Does orthopaedic surgery improve quality of life and function in patients with mucopolysaccharidoses?

PURPOSE: Mucopolysaccharidoses (MPS) are a group of rare lysosomal storage disorders associated with involvement of multiple organs along with a generalised skeletal dysplasia. Both haematopoetic stem cell transplant and enzyme replacement therapy have improved the outlook for patients while surgery remains high-risk and there is little information on clinical or functional outcome to justify many of the surgical procedures performed. This paper aims to summarise the orthopaedic surgical procedures in MPS patients for which quality of life (QoL) and functional data are available and to describe additional QoL and functional measurement tools of relevance to the assessment of orthopaedic outcomes in MPS. METHODS: We reviewed the available literature to look for reported outcomes of orthopaedic surgery to lower and upper limbs and the spine. In addition, we describe the general and MPS-specific health measures that might be of relevance to the orthopaedic surgeon. RESULTS: There is some evidence in the literature that orthopaedic surgery may improve QoL and function in some specific aspects of the MPS condition (in relation to genu valgum, carpal tunnel syndrome and trigger digits); however, the literature is sparse and consists of level 4/5 studies only. Further studies of these conditions should include QoL and functional assessment in order to confirm or refute these reports. In other areas (spine and hip), outcomes are judged largely on radiographic appearances with little clinical correlation and short follow-up; however, one long-term study of function following hip dysplasia surgery suggests poor outcomes. Anaesthetic morbidity/mortality is not insignificant in these complex patients with multi-organ involvement. Careful assessment is required, particularly when there is neurological involvement. CONCLUSIONS: Orthopaedic surgeons involved with MPS patients should be encouraged to use and report measures of QoL and function with respect to musculoskeletal manifestations and response to surgery, recognising that such assessments in these complex and challenging patients may require a multidisciplinary approach

Topical glyceryl trinitrate for the treatment of tendinopathies: a systematic review

Objective: To produce a best evidence synthesis of the clinical effects of topical glyceryl trinitrate (GTN) in the treatment of tendinopathies. Design: A systematic review of published randomised controlled trials (RCTs) of the use of GTN in patients with tendinopathy. Data sources: MEDLINE, Embase, Scopus and CINAHL from database inception to January 2018. Methods: We examined RCTs comparing the effects of topical GTN with either placebo or other treatments on tendinopathy. Overall quality of each eligible study was determined based on a combined assessment of internal validity, external validity and precision. The level of evidence for each assessed parameter was rated based on the system by van Tulder et al. Results: A total of 10 eligible RCTs were identified including patients with tendinopathy of the rotator cuff (n=4), wrist extensors (n=3), Achilles (n=2) and patellar (n=1) tendons. For all tendinopathies, improvements in pain were significant when comparing GTN versus placebo in the short term (<8 weeks; poor evidence). Significant improvements in midterm outcomes for treatment with GTN versus placebo included the following: patient satisfaction (strong evidence); chances of being asymptomatic with activities of daily living (strong evidence); range of movement (moderate evidence); strength (moderate evidence); pain (at night and with activity; poor evidence) and local tenderness (poor evidence). Patients treated with topical GTN reported a higher incidence of headaches than those who received placebo (moderate evidence). Conclusions and relevance: Treatment of tendinopathies with topical GTN for up to 6 months appears to be superior to placebo and may therefore be a useful adjunct to the treating healthcare professions

Effects of calcium intake on the cardiovascular system in postmenopausal women [Review]

The use of calcium supplements for the prevention of complications of osteoporosis has significantly increased during the last years. The effects of calcium intake in postmenopausal women on cardiovascular parameters such as blood pressure, serum lipids and cardiovascular events are controversial. Even though transient beneficial effects of calcium supplementation have been reported, especially in women with low dietary calcium intake, their long-term outcomes are inconclusive. Only a very few studies investigating serum lipids in postmenopausal women have been described and these showed significant increases in high-density lipoprotein and high-density lipoprotein to low-density lipoprotein ratio. With regards to cardiovascular events in this population group adverse effects have been reported on the rates of myocardial infarction and stroke with increased calcium intake by some authors, however, others described no effects or even beneficial outcomes. We present a review of the current literature which provides a balanced summary of the possible beneficial and adverse effects of calcium intake in postmenopausal women on cardiovascular parameters. Taking into account the modest effect of calcium supplementation in reducing fracture rates, a reassessment of the role, benefits and adverse effects of calcium supplements should be conducted in postmenopausal women

The mucopolysaccharidoses: advances in medical care lead to challenges in orthopaedic surgical care

The mucopolysaccharidoses (MPS) are a group of inherited lysosomal storage disorders with clinical manifestations relevant to the orthopaedic surgeon. Our aim was to review the recent advances in their management and the implications for surgical practice. The current literature about MPSs is summarised, emphasising orthopaedic complications and their management. Recent advances in the diagnosis and management of MPSs include the recognition of slowly progressive, late presenting subtypes, developments in life-prolonging systemic treatment and potentially new indications for surgical treatment. The outcomes of surgery in these patients are not yet validated and some procedures have a high rate of complications which differ from those in patients who do not have a MPS. The diagnosis of a MPS should be considered in adolescents or young adults with a previously unrecognised dysplasia of the hip. Surgeons treating patients with a MPS should report their experience and studies should include the assessment of function and quality of life to guide treatment.N. Williams, D. Challoumas, D. Ketteridge, P. J. Cundy, D. M. Eastwoo

Multiple iliopsoas tendons: a cadaveric study and treatment implications for internal snapping hip syndrome.

PURPOSE: This cadaveric study aimed at describing the anatomical variations of the iliopsoas complex. METHODS: The iliopsoas complex was dissected unilaterally in 28 formalin-embalmed cadavers-13 males and 15 females with a mean age of 85.6 years. The number, courses and widths of the iliacus and psoas major tendons were determined. Patients with previous hip surgery were excluded. The following measurements were taken from the mid-inguinal point: the distance to the point of union of the psoas major and iliacus tendon; and the distance to the most distal insertion of iliopsoas. RESULTS: The presence of single, double and triple tendon insertions of iliopsoas were found in 12, 12 and 4 of the 28 specimens, respectively. When present, double and triple tendons inserted separately onto the lesser trochanter. The average length of the iliopsoas tendon from the mid-inguinal point to the most distal attachment at the lesser trochanter was 122.3 ± 13.0 mm. The iliacus muscle bulk merged with psoas major at an average distance of 24.9 ± 17.9 mm proximal to the mid-inguinal point. In all cases, the lateral-most fibres of iliacus yielded a non-tendinous, muscular insertion on to the anterior surface of the lesser trochanter and the femoral shaft, rather than joining onto the main iliopsoas tendon(s). The average total width of the psoas major tendon decreased with an increasing number of tendons: 14.6 ± 2.2 mm (single tendon), 8.2 ± 3.0 mm (2 tendons present) and 5.9 ± 1.1 mm (3 tendons present) (P < 0.001). CONCLUSIONS: The results of this study suggest that multiple tendinous insertions of iliopsoas are present as an anatomical variant in more than 50% of the population. The non-tendinous muscular insertion of the iliopsoas on to the anterior surface of the lesser trochanter and femoral shaft found represents a novel anatomical variant not previously described. LEVEL OF EVIDENCE: Level V