Bilateral Ankle Fusion in Leak Syndrome Induced Deformity: A Case Report

Systemic capillary leak syndrome (SCLS) is a rare condition of unknown etiology defined by sudden episode of hypotension, high hematocrit, and low serum protein concentration due to capillary hyperpermeability. Several treatments have been published for this pathology and eventual compartment syndrome but not for the disabling consequences. This clinical case highlights the negative orthopedic consequences of a severe systemic attack and reports a subsequent deformity treatment option that resulted in the patient quality of life improvement. To our knowledge, orthopedic surgery for SCLS induced foot and ankle deformity is presented for the first time.

A 40-year-old Caucasian female patient was referred to our institution for bilateral foot and ankle deformity after compartment syndrome during SCLS attack. She presented a bilateral rigid equino-cavo-varus-adductus deformity causing walk impairment. Bilateral ankle fusion by retrograde nailing was performed in a two-staged procedure. Rigid claw toes were also surgically addressed in an additional procedure. Radiologically, apparent ankle fusion occurred at 3 months post-operative in both sides. Deformity correction allowed the patient to regain walking capacity. A right ankle tibiotalar failed arthrodesis was noticed at 24 months post-operative. Prophylactic human immunoglobulin therapy was initiated after the last surgery with no recurrent attack registered during the 38 months follow-up period.

Bilateral ankle fusion improved this patient quality of life by restituting walking capacity and lowering the probability of lower limb deformity related ulcers and infections. Major and minor complications are also discussed. This report contributes to the global knowledge about this syndrome and elicits the importance of the induced deformities surgical correction.