Non-surgical correction of knee flexion deformity in persons with haemophilia: A staged multidisciplinary approach.

INTRODUCTION: Flexion deformity of the knee is a common complication following recurrent haemarthrosis in persons with haemophilia (PWH) on episodic factor replacement therapy, restricting independent mobility. There is limited literature on the comprehensive management of this condition. This report provides the outcome of a staged multidisciplinary approach for the correction of knee flexion deformity (KFD) even in limited resource settings. PATIENTS AND METHODS: The data of 49 consecutive PWH who were treated for KFD were analysed. The approach included graded physical therapy (PT), followed by serial casting and/or mobilisation under anaesthesia (MUA). MUA was done in carefully selected knees. Surgical correction was opted when non-surgical methods failed. RESULTS: Of the 49 patients (55 knees), with a median KFD of 40 degrees (range: 10-90), 26/55 (47%) were corrected by graded PT. With serial casting, 9/19 (47%) knees had their KFD corrected. MUA was done for 11 knees of which five achieved correction (45%). Surgical correction was required for only seven knees (12.7%). Following this approach, KFD improved from 40 degrees (range: 10-90) to 15 degrees (range: 0-40), with only minor loss of flexion from 105 (range: 60-155) to 90 degrees (range: 30-150). Out of 55 KFD, 46 (83.6%) KFD were corrected; non-surgical, 39 (70.9%) and surgery, seven (12.7%). The remaining patients (nine KFD; 16.4%) were able to achieve their functional goal despite not meeting the correction criteria. CONCLUSION: This study shows that in PWH, functionally significant KFD correction can be achieved in about 71%, through non-surgical methods, even without prophylactic factor replacement.

Mobilization under anaesthesia for correction of knee flexion deformity in haemophilia.

INTRODUCTION: Haemophilia patients not treated with primary or secondary prophylaxis, often present with recurrent haemarthrosis. Knee is the most frequently involved joint leading to disabling knee flexion deformity (KFD). Here, we present a retrospective study of our experience on the role of mobilization under general anaesthesia in the correction of KFD. AIM: To study the effectiveness of mobilization under anaesthesia (MUA) for correction of knee flexion deformity (KFD) in persons with haemophilia (PWH). METHODS: Outcome of all patients managed with MUA in our multidisciplinary haemophilia clinic from 2008 to 2019 were included for analysis. PWH with KFD > 20 degree who underwent MUA were included in the study. Under general anaesthesia and cover of clotting factor replacement, gentle joint mobilization was done to achieve maximal correction in flexion deformity, followed by above knee casting in this position. The outcome measures assessed were reduction in knee flexion deformity following MUA and complications, if any. RESULTS: Thirty patients (34 knees) with knee flexion deformity were included in the study. Mean age of the study population was 14.23 years ± 8.3. Study population was analysed in two groups, Group 1 included patients who underwent single MUA and Group 2, patients who underwent two or more MUA. There was significant improvement in KFD correction in both groups. [Group 1; Mean difference: 22 ± 13.7, p value -0.01, 95% CI (16.4-27.5) and Group 2; Mean difference 48.8 ± 19.8, p value -0.00, 95% CI (34.2-64.5)]. CONCLUSION: MUA can be effective in the short-term correction of KFD in PWH particularly those below 15 years of age. It should be done judiciously when target correction is not achieved with other physical methods.

Role of Ultrasonography in Upper Airway Assessment for Decannulating Tracheostomy in Acquired Brain Injury-A Pilot Study.

OBJECTIVE: To compare the findings of ultrasonography of the upper airway with flexible fiberoptic laryngoscopy and determine the efficacy of transcutaneous laryngeal ultrasonography for decannulation. DESIGN: Prospective cross-sectional study. SETTING: Tertiary care referral center in South India. PARTICIPANTS: Twenty-four patients with acquired brain injury (N=24). MAIN OUTCOME MEASURES: Participants underwent an airway assessment by ultrasonography followed by assessment of airway by flexible laryngoscopy done within the next 72 hours. RESULTS: Vocal cord assessment by ultrasonography revealed a sensitivity of 81.2% and specificity of 87.5%. A statistically significant association between vocal cord mobility as assessed by ultrasonography and decannulation was observed (sensitivity of 81.25%, specificity of 87.5%, P=.002). Although aspiration was not assessed by ultrasonography, a statistically significant association was observed between vocal cord mobility on ultrasonography and aspiration as assessed by laryngoscopy (sensitivity of 81.25%, specificity of 87.5%, P=.011). CONCLUSION: Laryngeal ultrasonography is an emerging diagnostic modality with a potential role for assessing vocal cord mobility and airway prior to decannulation in centers that lack the expertise and the infrastructure to perform a flexible laryngoscopy.

Body composition and metabolic parameters in men with chronic traumatic paraplegia - A pilot study from India.

