[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"detail-sidebar-cat-0-en-105":3,"doc-seo-647754-105":59,"doc-detail-647754-en":135},{"code":4,"msg":5,"data":6},0,"success",[7,13,18,23,28,33,38,43,48,51,55],{"id":8,"doc_module":4,"doc_module_name":9,"category_name":10,"show_sort_weight":11,"slug":12},1,"Document","Story & Novel",90,"story-novel",{"id":14,"doc_module":4,"doc_module_name":9,"category_name":15,"show_sort_weight":16,"slug":17},2,"Literature",80,"literature",{"id":19,"doc_module":4,"doc_module_name":9,"category_name":20,"show_sort_weight":21,"slug":22},4,"Exam",70,"exam",{"id":24,"doc_module":4,"doc_module_name":9,"category_name":25,"show_sort_weight":26,"slug":27},5,"Comic",60,"comic",{"id":29,"doc_module":4,"doc_module_name":9,"category_name":30,"show_sort_weight":31,"slug":32},6,"Technology",50,"technology",{"id":34,"doc_module":4,"doc_module_name":9,"category_name":35,"show_sort_weight":36,"slug":37},7,"Healthcare",40,"healthcare",{"id":39,"doc_module":4,"doc_module_name":9,"category_name":40,"show_sort_weight":41,"slug":42},8,"Research & Report",30,"research-report",{"id":44,"doc_module":4,"doc_module_name":9,"category_name":45,"show_sort_weight":46,"slug":47},9,"Religion & Spirituality",20,"religion-spirituality",{"id":46,"doc_module":4,"doc_module_name":9,"category_name":49,"show_sort_weight":46,"slug":50},"World Cup","world-cup",{"id":52,"doc_module":4,"doc_module_name":9,"category_name":53,"show_sort_weight":52,"slug":54},10,"Lifestyle","lifestyle",{"id":56,"doc_module":4,"doc_module_name":9,"category_name":57,"show_sort_weight":24,"slug":58},19,"General","general",{"code":4,"msg":60,"data":61},"ok",{"site_id":62,"language":63,"slug":64,"title":65,"keywords":66,"description":67,"schema_data":68,"social_meta":128,"head_meta":130,"extra_data":132,"updated_unix":134},105,"en","upper-cervical-fusion-in-children-with-morquio-syndrome-intermediate-to-long-term-results","Upper Cervical Fusion in Children with Morquio Syndrome - Intermediate to Long-Term Results","","Study reports intermediate to long-term outcomes of upper cervical spine fusion in children with Morquio syndrome, a mucopolysaccharidosis associated with upper cervical instability and spinal cord compression. Twenty patients (nine female, eleven male) underwent fusion at a mean age of 63 months and were analyzed retrospectively using hospital records, with radiographic and clinical results documented. Mean follow-up was 8 years 10 months; fusion succeeded in all but one patient. Results show reliable neurological stability while highlighting distal junctional instability and cervicothoracic or thoracolumbar kyphotic deformity as key late concerns.",{"@graph":69,"@context":127},[70,84,106],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/upper-cervical-fusion-in-children-with-morquio-syndrome-intermediate-to-long-term-results/647754/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/upper-cervical-fusion-in-children-with-morquio-syndrome-intermediate-to-long-term-results/647754.png","ImageObject",300,407,{"name":92,"@type":93},"Emma Wilson","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-11-10","2026-10-23",true,{"@type":102,"interactionType":103,"userInteractionCount":105},"InteractionCounter",{"@type":104},"ViewAction",11,{"@type":107,"mainEntity":108},"FAQPage",[109,115,119,123],{"name":110,"@type":111,"acceptedAnswer":112},"What was the purpose of this study in children with Morquio syndrome?","Question",{"text":113,"@type":114},"To report intermediate to long-term results of upper cervical spine fusion for treating spinal cord compression due to upper cervical instability.","Answer",{"name":116,"@type":111,"acceptedAnswer":117},"How many patients were analyzed and what was the follow-up duration?",{"text":118,"@type":114},"Twenty patients were retrospectively analyzed, with an average follow-up of eight years and ten months.",{"name":120,"@type":111,"acceptedAnswer":121},"What were the main outcome findings regarding fusion and neurological status?",{"text":122,"@type":114},"Fusion was achieved in all patients except one (who required revision), and all patients were neurologically stable at the latest follow-up visit.",{"name":124,"@type":111,"acceptedAnswer":125},"What major long-term complication was emphasized in the conclusions?",{"text":126,"@type":114},"Distal junctional instability was identified as a major problem at long-term follow-up, along with possible kyphotic deformity at cervicothoracic and thoracolumbar junctions.","https://schema.org",{"og:url":83,"og:type":129,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":131,"canonical":83},"index,follow",{"doc_id":133,"site_id":62},647754,1792790035,{"code":4,"msg":5,"data":136},{"doc_id":133,"user_id":137,"nickname":92,"user_avatar":138,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":139,"file_id":140,"file_url":141,"file_type":142,"file_size":143,"view_count":105,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":39,"language":144,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":145,"faqs":146,"seo_title":147,"seo_description":67,"update_tm":134,"read_time":46,"preview_page_count":4},3848291630094,"https://eur-avatar.wpscdn.com/davatar_085a072bc5b1113ac321206ff7593b45","# Upper Cervical Fusion in Children withMorquio Syndrome\n\nIntermediate to Long-Term Results  \nOzgur Dede,MD,Mihir M.Thacker,MD,Kenneth J.Rogers,PhD,ATC,Murat Oto,MD,Mohan V.Belthur,MD,FRCS(Tr&Orth),Wagner Baratela,MD,and William G.Mackenzie,MD,FRCS(C)  \nInvestigation performed at the Department of Orthopaedic Surgery,Nemours/Alfred I.duPont Hospital for Children,Wilmington,Delaware  \nBackground:Paraplegia or death secondary to upper cervical spine instability and spinal cord compression are knownconsequences of Morquio syndrome.Decompression and fusion of the upper cervical spine are indicated to treat spinalcord compression.The purpose of this study was to report the intermediate to long-term results of upper cervical spinefusion in children with Morquio syndrome.  \nMethods:Twenty patients(nine female and eleven male)with Morquio syndrome who underwent upper cervical spinefusion at a mean age of sixty-three months were retrospectively analyzed with use of hospital records.Radiographic andclinical results were reported.  \nResults:The average follow-up period was eight years and ten months.Fusion was achieved inallpatients except one;this patient underwent a revision with transarticular C1-C2 screw fixation.Seven patients developed symptomatic in-stability below the fusion mass that required extension of fusion to lower levels at a mean of ninety-one months after theinitial operation.Two patients required decompression and fusion of a site other than the upper cervical spine.Asymp-tomatic cervicothoracic and thoracolumbar kyphosis was prevalent among our patients.All patients were neurologicallystable at the time of the latest follow-up visit  \nConclusions:Upper cervical spine fusion provides reliable fusion and a stable neural outcome in patients with Morquiosyndrome.However,distal junctional instability is a major problem at long-term follow-up.Kyphotic deformity of thecervicothoracic and thoracolumbar junction may be present in a large number of patients with Morquio syndrome andevaluation for spinal stenosis at these levels should also be considered.  \nLevel of Evidence:Therapeutic Level IV.See Instructions for Authors for a complete description of levels of evidence.  \nMorquio syndrome is the type ofmucopolysaccharidosis(type IV)with the most frequent skeletal conse-quences.In addition to extremity alignment ab-normalities,the natural progression of the disease may lead toquadriplegia and sudden death secondary to upper cervical spineinstability and cord compression'³.Specifically,children withMorquio syndrome have odontoid hypoplasia,ligamentous lax-ity,and extradural mucopolysaccharide deposition anterior tothe spinal cord at the C1 level that can result in atlantoaxialsubluxation,stenosis,and cervical myelopathy.The extraduralmucopolysaccharide deposition can accentuate cord compression,  \nDisclosure:None of the authors received payments or services,either directly or indirectly(i.e.,via his or her institution),from a third party in support ofany aspect of this work.None of the authors,or their institution(s),have had any financial relationship,in the thirty-six months prior to submission of thiswork,with any entity in the biomedical arena that could be perceived to influence or have the potential to influence what is written in this work.Also,noauthor has had any other relatonships,or has engaged in any other activities,that could be perceived to influence or have the potential to influence whatis written in this work.Thecomplete Disclosures of Potential Conflicts of Interest submitted by authors are always provided with the online version of thearticle.  \nand thus,the magnitude of cord compression could be more thanexpected on the basis of standard radiographs²45.The outcome ofdisease progression led several authors to recommend fusion ofthe upper cervical spine once instability is demonstrated²4-8.  \nSeveral studies have shown the results of upper cervicalfusion in patients with Morquio syndrome;however,long-termresu","cbCaik5G9BG2H95k","https://ap.wps.com/l/cbCaik5G9BG2H95k","pdf",6888002,"English","# Background\n# Methods\n# Results\n# Conclusions\n# Level of Evidence\n## Materials and Methods","[{\"question\":\"What was the purpose of this study in children with Morquio syndrome?\",\"answer\":\"To report intermediate to long-term results of upper cervical spine fusion for treating spinal cord compression due to upper cervical instability.\"},{\"question\":\"How many patients were analyzed and what was the follow-up duration?\",\"answer\":\"Twenty patients were retrospectively analyzed, with an average follow-up of eight years and ten months.\"},{\"question\":\"What were the main outcome findings regarding fusion and neurological status?\",\"answer\":\"Fusion was achieved in all patients except one (who required revision), and all patients were neurologically stable at the latest follow-up visit.\"},{\"question\":\"What major long-term complication was emphasized in the conclusions?\",\"answer\":\"Distal junctional instability was identified as a major problem at long-term follow-up, along with possible kyphotic deformity at cervicothoracic and thoracolumbar junctions.\"}]","Upper Cervical Fusion in Children with Morquio Syndrome - Intermediate to Long-Term Results | PDF"]