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 Oski’s 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. Wintrobe’s Clinical
Hematology. 10th ed. Baltimore, Md: Williams & Wilkins; 1999:1682-1692