Portal vein thrombosis, livedo reticularis, polymicrobial sepsis and high antiphospholipid antibody titers in a newborn: A clinicopathological conference of antiphospholipid-associated neonatal syndrome

Abstract

Neonatal Antiphospholipid syndrome (APS) is a rare disease related to transplacental passage of antiphospholipid (aPL) antibodies from the mother or de novo production of aPL in a newborn. Neonatal aPL antibodies have rarely been associated with thrombosis. We describe a 5-week-old infant who developed fever, portal vein thrombosis and livedo reticularis like skin rash. Evaluation for thrombosis revealed high titers of antiphospholipid (aPL) antibodies (dual positive) in the child without any evidence of aPL antibodies in the mother, suggesting a de novo production in the child. Autopsy findings revealed umbilical vein sepsis with thrombosis of portal vein secondary to gram positive cocci which led to multiple liver and lung abscesses. Additionally, the baby had disseminated Cytomegalovirus (CMV) disease (acquired postnatally) involving walls of umbilical and portal vein, liver, lungs, adrenals, pancreas, thymus, and kidneys. Our case highlights the need for testing of aPL in every neonate with arterial or venous thrombosis even when the mother may have no features suggestive of an autoimmune disease.

Suppurative Thrombosis of the Portal Vein (Pylephlebits): A Systematic Review of Literature

“…Metastatic abscesses due to the hematogenous dissemination of the pyogenic portal focus were the second most common complication. Our review includes an infant who developed pulmonary abscesses as a complication of pylephlebitis, with their hospital course complicated by respiratory failure, mechanical ventilation, and severe sepsis, culminating in death within a month following presentation [ 67 ]. In another case, even though a troublesome focus of brain abscess was diagnosed, the patient had an indolent clinical course and successfully recovered without any complications [ 7 ].…”

Pylephlebitis: A Systematic Review on Etiology, Diagnosis, and Treatment of Infective Portal Vein Thrombosis

“…Pyogenic liver abscesses can complicate up to 37% of cases of pylephlebitis [2,102,104,207]. Other rarer sites include metastatic abscesses in the lung [145] and the brain [114].…”

De novo antiphospholipid antibody syndrome in two infants

“…7,8 De novo aPLs are associated with similar, or potentially increased, morbidity and mortality compared to acquired aPLs, especially in the presence of other thrombotic risks. [9][10][11] While knowledge of the pathophysiology and outcomes of infants with de novo aPL production remains limited, we present two cases of infants with presumed de novo aPL production and APS.…”