Melorheostosis–an unusual cause of amputation

Graham, Lesley; Parke, R. C.

doi:10.1080/17461550500066808

Cited by 9 publications

(4 citation statements)

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“…Melorheostosis usually affects only one bone or one limb, and commonly involves the neurocranium and the hip bones (Leri and Joanny, 1992;Freyschmidt, 2001;Subhas et al, 2008;Abdullah et al, 2011). Radiographic images of this disease reveal cortical and endosteal involvement (Graham and Parke, 2005). Thus, we concluded that the specimens we evaluated is insufficiently compatible with melorheostosis.…”

Section: Discussionmentioning

confidence: 53%

Characterization of hypertrophic osteoarthropathy in an identified skeleton from Évora, Portugal, using combined and comparative morphology and microscopy

Anselmo

Silva

Fernandes

2016

International Journal of Paleopathology

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a b s t r a c tHypertrophic osteoarthropathy (HOA) is a bone pathology that can be associated with a number of focal or systemic diseases, particularly those affecting the respiratory system. HOA is characterized by proliferative periostosis of the long bones, especially in their distal and periarticular regions. This report presents a probable case of HOA in the skeleton of an elderly Portuguese male from Évora, Portugal, who died in 1970.The skeleton was evaluated by morphological, radiological, and histological methods. We found bilateral periosteal proliferation of the tubular bones, remodeling of the phalanges of the feet, and new bone formation on rib visceral surfaces. Bone alterations are more severe on the left tibia and fibula, where lesions display a 'tree bark' appearance, leading to severe thickening of the diaphysis.Microscopically, we observed deposition of porous woven bone. Our evaluation also considers a differential diagnosis of the lesions, which includes melorheostosis, hypervitaminosis A, fluorosis, thyroid acropachy, tuberculosis, and treponematosis.

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Section: Discussionmentioning

confidence: 53%

Characterization of hypertrophic osteoarthropathy in an identified skeleton from Évora, Portugal, using combined and comparative morphology and microscopy

Anselmo

Silva

Fernandes

2016

International Journal of Paleopathology

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“…In some particular cases, due to massive extension into the adjacent tissues and the complications related to the mass effect on the vascular and nervous structures, radical interventions such as the resection and reconstruction of the bone segment with tumor prostheses, arthroplasty, or even amputation may be required [ 34 , 35 , 36 ]. Possible complications of the surgery in our case are related to the disposition of the lesion in intimate contact with the vessels and the femoral nerve, the possibility of restoring limb length, and prosthetic difficulties, considering that at least 10 cm of intact bone was needed for fixation in the proximal segment.…”

