Isolated Protein S Deficiency Presenting in Neonatal Inferior Vena Cava as Right Atrial Thrombus: A Case Report

Abstract

Critically ill neonates are at the greatest risk of developing thromboembolic disease. Multiple risk factors have been identified, one of which is isolated Protein S deficiency-a rare cause. The free form of Protein S plays a role in anticoagulation, and its deficiency can manifest as deep venous thrombosis, pulmonary emboli, and superficial thrombophlebitis. Hereby, the authors present a case report of a two-day-old female neonate, born to a gestational diabetic mother, who was initially managed for hypoglycaemia and polycythaemia. On day 4 of life, the baby was shifted to the mother’s side. Twenty-four hours later, she developed late-onset neonatal sepsis with disseminated intravascular coagulation and required management with a mechanical ventilator, Intravenous (IV) antibiotics, and inotropic support. Echocardiography (ECHO) revealed a thrombus (8.9×6.8 mm) attached to the septum of the right atrium, extending from the Inferior Vena Cava (IVC). Treatment with Low Molecular Weight Heparin (LMWH) was initiated, and a follow-up ECHO after three months showed complete resolution of the thrombus. Interval analysis revealed isolated Protein S deficiency with normal Protein C levels. The present report emphasises the importance of interval analysis in identifying causes of Neonatal Thrombotic Events (NTE). Early diagnosis and management can reduce associated morbidity and mortality.

Keywords

• central venous catheterisation
• coagulation disorder
• hereditary
• neonatal sepsis
• newborn