Retroperitoneal Pseudotumor Syndrome (Hemophilia B)

T. Pfammatter, U. Binkert, & E. Meili
Institute of Diagnostic Radiology
University Hospital, Zuerich/Switzerland
Case Reference No. CC-0400-06

A 34-year-old immigrant cook with severe hemophilia B (Factor IX=1%) presented with a chronic hematoma extending along the iliopsoas muscle down into the thigh, as well as laterally at the waist into the subcutaneous tissue (Figs. 1,2,3). With weekly factor IX substitutions the hematoma has been stable since the acute bleed in January 97. Back then a catheter angiography was performed to rule out a treatable bleeding source. As no vessel abnormality was found the deep left circumflex iliac artery was embolized. The lower extrremity venous edema and the femoral neuropathy improved over time. The angiography as well as a total right hip replacement for posttraumatic degenerative joint disease in 1981 were performed without significant hemorrhagic sequelae under adaquate factor substitution. For obvious reasons our surgeons are reluctant to adress the extended hemophiliac pseutotumor.

Questions:

1) Can/should this chronic hematoma be drained percutaneously?

2) Will it refill and how soon?

3) How high is the risk of provoking an abcess by a percutaneous drainage?

4) Is there a need to repeat an angiography?

Fig. 1. Coronal T1-w MR showing chambered chronic ilio-psoas hematoma extending into the thigh and left flank.

Fig. 2. Axial MR at the waist showing subcutaneous fluid collection, likely communicating with the reroperitoneal hematoma.

Fig. 3. Asymmetry of the thighs due to hematoma surrounding the femur.