Section: Discussionmentioning

confidence: 99%

Melorheostosis: A Review of the Literature and a Case Report

et al. 2023

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Background and Objectives: Melorheostosis, also referred to in the literature as Leri’s disease, is an unusual mesenchymal dysplasia with the clinical appearance of benign sclerosing bone dysplasia; it frequently occurs in late adolescence. Any bone in the skeletal system can be affected by this disease, though the long bones of the lower extremities are the most common, at any age. Melorheostosis has a chronic evolution, and symptoms are usually absent in the early stages. The etiopathogenesis is still unknown, however, numerous theories have been proposed that could explain the appearance of this lesion formation. An association with other benign or malignant bone lesions is also possible, and associations with osteosarcoma, malignant fibrous histiocytoma, or Buschke–Ollendorff syndrome have also been reported. There have also been reported cases of the malignant transformation of a pre-existing melorheostosis lesion into malignant fibrous histiocytoma or osteosarcoma. The diagnosis of melorheostosis can be made only based on radiological images, but, due to its polymorphism, additional imaging investigations are often necessary and sometimes only a biopsy can establish a definite diagnosis. Because there are currently no guidelines for treatment based on scientific evidence, due to the low number of cases diagnosed worldwide, our objective was to highlight the early recognition and specific surgical treatments for better prognosis and outcomes. Materials and Methods: We conducted a review of the literature consisting of original papers, case reports, and case series and presented the clinical and paraclinical characteristics of melorheostosis. We aimed to synthesize the treatment methods available in the literature as well as determine possible future directions related to the treatment of melorheostosis. Furthermore, we presented the results of a case of femoral melorheostosis admitted to the orthopedics department of the University Emergency Hospital of Bucharest in a 46-year-old female patient with severe pain in the left thigh and limitation of joint mobility. Following the clinical examination, the patient complained of pain in the middle third of the left thigh in the antero-medial compartment; the pain appeared spontaneously and was aggravated during physical activity. The pain started about two years prior, but the patient experienced complete pain relief after the administration of non-steroidal anti-inflammatory drugs. In the last six months, the patient presented an increase in pain intensity without significant improvement following the administration of non-steroidal anti-inflammatory drugs. The patient’s symptoms were mainly determined by the increase in the volume of the tumor and the mass effect on the adjacent tissues, especially on the vessels and the femoral nerve. The CT examination and bone scintigraphy showed a unique lesion in the middle third of the left femur and no oncological changes in the thoracic, abdominal, and pelvic regions; however, at the level of the femoral shaft, there was a localized cortical and pericortical bone lesion formation that surrounded approximately 180 degrees of the femoral shaft (anterior, medial, and lateral). It had a predominantly sclerotic structure but was associated with lytic areas with thickening of the bone cortex and areas of periosteal reaction. The next therapeutic gesture was to perform an incisional biopsy using a lateral approach at the level of the thigh. The histopathological result supported the diagnosis of melorheostosis. Additionally, immunohistochemical tests completed the data obtained after the microscopic examination through the classic histopathological technique The patient was discharged and included in a full medical recovery program for eight weeks in a specialized medical center, during which she also received analgesic treatment in maximum doses, but without improvement regarding her symptoms. Taking into account the chronic evolution of the pain, the complete lack of response to conservative treatment after eight weeks, and the lack of treatment guidelines in the case of melorheostosis, a surgical approach needed to be considered. The surgical option in this case, considering the circumferential location of the lesion at the level of the femoral diaphysis, was a radical resection. The surgical approach consisted of segmental resection to healthy bone tissue and reconstruction of the remaining defect with a modular tumoral prosthesis. At the 45-day postoperative control, the patient no longer complained of pain in the operated-on limb and was mobile with full support without gait difficulties. The follow-up period was one year, and the patient presented complete pain relief and a very good functional outcome. Results: In the case of asymptomatic patients, conservative treatment seems to be a good option with optimal results. However, for benign tumors, it remains unclear whether radical surgery is a viable option. Conclusions: Melorheostosis remains an incompletely understood disease, given the limited number of cases worldwide, and thus, there is a lack of clinical guidelines regarding specialized treatment.

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“…Radiological findings first described as "melting wax flowing down the side of a candle" 9 are characteristic of melorheostosis, but also sclerotic changes confined to a limb may be suggestive of this disease. 15 As a result, treatment must be individualized considering each case in isolation in order to obtain maximum benefits and faster recovery. 2 The disease usually has a chronic course with periods of exacerbations and arrest.…”

Section: Discussionmentioning

confidence: 99%

Arthroscopic Findings in Melorheostosis

Minarro

Perez²,

Benítez³

et al. 2015

JCR Journal of Clinical Rheumatology

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Melorheostosis is a rare dysplastic bone formation disease that can also affect the joints. We present a case of a patient with knee pain that was radiographically diagnosed as melorheostosis because of "dripping wax" image. An exploratory arthroscopy was made. In the joint, we found hyperplasic synovial tissue and an increased retropatellar Hoffa pad, which was surrounding an intra-articular ossification resulting from the disease. This was removed and led to a clinical and functional improvement.

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Melorheostosis–an unusual cause of amputation

Cited by 9 publications

References 8 publications

Characterization of hypertrophic osteoarthropathy in an identified skeleton from Évora, Portugal, using combined and comparative morphology and microscopy

Characterization of hypertrophic osteoarthropathy in an identified skeleton from Évora, Portugal, using combined and comparative morphology and microscopy

Melorheostosis: A Review of the Literature and a Case Report

Arthroscopic Findings in Melorheostosis

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