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Background: Few references exist regarding contemporary complications of pyloromyotomy (PM) for infantile hypertrophic pyloric stenosis (IHPS). Therefore, we reviewed the frequency and outcome of patients with IHPS who developed complications from PM., Method: A 25-year retrospective review was performed in two populations. The first group included all infants who had a PM for IHPS at two pediatric surgery centers. The second group included all infants referred from other institutions who developed complications following PM for IHPS., Results: Between 1969 and 1994, 901 PMs were performed. Intraoperative complications occurred in 40 patients (4%), including 39 duodenal perforations and 1 difficult intubation requiring prolonged ventilation. No unrecognized duodenal perforations or incomplete PMs were found. Postoperative complications developed in 52 patients (6%). The wound infection rate was less than 1%. Postoperative vomiting occurred in 31 infants (3%). The mortality rate was 0.1%, with 1 death due to sepsis from delayed diagnosis of Hirschsprung's disease. During the same study period, 11 patients were referred from other hospitals for postoperative complications. Five had persistent vomiting treated successfully with expectant management. Six infants needed reoperation: 3 for persistent IHPS, 1 for gastric outlet obstruction, and 1 for small bowel obstruction secondary to adhesions; 1 required wound abscess drainage., Conclusion: Pyloromyotomy is not without complications. Duodenal perforation should be infrequent, but when it occurs, it can usually be readily recognized and treated with minimal morbidity. Postoperative vomiting can be managed nonoperatively, but if it persists longer than 5 days, radiologic evaluation should be performed. Incomplete PM is uncommon and should not occur. A second myotomy is needed when the diagnosis of incomplete myotomy is established. A single standard of care should be expected of all surgeons who perform PM for IHPS.

Congenital cystic adenomatoid malformation (CCAM) is a regional overgrowth of bronchioles with suppression of alveolar development in fetal and newborn lung. Twelve patients with CCAM were treated. Six premature infants had acute respiratory distress. Prenatal ultrasound was performed in only five patients and was abnormal in all five. Five premature neonates also had patent ductus arteriosus complicating their courses. Six older children presented with recurrent pneumonitis. Radiographs showed asymmetry of the chest and radiolucent masses in all 12 patients. Seven had type I lesions, two had type II lesions, and three had type III lesions. Hydramnios and hydrops were present in three, and hydrops alone was present in one of the six neonates. These four patients died. One other neonate died of respiratory failure and persistent fetal circulation. Seven patients survived for an extended period. Ultrasound makes the prenatal diagnosis of hydramnios and hydrops possible. It is in this group that fetal interventions can be considered. For the remaining patients, surgical intervention is indicated at the time of diagnosis.

Long-term follow-up of children with gastroschisis has been made possible by the increased survival of these infants over the past 20 years. We have observed that children with isolated gastroschisis defects exhibit normal growth and development beyond 5 years of age without significant bowel sequelae. Infants with gastroschisis with concomitant bowel atresia or complications who require small bowel resection are at a greatly increased risk for long-term bowel problems and abdominal complaints.

Appendicitis is a disease that continues to be characterized by a high morbidity rate that has changed little over the past 50 years. A significant proportion of patients (39 percent in this study) still present with advanced disease (gangrene, perforation, or abscess), as determined at operation. Duration of symptoms was the factor most closely associated with advanced disease. Patients with advanced disease had 88 percent of the morbidity. Primary care physicians referred patients who had symptoms for a longer period of time and who ultimately were found to have a more advanced stage of disease compared with patients who were referred from emergency rooms. This difference did not correlate with third party insurance coverage, as both referral groups exhibited a similar profile of coverage. In this study, the number of normal appendices removed was 5 percent. Early intervention remains the most promising means to reduce morbidity, mortality, and discomfort for the child and expense to the family or insurance carrier of a child with suspected appendicitis.

