Total Hip Joint Replacement in a Patient with Colchicine-Resistant Familial Mediterranean Fever under Canakinumab Treatment.

Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent episodes of fever and serositis. Periodic febrile attack can be managed with biologic medication in colchicine-resistant FMF patients, however, no reports or guidelines exist regarding the postoperative management of elective joint surgery in these patients. Although it is not clear how FMF attacks are triggered, they may be precipitated by stress including anesthesia or surgery. This study reports the case of a 51-year-old FMF patient who received total hip replacement under canakinumab (a specific interleukin-1ß monoclonal antibody) treatment. He had highly active FMF, which was resistant to colchicine; however, his recurrent febrile attack with serositis was successfully controlled with canakinumab. Four months later from the start of canakinumab treatment, his hip osteoarthritis was required for total hip replacement (THR) because of the traumatic fracture. THR was successfully done and FMF attack was not occurred after this elective surgery. Discontinuation of canakinumab 3 weeks before surgery and resumption 6 weeks after led to favorable outcome without complications. This case addresses the differential management concerning stopping and restating of canakinumab in the perioperative setting in contrast to the other biologics such as tumor necrosis factor-α (TNF-α) or interleukin-6 (IL-6) blocking agents. This case report suggests that canakinumab may represent a safe and effective therapy for the colchicine-resistant FMF, even in the patients requiring THR therapy.

Congenital incomplete duplication of lower extremity (polymelia) accompanied with anorectal malformation and unilateral deafness.

Polymelia (supernumerary limbs) is an extremely rare congenital condition in humans. Because congenital anorectal malformation is a relatively common complication of polymelia and it may require emergency surgery as in the present case, accompanying malformations must be investigated.

Periprosthetic Fracture Resembling Atypical Femoral Fracture After Fixation With Retrograde Intramedullary Nail in Elderly Women: A Report of Two Cases.

BACKGROUND/AIM: The number of individuals diagnosed with and requiring medical treatment for osteoporosis continues to increase due to global population aging and the high awareness of osteoporosis. Bone-modifying agents (BMAs) including bisphosphonate and denosumab are widely used for osteoporosis, and atypical femoral fracture (AFF) is also gaining attention as a severe potential side effect of long-term BMA treatment. The definition of AFF excludes periprosthetic femoral fracture; here, we describe two cases of a periprosthetic femoral fracture that resembled AFF. CASE REPORT: The fractures occurred at the proximal tip of the retrograde femoral nail after an internal fixation for a distal femoral shaft fracture in elderly Japanese women. Each woman had been treated with bisphosphonate therapy for >2 years and had continued the bisphosphonate after undergoing surgery for a distal femoral shaft fracture. Each patient had noticed thigh pain before falling down, and plain radiographs showed a short oblique or transverse fracture with medial spike and localized periosteal reaction of the lateral cortex in each case. The fractures were re-fixed with an antegrade intramedullary nail, and bone union was achieved at >1 year after the second operation. Although these two cases were classified as periprosthetic fractures, they fulfilled the characteristics of AFF. CONCLUSION: Physicians should conduct a thorough interview of patients with a history of BMA treatment in order to correctly diagnose periprosthetic fractures that resemble AFFs, and they should be aware that symptomatic fractures can be prevented by prophylactic fixation. The discontinuance of BMA therapy and the introduction of another drug such as teriparatide may lead to faster healing of surgically treated AFFs.