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Melorheostosis: a rare sclerosing bone dysplasia. Usefulness of bone scintigraphy
Melorreostosis: displasia ósea esclerosante infrecuente. Utilidad de la gammagrafía ósea
E.R. Marqués Aparicio
Corresponding author
enro5@hotmail.com

Corresponding author.
, J. Dolado Ardit, A. Crespo-Jara, A. Martínez Caballero, M.A. Antón Leal, J.M. Clavel Claver, M.M. Riera Ayora, J. Verdú Rico
Servicio de Medicina Nuclear, Hospital Universitario de Sant Joan d’Alacant, Alicante, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 35-year-old patient was diagnosed with melorheostosis 10 years previously and was treated with analgesics during symptomatic periods&#46; The patient was referred to nuclear medicine to undergo a whole body bone scintigraphy &#40;BS&#41; due to a new exacerbation of lower right limb pain&#46; Seven hundred forty MBq of <span class="elsevierStyleSup">99m</span>Tc-methylene diphosphonate were administered&#44; obtaining images with a dual head gamma camera &#40;Symbia<span class="elsevierStyleSup">&#174;</span>&#44; Siemens&#41;&#44; with low energy high resolution &#40;LEHR&#41; collimators at 2<span class="elsevierStyleHsp" style=""></span>h after injection of the radioisotope&#46; BS was performed with the following parameters of acquisition&#58; matrix of 256<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>1024 and acquisition speed of 16<span class="elsevierStyleHsp" style=""></span>cm&#47;min&#46; Polyostotic involvement of the lower limb and right hip was shown with no involvement of other bone structures &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Conventional radiology images &#40;X-ray&#41; and computed tomography &#40;CT&#41; are also shown &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41; showing typical findings of this disease&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Melorheostosis is also known as hyperostotic osteopathy&#44; monomelic eburnizing osteitis and Leri and Joanny disease&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> It is a benign process which&#44; according to the Fair-Bank classification&#44; is included among sclerosing dysplasias &#40;SD&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">2</span></a> Melorheostosis is very infrequent&#44; with only 400 cases having been reported worldwide since its first description in 1922 by Leri and Joanny&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">3</span></a> The etiology of this disease is not known&#44; and adjacent bone and soft tissue may be affected&#46; It may be monostotic or polyostotic&#44; with involvement of only one side of the bone being characteristic&#46; This disease may present at any age with no differences between sexes&#44; with approximately half of the cases being diagnosed at around 20 years of age&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">2</span></a> It is a chronic benign process that is not free of morbidity and limitations in everyday life due to pain&#44; contractures&#44; limitation in articulation mobility and bone deformities&#46; There is no specific treatment and symptomatic treatment of pain has classically been used as well as surgery for bone deformities or when there is important involvement of the soft tissues&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Imaging techniques play an important role in the diagnosis of melorheostosis&#44; and can rule out the need for more aggressive procedures such as biopsy&#46; In addition&#44; imaging studies are important in patient follow-up with evaluation of extension and personalized therapeutic management for these patients&#46; Useful imaging techniques include BS with <span class="elsevierStyleSup">99m</span>Tc-methylene diphosphonate which shows increased uptake in affected bones produced by hypervascularization and an increase in osteoblastic activity&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">3</span></a> There is an excellent correlation between BS and X-ray&#47;CT with respect to the distribution of the lesions&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">3</span></a> BS presents the advantage of providing a whole body bone study able to demonstrate other possible localizations of skeletal involvement by melorheostosis with a lower dose of radiation&#46; In addition&#44; BS can evaluate the site of activity during painful excerbation periods&#46; On the other hand&#44; BS helps to differentiate melorheostosis from other SD such as osteopoikilosis and striated osteopathy which present alterations in uptake&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">2&#44;3</span></a></p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Marqu&#233;s Aparicio ER&#44; Dolado Ardit J&#44; Crespo-Jara A&#44; Mart&#237;nez Caballero A&#44; Ant&#243;n Leal MA&#44; Clavel Claver JM&#44; et al&#46; Melorreostosis&#58; displasia &#243;sea esclerosante infrecuente&#46; Utilidad de la gammagraf&#237;a &#243;sea&#46; Rev Esp Med Nucl Imagen Mol&#46; 2019&#59;38&#58;243&#8211;244&#46;</p>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Bone scintigraphy with <span class="elsevierStyleSup">99m</span>Tc-methylene diphosphonate&#58; intense diffuse uptake in right femur&#44; extending outside the borders of the bone and deforming the bone&#46; The anterior border of the iliac crest is also affected as well as the acetabular region and homolateral patella&#46; Additional osteoblastic focus in the upper third of the tibia&#46;</p>"
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          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">&#40;a and b&#41; X-ray of right femur &#40;AP and lateral&#41;&#46; Excentric&#44; thick and diffuse hyperostosis in the right femur showing the classical image of &#8220;dripping candle wax&#8221;&#46; &#40;c&#41; Coronal CT image of the femur&#46; Thick hyperostosis in the internal slope of the femur&#46; &#40;d&#41; Axial CT image of the pelvis&#46; Osteoblastic involvement in the internal slope of the anterosuperior acetabular margin and internal region of the head of the femur&#46; In addition&#44; calcification is identified in the soft tissues and anterior lower right iliac spine&#46;</p>"
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Original language: English
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