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.

An Unusual Case of Posttraumatic Visual Agnosia Posing Challenges to Rehabilitation.

ABSTRACT: Acquired focal visuoperceptual deficits are rarely reported, and rehabilitation strategies are not well established. This is a report on visuoperceptual deficit after traumatic brain injury that initially went unnoticed. Missing visual agnosia is not unusual especially when perceptual deficits present with visual field defects, impaired insight, and inattention. This case is made more interesting because of the rarity of visual agnosia with predominant ventral pathway involvement, affecting object and face recognition. This report provides a brief discussion on visual agnosia spectrum deficits and rehabilitation measures.

Evaluating Bone Loss with Bone Turnover Markers Following Acute Spinal Cord Injury.

STUDY DESIGN: Prospective observational study. PURPOSE: To evaluate bone turnover markers (BTMs) in individuals with acute spinal cord injury (SCI) and to compare the results with those of healthy controls and postmenopausal females. OVERVIEW OF LITERATURE: SCI significantly impacts bone health. Change in bone mineral density appears 6 months after SCI and rapid bone loss during the acute phase is often underestimated, resulting in osteoporosis and a high risk of sublesional fractures. However, few studies have evaluated BTMs in the Indian SCI population. Despite a high risk of fracture, there are no guidelines for the diagnosis, monitoring, and management of SCI-induced osteoporosis. METHODS: Twenty patients within 1 month of traumatic SCI who had been admitted to a tertiary care rehabilitation center were included in this study. Serum BTMs, C telopeptide (CTX) as a bone resorption marker, and osteocalcin as a bone formation marker, were serially measured at baseline, and 3 and 6 months after SCI. BTMs of SCI patients were compared with those of a control group of age-matched healthy males, premenopausal females, and a vulnerable group of postmenopausal females. RESULTS: BTMs were significantly elevated in patients with SCI, with maximum levels observed at the 3rd month of injury. At baseline, the bone resorption marker CTX was approximately 3 times higher in SCI patients than in the control male population and premenopausal females, and about double that of postmenopausal females. The rise in the bone formation marker was marginal in comparison to that of the bone resorption marker. BTMs were persistently elevated and did not reach the normative range until the 6th month of SCI. CONCLUSIONS: Raised bone resorption markers in comparison to bone formation markers indicate hyper-resorption-related bone loss following acute SCI. Markedly elevated bone resorption markers in the SCI population, compared with those in control and vulnerable groups, emphasize the need for early bone health monitoring and management.

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.