Treatment of considerations in patients with compartment syndrome and an inherited bleeding disorder.

In addition to consultation with an experienced hematologist, the following are recommendations regarding compartment syndrome in a patient with an inherited bleeding disorder. Von Willebrand's Disease. Humate-P (rich in von Willebrand factor) is the replacement therapy of choice for surgical procedures in patients with von Willebrand's disease. In general, in the perioperative period, factor VIII levels between 50% and 100% are ideal with a gradual tapering to maintain levels at 50% for approximately 2 weeks. Adjuncts to therapy are DDAVP and EACA. Hemophilia A. During the initial evaluation and with measurement of compartment pressures, factor VIII replacement to levels in the range of 40% to 60% of normal is appropriate replacement therapy. For fasciotomy, however, factor VIII levels greater than 50% to 100% are required. In patients who have developed antibodies to factor VIII, a number of options are available. With low titers of factor VIII inhibitor, higher doses of factor VIII may be successful in overriding the inhibitor. In patients with higher titers of inhibitor, activated factor VII or porcine factor VIII is recommended. Hemophilia B. Highly purified factor IX replacement aimed at keeping factor levels between 50% and 100% in the perioperative period, followed by maintenance at 50% for approximately 2 weeks, is optimal management. Treatment Algorithm: The Figure outlines an algorithm to aid in the diagnosis and treatment of compartment syndrome in the patient with an inherited bleeding disorder. In a suspected case of compartment syndrome due to a soft-tissue hemorrhage or injury, factor replacement as outlined above should be initiated. Unequivocal clinical findings in the normal patient usually would be an indication to proceed to fasciotomy without obtaining compartment pressures. In the patient with an inherited bleeding disorder, however, factor replacement and subsequent normalization of the clotting cascade may help lowe compartment pressures. Therefore, we advocate obtaining initial pressures even with clinical findings of an acute compartment syndrome. At our institution, we advocate using an automated handheld pressure monitor (Stryker, Ontario, Canada) or the needle injection technique as described by Whitesides et al. In interpreting the obtained pressures, we choose to use the guidelines as described by Heppenstall et al. Briefly, Heppenstall et al determined that the pressure threshold at which cellular damage occurred was related more closely to the difference between the mean arterial blood pressure and compartment pressure than with the absolute compartment pressure alone; this measurement is called delta P. If delta P is > 30 mm Hg, then one should continue factor replacements and perform serial clinical and pressure examinations. Pressures should be taken every hour for 2 hours total. If the patient worsens in either respect, then the physician should enter the other limb of the algorithm for delta P < 30 mm Hg. For the patient with a delta P < 30 mm Hg, the amount of time since onset of symptoms must be considered. Since the patient may improve with adequate factor replacement, a delta P < 30 mm Hg mercury does not dictate automatic fasciotomy. An adequate time trial of replacement therapy may be attempted. In patients whose pressures do not begin normalizing, we advocate proceeding to fasciotomy. Patients who begin to normalize pressures during a 2-hour trial can be followed with serial clinical and pressure examinations. Any worsening in either scenario is an indication for fasciotomy; otherwise, observation and factor replacement may be continued. After initial decompression, staples may be placed in both wound edges with an elastic vascular loop woven between the two edges in a "shoelace" pattern. Then while waiting for closure, the loops can be gradually tightened at the bedside. Definitive closure should be attempted around the fifth postoperative day. All closure techniques should be pre