Neonatal necrotizing enterocolitis was seen in 42 of 5,030 infants (0.83 percent) admitted to our neonatal intensive care units. The condition developed more than 1 week after birth in most infants. This group included most of the very low birth weight infants. Forty-one of the 42 infants had enteral feedings before the onset of enterocolitis, which developed within 72 hours from the start of feedings in 19 infants (45 percent). Twenty-nine patients (69 percent) underwent surgery. Mortality in this group was 38 percent overall and 28 percent in the infants considered salvageable at laparotomy. The interval from diagnosis to surgery was 24 hours or less in 65 percent of cases, emphasizing the fulminant nature of the disease in many patients. Three of the 14 patients who died underwent no surgery. Pneumoperitoneum was one of the initial radiologic signs in 10 patients (24 percent). The necrotizing enterocolitis observed was similar clinically to that reported by others. The strikingly low incidence of enterocolitis in the neonatal intensive care units in Oregon (0.83 percent) contrasts with the generally reported incidence of 3 to 8 percent in other neonatal intensive care units. We attribute the low incidence of necrotizing enterocolitis in our experience to delay of enteral feedings and reliance on prolonged peripheral or central total parenteral nutrition, or both, for nutritional maintenance of stressed or low birth weight infants.

Colon replacement for the esophagus was used successfully in 20 of 21 pediatric patients. Intrathoracic placement of the colon segment was done in 18. Careful preparation and operative experience combined to reduce operative morbidity and mortality in an unfortunate group of patients. Satisfactory long-term results have been achieved. Careful technique, attention to detail, and perfection of a single technique may be more important than performing new surgical techniques when they are reported. Colon interposition performed by the Waterston technique utilizing the transverse colon is an effective means of substitution for a diseases esophagus in children.

The demographic data and the distribution of the extent of involved bowel in Hirschsprung's disease have remained unchanged over the past 25 years and are similar to those reported by others. The age at which the diagnosis was established decreased initially but increased in the last 10 year period. This increase has been accompanied by an increase in the number of breast-fed infants. Definitive operation for Hirschsprung's disease is now usually performed in children less than 18 months of age. The procedures used were selectively applied in the most recent 10 years. Arbitrary dependence on a single procedure is no longer appropriate and individualized judgment should be used in the application of specific treatment alternatives in the surgical management of Hirschsprung's disease. Mortality after definitive operation is now confined to problems other than Hirschsprung's disease. The patient with undiagnosed Hirschsprung's disease may not survive enterocolitis despite aggressive contemporary resuscitative treatment.

A retrospective study of 36 infants and children with diaphragmatic hernia was carried out. Mortality was confined to the group of patients identified between birth and 6 hours of life. Survivors appeared to be separable early in life from those who died on the basis of clinical status at birth measured by the Apgar, by ventilatory capacity of the lung reflected by carbon dioxide pressure and by acid base balance. Oxygenation, time from birth surgery, maternal factors, and labor and delivery appeared to play no role in survival. Hypoplasia of the lung reflected in low lung weights in those who died did not correlate with initial clinical status or blood gas data. Surgical adjuncts to reduction and closure of the hernia did not appear to have an effect on survival. Definition of patients at high risk of dying will permit critical application an evaluation of modes of treatment beyond those now used regularly in the care of these desperately ill infants.

Pancreatic resection was required in 11 pediatric patients over a 14 year period for control of persistent hypoglycemia. A standard surgical approach based on pathologic considerations was utilized. A subtotal distal pancreatectomy, liver biopsy, and removal of additional adenomatous tissue when found was performed in each patient. This approach precluded the need for routine preoperative visceral angiography, which is currently reserved for patients who require a second exploration. Surgery was curative in patients with adenomatous disease, although patients with diffuse pancreatic lesions usually required continued pharmacologic therapy postoperatively. Two patients thus far have had a total pancreatectomy performed at a second laparatomy due to persistent intractable disease. Seizure disorders, mental retardation, and behavioral problems frequently were unresponsive to improved control of hypoglycemia. An aggressive approach to management of this disorder is required if permanent neurologic damage is to be minimized.