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Case report
Bilateral congenital seudarthrosis of the clavicle. A clinical case
Seudoartrosis congénita de clavícula bilateral. Caso clínico
A. Nieto Gil
Corresponding author
a.nieto@hotmail.es

Corresponding author.
, A. Gómez Navalón, P. Zorrilla Ribot
Servicio de Traumatología y Cirugía Ortopédica, Hospital General Universitario de Ciudad Real, Ciudad Real, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical case</span><p id="par0005" class="elsevierStylePara elsevierViewall">We present the case of a male aged 3 months&#44; the third of three siblings&#44; whose delivery was normal with cephalic presentation&#46; We detected a family history of &#8220;coffee stain&#8221; marks suggestive of neurofibromatosis inherited from the maternal grandmother and a sister&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">In the study conducted by the neuropaediatric unit&#44; no neurological changes were found in the patient&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">The child was referred to the Department of orthopaedics and traumatology by his paediatrician after it was noted that he had a small lump in both clavicles&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Examination revealed normal movement of the neck and arms&#46; There was an absence of asymmetry and there were small nodules in the middle third of both clavicles&#46; The parents mentioned that at no time since birth had the child suffered from pain or impaired mobility&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The X-ray at one week old &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#46;&#41; shows an image compatible with a fracture versus bilateral pseudarthrosis of the clavicles&#46; The study was repeated at 4 months &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41; and the persistence of the image of pseudarthrosis may be observed in both clavicles&#46; Since there was neither sign of consolidation nor any background of a traumatic birth or previous trauma&#44; bilateral congenital pseudarthrosis of the clavicle was diagnosed&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Discussion</span><p id="par0030" class="elsevierStylePara elsevierViewall">Congenital pseudarthrosis of the clavicle is a rare pathology and there are few references to it in literature&#46; 200 cases have been described in literature&#44; most frequently in girls&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> Bilateralism is uncommon&#44; and there are 7 published cases&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Its aetiology has not been clarified with certainty&#46; Several authors believe that this is due to a failure in the consolidation of the two ossification centres of the clavicle&#46;<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3&#44;4</span></a> Others believe it originates from a defect in embryological development during the initial stages&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> It has also been attributed to a causal link between the foetal head and cephalic delivery&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> Several authors have found a certain relationship between congenital pseudarthrosis of the clavicle and narrow superior thoracic outlet syndome&#44;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> and between Ehlers&#8211;Danlos syndrome<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> and neurofibromatosis&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Although cases of autosomal inheritance have been described&#44; in general their presentation is sporadic&#46; However&#44; autosomal dominant genetic transmission is a possibility&#46; Cases of family involvement also exist&#46; A relationship with the chromosome 10p11&#46;21p12<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> has also been found&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Diagnosis is clinical and radiological&#46; It presents as a painless deformity at the clavicle in the absence of any background of obstetric trauma and with no mobility impairment&#46; It is usually diagnosed in the first few years of life&#44; due to the aesthetic defect it entails&#46; Usually the mother realises there is a lump&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">In the majority of cases a biopsy is not necessary&#46; An X-ray reveals the bone involvement at both ends of the clavicle with the middle and anterior bone fragment being wider than the lateral one&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">If hypertrophic pseudarthrosis is present the anatomopathological imaging shows up hyaline cartilage<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> with chondrocytes in different stages of maturity with the same pattern as that of the growth plate&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> If it is a case of atrophic pseudarthrosis we may have a pattern of fibrous connective tissue&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;5</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Differential diagnosis must primarily take place with obstetric fracture of the clavicle&#46; In these cases there may be a background of trauma&#44; from&#44; for example&#44; a delivery with dystocia&#44; macrosomia or the use of forceps&#46; The fracture callus would be visible in an X-ray and there would be pain on physical examination&#46; It is also necessary to differentiate