Congenital bronchoesophageal fistula is a rare clinical entity in adults. This anomaly may cause various symptoms such as respiratory infections, coughing bouts when eating or drinking, and even hemoptysis. The fistula can cause symptoms in childhood but may not appear until adulthood. We recently experienced a case of congenital bronchoesophageal fistula associated with esophageal diverticulum in an adult. A 63-year-old woman was admitted to our hospital due to chest discomfort, sore throat and coughing bouts when eating. An empyema with lung abscess had occurred eight years previously. Results of the physical examination were unremarkable. A Barium swallowing revealed a medium-sized diverticulum at the right anterior aspect of the esophagus, which had developed a fistulous connection with the right lower lobe bronchus. The patient was treated by fistulectomy and lobectomy of the right lower lobe. The postoperative course was smooth and uneventful.
Bronchial Diseases/congenital* ; Bronchial Diseases/complications* ; Case Report ; Diverticulum, Esophageal/complications* ; Esophageal Diseases/congenital* ; Esophageal Diseases/complications* ; Female ; Fistula ; Human ; Middle Age

BACKGROUND: During the past few years, transesophageal echocardiography(TEE) became one of most impartant method to detect various cardiac disease. But it was performed less than 10% of transthoracic echocardiography(TTE) in most of echocardiographic laboratoties because of major and minor complicatios associated with this procedure. The purposes of this study are to evaluate the clinical applications and the usefulness of transesophageal echocardiography in various cardiac disorders. METHOD: From 1990 to 1995, 942 cases were included in this study. All subjects were taken TTE and TEE simultaneously The causes of request, related side effect, cause of failed cases, and the benefit of TEE compared to TTE were analysed. The benefit of TEE were classified into major and minor benefit. Major benefit was defined as to diagnose the disease or to change the therapeautic method by TEE. Minor benefit was defined as to have additional findings by TEE whic could not be enrolled in major benefit. RESULTS: 1) The success rate of TEE was 95.5%(900/942). In 42 failed cases(4.5%), 19 were interrupted procedure earlier, 16 were failed to insert the TEE probe, 4 cases showed arrhythmia, and 3 cases had esophageal stricture or varices. 2) The causes of request were to assess the prosthetic valve function(35.5%), cardiac source of embolism(23.7%), valvular heart disease(22.7%), congenital heart disease(10.2), aortic disease(2.7%), endocarditis(2.3%), cardiac mass(1%), and coronary artery disease(1%), in orders. 3) The benefit of TEE was 77%, congenital heart disease was revealed greatest benefit(97%), but major benefit was highest for the evaluation of cardiac source of emboli(100%). CONCLUSION: TEE was very useful method for the detection of intra-cardiac and aortic abnormalities. TEE is a relatively safe procedure but it is a semi-invasive method and has few complications. So it is wise to apply TEE as adjuvant method of TTE.
Arrhythmias, Cardiac ; Coronary Vessels ; Echocardiography ; Echocardiography, Transesophageal ; Esophageal Stenosis ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Methods ; Varicose Veins

OBJECTIVETo introduce the technique of intrathoracic surgery performed through vertical transaxillary minithoracotomy.

METHODSFrom March 1989 to March 2001, 316 patients underwent intrathoracic surgery through a vertical transaxillary minithoracotomy. 285 patients suffered from patent ductus arteriosus (PDA), 10 congenital esophageal atresia, 8 congenital pulmonary cysts, 6 congenital emphysema, 1 pulmonary sequestration, 5 mediastinal tumor, and 1 eventration of the diaphragm.

RESULTSAll of the patients were successfully treated under satisfactory exposure. No operative mortality and severe postoperative complications occurred.

CONCLUSIONSIntrathoracic surgery performed through a vertical transaxillary minithoracotomy appears to be less invasive, and is a simple, safe, cosmetically acceptable and efficient approach.

Adolescent ; Axilla ; Child ; Child, Preschool ; Cysts ; congenital ; surgery ; Ductus Arteriosus, Patent ; surgery ; Esophageal Atresia ; surgery ; Female ; Humans ; Infant ; Infant, Newborn ; Lung Diseases ; congenital ; surgery ; Male ; Pulmonary Emphysema ; congenital ; surgery ; Thoracotomy ; methods

Congenital hepatic fibrosis is an inherited, congenital disorder of the liver characterized by portal hypertension and hepatic fibrosis. We experienced a case of congenital hepatic fibrosis with esophageal varix in a 9-year-old male. He complained hematemesis, hematochezia, dizziness. In laboratory examination, AST/ALT was slightly increased. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and hypoechoic lesion of periportal area were seen by abdominal CT scanning. Histologic finding of liver biopsy showed fibrous tracts containing dilated bile ductules connecting adjacent portal spaces that were widened by mature fibrosis. Endocopic sclerotherpy and ligation was done. We summarized a case with review of literatures.
Bile ; Biopsy ; Child ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Dizziness ; Esophageal and Gastric Varices* ; Fibrosis* ; Gastrointestinal Hemorrhage ; Hematemesis ; Humans ; Hypertension, Portal ; Ligation ; Liver ; Male ; Tomography, X-Ray Computed

