John C. German

ESOPHAGEAL ATRESIA AND ASSOCIATED ANOMALIES

One hundred and two patients with esophageal atresia possessed 237 additional anomalies. Risk grouping of these patients was useful and showed a direct relationship between the increased incidence of anomalies, low birth weight, and nonsurvival. Fifty-seven per cent of the patients with additional anomalies possessed multiple anomalies, most frequently occurring in the gastrointestinal, cardiovascular, and musculosketetal organ systems. Fifty-five per cent of the deaths appeared to result from the coexistent severe anomaly, while the remaining 45% of the deaths appeared to occur as a result of the presence of esophageal atresia. It is our suspicion that the insult producing esophageal atresia with a high incidence of associated anomalies occurs at a more critical time in organogenesis than that event that produces the esophageal atresis alone or with one moderate anomaly.

Colon interposition for the replacement of the esophagus in children

Thirty-two new cases of colon interposition for esophageal reconstruction are presented. Only one late death occurred in this series, which was unrelated to the colon interposition. Fifteen severe complication were observed in five patients with preexisting esophageal atresia and previously complicated courses following disastrous primary esophageal repairs. The most common complication was pneumonia which occurred 13 times. Five late strictures responded well to dilatation or revision. Long-term weight gain was compared between a group of colon transplant patients and a group of patients with repaired esophageal atresia. Although the esophageal atresia group followed the 25th percentile and the colon interposition group followed the 3rd percentile on the growth curve, no significant difference existed in weight gain over 12 yr.

Management of pulmonary insufficiency in diaphragmatic hernia using extracorporeal circulation with a membrane oxygenator (ECMO)

Persistent fetal circulation (PFC) causes severe pulmonary insufficiency in patients who have demonstrated adequate lung function following diaphragmatic hernia repair. Patent ductus arteriosus (PDA) ligation corrects this condition, but carries the risk of sudden right ventricular failure. Pharmacologic reversal of PFC may be attempted, and if unsuccessful, prolonged venoarterial bypass becomes necessary to provide effective pulmonary support. PDA ligation can then be performed safely and maturation of the pulmonary vasculature allowed to occur. Pulmonary artery pressure monitoring is essential.

Technical aspects in the management of the meconium aspiration syndrome with extracorporeal circulation

Sixteen neonatal patients diagnosed as having the meconium aspiration syndrome were selected for management with extracorporeal circulation with a membrane oxygenator (ECMO) with 8 survivors over 4 yr. All patients weighed greater than 2 kg. Each was placed in the 100% mortality group according to a Neonatal Pulmonary Insufficiency Index (NPII) based on hourly pH and FiO2 determinations. The typical patient course on ECMO was stabilization for the first 12 hr then improvement on high bypass flow rates for 12-24 hr to maintain a pAO2 for 50-60 mm Hg with minimal ventilator settings with an FiO2 of 0.3-0.4. Bypass flow rates were reduced to maintain adequate pAO2 with similar ventilator settings for another 24 hr. Survivors were taken off bypass and decannulated while on similar ventilator settings. Nonsurvivors did stabilize or improve but usually exhibited symptoms of intracranial hemorrhage by 48 hr. Intracranial hemorrhage appeared to be related to the degree of prebypass acidosis. Successful ECMO support reduced the expected mortality from severe meconium aspiration from 100% to 50%. Early institution of ECMO, before acidosis worsens, seems to be indicated to reduce the morbidity of conventional ventilator management and to prevent intracranial hemorrhage from severe prebypass acidosis. Long term followup indicates that these patients have progressed satisfactorily according to developmental testing for as long as 4 yr.

Prospective application of an index of neonatal necrotizing enterocolitis.

Fifty-five patients were scored 1 to 3 according to the criteria: the character of stools, abdominal findings on palpation, x-ray evidence of pneumatosis intestinalis, the development of pulmonary insufficiency, and the duration of symptoms to positive x-rays. Fifteen patients with scores of less than five were considered to have subclinical NEC with one late death. Twenty-nine of 30 patients with scores of 5-10 responded to medical management with 2 deaths related to recurrent bouts of sepsis without recurrent NEC. Eleven patients required surgery with index scores of 10-14 with 6 deaths occurring uniformly in those patients with scores of 12 or more. Two patients were scored inappropriately low due to the lack of the passage of a stool for analysis. One patient with a score of 4 did not pass a stool but had the other diagnostic criteria for the single false negative of the series. This index correctly determined the severity of NEC of 53 of 55 patients, identified the patients who required surgical intervention and predicted survival.

Pulmonary artery pressure monitoring in persistent fetal circulation (PFC)

The use of the Swan-Ganz catheter to monitor pulmonary artery pressure in adults with cardiopulmonary failure has become commonplace. Our meager experience was with four neonates diagnosed as having persistent fetal circulation monitored by the use of this PA catheter. Tolazoline was infused directly into the pulmonary circulation via the catheter. Pulmonary artery pressure was temporarily reduced by tolazoline administration, with a marked increase in PaO2. More experience is required to define the role of the Swan-Ganz catheter in neonatal physiologic monitoring.

Computer utilization for intravenous nutrition in surgical neonates preliminary report

A program was developed to store important numerical clinical data such as daily weights, intravenous caloric intake, and laboratory test results on surgical neonates who required total intravenous nutrition. Preliminary experience with the data from the first 5 surgical neonates requiring intravenous nutrition indicated that this data processing is easily achieved. Data retrieval by the development of output programs provided rapid access to stored records, rapid data tabulation, and graphic print-out of parameters, such as intravenous calories per kilogram per day and weight gain, that can be placed in the patients record.

The twin with esophageal atresia.

A retrospective analysis of esophageal atresia occurring in patients who are members of a twin set indicated that twinning does occur more frequently in patients with esophageal atresia. These patients tend to be small for date, but have a similar occurrence of associated anomalies as singletons with esophageal atresia. Applying risk-grouping to the entire series and twins indicated no real difference in survival or additional anomalies by organ system, except more cardiovascular anomalies occurred in twins. Long-term follow-up of five survivors revealed severe growth retardation.