Destruction of a cervical vertebra in a patient with end-stage renal failure

(Section Editor: G. H. Neild)

Brian Camilleri, Donald Richardson and Alex M. Davison

Department of Renal Medicine, St James's University Hospital, Leeds, UK

Keywords: lupus erythematosus; sclerotic bone disease; end-stage renal failure; tertiary hyperparathyroidism

Case

We present a patient who was diagnosed with systemic lupus erythematosus (SLE) in 1979 at the age of 19 years. Six months after presentation she developed nephrotic syndrome and was treated with prednisolone and azathioprine. However, in the following 4 years she progressed to end-stage renal failure (ESRF) and started haemodialysis. In 1984 she received a cadaveric renal transplant but underwent a transplant nephrectomy 1 month later, due to steroid-resistant rejection. In 1986, she received a second cadaveric renal transplant but returned to dialysis 2 years later due to chronic allograft nephropathy. After a brief period of time on haemodialysis she changed modality to peritoneal dialysis.

In July 1990, she complained of a 10-day history of severe right-sided neck pain radiating to the right shoulder. This was extremely painful, despite regular analgesics, and was associated with severely limited range of movement of the neck. Neurological examination was unremarkable. A skeletal survey performed 1 year previously had shown widespread erosions in the middle phalanges of all fingers and some of the terminal tufts, and calcification of the medial sclerosis type affecting arteries of the feet, hands, forearms and pelvis. Investigations showed elevated alkaline phosphatase 1983 iu/l (70–300), grossly elevated parathyroid hormone (PTH) 1597 ng/l (10–65), calcium 2.22 mmol/l (2.2–2.6), phosphate 1.27 mmol/l (0.8–1.3), C-reactive protein 18 mg/l (<10), and aluminium 1.15 µmol/l (acceptable level <1.9). A plain film of the cervical spine (Figure 1Go) showed absence of C5 vertebra. A hard neck collar was fitted and a CT-guided biopsy of the vertebrae was carried out. However, the histology specimen consisted mainly of blood and a core of fibrocartilage, muscle and fatty tissue. Stains for amyloid were negative but the tissue was inadequate for diagnosis. Our patient underwent an urgent total parathyroidectomy in August 1990. Post-operative PTH levels were 4 ng/l. A repeat film of the cervical spine performed 6 months after initial presentation is shown in Figure 2Go. At this stage an orthopaedic opinion recommended it was safe to remove the collar in view of the good range of movement of the cervical spine.



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Fig. 1. A plain film of the cervical spine showing absence of C5 vertebra.

 


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Fig. 2. A repeat film of the cervical spine performed 6 months after initial presentation.

 
In 1993 a third cadaveric renal transplant was performed but failed after 3 years, due to chronic allograft nephropathy and the patient returned to peritoneal dialysis. In 1997, she complained of pains across both shoulders and radiating down both arms and elbows. This was accompanied by a cold feeling affecting the right foot. Neurological examination was grossly unremarkable apart from absent ankle jerks bilaterally. Plain cervical films showed severe sclerotic bone disease with destruction of the cervical vertebral body and bridging ossification between C4 and C6. A magnetic resonance image (MRI) of the spine showed complete loss of C5 with some compression of the cord at this level (Figure 3Go).



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Fig. 3. A magnetic resonance image (MRI) of the spine showing complete loss of C5 with some compression of the cord at this level.

 
Neurosurgical opinion was sought and it was felt that no intervention was required unless the patient developed any further symptoms or signs. To date, she is well with no symptoms or signs suggestive of progressive cord compression. She has full range of movement in the neck and, despite the previous ‘total’ parathyroidectomy, her current PTH levels are 147 ng/l suggesting remnant parathyroid tissue.

