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

Sacras, ML; Ladeira, C; Carmo, S; Kjöllerström, P

http://hdl.handle.net/10400.17/4247

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Abstract(s)

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.

Keywords

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