1. Pediatric Surgery Department, Hospital de Dona Estefânia, CHULC, 2. Hematology Unit, Hospital de Dona Estefânia, CHULC.
- Case Report - Port J Pediatr 2021;52:205-8 (DOI: https://doi.org/10.25754/pjp.2021.20470)
Resumo: 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. Na isolated prolonged partial thromboplastin time was found, and hemophilia was suspected. Factor VIII levels less than 1% were consistent with severe hemofilia A. The patient received recombinant factor VIII perioperatively. Fasciotomy was performed and the patient was kept on antibiotics until closure.
Palavras Chave: Compartment Syndromes, Fasciotomy; Hemophilia A, Newborn