General Spine
Puneet, S., Pawha, MD
Daniel, Lerner, MD, Non ASSR Member
Amish, H., Doshi, MD, Non ASSR Member
Excerpta
Medullary infarctions in the appendicular skeleton are commonly surrounded by a curvilinear border of hypointense signal on T1 and T2 weighted imaging, reflecting the reactive interface between reparative and necrotic bone (1). This appearance is well recognized in the metaphyses and epiphyses of long bones, but is rarely appreciated in vertebral body infarction (also known as vertebral osteonecrosis or avascular necrosis).
We present a 63 year old female with Gaucher disease who has deep medullary vertebral body infarction at the L4 level, sharply outlined by a hypointense border on MR imaging in the absence of vertebral collapse or cleft (Figs. 1 and 2). MRI demonstrates triangular geographic regions of signal abnormality adjacent to the central portions of both the inferior and superior endplates of a single vertebral body, a distribution known to be particularly vulnerable to ischemia. These geoqraphic abnormalities are sharply outlined by a peripheral hypointense border on both T2 weighted and T1 weighted imaging. Given the high incidence of bone infarction in patients with Gaucher disease, the presence of multiple extensive medullary bone infarctions in both lower extremities of this patient (Fig. 3), and the characteristic distribution of the abnormality, these findings are consistent with deep medullary vertebral body infarction. The patient has a surgical history significant for splenectomy and right total hip arthroplasty, with no prior spine intervention.
To our knowledge, this appearance has been reported twice in the vertebral body. One of these cases. was secondary to delayed radionecrosis of the vertebral body and spinal cord and the other case presented by Javier et al. was of uncertain etiology in a patient with lymphoma and osteoporosis (2,3). Subclinical deep medullary infarcts of the vertebral body are known to occur in patients with Gaucher disease often resulting in vertebral collapse (4).
Discussion
The double-line sign is a well known and specific MR imaging appearance of skeletal infarction typically seen in the metaphyses and epiphyses of long bones (5). This sign refers to an outer rim of low signal surrounding an inner parallel line of high signal on T2 weighted imaging. The peripheral hypointense line reflects sclerotic reparative bone, while the inner high signal is thought to represent hyperemic granulation tissue. In our case, the internal T2 hyperintensity was more diffuse rather than linear and may reflect a more longstanding appearance.
The posterior central arteries of the vertebral body supply the posterior and central portions, while the anterior central arteries supply the antero-lateral portions of the vertebral body, with the end-arterial branches of both arteries supplying the superior and inferior endplates. The watershed regions are in the center (deep medullary portion) of the vertebral body. The watershed zones and end-arterial zones of the vertebral bodies overlap in triangular (conical) regions based along the central portions of both the superior and inferior endplates (6). These regions are particularly susceptible to infarction, and correlated well with the distribution of abnormality in our case.
Gaucher disease is an autosomal recessive lysosomal storage disease caused by deficiency of the enzyme glucocerebrosidase. This leads to the accumulation of lipid-laden macrophages (Gaucher cells) with associated inflammatory response and results in chronic multi-organ damage particulary affecting the spleen, liver and bone marrow (7). Skeletal manifestations, and particularly bone infarctions, are a significant cause of morbidity in these patients. Analysis of the Gaucher registry of patients revealed that most have some degree of skeletal involvement and 25% of patients have bone infarcts (8). Infiltration with Gaucher cells may result in diffuse bone marrow infiltration replacing the normal marrow signal on MR imaging (9). The mechanism of bone infarction in these patients is incompletely understood, but may relate to direct occlusion of the microvasculature by Gaucher cells, or to marrow infiltration and resultant increased intraosseous pressure causing vascular compression and ischemia (10).
More commonly recognized imaging signs of vertebral body infarction include triangular areas of T2 hyperintensity along the endplates, vertebral body collapse, and vertebral body clefts filled with air or fluid. Sclerotic outlining of a geographic vertebral body lesion in the appropriate clinical setting should alert the radiologist to consider the diagnosis of vertebral body infarction.
References/Financial Disclosures
No financial disclosures.
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