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Introduction: Avascular necrosis of femoral head secondary to rare metabolic Gaucher’s disease (GD) can cause debilitating hip arthritis in young adults. It is an autosomal recessive disorder caused due to deficiency of lysosome enzyme glucocerebrosidase resulting in accumulation of its substrate in macrophages. The activated macrophages or the Gaucher cells causes hepatosplenomegaly, anemia, and thrombocytopenia. Extensive marrow involvement causes bony deformity, necrosis, and pathological fractures in non-neuropathic GD. Total hip replacement (THR) for young adult with secondary arthritis due to avascular necrosis (AVN) of femoral head in GD is complex and has high failure rate. As the abnormal cell infiltration involves both femoral head and the acetabulum. It becomes even more challenging, when associated spinopelvic fusion preexists. The altered biomechanics needs special attention to the anteversion of the cup placement and deciding the combined ante-version angle (CAVA). Case Presentation:We report a case of GD with avascular necrosis of the femoral head, who underwent spinopelvic fusion to address his osteonecrosis of lumbar vertebra. Previously unreported, here we will discuss the pre-operative radiological evaluation and other intra-operative challenges in the management of GD post-enzyme replacement therapy (ERT) with secondary hip arthritis by THR. Conclusion:Hip replacement surgery in patients with Gaucher disease related to secondary arthritis restores pain free mobility. Despite the young age of the patients with GD, prognosis remains better with THR after enzyme replacement therapy. The pre-operative planning, anticipation of complications in metabolically abnormal hip joints makes it a complex primary THR. However, in patients with the spinopelvic fusion placement of the cup, at the narrow range of angle of version with altered spinopelvic rhythm plays an important role in post-operative prosthetic hip stability and patient mobility. Keywords:Gaucher’s disease, spinopelvic fusion, total hip arthroplasty, enzyme replacement therapy.

<p class="abstract">Elbow injures in children accounts for 10% of all fractures, ranges from simple sprain to elbow fracture dislocation. Ligamentous injury accounts for 2-3% only and are mostly sports related seen in overhead athletes. Trash lesions (the radiological appearances seemed harmless) around the elbow are reported in children under 10 years which are high energy trauma with initial normal looking X-rays. These are osteochondral or physical injures presenting with soft tissue swelling without any fractures. A high index of suspicion, early additional imaging such as MRI, CT scan, ultrasound examination and aggressive surgical care are usually necessary for prompt diagnosis and successful treatment of these injuries before complications arise. We presented a case of an elbow injury in a 15 year old girl, following a fall on her outstretched hand with initial normal findings in her X-rays. 3 months following the injury she had persisting pain and instability with ulnar nerve impingement symptoms. Her elbow CT scan, nerve conduction study confirmed the avulsion fracture of medial epicondyle with ulnar nerve neuritis and cubital tunnel syndrome. Valgus stress test proved the extent of additional medial instability. Medial epicondyle avulsion fractures are always challenging for fixation. The retracting UCL forces prevents healing and were prone for failure with non-operative splinting once instability is defined. Attempted fixation can also result in failure of fixation. Our case study highlights the possibility of such trash lesion in adolescent and also the modified technique of ulnar collateral ligament (UCL) reconstruction in a cost effective manner.</p>

Introduction: Bosworth ankle injuries (BAIs) are rare talocrural dislocations with bi or trimalleolar fractures. Attempted closed reduction fails due to incarcerated fibular fracture behind the distal tibia. The diagnostic delay and failed attempts to restore anatomical reduction result in significant ankle swelling, compromised skin, and imminent compartment syndrome. In neglected cases with unreduced dislocation over months, capsular and tendo-Achilles contracture, additional disuse osteoporosis, adds to the complexity of surgical management. In chronic and neglected cases, literatures have no clear consensus of opinion on the type of surgical intervention and on the extent of optimization. In acute BAI, open reduction is recommended but the soft-tissue swelling may warrant staged internal fixation, after temporary external fixation. Case study: In this retrospective study we present the clinico-radiological outcome of surgically treated delayed presentation of BAI. The primary fixation in delayed cases and the role of reverse talar dome osteotomy for fusion in the chronic neglected case will be discussed. Patients were aged between 25 and 62 years, presented with isolated closed ankle injuries. Initial X-rays were consistent with BAI. The delay in presentation ranges from 2 to 49 days. All had open reduction and stabilization of the ankle. Chronic neglected case with 7 months delay had “Reverse talar dome osteotomy” for reduction of the neglected dislocation and proceeded for the primary fusion. Patients gained back their full weight-bearing mobility at an average of 12 weeks following delayed fixation. Fused ankle gained full weight-bearing mobility at 5 months post-operative period. ConclusionBosworth type of ankle dislocations are difficult injuries for closed reduction. Early diagnosis and recognition of fibular impingement require open reduction of the joint. In cases of delayed presentation, it could be challenging. Even after open reduction, a staged procedure may be warranted based on the soft tissue status. Osteotomy for joint reduction and primary fusion remains a viable option in neglected cases with an ultimate aim to regain plantigrade foot for mobility. Keywords: Bosworth ankle fracture, Irreducible ankle dislocation, Neglected ankle fracture, Primary ankle fusion, Reverse talar dome osteotomy.