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Introduction: Melorheostosis was initially identified in 1922 by Leri and Joanny. It is often referred to as candle bone disease, melting wax syndrome, or Leri disease. This mesenchymal condition is uncommon, benign, and non-heritable. There is still a lack of clarity regarding the underlying cause of the condition. The estimated incidence is 0.9% per million. No inherited traits have been identified, and both sexes are equally affected. Pain or swelling is the most typical symptoms, and the onset is insidious. People often call this condition "candle disease of the bone" because the radiological signs of it look like dripping candle wax. Case Report: A 56-year-old female presented with an 8-year history of the left knee pain, swelling, and movement restriction. On examination, a hard, bony lump measuring 12×5 cm was located on the outer aspect of the left knee. The skin over the lump's surface was neither erythematous nor warm. Plain radiographs revealed a wide, dense, undulating, or irregular cortical hyperostosis, resembling candle wax, spreading along the length of the fibula bone as well as an extra-osseous calcification mass across the lateral aspect of the left knee. The patient underwent excision with complete left knee motion. Conclusion: Melorheostosis' actual etiology is yet unknown. There is no established treatment for this condition. Only symptomatic treatment helps patients feel better; in some circumstances, the excision of a bone mass yields more beneficial results.
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Femoral shaft fractures are major life- and limb-threatening injuries. Such injuries, when neglected for months or years, can lead to a range of debilitating consequences. In the Indian subcontinent, there are multiple such cases that are presented to tertiary care hospitals late due to a lack of awareness and low socioeconomic constraints. These neglected cases on presentation are usually in a state of nonunion or malunion, with gross shortening and deformity affecting adjacent joint mobility. The management of neglected femur shaft fractures (NFFs) demands uphill tasks, such as achieving mechanical stability, restoring length and alignment, and having strong rehabilitation schedules. The functional outcomes of these cases are always not proportional to their radiological counterparts and must be taken care of separately. In this study, intramedullary nailing of the fracture after initial distraction with the Ilizarov fixator aims to reduce deformity and shortening while restoring near-optimal functional life. The study aimed to evaluate the functional and radiological outcomes of NFFs operated with distraction by an Ilizarov fixator followed by intramedullary nailing. METHODS: Fourteen cases of NFFs presented to Kalinga Institute of Medical Sciences, Bhubaneswar, India, between January 2020 and June 2022 were recruited for the study. After preoperative evaluation and explanation of available treatment options to patients, they were operated on with a two-stage procedure of Ilizarov fixator application, followed by intramedullary interlocking nailing, with a period of gradual distraction in between. They were then followed up for a minimum of 12 months to assess functional and radiological outcomes. RESULTS: The average time for all of the fractures to heal was 25.2 weeks. The average knee flexion increased from 28.2 degrees before surgery to 87.1 degrees after surgery. All 14 patients could walk with complete weight bearing on the operated limb postoperatively after proper pain control measures were taken. The mean Tegner Lysholm knee score was 77.8. There was residual limping in six of the cases, which could be attributed to muscle atrophy and/or shortening in the affected limbs. In three cases, skin blisters were formed due to the acute nature of the distraction, but they all healed with a scab and scar, otherwise uneventfully. The shortening, in 13 cases, came down to 4 cm or less, which was managed with a shoe raise. The one case with a residual 5 cm shortening had a short, limping gait, and it was attributed to an extremely overriding osteopenic femur preoperatively. CONCLUSION: A two-stage operation with distraction by an Illizarov fixator followed by an intramedullary fixation provides the basic advantage of not having to excise an excessive amount of bone, which may be required in primary open reduction and intramedullary fixation. It also allows the patient to carry on his daily activities as mobilization is not restricted, which is the case in an individual to whom skeletal traction is applied. Hence, in any NFF case, this algorithm of management can be considered a frontrunner in the comprehensive management of disability and deformity.
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BACKGROUND: Treatment for osteoporosis can have catastrophic side effects, including the uncommon fracture known as an atypical femur fracture (AFF), which is related to the long-term usage of antiresorptive agents. Bisphosphonate therapy may lead to significant and chronic suppression of bone turnover, impairing the bone's remodelling property and finally leading to incomplete or complete atypical femur fracture. AFF was defined by the American Society for Bone and Mineral Research (ASBMR) Task Force in 2010 and is far less prevalent than proximal femur (hip) fracture, with an incidence of 2 to 78 per 100,000 patients per year following two to eight years of bisphosphonate therapy, respectively. Due to the rarity of the fracture, it is still not clear what the functional and radiological outcome will be after surgery. AIM: To identify the functional and radiological outcomes of surgical fixation of atypical femur fractures. METHODS: The study was conducted in a tertiary healthcare centre after scientific and ethical clearance from the competent authority. Between January 2018 and December 2021, individuals who were diagnosed with an atypical subtrochanteric femoral fracture associated with the use of bisphosphonates and treated surgically were retrospectively evaluated. The study's inclusion and exclusion criteria were used to include 20 patients. The features of an atypical subtrochanteric fracture were congruent with the radiographic findings. Most of the patients were treated with internal fixation with intramedullary osteosynthesis in standard with or without plate osteosynthesis. They were then followed up for a year to look at the functional and radiological outcomes. RESULTS: All of the 20 patients who were included had an atypical subtrochanteric fracture, with 15 of them being female and 5 of them being male. The patients' mean age at surgery was 65.12 (range 49 to 82) years, and their average history of bisphosphonate use was 3 (range 2.5 to 5) years. All patients were treated surgically. We found that five months was the mean period for bone union (p = 0.990). Within six months, bone union was achieved in 11 patients (55 %) (p = 0.884). Five patients (about 25%) had implant failure and non-union, requiring two to three revision surgeries. At three, six, and nine months, the mean visual analogue score (VAS) was 4.14, 3.12, and 1.85, respectively. The modified Hip Harris Score had a mean of 72.66 and 15 patients (about 75% of them) could walk normally again after a fracture. The mean of the modified HHS was 72.66, and the VAS at three, six, and nine months was 4.14, 3.12, and 1.85, respectively. CONCLUSION: AFFs are rare fractures that must be treated effectively, and most of them require surgery. Successful treatment of AFF is possible by the use of intramedullary fixation, which enhances axial stability, serves as an internal splint, and lessens the likelihood of implant failure. A good functional and radiological prognosis can come from a stable fixation and a fracture that has been reduced anatomically.
