Acute Compartment Syndrome in a Neonate: May Be the First Manifestation of Hemophilia A

Abstract

Abstract Background: Acute compartment syndrome (ACS) most commonly results from traumatic or ischemic injuries, though rarely it may result from an underlying bleeding disorder. Reports of ACS in hemophilia are present; however, neonatal presentation secondary to hemophilia A is extremely rare. Clinical Description: A 5-day-old neonate presented with increasing swelling and hematoma over the dorsum of both hands following venipuncture. There was no other bleeding manifestations. Antenatal history and natal history were uneventful, and the baby had received a birth dose of Vitamin K. On examination, the hematoma was larger on the right hand than the left, extending from the proximal interphalangeal joints of all the digits to the wrist proximally, with tension on palpation and increased capillary refilling time, associated with bluish discoloration of hands and fingers with claw hand appearance. Oxygen saturation on pulse oximeter was 80%–85%. Management and Outcome: The baby had a hemoglobin of 16.3 g/dL, white blood cell count of 5300/mm3, platelet count of 2.33 lakhs/mm3, and elevated activated partial thromboplastin time >120 s with a normal prothrombin time of 12 s. Factor assay confirmed severe factor VIII deficiency (<1%). Ultrasonography Doppler of the hand confirmed compartment syndrome. Cryoprecipitate was given perioperatively, and fasciotomy was done. Perfusion of the hand improved, and oxygen saturation normalized. Healing was accomplished without any closure, with good cosmesis and complete functional recovery by 14 days. Conclusion: ACS may be an unusual primary presentation of hemophilia as early as in the neonatal period. Timely identification of ACS and early suspicion of an underlying bleeding disorder can help in undertaking appropriate evaluation and management strategies and preventing catastrophic consequences.