Anaesthetic management of an infant with severe protein C deficiency and septal cardiac defects—a case report

Background
Protein C deficiency is a rare genetic disorder with varying severity of symptoms and disease. The disorder may vary in presentation from a complete symptomless state to a less severe form like venous thromboembolism. The most severe form of disease is a rare condition called neonatal purpura fulminans (NPF) which is characterized with sudden progressive dermal hemorrhage and necrosis due to vascular thrombosis and disseminated intravascular coagulation. In contrast, congenital atrial and ventricular septal defects are the commonest congenital heart diseases found in pediatric population. An infant presenting with systemic vascular thromboembolism secondary to protein C deficiency along with the cardiac septal defects posted for surgery will be a very challenging task to manage in perioperative period. Also, physiological mechanisms during perioperative period and surgery will promote thromboembolism leading to worsening of the situation further. So, perioperative management of such patient pose a great challenge to the anaesthesiologist. Due to rarity of the condition, there is very limited literature available.
Case presentation
We report the perioperative management of a 2-month-old child suffering with neonatal purpura fulminans with atrial and ventricular septal cardiac defect, scheduled for bilateral foot amputation. The patient was a diagnosed with complete occlusion of abdominal aorta leading to foot gangrene. After initiation of anticoagulant therapy, symptoms were relieved and patient was posted for amputation of gangrenous feet.
Conclusions
There could be an increased risk of thromboembolism and bleeding due to protein C abnormality along with the chances of shunt reversal, paradoxical embolism, and other cardiac morbidities secondary to septal defects. Wise selection of anaesthetic agents like limiting the use of nitrous oxide, ketamine as much as possible to be considered. Conditions like tachycardia, hypotension, and hypothermia should also be prevented perioperatively as these could increase the chances of thrombosis.