Postural control during a transition task in haemophilic children, adolescents and young adults with haemophilic ankle arthropathy.

BACKGROUND: The aim of this study was to determine whether young haemophilic boys with and without MRI-based signs of ankle arthropathy demonstrate reduced balance ability during a transition task with eyes open and eyes closed. METHODS: Thirty-four haemophilic bodies and 28 typically developing boys aged 6-20 years participated to this study. Structural integrity of the tarsal foot joints of all haemophilic boys was assessed with MRI. All participants performed a standard transition task from double-leg stance to single-leg stance with eyes open and eyes closed. Comparison of balance features derived from the centre of pressure displacement captured by a single force platform was performed between the different haemophilia subgroups and sex-age-height matched peers. FINDINGS: The haemophilic boys without signs of arthropathy presented only a higher intermediate phase velocity during the eyes closed condition (P = .05). The haemophilic boys with signs of arthropathy had significantly higher displacement after the time to new stability point, and 95% Ellipse Sway Area and Balance Area compared to their matched peers during eyes open test (P < .05). Similar findings were observed during the eyes closed test for the displacement after the time to new stability point and 95% Ellipse Sway Area (P < .05). No significant differences were observed between affected and non-affected side of the unilateral affected patients. INTERPRETATION: We suggest that the pathophysiological cascade associated with chronic bleeding episodes should not be considered as a "simple" musculoskeletal injury, hence more as a complex neurophysiological dysfunction which may originate both from unilateral and bilateral deterioration of the musculoskeletal system.

Reliability and clinical features associated with the IPSG MRI tibiotalar and subtalar joint scores in children, adolescents and young adults with haemophilia.

OBJECTIVES: To assess the reliability of the IPSG MRI scale for tibiotalar (TTJ) and subtalar joint (STJ) changes in young haemophilic patients, correlating MRI findings with functional scores and 3D-rearfoot kinematics. METHODS: A total of 37 haemophilic patients underwent bilateral MRI of the footankle, clinical evaluation and quantitative assessment of their 3D-rearfoot kinematics during walking. TTJ and STJ soft tissues were assessed twice along with osteochondral changes by two radiologists using the IPSG MRI scale. Inter- and intra-observer reproducibility of MRI scoring were tested by means of kappa statistics. Correlational analyses were performed between MRI findings and the Haemophilia Joint Health Score 2.1 (HJHS) and 3D-rearfoot kinematic data. RESULTS: The intra-reader reliability of MRI scoring was good to excellent (Kappa: 0.62-1), whereas the inter-reader reliability was moderate to good (Kappa: 0.54-0.79). Weak yet significant correlations were found between the frontal plane rearfoot range of motion (ROM) during loading response of gait and STJ score, as well as between frontal plane rearfoot ROM during the terminal stance phase and the rearfoot osteochondral lesions. CONCLUSION: The IPSG score appears applicable to not only the TTJ but also the STJ. Contrary to TTJ lesions, those of the STJ do not correlate with the HJHS but do with 3D-rearfoot kinematic data.

Deficits of ankle muscle strength not found in children, adolescents and young adults with haemophilic ankle arthropathy.

OBJECTIVES: Adequate management of haemophilia patients requires early detection of joint impairment in relatively asymptomatic patients. This study sought to quantify the impact of the ankle's structural impairment on muscle strength in children, adolescent and young adults with haemophilia (CAAwH). METHODS: Twenty-three CAAwH underwent bilateral magnetic resonance imaging (MRI) assessing the anatomical status of tibiotalar joint (TTJ) and subtalar joint (STJ) using the International Prophylaxis Study Group MRI scale. An isokinetic dynamometer enabled a detailed evaluation of muscle strength at slow and fast speed. In parallel, 10 typically developing healthy boys (TDB) participated in a 1-week interval test-retest assessment to assess the test's reliability. RESULTS: Forty-six MRI ankle scores were obtained, with 11 patients unilaterally affected and one bilaterally. Of the 13 affected feet, nine showed abnormalities at TTJ, three at the posterior STJ and the remaining one at both joints. Muscle strength was not reduced in CAAwH exhibiting TTJ and/or STJ arthropathy, as compared to healthy TDB, nor was there any difference between the CAAwH's affected or unaffected sides. CONCLUSION: Contrarily to adult patients, CAAwH with repeated ankle bleeding may be less impaired than current structural evaluations imply, with possibly a latency between the occurrence of structural and functional damage.

Falling and fall risk factors in adults with haemophilia: an exploratory study.

Falls are a particular risk in persons with haemophilia (PWH) because of damaged joints, high risk of bleeding, possible impact on the musculoskeletal system and functioning and costs associated with treatment for these fall-related injuries. In addition, fall risk increases with age and PWH are increasingly entering the over 65 age group. The aim of this study was to determine the occurrence of falls during the past year and to explore which fall risk factors are present in community-dwelling PWH. Dutch speaking community-dwelling adults were included from the age of 40 years with severe or moderate haemophilia A or B, independent in their mobility and registered at the University Hospitals Leuven. They were asked to come to the haemophilia centre; otherwise a telephone survey was conducted. Demographic and social variables, medical variables, fall evaluation and clinical variables were queried. From the 89 PWH, 74 (83.1%) participated in the study. Twenty-four (32.4%) fell in the past year, and 10 of them (41.7%) more than once with an average of four falls. Living conditions, physical activity, avoidance of winter sports due to fear of falling, orthopaedic status, urinary incontinence and mobility impairments are potential fall risk factors in adult PWH. This exploratory study indicates that PWH are attentive to falling since they are at higher risk for falls and because of the serious consequences it might have. Screening and fall prevention should be stimulated in the daily practice of haemophilia care.

20 years of arterial switch operation for simple TGA.

BACKGROUND: Arterial switch operation became the golden treatment for simple transposition of the great arteries (sTGA). We describe our experience with the arterial switch operation regarding long-term outcome and the need for re-intervention. Nevertheless, supravalvular pulmonary stenosis (SPS) remains a concern in the long run. We assess the evolution of SPS over time and evaluate the effect of technical modifications on SPS during our experience. METHODS: We performed a retrospective study on 133 patients operated with ASO for TGA between October 1991 and November 2009. Last report method was used. We reviewed our pediatric cardiology and cardiac surgery database to examine the echocardiographic data and electrocardiograms. A mean follow-up of 9.2 years (+/- 5.83 SD) was reached. RESULTS: One (0.8%) patient deceased postoperatively due to cardiogenic shock. The overall actuarial freedom from reoperation (open and percutaneous) was 88.1%, 78.5% and 76.9% at 1, 5 and 10 years. SPS needed to be treated in 17 patients. Valve regurgitation at final investigation was maximal moderate in 5 patients for the aortic valve, 10 for pulmonary valve and 3 in tricuspid valve. CONCLUSIONS: ASO shows excellent long-term results in sTGA with a very low morbidity and mortality and is therefore the procedure of choice. Re-intervention rate is determined by SPS. Since the extensive mobilization of the pulmonary arteries and the creation of a longer neo-pulmonary root, reduction in SPS was seen with no re-interventions in the second half of the group. To obtain a final comparison with the atrial switch operation, a longer Follow-up is necessary.