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Table of Contents    
Year : 2015  |  Volume : 63  |  Issue : 6  |  Page : 965-967

Dialysis-related amyloidosis: An unusual etiology for pathological odontoid fracture

1 Department of Neurosurgery, Rush University Medical Center, Chicago, Illinois 60612, USA
2 Department of Pathology, Rush University Medical Center, Chicago, Illinois 60612, USA

Date of Web Publication20-Nov-2015

Correspondence Address:
Manish K Kasliwal
Department of Neurosurgery, Rush University Medical Center, Chicago, Illinois 60612
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.170091

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How to cite this article:
Kasliwal MK, Moore KA, Tan LA, Harbhajanka A, Gattuso P, O'Toole JE. Dialysis-related amyloidosis: An unusual etiology for pathological odontoid fracture. Neurol India 2015;63:965-7

How to cite this URL:
Kasliwal MK, Moore KA, Tan LA, Harbhajanka A, Gattuso P, O'Toole JE. Dialysis-related amyloidosis: An unusual etiology for pathological odontoid fracture. Neurol India [serial online] 2015 [cited 2022 Aug 16];63:965-7. Available from: https://www.neurologyindia.com/text.asp?2015/63/6/965/170091


Dialysis-related amyloidosis (DRA) is a subtype of amyloidosis resulting from deposition of fibrils derived from beta2-microglobulin (beta2-m). These fibrils can accumulate in patients who are on long-term dialysis and have a predilection for osteoarticular structures.[1],[2] Spinal involvement secondary to DRA is also known as destructive spondyloarthropathy (DSA) and generally presents as spinal stenosis and/or segmental instability due to ligamentous laxity.[3],[4],[5] We report a rare case of a pathological odontoid fracture resulting from DRA.

An 81-year-old male with a past medical history of bladder and prostate cancer under remission, as well as end-stage renal disease (ESRD) on daily peritoneal dialysis, presented with right occipital headache for 3 months. His physical examination was unremarkable with no detectable sensorimotor deficit or signs of myelopathy. His laboratory studies revealed significant abnormalities in the values of hemoglobin (9.4 mg/dl), blood urea nitrogen (81 mg/dl), and creatinine (13.85 mg/dl), all of which were consistent with ESRD. Computed tomography (CT) of the cervical spine was obtained and demonstrated a type II odontoid fracture with mild anterior displacement, as well as a lytic lesion involving the right lateral mass of C1 with associated soft tissue swelling [Figure 1]. Magnetic resonance imaging of the cervical spine did not reveal any significant spinal cord compression [Figure 1]. Given the presence of odontoid fracture and atlantoaxial instability, a decision was made to take the patient to the operating room for open biopsy of the C1 lesion with occiput-C2 posterior fusion and instrumentation. The procedure was performed without complication, and the patient was discharged in a stable condition with advice to undergo rehabilitation. Final histological examination of the biopsy specimens demonstrated the presence of dense fibrillary eosinophilic material, which showed apple-green birefringence after Congo red staining and polarized light microscopy, and a final diagnosis of amyloidosis was made [Figure 2].
Figure 1: Sagittal (a) and coronal (b) reconstruction of the cervical spine computed tomography showing type II fracture of the odontoid with mild anterior displacement and lytic radiolucency involving the right lateral mass of C1 (arrow). Sagittal T1-weighted (c), T2-weighted (d), postcontrast sagittal (e) and axial T2-weighted (f) magnetic resonance imaging demonstrating a type II odontoid fracture with diffuse enhancement at the fracture site and mild anterior listhesis of odontoid fragment and C1 relative to the body of C2. There is absence of any other abnormal signal or enhancement

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Figure 2: Microphotograph demonstrating the presence of dense fibrillary eosinophilic material with fragments of bony trabeculae (a; H and E, ×400), staining positive for amyloid P (b; ×200) characteristic of amyloidosis

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Patients with chronic kidney disease are predisposed to various musculoskeletal abnormalities which may include secondary hyperparathyroidism, osteomalacia, osteosclerosis, osteoporosis and amyloidosis.[6] Beta2-m amyloidosis seen in patients on long-term dialysis has been reported to cause various pathological conditions including carpal tunnel syndrome, trigger finger, DSA, bone cysts, pathologic fractures, ischemic damage of the viscera, urolithiasis, and subcutaneous and periarticular amyloidomas.[2],[4] Spinal involvement in patients on chronic hemodialysis was first described by Kuntz, et al., and termed as "DSA".[7] The potential risk factors for DRA include increasing age, age at onset and duration of hemodialysis, use of low-flux dialysis membranes, use of bio-incompatible dialysis membrane and lack of residual renal function.[8] Involvement of the occipitoatlantoaxial regions is very uncommon in DRA.[4],[9] While renal transplantation remains the best treatment modality, there has been an absence of any other medical treatment which can halt the progression of DRA. Patient with DSA may require surgical treatment in the presence of instability of the spine or symptomatic spinal stenosis.

