Massive foetal pericardiomegaly causing pulmonary hypoplasia, associated with intra-pericardial herniation of the liver.

The clinical features, investigation, treatment and outcome of four newly born babies with the following recognisable triad of findings are presented: Bilateral pulmonary compression with or without hypoplasia. Massive pericardial effusion without cardiac compromise. An intrapericardial hernia containing part of the liver. The primary event in the causation of this triad is a congenital defect in the central tendon of the diaphragm. Compromised hepatic venous outflow involving the herniated part of the liver is the postulated origin of the fluid within the pericardium (Budd-Chiari-like effect). Although rare, this triad is clinically identifiable. Sonar imaging clinches the diagnosis. Surgical correction is simple but the prognosis depends on the presence of pulmonary hypoplasia which caused death in two cases and on other described lethal associated anomalies which were not encountered in the reported patients.

The effect of a heat and moisture exchanger on gas flow in a Mapleson F breathing system during inhalational induction.

Heat and moisture exchangers (HMEs) humidify, warm and filter inspired gas, protecting patients and apparatus during anaesthesia. Their incorporation into paediatric anaesthetic breathing systems is recommended. We experienced delays in inhalational induction whilst using a Mapleson F breathing system with an HME. We have demonstrated that the HME significantly alters gas flow within the breathing system. Approximately half of the fresh gas flow is delivered to the patient, the remainder being wasted into the expiratory limb of the breathing system. We suggest that the HME should be removed from the Mapleson F breathing system until inhalational induction is complete, or that the reservoir bag is completely occluded until an effective seal is obtained with the mask.

Megacystis - microcolon - intestinal hypoperistalsis syndrome.

Two non-related female neonates with the megacystis-microcolon-intestinal hypoperistalsis syndrome are described. One presented with a family history of a similar condition. In one child no intestinal peristalsis was observed, while in the other decreased peristalsis with occasional rectal evacuation occurred. Both had fatal outcomes. These two cases demonstrate the wide variation in the clinical course of this rare and fatal syndrome.