Perioperative Management of Emergency Craniotomes in Children With Cyanotic Congenital Heart Disease: A Case Series.

While congenital heart disease is not uncommon, cyanotic congenital heart disease (CCHD) accounts for a minor fraction of them. However, when cyanosis is present, it usually indicates a severe or critical illness. Tetralogy of Fallot (TOF) is one of the common CCHDs, representing 7-10% of all congenital cardiac malformations. Double-outlet right ventricle (DORV) is another CCHD similar to the TOF and associated with decreased pulmonary flow, ventricular septal defect (VSD), and aorta receiving blood from both ventricles. Reduced oxygen arterial saturation and increased viscosity by polycythemia induce focal cerebral ischemia, often in the area supplied by the middle cerebral artery leading to brain abscess. Brain abscesses require craniotomy, which is a major surgery. These patients also often show features of sepsis and increased intracranial pressure. The presence of CCHD further complicates the situation, making perioperative management even more challenging. There are studies in the literature on the management of similar cases, and they report successful management in most of them. However, not all such cases need intensive postoperative management. We present four pediatric cases who had either TOF or DORV and had to undergo craniotomy for brain abscess or ventriculoperitoneal shunt placement. We describe case management and highlight the critical features and cases that require prolonged postoperative critical care management.