The pathogenesis of spinal involvement in patients on dialysis is multifactorial and not completely understood. Initially, hydroxyapatite crystal deposition and aluminum intoxication secondary to uremia were implicated in the development of spinal abnormalities in patients with renal failure. However, recent evidence has demonstrated that amyloid deposits consisting of beta2-m with subsequent reactive inflammation may contribute to the development of DSA in patients undergoing chronic hemodialysis.[10] Besides the destructive changes, amyloid or calcium deposits in the spinal canal and ligamentum flavum cause spinal canal stenosis leading to spinal cord or cauda equina compression.

Plain X-rays may show vertebral endplate destruction with subchondral sclerosis, vertebral body collapse, and disc-space narrowing in the absence of significant osteophytosis.[4] Typically, amyloid deposition and the resultant lytic lesions show hypointensity on both T1- and T2-weighted imaging, which can help differentiate it from other inflammatory (e.g., synovitis), infectious, or neoplastic processes.[4],[9] However, some lesions may show foci of increased T2 signal, and the diagnosis must be aided by clinical history and presenting symptoms. Computed tomographic scan can demonstrate well-defined radiolucencies often involving the facet joints or any other part of the vertebrae, as seen in our patient. DSA mainly involves the cervical spine with occipitoatlantoaxial regions being affected less commonly.[4],[9] Certain blood markers such as elevated serum beta2-m is characteristic of DRA and may be helpful in diagnosis.[6]

Deposition diseases in patients with chronic renal failure have the propensity to involve the spine and can result in spinal stenosis or pure bony involvement manifesting as a pathological fracture. DSA presenting as a pathological odontoid fracture is exceptionally rare. Though biopsy is the gold standard to diagnose the various deposition diseases, characteristic radiological findings in the appropriate setting may be suggestive and helpful in appropriate work up and treatment.

Dr. O'Toole is a consultant for Federal Drug Administration, Globus Medical, RTI Surgical and has received royalties from Globus Medical, RTI Surgical and is a stockholder in Theracell, Inc.

Financial support and sponsorship


Conflicts of interest

Dr. O'Toole is a consultant for Federal Drug Administration, Globus Medical, RTI Surgical and has received royalties from Globus Medical, RTI Surgical and is a stockholder in Theracell, Inc.

  References Top

Digenis GE, Davidson G, Dombros NV, Katz A, Bookman A, Oreopoulos DG. Destructive spondyloarthropathy in a patient on continuous ambulatory peritoneal dialysis for 13 years. Perit Dial Int 1993;13:228-31.  Back to cited text no. 1
Ohashi K. Pathogenesis of beta2-microglobulin amyloidosis. Pathol Int 2001;51:1-10.  Back to cited text no. 2
Abumi K, Ito M, Kaneda K. Surgical treatment of cervical destructive spondyloarthropathy (DSA). Spine (Phila Pa 1976) 2000;25:2899-905.  Back to cited text no. 3
Theodorou DJ, Theodorou SJ, Resnick D. Imaging in dialysis spondyloarthropathy. Semin Dial 2002;15:290-6.  Back to cited text no. 4
Yuzawa Y, Kamimura M, Nakagawa H, Kinoshita T, Itoh H, Takahashi J, et al. Surgical treatment with instrumentation for severely destructive spondyloarthropathy of cervical spine. J Spinal Disord Tech 2005;18:23-8.  Back to cited text no. 5
Chang CC, Sung CC, Hsia CC, Lin SH. Uremic tumoral calcinosis causing atlantoaxial subluxation and spinal cord compression in a patient on continuous ambulatory peritoneal dialysis. Int Urol Nephrol 2013;45:1511-6.  Back to cited text no. 6
Kuntz D, Naveau B, Bardin T, Drueke T, Treves R, Dryll A. Destructive spondyloarthropathy in hemodialyzed patients. A new syndrome. Arthritis Rheum 1984;27:369-75.  Back to cited text no. 7
Jadoul M, Garbar C, Vanholder R, Sennesael J, Michel C, Robert A, et al. Prevalence of histological beta2-microglobulin amyloidosis in CAPD patients compared with hemodialysis patients. Kidney Int 1998;54:956-9.  Back to cited text no. 8
Flipo RM, Cotten A, Chastanet P, Ardaens Y, Foissac-Gegoux P, Duquesnoy B, et al. Evaluation of destructive spondyloarthropathies in hemodialysis by computerized tomographic scan and magnetic resonance imaging. J Rheumatol 1996;23:869-73.  Back to cited text no. 9
García-García M, Argilés, Gouin-Charnet A, Durfort M, García-Valero J, Mourad G. Impaired lysosomal processing of beta2-microglobulin by infiltrating macrophages in dialysis amyloidosis. Kidney Int 1999;55:899-906.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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