from cleidocranial dysostosis&#44;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> where the patient would also present with bulging fontanelles&#44; an impairment in the development of the sternum&#44; spine and pelvis in addition to having bilateral involvement and a family history of the condition&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">With regards to treatment&#44; this is controversial&#46; Several authors prefer conservative treatment&#44; provided that there are no functional limitations&#44;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> vascular nerve compression and that the aesthetic defect is tolerated by the patient&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> Those who prefer surgical treatment advise non-intervention until the age of 4&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> The majority of authors only recommend surgery in the case of symptomatic patients or those with major deformities&#46; Surgical correction basically consists of the resection of fibrous tissue and osteosynthesis using Kirschner<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> needles or plates in addition to grafts&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2&#44;6</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Prakash Chandran et al&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> in their study refer to a lower rate of infection and complications with the use of plates compared to osteosynthesis with Kirschner needles&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">Unlike congenital pseudarthrosis&#44; in post-traumatic pseudarthrosis the majority of patients require surgical treatment due to pain and impaired mobility associated with these lesions&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">To conclude&#44; we presented this case due to its exceptional nature&#44; low prevalence and because it concerned a male patient with bilateral involvement&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">We would highlight the scarcity of published literature in this regard and the absence of long term studies&#46; As a result&#44; from our point of view&#44; it would be essential to carry out a follow-up of the patient to periodically assess the evolution of the condition&#46; Should clinical symptoms&#44; outstanding deformity and&#47;or impairment of mobility occur we would suggest surgery be performed&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Ethical disclosures</span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Protection of people and animals subjects</span><p id="par0090" class="elsevierStylePara elsevierViewall">The authors declare that no experiments have been carried out on humans or animals for this investigation&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Confidentiality of data</span><p id="par0095" class="elsevierStylePara elsevierViewall">The authors declare that they have adhered to the protocols of their centre of work regarding the publication of patient data&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Right to privacy and informed consent</span><p id="par0100" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Funding</span><p id="par0105" class="elsevierStylePara elsevierViewall">There was no type of financing&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflict of interests</span><p id="par0110" class="elsevierStylePara elsevierViewall">The authors have no conflict of interests to declare&#46;</p></span></span>"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Congenital pseudarthrosis of the clavicle is a rare malformation in which the aetiology is still unclear&#46; Bilateral involvement is exceptional&#46; Although it is a congenital malformation&#44; it may not be diagnosed until late childhood&#44; with patients presenting with a painless deformity of the middle third of the clavicle in the absence of prior trauma&#46; The treatment is controversial&#44; and may be surgical&#44; depending on the functional impact and aesthetics&#46; A case of bilateral involvement is presented&#44; together with a review of the relevant literature&#46;</p></span>"
      ]
      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La seudoartrosis cong&#233;nita de clav&#237;cula es una rara malformaci&#243;n de etiolog&#237;a todav&#237;a no aclarada&#46; La afectaci&#243;n bilateral es excepcional&#46; Aun siendo una malformaci&#243;n cong&#233;nita su diagn&#243;stico puede prolongarse hasta avanzada la ni&#241;ez&#44; presentando los pacientes una deformidad indolora del tercio medio de la clav&#237;cula en ausencia de traumatismo previo&#46; El tratamiento es controvertido&#44; y puede ser quir&#250;rgico o no seg&#250;n la repercusi&#243;n funcional y est&#233;tica&#46; Presentamos un caso de afectaci&#243;n bilateral y analizamos la bibliograf&#237;a encontrada al respecto&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Nieto Gil A&#44; G&#243;mez Naval&#243;n A&#44; Zorrilla Ribot P&#46; Seudoartrosis cong&#233;nita de clav&#237;cula bilateral&#46; Caso cl&#237;nico&#46; Rev Esp Cir Ortop Traumatol&#46; 2016&#59;60&#58;397&#8211;399&#46;</p>"
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                      "titulo" => "Congenital pseudoarthrosis of the clavicle"
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ISSN: 19888856
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es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos