A seventy-eight-year-old man with osteogenesis imperfecta (Sillence type IV) was admitted to the hospital with severe pelvic pain following pelvic fractures in five locations (the superior and inferior pubic rami on both the left and right sides and the sacrum), which had been sustained three months earlier after a fall. The initial office visit had been in a specialized trauma center. The patient had been treated with bisphosphonate therapy (alendronate followed by ibandronate) for a minimum of ten years.
Since the occurrence of the pelvic fractures, the patient had been confined to a wheelchair and had been dependent on analgesics (tramadol hydrochloride, metamizol, diclofenac) for severe, chronic pain. The earlier fracture history had consisted of numerous nonvertebral fractures after minor trauma in the left radius, right malleolus, right thumb, left femoral neck (followed with total endoprosthesis), ilium, and multiple ribs. The spine had been stabilized from T12 to L2, with posterior lumbar interbody fusion for treatment of a vertebral fracture at L1. The medical history also revealed arterial hypertension and a cerebrovascular insult in the basal ganglia that had occurred fifteen years earlier, with a remaining mild left gluteal hemiparesis.
When admitted to the hospital, the patient (body mass index, 30.3; weight, 94 kg; height, 176 cm) was hypertensive. The neurological status revealed reduced strength of the left lower extremity (a result of the prior stroke) and insufficiency of the hip muscles.
Radiographs and multislice computed tomography of the pelvis showed displaced fractures of the pubic and ischial rami on both the right and left sides and a fracture of the sacrum, with minimal callus formation at the fracture sites (Figs. 1-a and 1-b). A spine radiograph revealed multiple fractures of the lumbar and thoracic vertebrae as well as the ribs.
Bone mineral density measurements (measured by dual-energy x-ray absorptiometry) demonstrated moderately decreased T-scores at the radius (−1.9), total hip (−2.4), femoral neck (−3.3), and calcaneus (−3.9), with a decrease of approximately 5% compared with values measured four years earlier. The values at the lumbar spine could not be evaluated because of the prior spinal surgery.
Structure analysis with high-resolution peripheral quantitative computed tomography (SCANCO) showed profound inhomogeneity of the trabecular network and a substantially reduced cortical thickness of the radius (0.43 mm) and the tibia (0.25 mm). The indices of trabecular number, thickness, and separation were within the normal range.
Laboratory investigations demonstrated an elevated alkaline phosphatase level (335 U/L; reference range, 40 to 129 U/L). Levels of calcium, phosphate, PTH, and 25-hydroxy vitamin D were all within the normal range. Levels of type-1 collagen cross-linked C-telopeptide and procollagen aminoterminal propeptide type I were suppressed, which was related to the long-term bisphosphonate use. Secondary metabolic bone disorders were excluded with full clinical investigations and blood analysis.
Because of its anabolic effects on bone and the evidence of enhanced fracture-healing in preclinical studies, treatment with teriparatide (20 μg once daily) was initiated to potentially stimulate fracture repair. Additionally, calcium (1000 mg daily) and vitamin D (800 IU daily) were given to prevent vitamin D deficiency and to preserve 25-hydroxy vitamin D levels above the threshold of 30 ng/mL.
After six weeks of treatment with teriparatide, marked increase of callus formation at every fracture site was demonstrated by computed tomography (Figs. 1-c and 1-d).
In addition, pelvic pain decreased substantially, and the patient used fewer analgesic medications. He regained mobility, no longer needed a wheelchair, and could stand and walk without a walker. Serum levels of calcium, phosphate, vitamin D, and PTH remained in the normal range during and after treatment. Type-1 collagen cross-linked C-telopeptide and procollagen aminoterminal propeptide type I were increased, which was a sign of response to teriparatide therapy.
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