Hemophilia: Presenting with swelling/hematoma

Children who have hemophilia can develop severe peripheral neurologic deficits when hematomas compress nerves. Because such hematomas usually do not involve the joint, they may be assessed as less serious soft-tissue hematomas. Most smaller soft-tissue hematomas in less critical sites do not require treatment, but compression within closed spaces, such as the forearm, can result in compartment syndrome that can compromise nerve function and blood flow. Vascular compromise is usually obvious, but peripheral nerve involvement may be overlooked. These neurologic deficits often are slow to resolve and may result in permanent deficit. In older children, increasing pain out of proportion to the size of the hematoma, numbness, and paresis are critical signs. Frequently weakness is mistaken for intentional disuse because of pain. This is even more likely to occur in younger children who are less verbal.

As with many bleeding episodes in children who have hemophilia, there often is no history of trauma. A delay of hours to days from the time of injury to the development of a clinically evident hematoma may result in the child forgetting the trauma. In addition, many affected children develop hematomas after injuries that would have been considered trivial. Such episodes are more likely to occur in those who have severe hemophilia, but they are seen in children who have moderate and even mild disease.

Significant blood loss can complicate hematoma formation, but this usually occurs in the thigh or retroperitoneum where substantial blood loss may not be readily apparent on physical examination.

Iatrogenic hematomas can be serious. Routine venipuncture, when performed appropriately, is without danger, but it should be followed by at least 5 minutes of firm finger pressure. In contrast, femoral or jugular venipuncture and any arterial puncture should not be undertaken without prior factor replacement. Otherwise, serious hematomas can result. In the jugular area, this can lead to compression of vital structures, as exemplified by vocal cord hematomas and subsequent asphyxia resulting from local anesthetic blocks during dental work. In the femoral triangle, large hematomas may form before swelling is obvious. Routine intramuscular immunizations can be administered safely when followed by 5 minutes of pressure to the site. Large intramuscular injections should be avoided.

Serious and permanent muscular damage is not frequent among children who have hemophilia. Tendon shortening can occur, but it usually is the result of more chronic and severe hemarthroses, due to peripheral nerve damage, or caused by limited mobility for several days or longer following inadequately treated soft-tissue or joint bleeding.

References:
DiMichele D. Hemophilia 1996. New approach to an old disease. Pediatr Clin North Am. 1996;43:709-736
Montgomery RR, Cox Gill J, Scott JP. Hemophilia and von Willebrand disease. In: Nathan DG, Orkin SH, Oski FA, Ginsburg D, eds. Nathan and Oskiís Hematology of Infancy and Childhood. 5th ed. Philadelphia, Pa: WB Saunders Co; 1998:1631-1644
Rodgers GM, Greenberg CS. Inherited coagulation disorders. In: Lee GR, Foerster J, Lukens J, Paraskevas F, Greer JP, Rodgers GM, eds. Wintrobeís Clinical Hematology. 10th ed. Baltimore, Md: Williams & Wilkins; 1999:1682-1692