Sympathetic Blocks for Raynaud's Phenomena in Pediatric Rheumatological Disorders.

OBJECTIVE: Sympathetic blocks are invaluable to prevent morbidity from Raynaud's phenomenon (RP). RP may occur in children with rheumatological disorders and causes severe pain, discoloration of digits, gangrene, and auto-amputation. We describe the planning and execution of sympathectomy blocks in children with rheumatological disorders presenting with RP. METHODS: With upper-limb involvement, ultrasound-guided stellate ganglion block (USGB) was given with ropivacaine and clonidine. When all four limbs were involved, intrathecal block with bupivacaine and clonidine was also given. RESULTS: A total of 68 sympathectomy blocks were performed: 28 bilateral USGBs, two unilateral USGBs, and 10 intrathecal injections. Multiple interventions in a single day were frequently required. For safety, all USGBs were performed with an ultrasound with strict adherence to local anaesthetic volume was maintained, with periprocedure monitoring of 2-3 hours. All blocks were performed by an experienced specialist. All children reported immediate pain relief with prevention of major amputation. CONCLUSION: With meticulous planning, monitoring, and precautions, sympathectomy of limbs in pediatric rheumatological disorders with RP can be safely undertaken. Bilateral stellate ganglion block with ultrasound is safe in children, and clonidine is a useful adjunct for vasodilation and prolongation of the effect of sympathectomies in children.

GNE myopathy - A cross-sectional study on spatio-temporal gait characteristics.

GNE myopathy is a rare, predominantly distal myopathy, involving mainly the lower limbs and presenting with gait disturbances. In this cross-sectional study gait evaluation of 23 (14 men) genetically confirmed GNE myopathy patients was done using Instrumented walkway analysis (GAITRite®) along with video gait capture. We recorded the topographical pattern of muscles involvement in lower limbs and correlated Functional Ambulation Profile-FAP and Medical Research council-MRC grading of lower limb scores with duration of illness. Early foot flat, foot drop gait with wider out-toed stance and higher perturbations with increased pressure at heel and decreased arm swing were noted. Muscle topography showed predominant weakness in ankle dorsi-flexors, flexor hallucis longus, extensor hallucis longus, hip adductors and knee flexors with stark sparing of quadriceps and relative sparing of hip- abductors, extensors, flexors and ankle plantar-flexors. Gait parameters in women were significantly more affected than men (p < 0.05) for the same duration of illness. FAP score and MRC grading of lower limb scores correlated significantly with duration of illness (p < 0.05). We observed that ankle dorsiflexors were affected earliest with sparing of quadriceps muscles in these patients.