Umbilical bleeding revealing congenital factor XIII deficiency in a newborn: A case report and literature review

Factor XIII Deficiency is a rare congenital coagulopathy, frequently revealed during the neonatal period by unusual bleeding manifestations, particularly umbilical bleeding, while routine coagulation tests are often normal, leading to delayed diagnosis. We report the case of a female neonate born to first-degree consanguineous parents, admitted on day 15 of life for umbilical bleeding occurring at the time of cord separation. Initial clinical examination was unremarkable apart from active umbilical bleeding. Routine coagulation investigations were within normal limits. The diagnosis was confirmed by a factor XIII activity level of 6%. The clinical course was marked by recurrent post-traumatic bleeding episodes, managed with fresh frozen plasma and tranexamic acid in the absence of specific replacement therapy. Factor XIII deficiency should be suspected in any neonate presenting with umbilical bleeding despite normal routine coagulation tests. The diagnosis relies on specific factor XIII assays and is essential for appropriate therapeutic management. Limited access to replacement therapy may delay prophylactic treatment and necessitate an on-demand therapeutic approach.