Author(s): Sacras, ML ; Ladeira, C ; Carmo, S ; Kjöllerström, P
Date: 2021
Persistent ID: http://hdl.handle.net/10400.17/4247
Origin: Repositório do Centro Hospitalar de Lisboa Central, EPE
Subject(s): 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.
