Perioperative Mx of a neonate with Cantrell's pentalogy
Pentalogy of Cantrell (POC) is a rare congenital anomaly with multiple defects involving sternum, pericardium, diaphragm, and anterior abdominal wall. Survival of these babies depends on associated ectopia cordis and intracardiac anomalies. Published in the Indian Journal of Anaesthesiology, the authors report the perioperative management of a newborn with POC for surgical repair of omphalocoele.

A 5-day-old 2.5 kg male child with thoraco-abdominal defect was referred to our institute for surgical management. Parents deferred termination of pregnancy when the antenatal scan was suggestive of POC, and the baby was delivered at term by Caesarean section.

The child had midline defect extending from the mid-sternum to umbilicus with the heart outside the thoracic cavity and bowel loops prolapsing through the defect. However, there was no respiratory distress or cyanosis at birth. The child was referred on day four of life and was found to have dehydration, hypothermia, and elevated renal parameters (Urea – 110 mg/dl, creatinine – 2.5 mg/dl).

The child was resuscitated with intravenous fluids, dopamine, and dobutamine infusions at 5 μg/kg/min. Echocardiography revealed pulmonary atresia, patent ductus arteriosus (PDA), and major aortopulmonary collateral arteries (MAPCAs).

The child was scheduled for omphalocoele repair under general anaesthesia. In the operating room, the child had a baseline heart rate of 145/min, blood pressure of 60/36 mmHg, and room air saturation was 94%. Anaesthesia was induced with intravenous fentanyl 5 μg, ketamine 6 mg, and atracurium 1.5 mg.

Trachea was intubated with 3.5 mm inner diameter uncuffed endotracheal tube. The right femoral artery was cannulated for continuous blood pressure monitoring. Anaesthesia was maintained with sevoflurane in O2/air with FiO2 50–60%. The lungs were ventilated with peak inspiratory pressure of 16 cmH2O. The diaphragmatic defect was repaired and omphalocoele was covered with mobilized skin. There was no change in peak airway pressure.

Total duration of surgery was 40 min; blood loss was minimal, urine output was 3 ml, and the child received 15 ml of crystalloid. The child was shifted to neonatal intensive care for further management. Eventually, the sepsis and kidney injury worsened and the child expired on the fourth postoperative day.

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Dr. S●●●●●a P●●●●●a
Dr. S●●●●●a P●●●●●a Anaesthesiology
Very sad.thorough ANC is must.
Oct 17, 2018Like