Spinal Stenosis: An Emerging Complication of Ageing in People With Haemophilia

Abstract

Introduction: Advances in haemophilia care have brought the challenges of ageing for people with haemophilia (PWH) to the forefront. Age-related spinal degeneration may result in spinal stenosis; however, the rates in PWH are unknown. We sought to systematically review Irish PWH to address this gap in the current literature.

Methods: Clinical and radiological notes of all patients ≥40 years old (yo) registered with severe or moderate haemophilia A or B were reviewed, recording Haemophilia Joint Health Scores (HJHS), radiological imaging and orthopaedic/pain interventions.

Results: Of 100 males included with moderate or severe haemophilia, 13% had radiologically confirmed symptomatic spinal stenosis (reported rates 4% in the general population aged >60 yo). Persons with stenosis were older (median age 69yo vs. 55 yo, p = 0.004) with similar rates observed between those with moderate and severe haemophilia (4/35, 11.4% vs. 9/65, 13.8%). HJHS did not differ between those with and without stenosis (median 30 vs. 35, p = 0.6). On regression analysis, only age >60 yo was associated with an increased likelihood of spinal stenosis; severity of haemophilia (moderate vs. severe) was not significantly associated.

Conclusions: These data identify symptomatic spinal stenosis as a novel complication of ageing for PWH. Spinal stenosis rates were higher than expected for age in comparison to reported rates in the general population. Current joint assessments fail to capture spinal pathology, highlighting limitations of HJHS in older PWH. Increased awareness amongst PWH and health care providers of spinal stenosis is directly required; however, optimal management strategies for PWH with established stenosis are yet to be defined.

Keywords: ageing; arthropathy; haemophilia; spinal stenosis; spine; surgery.

FIGURE 1

Radiological changes of spinal stenosis or symptoms of back pain may exist in isolation, symptomatic spinal stenosis requires the presence of both.

FIGURE 2

Scatter plot (Figure 2A) comparing ages of those with haemophilia A (circle, median 55 years old, yo) and haemophilia B (squares, median 60yo, p = 0.1). People with moderate haemophilia (Figure 2B, triangles) were significantly older (median 64yo) than those with severe haemophilia (squares, median 53 yo, p = 0.0002). Overall, the age profile of those with spinal stenosis (Figure 2C, squares) was older (median 69 vs. 55 yo, p = 0.004). For those with spinal stenosis, the age profile was similar between those with haemophilia A (Figure 2D, circles, median 69yo) and haemophilia B (open squares, median 67.5yo, p = 0.9). Figure 2E displays a Forest plot of clinical determinants, including age, body mass index (BMI), severe haemophilia, prior joint replacement, and abnormal DEXA scan, in relation to a diagnosis of spinal stenosis. Squares represent the odds ratios (OR), and whiskers indicate the corresponding 95% confidence intervals. Only age was associated with an increased likelihood of a spinal stenosis diagnosis following logistic regression analysis. Figure 2F shows a Kaplan–Meier curve assessing the cumulative incidence of spinal stenosis development for those with moderate (green line) or severe haemophilia (red).

FIGURE 3

Scatter plots showing baseline arthropathy as assessed by the HJHS were similar between those with (Figure 3A, circles, median 30) and without spinal stenosis (triangles, median 35, p = 0.55). When those with (circles) and without (triangles) spinal stenosis were compared, no significant differences were seen in HJHS scores for elbows (Figure 3B median 8 vs. 11, p = 0.62), knees (Figure 3C median 5.5 vs. 9, p = 0.83) or ankles (Figure 3D median 11.5 vs. 12, p = 0.83). The age at first joint surgery was significantly higher in those with SS (median 55.5yo vs. 46yo, p = 0.02, Figure 3E). Figure 3F: Box chart DXA bone mineral density (BMD) scores for those with (n = 10) and without (n = 53) spinal stenosis. T and Z scores are compared by location; spine (T 0.9 vs. −0.4, p = 0.007; Z 0.7 vs. −0.5, p = 0.0007), femoral neck (T 1.2 vs. 1.2, p = 0.942; Z −0.45 vs. −0.9, p = 0.255) and hip (T 0.45 vs. 0.4, p = 0.600; Z −0.35 vs. −0.6, p = 0.26).