Reumatología Clínica en imágenes
Crowned dens syndrome as a rare cause of severe cervical pain
Síndrome de Crowned Dens como causa rara de dolor cervical severo
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array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 2 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] 3 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Carlos" "apellidos" => "Vaz" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Rheumatology Department, University Hospital Center of São João, Porto, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Medicine of Faculty of Medicine, University of Porto, Porto, Portugal" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Center for Health Technology and Services Research (CINTESIS), Faculty of Medicine, University of Porto, Porto, Portugal" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome de Crowned Dens como causa rara de dolor cervical severo" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 591 "Ancho" => 1305 "Tamanyo" => 74450 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Axial (A) and sagittal (B) CT images of the patient's cervical spine, representing calcium pyrophosphate dihydrate crystals deposition around the odontoid process of C2 (red arrows).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Cervico-occipital computed tomography (CT) demonstrated deposition of calcium pyrophosphate dihydrate crystals around the odontoid process of axis in a 87-year-old female patient with initial diagnosis of polymyalgia rheumatica (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A, B). Patient had an acute severe cervico-occipital pain and markable neck stiffness. The patient had no shoulder or pelvic girdle pain and no jaw claudication. There is no history of trauma. There was a long personal history of knee pain due to calcium pyrophosphate dehydrate crystal deposition disease. Physical examination revealed limited passive cervical rotation without focal neurologic deficit. Laboratory results revealed elevation of erythrocyte sedimentation rate of 54<span class="elsevierStyleHsp" style=""></span>mm/h and a normal C-reactive protein (5<span class="elsevierStyleHsp" style=""></span>mg/dL). A knee radiography confirmed peripheral calcium pyrophosphate deposition disease. CT images showed atlantoaxial synovial calcifications in a crown around the odontoid process. A diagnosis of crowned dens syndrome (CDS) or periodontoid calcium pyrophosphate dehydrate crystal deposition disease was made. The patient was improved with a combination of prednisolone and nonsteroidal anti-inflammatory drug (NSAID) in a week. CDS is a rare clinical and radiological entity described firstly in 1985 and which pathophysiologic is not completely clear.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> This condition is accompanied by acute cervico-occipital pain and stiffness with severe limitation of neck rotation.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> The elevation of inflammatory markers is common.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> CDS can be misdiagnosed as various rheumatic diseases namely rheumatoid arthritis, spondyloarthritis, giant cell arthritis and PMR, as in our case.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4,5</span></a> For diagnosis, cervico-occipital CT, showing calcification around the odontoid process, is the gold standard and should be done as soon as symptoms appear.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> Patients respond well to corticosteroids, NSAIDs and colchicine.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> Surgical procedure is performed rarely. CDS is a rare disease entity with various differential diagnosis. Thus, a high index of clinical suspicion is needed to obtain an early accurate diagnosis avoiding unnecessary exams or treatments.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0010" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2021-12-20" "fechaAceptado" => "2022-02-10" "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 591 "Ancho" => 1305 "Tamanyo" => 74450 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Axial (A) and sagittal (B) CT images of the patient's cervical spine, representing calcium pyrophosphate dihydrate crystals deposition around the odontoid process of C2 (red arrows).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Acute neck pain due to calcifications surrounding the odontoid process: the crowned dens syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "J.P. Bouvet" 1 => "J.M. le Parc" 2 => "B. Michalski" 3 => "C. Benlahrache" 4 => "L. Auquier" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1002/art.1780281215" "Revista" => array:6 [ "tituloSerie" => "Arthritis Rheum" "fecha" => "1985" "volumen" => "28" "paginaInicial" => "1417" "paginaFinal" => "1420" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/4084331" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0040" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The crowned dens syndrome: a rare form of calcium pyrophosphate deposition disease" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "T. Baysal" 1 => "O. Baysal" 2 => "R. Kutlu" 3 => "I. Karaman" 4 => "B. 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Auquier" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:7 [ "tituloSerie" => "Rev Rhum Mal Osteoartic" "fecha" => "1982" "volumen" => "49" "paginaInicial" => "549" "paginaFinal" => "551" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/7123130" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S1873994612004564" "estado" => "S300" "issn" => "18739946" ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/1699258X/0000001800000008/v1_202210010847/S1699258X2200047X/v1_202210010847/en/main.assets" "Apartado" => array:4 [ "identificador" => "93550" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Reumatología Clínica en imágenes" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/1699258X/0000001800000008/v1_202210010847/S1699258X2200047X/v1_202210010847/en/main.pdf?idApp=UINPBA00004M&text.app=https://reumatologiaclinica.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1699258X2200047X?idApp=UINPBA00004M" ]
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