Neonatal Acute Compartment Syndrome as First Manifestation of Hematologic Disease: Case Report

• Autor(a) principal: Sacras, ML
• Data de Publicação: 2021
• Outros Autores: Ladeira, C, Carmo, S, Kjöllerström, P
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://hdl.handle.net/10400.17/4247
• topic: Acute Disease; Compartment Syndromes/ diagnosis; Compartment Syndromes/etiology; Compartment Syndromes/therapy; Fasciotomy; Hemophilia A/complications; Hemophilia A/diagnosis; Infant, Newborn; Case Report; HDE CIR PED; HDE HEM PED

Hemophilia A, characterized by deficiency of functional plasma clotting factor VIII, is an X-linked disorder. Signs and symptoms depend on factor VIII levels. Severe hemophilia A (factor levels less than 1%) is usually diagnosed in the first two years of life. Pediatric compartment syndrome is most associated with trauma, but infectious and vascular causes must also be considered. We report a case of a newborn who presented with a severe hand hematoma after venous puncture, complicated by compartment syndrome. An isolated prolonged partial thromboplastin time was found, and hemophilia was suspected. Factor VIII levels less than 1% were consistent with severe hemophilia A. The patient received recombinant factor VIII perioperatively. Fasciotomy was performed and the patient was kept on antibiotics until closure.