There are considerable controversies in the management of congenital diaphragmatic hernia. By 1997, early operation, routine chest tube on the ipsilateral side and maintainingrespiratory alkalosis by hyperventilation were our principles (period I). With a transition period from 1998 to 1999, delayed operation with sufficient resuscitation, without routine chest tube, and permissive hypercapnia were adopted as our practice. High frequency oscillatory ventilation (HFOV) and nitric oxide (NO) were applied, if necessary, since year 2000(period II). Sixty-seven cases of neonatal Bochdalek hernia from 1989 to 2005 were reviewed retrospectively. There were 33 and 34 cases in period I and II, respectively. The neonatal survival rates were 60.6 % and 73.5 %, respectively, but the difference was not significant. In period I, prematurity, low birth weight, prenatal diagnosis, inborn, and associated anomalies were considered as the significant poor prognostic factors, all of which were converted to nonsignificant in period II. In summary, improved survival was not observed in later period. The factors considered to be significant for poor prognosis were converted to be nonsignificant after change of the management principle. Therefore, we recommend delayed operation after sufficient period of stabilization and the avoidance of the routine insertion of chest tube. The validity of NO and HFOV needs further investigation.
performed at the time of discharge. There was only one recurrence of adirect inguinal hernia. Necrotizing enterocolitis developed in 17 patients, 11 were operated upon, two had peritoneal drainages, and 9 had enterostomies. Five of 11 surgical infants died after operation and three of the nonsurgical infants died of various complications. Although micropremies have potentially high risks of serious complications and death, the outcome can improve with careful surgical observation and judgment.
This study reviews 14 years' experience of esophageal atresia with special emphasis on the clinical profile and the outcome. From May 1989 to February 2003, 65 cases of esophageal atresia (EA) were treated at Asan Medical Center. Boys outnumbered girls 2.4 to 1. Prematutity and low birth weight were 27.7% and 38.5%. Esophageal atresia with distal tracheoesophageal fistula (TEF) was the most common type (87.7%), followed by pure EA and H type fistula. Forty-six patients (70.8%) had one or more associated anomalies, cardiac malformations were the most common. Duodenal atresia was found in 7 cases. There were 6 patients (9.2%) with VATER cluster. VACTERL cluster was present in 18 patients (27.7%), one of who fulfilled the complete syndrome. Waterston group A, B and C made up 21.5%, 40.0% and 38.5% of the total group. Surgical treatment was attempted in 63 patients and deferred in 2 who had severe associated malformations. For EA with distal TEF, primary esophago-esophagostomy was carried out in 51 cases, and division of TEF and gastrostomy in 4 cases and no operation in 2 cases. For pure EA, colonic graft was done in 2 after gastrostomy and esophagostomy, and esophago-esophagostomy was performed in 2 after gastrostomy. Two TEF was carried out in 2 cases with H type TEF. The overall survival rate was 76.9%, and survival by Waterstuon classification was 100% in group A, 80.8% in B and 60.0% in C. Thorough workup for associated anomalies, interdepartmental approach and more careful surgical decision and technique are required to improve the outcome of EA.
Necrotizing enterocolitis (NEC) is usually a disease of premature infants, but occasionally it affects the term neonate. Twenty-five infants with NEC were treated at Asan Medical Center between January 2000 and December 2002. and 13 of them were term infants. In each case, the diagnosis of NEC was established by a clinical illness fulfilling the Bell's stage II or III NEC as modified by Walsh or by surgical findings. There were six males and seven females. The birth weight was from 1,960 to 3,700 g. The age at diagnosis was from 1 to 40 days. Four patients had congenital heart disease; one of them had hypothyroidism and cleft palate. Abdominal distension was present in all, and bloody stools in four. One patient had history of hypoglycemia, three had Rota viral infection. Eight patients had leucopoenia (<5.0 × 109/L), Seven had thrombocytopenia (<100 × 109/L), and three severe thrombocytopenia (<50 × 109/L). Laparotomy was required in 10 of the 13 patients. Indications for operation in acute phase were failure to respond to aggressive medical therapy in five, and perforation in three patients. There were two late phase operation for intestinal stricture and fistula. There was no operative complications. Ten of thirteen patients survived (76.9%). Two patients died of septic complication. There was a delayed death due to heart failure. There was a significant difference in survival according to platelet count (50 × 109/L) (p<0.05). Congenital heart disease and Rota viral infection is associated with NEC in term infants and thrombocytopenia and leucopenia in the course may be surgical indications.
Diagnosing Hirschsprung's disease is a clinical challenge. Hirschsprung's disease should be considered in any child who has a history of constipation dating back to the newborn period. We examined diagnostic methods and their results retrospectively in 37 neonates and infants who underwent both barium enema and anorectal manometry for the diagnosis of Hirschsprungs disease at Asan Medical Center between January 1999 and April 2001. Two radiologists and a surgeon repeatedly reviewed both of the diagnostic results. In anorectal manometry, thirty-four studies were in agreement with the definitive diagnosis, giving an overall diagnostic accuracy of 91.9 % (neonate; 100 %, infant; 85.7 %). The accuracy and specificity of barium enema was lower than those of anorectal manometry, but sensitivity was higher. There was no significant difference between the two methods. Both studies showed findings consistent with the final diagnosis. However, discordant results needed further evaluation or close observation to diagnose accurately. We conclude that Hirschsprungs disease should not be diagnosed by only one diagnostic method.