Physicians described the case of an individual with hemophilia B who experienced a gunshot wound to his lower leg that was complicated by the development of acute compartment syndrome (ACS), in Blood Research.

Hemophilia B, which is reported in 1 of 30,000 live male births, is caused by a deficiency in factor IX. The patient in the current case was born in 1983 and was diagnosed with hemophilia B at 10 years of age, following prolonged bleeding after a tooth extraction. At that time, his factor IX level was 4 IU/dL. The man had never experienced any significant spontaneous hemorrhage, only having received factor IX replacement therapy in 2015 for pilonidal sinus surgery.

Immediately following the current injury, he pressed the wound with gauze in an effort to halt the intense bleeding, not having any factor IX concentrate at home. He ultimately presented to the emergency department at the University of Belgrade, in Belgrade, Serbia, on January 6, 2019, with a penetrating gunshot wound to his right lower leg, which he sustained after a bullet from a 7.65-mm caliber pistol backfired.


Continue Reading

When he was examined in the emergency department, a round entrance wound surrounded by an abrasion ring with a minor irregular exit wound on the opposite side was observed on the lateral side of his distal right lower leg, about 5 cm above the lateral malleolus. No bleeding from the injury was seen. His entire right calf was painful, swollen, and pale.

When measured to the greatest extent, the difference in size between his right and left calves was 4 cm. His muscles were taut, and stretching only served to intensify his pain. Tibialis posterior and dorsalis pedis were not palpable on his right side, but could be felt in his left leg. Radiography and computed tomography scans revealed transection of the tibial artery and vein, supporting the clinical picture of ACS, and the patient was scheduled to undergo urgent surgery.

Learn about the types of hemophilia

The patient’s surgery began 5 hours after he sustained the injury. Immediately prior to the operation, he received
80 IU/kg of plasma-derived factor IX concentrate. His vascular structures were ligated, which stopped the bleeding. He underwent a decompressive fasciotomy of his right calf. Total blood loss during his surgery was 150 mL. In the initial 3 days following surgery, his factor IX trough level was maintained at approximately 80 IU/dL and then at approximately 60 IU/dL for the next 7 days.

He continued to receive factor IX concentrate 20 IU/kg once daily without any pharmacologic thromboprophylaxis. He was subsequently transferred to the plastic surgery unit for skin grafting. Four weeks after his injury, a skin graft was placed on his right calf using a split-thickness homologous graft that was harvested from his right thigh. Immediately prior to his surgery, he received 50 IU/kg factor IX concentrate, then continued to received 20 IU/kg for the next 7 days. After receiving prolonged physical therapy, he regained muscle function in his right leg and ultimately recovered completely.

The authors noted that “ . . . the treatment of ACS in patients with hemophilia remains controversial, mainly because the available data are based on scattered case reports and small case series. If it occurs spontaneously, care should focus on early recognition and factor concentrate replacement therapy. . . .However, surgical management of ACS, including fasciotomy, may be necessary and should not be delayed if indicated.”

Reference

Bodrozic J, Lekovic D, Koncar I, Dzelatović NS, Miljic P. Rapid development of lower leg compartment syndrome following firearm injury in a patient with moderate hemophilia B. Blood Res. Published online November 9, 2022. doi:10.5045/br.2022.2022112