Congenital hepatic fibrosis is an inherited, congenital disorder of the liver, and is occasionally associated with cystic disease of the liver and kidney. We present a case of congenital hepatic fibrosis with Caroli's disease. A 21-year-old woman had suffered from an episodic fever with headaches for 3 years. In laboratory examination, the liver function test was within the normal limits. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and multiple dilated bile ducts were seen by abdominal CT scanning. An orthotopic whole liver transplantation was done. The liver was fibrotic and enlarged. Multiple cystically dilated intrahepatic ducts were noted. Microscopically, diffuse portal fibrosis and widening with proliferation of bile ductules were seen. Intrahepatic bile ducts were markedly dilated and tortuous. The liver cell cords were well preserved.
Bile ; Bile Ducts ; Bile Ducts, Intrahepatic ; Caroli Disease* ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Esophageal and Gastric Varices ; Female ; Fever ; Fibrosis* ; Headache ; Humans ; Kidney ; Liver ; Liver Function Tests ; Liver Transplantation ; Tomography, X-Ray Computed ; Young Adult

A study was conducted to present clinical and statistical analysis of pediatric patients admitted to Severance Hospital between Jan. 1966 and Dec. 1975. Remakable progress has been made in the field of pediatrics and pediatric surgery, particularly in physiology, anesthesiology and operative technique. However, the ultimate result hasn't been totally satisfactory, largely due to the lack of cognizance of early diagnosis and prompt treatment. Futhermore, most of the patients with associated abnormal conditions, such as congenital heart disease, other associated G.I. anomalies and chromosomal anomalies, require surgery, urgently A total of 250 cases were analyzed according to systems. The following results were obtained; 1. 1. In the sex ratio male to female was 6-7:1. 2. 2. The order of frequency of diseases was as follows; Congenital megacolon 50 cases(20.0%), Imperforated anus 45 cases (18.0%), Congenital hypertrophic pyloric stenosis 41 cases(16.4%), Congenital anomalies of intestine 29 cases (11.6%), Congenital anomalies of hepatobiliary system 25 cases(10.0%), Meckel's diverticulum 18 cases(7.2%), Situs inversus 11 cases (4.4%), Congenital diaphragmatic hernia 6cases(2.4%), Congenital anomalies of cecum 6 cases(2.4%), Esophageal diverticulum 6 cases (2.4%), Esophagealatresia 4 cases (1.6%), Congenital anomalies of pancreas 4 cases (1.6%), Patent omphalomessentric duct 2 cases (0.8%), Congenital intestinal perforation 2 cases (0.8%), and Congenital stomach perforation 1 cases (0.4%). 3. There was an overall mortality rate of 36 cases (18.9%), the causes of death were frequently associated with respiratory failure. 4. Associated congenital anomlies were found in 57 cases. They were G. I. Anomalies, congenital heart diseases, Down's syndrome and cleft palate. 5. Of significance was the fact in this study, congenital megacolon had the highest incidence which was in sharp contrast to accidental series in which congenital hypertrophic pyloric stenosis showed the peak incidence. These figures are very similar to that reported in various publications. This analysis suggest that a more keen understanding of neonatal physiology, embryology, surgical technique and improved post-operative care for anomalous patients, may certainly improve the ultimate outcome of corrective surgery.
Anal Canal ; Anesthesiology ; Cause of Death ; Cecum ; Cleft Palate ; Digestive System* ; Diverticulum, Esophageal ; Down Syndrome ; Early Diagnosis ; Embryology ; Female ; Heart Defects, Congenital ; Heart Diseases ; Hernia, Diaphragmatic ; Hirschsprung Disease ; Humans ; Incidence ; Intestinal Perforation ; Intestines ; Male ; Meckel Diverticulum ; Mortality ; Pancreas ; Pediatrics ; Physiology ; Pyloric Stenosis, Hypertrophic ; Respiratory Insufficiency ; Sex Ratio ; Situs Inversus ; Statistics as Topic* ; Stomach

PURPOSE: Perinatal mortality rates have been used as a summary statistic for evaluating child health and medical status. Neonatal mortality rates have decreased over the past 30 years in Korea. To understand the current status of neonatal surgical gastrointestinal diseases in Daegu?Busan area, we have studied about neonatal gastrointestinal diseases with their clinical features, postoperative outcome, and mortality rates. METHODS: A clinical analysis on 202 neonates who underwent neonatal surgery from January 1996 to July 2003 at Pusan National University, Kyungpook National University, Youngnam University, and Daegu Catholic University was carried out. RESULTS: The main diseases of surgical conditions were anorectal malformation (23.8%), atresia/ stenosis of midgut (13.4%) and pyloric stenosis (13.4%). The male to female ratio was 2.8:1. Thirty-five cases (17.0%) had one or more associated anomalies including congenital heart disease, cryptoorchidism, hydronephrosis, and chromosomal anomaly. Twenty cases (10.0%) were diagnosed by antenatal ultrasound. Patients with esophageal atresia had the longest hospitalization for 54.6 days. Postoperative complications occurred in 18 cases (8.9%). The main postoperative complications were wound infection (3.5%) and anastomotic leakage (2.5%). Overall mortality was 5.9%. Diaphragmatic hernia showed the highest mortality rate (37.5%), and esophageal atresia (28.6%) and omphalocele (20.0%) were followed. CONCLUSION: The current status of neonatal surgical gastrointestinal diseases in Daegu?Busan area has improved because the disease categories are various, postoperative complications and mortality rates are decreased.
Anastomotic Leak ; Busan ; Child ; Child Health ; Constriction, Pathologic ; Daegu ; Esophageal Atresia ; Female ; Gastrointestinal Diseases* ; Gyeongsangbuk-do ; Heart Defects, Congenital ; Hernia, Diaphragmatic ; Hernia, Umbilical ; Hospitalization ; Humans ; Hydronephrosis ; Infant ; Infant Mortality ; Infant, Newborn ; Korea ; Male ; Mortality ; Perinatal Mortality ; Postoperative Complications ; Pyloric Stenosis ; Ultrasonography ; Wound Infection