Subchondral insufficiency fracture of the femoral head in a patient with alkaptonuria

Abstract

We report a patient with alkaptonuria accompanied by bilateral rapidly destructive arthrosis of the hip. The destruction of the left hip joint with its severe functional impairment necessitated total hip arthroplasty (THA). The outcome was satisfactory. Both magnetic resonance imaging (MRI) and pathologic findings were compatible with a subchondral insufficiency fracture. A year and half later, during a follow-up visit, the patient complained of right coxalgia. Radiography showed that the right femoral head had already disappeared, requiring THA of the right hip. Although there have been a few reports of rapid destructive hip osteoarthritis associated with ochronotic arthropathy, the pathogenesis of the destructive change is not clear. Subchondral insufficiency fracture was diagnosed on MR imaging and pathologically confirmed in our patient with alkaptonuria, suggesting that subchondral insufficiency fracture is one of the causes of ochronotic hip destruction.

The mysteries of rapidly destructive arthrosis of the hip joint: a systemic literature review

“…Several potential risk factors have been proposed in the literature, including: (I) ageing; (II) female gender; (III) underlying health problems such as rheumatoid arthritis, diabetes mellitus and systemic lupus erythematosus; (IV) lower bone mineral density; (V) higher BMI; (VI) medicinal drug use including NSAIDs and corticosteroid; (VII) alcoholism (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(19)(20)(21)(22)(23)(24)27,29). In this study, we evaluated the risk factors and found the only prevalent factors were: (I) age greater than 60 years; (II) female gender; (III) underlying systemic disease such as rheumatoid arthritis, diabetes mellitus or systemic lupus erythematosus (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(19)(20)(21)23,24). There is insufficient evidence to support the relationship between BMD, BMI, medication and alcoholism and the o...…”

“…It was reported that a unique presentation of severe bone destruction as a manifestation of chronic myeloid leukemia in the absence of blast crisis (33). There were bone marrow edema on acetabulum, femoral head and neck and the weight-bearing area and the articular cartilage was absent with the subchondral insufficiency fracture or subchondral bone destroyed (2,3,(5)(6)(7)(8)10,34,35).…”

“…Although cases with delayed replacement surgery showed no signs of malignancy or contralateral involvement (26), these patients showed negligible results on conservative treatment. Although Low-dose aspirin and monoclonal antibody against human receptor activator of nuclear factor-κ B ligand (RANKL) were benefit for maintaining bone mass and qualities by activation of osteoblastic bone formation and inhibition of osteoclast activities via cyclooxygenase-independent manner (37,38), the use of drugs such as NSAIDs, alendronate sodium hydrate and alfacalcidol had only mild or even no effect on the patients with RDA (3,5,6,20,24,39,40). Total hip replacement is probably the treatment of choice for these patients.…”

“…All of these studies were retrospective. Fourteen were reported in Asia (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15), six in Europe (16)(17)(18)(19)(20)(21) and three in America (22)(23)(24). In total, 17 detailed patient cases were included (one article reported two cases, see Table 1).…”

The Distribution of Ochronosis in Osteoarthritic Articular Cartilage in a Knee

“…Of particular interest in our case was the lack of ochronotic pigment in a newly formed osteophyte, a finding we believe has not been previously reported. A recent paper by Yamada et al, however, does show gross lack of ochronotic staining in an osteophyte, although it is not noted [18]. The apparent forming osteophyte is in the formation phase of bone, with the synthesis of new organic matrix, not the catabolism of existing proteins.…”