Discussion

Cervical spine involvement in patients with ESRF, especially those undergoing dialysis, can be due to a number of reasons. Renal osteodystrophy (ROD) can be seen throughout the spine, but reports of significant cervical spine involvement are uncommon. Patients typically seek treatment for pain in the thoracic or lumbar area. The classical stigmata of ROD in the vertebrae is the ‘rugger-jersey’ spine which consists of bands of increased density paralleling the end-plates of the vertebral body and a band of radiolucency in the centre of the body [1]. Components of ROD include hyperparathyroidism, osteoporosis and osteomalacia. Hyperparathyroidism can lead to brown tumours (osteoclastomas), which appear as cysts in bone [1]. They are cavities in bone in which there is excessive osteoclastic resorption, fibrous tissue, necrosis and liquefaction. They can occur anywhere in the skeleton and may expand bone. When the cervical spine is involved in ROD, the need for surgical intervention for instability in the spine seldom arises. However, in 1993, Nair et al. [2] reported six patients treated over the previous 3 years who demonstrated cervical instability and cord compression, all of which required surgical intervention. These patients had been on haemodialysis for between 4 and 12 years. It is also of note that two patients had both ROD and superimposed osteomyelitis.

Infection should be included in the differential diagnosis of erosive changes on plain X-rays of the spine, especially due to the increased risk of infection in haemodialysis patients. Cervical vertebrae are the least common sites of infection in vertebral osteomyelitis and when it does occur, contiguous spread must also be considered [3]. This usually results in paravertebral or epidural masses. The latter are best identified on MRI and a high erythrocyte sedimentation rate and/or CRP should help distinguish infective discitis/osteomyelitis from destructive spondyloarthropathy (DSA).

DSA is another cause of erosive spondyloarthropathy in patients on long-term haemodialysis and usually involves the middle to lower cervical spine [4]. Spinal cord compression has been described but is rare [57]. MRI is useful in establishing a differential diagnosis with infection. The aetiology of DSA is debated but suggested causes are hyperparathyroidism, crystal deposition disease (apatite crystals) [1], amyloid (ß2-microglobulin deposition) [6], aluminium overload, ligamentous laxity, and neuropathy (in diabetic patients). Hyperparathyroidism appears to be the most important contributing factor. Parathyroidectomy is indicated to halt the progression of the erosive process.

In our patient, it is difficult to be certain whether there was an initial osteoclastoma preceding the complete destruction of C5 vertebra or whether this could be attributed entirely to DSA. In either case, however, the underlying pathogenesis was hyperparathyroidism, and an urgent total parathyroidectomy, with subsequent alfacalcidol supplementation, was the mainstay of treatment. This resulted in a stabilization of the lesion and her symptoms and was followed by ossification with maintenance of satisfactory cervical spine function.

Notes

Correspondence and offprint requests to: A. M. Davison, Department of Renal Medicine, St James's University Hospital, Beckett Street, Leeds LS9 7TF, UK. E-mail: alex.davison{at}leedsth.nhs.uk Back

References

  1. Adams JE. Renal bone disease: radiological investigation. Kidney Int1999; 56 [Suppl 73]: S38–S41[ISI]
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  3. Sapico FL, Montgomerie JZ. Vertebral osteomyelitis. Infect Dis Clin North Am1990; 4: 539–550[Medline]
  4. Kuntz D, Naveau B, Bandis T, Dreuke T, Treves R, Dryll A. Destructive spondyloarthropathy in haemodialysis patients. A new syndrome. Arthritis Rheum1984; 27: 369–375[ISI][Medline]
  5. Allard JC, Artze ME, Porter G, Ghandur-Mnaymned L, de Velasco R, Perez GO. Fatal destructive cervical spondyloarthropathy in two patients on long-term dialysis. Am J Kidney Dis1992; 19: 81–85[ISI][Medline]
  6. Sebert JL, Fardellone P, Marie A et al. Destructive spondyloarthropathy in haemodialysed patients: possible role of amyloidosis. Arthritis Rheum1986; 29: 301–302[ISI][Medline]
  7. Deforges-Lasseur C, Combe C, Cernier A, Vital JM, Aparicio M. Destructive spondyloarthropathy presenting with progressive paraplegia in a dialysis patient. Recovery after surgical spinal cord decompression and parathyroidectomy. Nephrol Dial Transplant1993; 8: 180–184[ISI][Medline]




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