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Gastroschisis complicated by intestinal atresia is a complex problem. Six cases are presented. A review of the literature and of our own cases shows a high mortality rate. Success or failure is related more to the pathology present than to any specific method of operative management. Resection and primary anastomosis is the favored method of treatment, as the intestine heals well in spite of its appearance. Primary closure of the abdominal wall musculature and skin is done whenever possible. A gastrostomy is used uniformly. Intravenous hyperalimentation is critical to survival of these babies and should be used early. The use of this therapeutic modality allows for the onset of gastrointestinal function spontaneously (often over prolonged periods of time) without nutritional deprivation. Intestinal atresias almost always are easy to identify in babies with gastroschisis. Extensive dissection and mobilization of this friable intestine is contraindicated in those babies in whom an atresia is not obvious but only suspected. In such cases the gastroschisis defect should be managed by whatever method is deemed appropriate and the baby observed while receiving intravenous nutritional support. If an atresia is present, it can be managed later in the baby's course by resection and primary anastomosis.

The anterior fundoplication described by Thal has been used in treating gastroesophageal reflux surgically in 362 children at The Children's Mercy Hospital and at St. Luke's Hospital in Kansas City, Missouri, because medical therapy had failed or was inappropriate. Long-term results have been evaluated in regard to relief of reflux and relief of symptoms attributed to reflux. Of the 335 patients followed from 1 to 8 years, 90% had a satisfactory initial result. Five percent required reoperation for a recurrence of reflux due to failure of the fundoplication or development of a hiatus hernia. All recurrences developed with 5 months of the initial operation. Fifteen of 335 patients (4.5%) had persistent symptoms despite correction of the gastroesophageal reflux; in these patients, attributing the symptoms to reflux was incorrect. There were no deaths in this series of patients as a result of operation. The success rate of the Thal fundoplication in children compares favorably with that of the Nissen Fundoplication.

One hundred consecutive patients with esophageal atresia or tracheoesophageal fistula, or both, were treated at The Children's Mercy Hospital during the past 14 years. Each patient was evaluated and a plan for therapy was formulated depending on the type and urgency of concomitant disease. Healthy patients were treated by primary repair. Those with respiratory disease were treated before repair by gastrostomy, upper pouch suction, and antibiotics. In the small premature infant and the patient with major associated anomalies, repair of the esophageal atresia and tracheoesophageal fistula was postponed by gastrostomy, upper pouch suction, and parenteral nutrition while the concomitant disease was treated. Fifteen patients with life-threatening associated anomalies required operation before esophageal repair and 13 of them survived. During the initial hospitalization two deaths related to esophageal atresia occurred, whereas three prerepair and two late (2 1/2 and 3 months) postrepair deaths related to associated anomalies occurred. Thus, 93 of the 100 patients left the hospital alive. The increased survival is due not only to improved care of esophageal atresia and tracheoesophageal fistula, but also to a more aggressive approach to the treatment of the serious associated anomalies.

Reaction of an isolated, unruptured sinus of Valsalva aneurysm is described. Detection was serendipitous in a patient with previous surgical resection and grafting of significant aortic coarctation. The philosophy and importance of proceeding with elective resection in these progressive lesions is emphasized.