OBJECTIVE: To study body composition, measures of insulin resistance and dyslipidemia in Indian men with paraplegia as compared to age and body mass index (BMI) matched able-bodied men. DESIGN: Cross sectional study. SETTING: Departments of Physical Medicine and Rehabilitation and Endocrinology. PARTICIPANTS: Males aged 18-45 years with chronic traumatic paraplegia versus age and BMI-matched able-bodied men. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Measures of body composition such as total body fat, lean mass, regional adiposity using dual energy x-ray absorptiometry (DXA), metabolic profile and insulin resistance. RESULTS: Subjects with paraplegia (n = 43), compared to controls (n = 36), had higher %Fat mass (FM) (25.5 (21.2-28.9) vs 20.2 (15.9-22.2); P < 0.01), lower trunk to leg ratio (0.66 (0.51-0.73) vs 0.87 (0.72-0.94); P < 0.01), lower lean mass index (14.38 (2.57) vs 17.80 (2.34); P < 0.01) and lower appendicular lean mass index (5.81 ± 1.26 vs 8.17 ± 1.12; P < 0.01). Fasting blood glucose (mg/dl) was higher (89.0(81.5-96.5) vs 80.0 (74.5-88.2); P < 0.01), Homeostasis model assessment of insulin resistance was higher (1.33 (1.03-2.12) vs 0.94 (0.52-1.78); P = 0.02), Quantitative insulin sensitivity check index (QUICKI) was lower (0.36 ± 0.04 vs 0.38 ± 0.05; P = 0.02) and HDL-C was lower (33.00 (30.00-42.75) vs 38.50 (33.00-43.25); P < 0.02) in cases compared to controls. QUICKI correlated positively with HDL-C and negatively with %FM, estimated VAT volume and TG. Trunk to leg ratio correlated positively with TG even after controlling for %FM. CONCLUSION: Men with chronic paraplegia had lower lean mass, higher total and regional fat mass, increased insulin resistance and low HDL-C when compared with BMI-matched able-bodied controls. Both total and regional adiposity correlated with poor metabolic profile.

Status dystonicus: Diagnosis and management of a rare and challenging entity.

We report the case of a six-year-old girl with Moyamoya disease who presented with bilateral internal carotid artery malignant infarct following encephaloduroarteriosynangiosis (EDAS). During her neurorehabilitation, she developed gradually worsening dystonic spasms with opisthotonic posturing, tachycardia, tachypnea and desaturation. This rare life threatening movement disorder was diagnosed as status dystonicus based on the history and clinical presentation. Status Dystonicus occurs commonly in children and the etiology is often diverse. It occurs in patients with preexisting dystonia or following an acute central nervous system insult of varied etiology. Status dystonicus is usually precipitated by one or more triggering factors. Rarity and lack of objective criteria for diagnosis often delays the management thereby increasing the risk of mortality and morbidity. Here, we discuss the challenges faced in the diagnosis and management of a child with denovo status dystonicus.

Wii-based interactive video games as a supplement to conventional therapy for rehabilitation of children with cerebral palsy: A pilot, randomized controlled trial.

OBJECTIVE: To assess the effect of interactive video gaming (IVG) with Nintendo Wii (Wii) supplemented to conventional therapy in rehabilitation of children with cerebral palsy (CP). DESIGN: Randomized, controlled, assessor-blinded study. PARTICIPANTS: Children with CP; 10 children each in the control and intervention groups. INTERVENTION: IVG using Wii, given as a supplement to conventional therapy, for 45 min per day, 6 days a week for 3 weeks. The children in the control group received conventional therapy alone. OUTCOME MEASURES: Posture control and balance, upper limb function, visual-perceptual skills, and functional mobility. RESULTS: Significant improvement in upper limb functions was seen in the intervention group but not in the control group. Improvements in balance, visual perception, and functional mobility were not significantly different between control and intervention groups. CONCLUSIONS: Wii-based IVG may be offered as an effective supplement to conventional therapy in the rehabilitation of children with CP.

Measurement of physical performance and objective fatigability in people with mild-to-moderate traumatic brain injury.

The aims of this study were to objectively measure the physical performance and physical endurance of patients with traumatic brain injury with minimization of cognitive and psychological fatigue, and to compare the physical performance of brain injured patients with that of healthy controls. This was a nonrandomized partially blinded controlled study. The study setting was the Outpatient Multidisciplinary Brain Injury Clinic in the Department of Physical Medicine and Rehabilitation of a tertiary care university teaching hospital. Participants included an experimental group that comprised independently ambulant men (age 18-55 years) with mild-to-moderate traumatic brain injury (n = 24) who complained of greater fatigue than before their injury and an age-matched and sex-matched control group (n = 24). The intervention included the Six-Minute Walk Test. The primary outcome measures were the Six-Minute Walk Distance, the Fatigue Severity Scale, Addenbrooke's Cognitive Examination, and the Fatigue Visual Numeric Scale; the secondary outcome measures were the Physiological Cost Index of Walking and the Borg Scale of Perceived Exertion. The Six-Minute Walk Distance of the experimental group (452.33+/-68.816) when compared with that of the control group (518.08+/-92.114) was reduced by 12.7 and 30.5%, respectively, when compared with the predicted Six-Minute Walking Distance (650.04+/-79.142) for the same age and sex. The mean Fatigue Severity Scale values were 2.51 and 1.62 for the experimental and control groups, respectively. The mean Addenbrooke's Cognitive Examination Score for the patients was 85.5+/-7.265. In conclusion, the Six-Minute Walk Test is useful in segregating physical fatigue from cognitive and psychological aspects of fatigue when cognitive and psychological dimensions are known. The Six-Minute Walk Test can be used as a tool for exercise intensity prescription in men with mild-to-moderate brain injury, to avoid the deleterious effects of fatigue.