most frequent presenting complaint. 6. Polyhydramnios occurred in two of the patients. 7. Diagnosis was possible with clinical symptom and simple abdomen. 8. The operative procedures performed were ; duodenoduodenostomy in five, duodenojejunostomy in two, excision of wind-sock membrane in one, and Ladd's procedure in eight. 9. A total of ten asociated congenital anomalies were found in six patients. 10. Postoperative complications occurred in five cases(31%).

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