Phlegmasia cerulea dolens with lower leg compartment syndrome.

Abstract

A 57-year-old man with a history of human immunodeficiency virus infection presented with sudden pain and swelling of the right leg that had developed over the preceding thirty minutes (Panel A). He reported having been taking antiretroviral drugs. Computed tomography (CT) demonstrates partial pulmonary artery embolism and massive deep vein thrombosis extending from the right external iliac to the popliteal veins. Despite commencing anticoagulation, he had severe pain and intense dysesthesia of the right leg. The intramuscular pressure in his right leg was 98 mmHg (reference: <10 mmHg), and his right dorsalis pedis pulse was not palpable. A clinical diagnosis of compartment syndrome secondary to phlegmasia cerulea dolens was made, and he underwent immediate fasciotomy (Panel B). As the dysesthesias did not improve by fasciotomy, catheter-directed venous thrombectomy was performed on day 5 of hospitalization using a 10-French Heartrail ST01 catheter (Terumo, Tokyo, Japan) via a cross-over 12-French DrySeal (WL Gore, Flagstaff, Arizona, USA) from the contralateral femoral vein after placing an inferior vena cava (IVC) filter. Although the final venography revealed restored venous flow, the filter could not be retrieved because of trapped thrombi (Panel C-E, see supplementary data online, Videos S1–S3). Postprocedural CT demonstrated complete thrombosis of the right external iliac vein, and swelling in the right thigh was persistent despite anticoagulation and intermittent pneumatic compression. After the second venous thrombectomy on day 15, the venous flow was completely restored, and the filter was retrieved (see supplementary data online, Videos S4 and S5). He was discharged on apixaban without symptoms of post-thrombotic syndrome (Panel F).