Hepatic Venous Outflow Obstruction Complicating Churg‐Strauss Syndrome in a 5‐year‐old Child

Abstract

H epatic venous outflow obstruction is uncommonly reported in children (1). Although it is a rare disease in populations with a high standard of living, it is a leading cause for liver-related hospital admission in others with a lower standard of living (2). Hepatic venous outflow obstruction may run an acute, chronic, or fulminant course. Although commonly manifested by abdominal pain, ascites, and hepatosplenomegaly, cases have been reported in which there was a complete absence of symptoms, this being strongly associated with large hepatic vein collaterals (3). The syndrome is caused by a wide variety of conditions, including congenital or acquired inferior vena caval webs, thrombotic, inflammatory, or neoplastic disorders (4).Churg-Strauss syndrome (CSS) is characterized by eosinophilia and systemic vasculitis and occurs in patients affected by asthma and allergic rhinitis (5). In 1990, the American College of Rheumatology developed diagnostic criteria for the syndrome, namely moderate to severe asthma, peripheral blood eosinophilia (>10%), mono-or polyneuropathy, nonfixed pulmonary infiltrates, paranasal sinus abnormality, and histological proof of vasculitis with extravascular eosinophilia. The presence of at least 4 of these criteria is required for diagnosis (6). Epidemiological association of CSS with the use of antileukotrienes suggested the possibility that, in some instances, CSS may be a drug-induced illness (7).

Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort

“…Literature on pediatric EGPA is scarce, and the specificities of pediatric cases remain poorly described. To the best of our knowledge, only 38 articles describing pediatric cases of EGPA are listed in the PubMed database, reporting 92 cases (mostly individual case reports) . The two largest reported pediatric EGPA series described, respectively, 8 and 13 pediatric cases.…”

Acute Eosinophilic Pneumonia Complicated with Venous Thromboembolic Disease—Diagnostic and Therapeutic Considerations

“…Thus, the balance between pro-coagulant and anticoagulant factors in eosinophilic diseases is disturbed, leading to hypercoagulability. Venous thromboembolism associated with tissue and/or systemic eosinophilia was previously reported in eosinophilic esophagitis [ 17 ], eosinophilic granulomatosis with polyangiitis (EGPA) [ 18 ], food protein-induced allergic proctocolitis [ 19 ], and idiopathic hyper-eosinophilic syndrome [ 20 , 21 , 22 ], nevertheless it was not reported in AEP.…”