Solitary epidermoid splenic cysts: occurrence in sibs.

The case reports of two siblings with solitary epidermoid cysts of the spleen are presented. This improbable chance occurrence raises the possibility of a transmissible genetic defect accounting for the formation of these cysts.

The mortality of congenital diaphragmatic hernia. Is total pulmonary mass inadequate, no matter what?

Much progress has been made over the past 20 years in neonatal surgery, but the mortality rate in cases of congenital diaphragmatic hernia (CDH) for babies presenting in the early hours of life has remained high. We have reviewed our experience with special reference to 40 autopsied patients. Hypoplastic lungs were seen in all patients. When the ratio of observed combined lung weight to the expected combined lung weight is calculated, the result is 0.33 +/- 0.17 when the expected lung weights are calculated from the babies' birth weight; and 0.36 +/- 0.17 when the expected lung weights are calculated from the gestational age. In all patients with high ratios, extensive pneumonia was confirmed. Those patients with pneumonia were eliminated from the calculations. We cannot state unequivocably that hypoplasia of both lungs is the cause of death in all patients with CDH. We can affirm that, in our experience, hypoplasia is present and is probably a major factor in the high mortality rate.

Congenital posterolateral diaphragmatic hernia: a retrospective study.

Congenital posterolateral diaphragmatic hernia continues to be associated with a high mortality from ventilatory failure. To evaluate the malformation and factors associated with survival, 43 children with congenital diaphragmatic hernia seen in the neonatal period were studied; 20 survived. The male:female ratio was 2.1. No consistent antenatal factors could be implicated, and, except for intestinal malrotation and lung hypoplasia, associated anomalies were uncommon. The most common cause of death was ventilatory failure and marked bilateral lung hypoplasia was a common autopsy finding. Most of those died were admitted within 8 hours of birth. Tolazoline has been used in 10 patients since 1975 and, of these, 4 survived. Postoperatively a transient period of satisfactory lung function correlated well with response to vasodilators, which may be of value in future therapy.

Congenital diaphragmatic hernia: pathophysiology and pharmacologic support.

The incidence of death from congenital diaphragmatic hernia appears to be unchanged in recent years despite advances in resuscitation, transport, and ventilatory support. Bilateral lung hypoplasia and abnormal pulmonary vascular reactivity as developmental consequences of the defect appear to play a major role in the continued high mortality rate. Recent advances in pharmacologic support have further elucidated the mechanisms of ventilatory failure in these patients and may represent means of improving survival in the future.

Vasodilator response and prediction of survival in congenital diaphragmatic hernia.

In an effort to find an accurate prediction of survival in congenital diaphragmatic hernia (CDH) patients with severe respiratory distress and persistent pulmonary hypertension, we reviewed the response to vasodilators and te clinical course of 13 patients with this condition. All patients had been operated upon within the first 24 hr of life and had postoperative descending aortic PaO2 values less than or equal to 50 torr on maximum ventilatory support. The only significant change in standard blood gas values after an intravenous test dose of 2 mg/kg of tolazoline was in the PaO2 (p less than 0.05). Patients with good response (PaO2 increase greater than 100 torr) survived. Patients with fair response (PaO2 increase between 20 and 100 torr) had equivocal survival. Patients with poor response (PaO2 increase less than 20 torr) died. There was a postoperative period up to several weeks of marked fluctuations in arterial oxygen tension in survivors before stability occurred. Vasodilator responsiveness appears to be an accurate, therapeutic indicator of the chances for survival in CDH patients with persistent pulmonary hypertension and right to left shunting.

Tolazoline therapy for persistent pulmonary hypertension after congenital diaphragmatic hernia repair.

To determine whether vasodilators are useful in persistent pulmonary hypertension associated with congenital diaphragmatic hernia, we reviewed the clinical course, laboratory data, and outcome of 37 patients with respiratory distress and diaphragmatic hernia requiring an operation before 24 hours of life. These patients were divided into two groups, Group I (n = 17) included patients treated prior to the use of tolazoline; Group II (n = 20) included those treated after tolazoline became available. Postoperative severe respiratory distress was observed in ten patients in Group I, and all died. In Group II, 16 patients had severe postoperative respiratory distress and four survived; 12 of these 16 patients received tolazoline, including all four survivors. Treated survivors had significantly higher increase in Pao2 after a test dose of tolazoline than did nonsurvivors. A transient "honeymoon period" of adequate oxygenation correlated with good response to tolazoline, and the presence of both was predictive of survival. No patient survived with the combination of no "honeymoon period" and no response to tolazoline, whereas response to tolazoline without a honeymoon period was sometimes followed by survival. All nonsurvivors had severe lung hypoplasia at autopsy.

Esophageal atresia with tracheoesophageal fistula: end-to-end versus end-to-side repair.

Despite early enthusiasm by some authors for the end-to-side repair of esophageal atresia with tracheoesophageal fistula, many have returned to the end-to-end technique. The present study compares the results of these two operative procedures. A retrospective analysis was made of 52 consecutive cases in which primary repair was performed. The patients were divided according to the three preoperative risk groups descibed by Waterston. The mortality for the end-to-end and end-to-side repairs was similar in each of the three risk groups. Similarly, there was no significant differnences in the incidence of anastomotic leak or recanalization of the fistula. However, the rate of anastomotic stricture in the end-to-end group was significantly higher (p less than 0.001) than in the end-to-side group. A description of the end-to-side technique is given, and its advantages are outlined.

Necrotizing enterocolitis (ischemic enteropathy) with the sequel of colonic atresia.

Two cases of acquired colonic atresia subsequent to necrotizing enterocolitis (ischemic enteropathy) in the neonatal period are reported. These infants were noted to have complete atresia of the colon in the region of the splenic flexure 8 and 11 weeks after small bowel resection and ileostomy formation. Recognition of this and other late sequelae of ischemic enterocolitis becomes critical to the successful outcome of these patients as survival rates increase.

Supportive use of total peripheral parenteral alimentation in children with severe pancreatic injuries.

Although blunt abdominal trauma is common during childhood, experience with major pancreatic injuries is rare. Three such cases were recently treated at the Montreal Children's Hospital. Because of the complicated problems related to these injuries, these children needed prolonged nutritional support. Total peripheral parenteral nutrition was used to provide complete rest of the pancreas and avoid the risks of sepsis associated with central venous feeding. This technique enables the maintenance of caloric intake and weight gain during difficult complications and was instrumental in the early complete recovery of these children.