[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"doc-seo-440967-105":3,"detail-sidebar-cat-0-en-105":80,"doc-detail-440967-en":130},{"code":4,"msg":5,"data":6},0,"ok",{"site_id":7,"language":8,"slug":9,"title":10,"keywords":11,"description":12,"schema_data":13,"social_meta":73,"head_meta":75,"extra_data":77,"updated_unix":79},105,"en","pediatric-triad-of-craniofacial-fibrous-dysplasia-chiari-malformation-type-i-and-syringomyelia-case-report","Pediatric triad of craniofacial fibrous dysplasia, Chiari malformation type I and syringomyelia - case report","","Fibrous dysplasia is a benign bone disorder in which normal bone is replaced by fibrous tissue, creating irregular skeletal structure. Craniofacial fibrous dysplasia combined with Chiari malformation type I and syringomyelia is extremely rare in children. This case report presents complex clinical manifestations of all three conditions and shows that surgical management substantially improved prognosis. Follow-up demonstrated near-complete resolution of syringomyelia and provides guidance for similar future cases.",{"@graph":14,"@context":72},[15,34,55],{"@type":16,"itemListElement":17},"BreadcrumbList",[18,23,27,31],{"item":19,"name":20,"@type":21,"position":22},"https://docshare.wps.com","Home","ListItem",1,{"item":24,"name":25,"@type":21,"position":26},"https://docshare.wps.com/document/","Document",2,{"item":28,"name":29,"@type":21,"position":30},"https://docshare.wps.com/document/research-report/","Research & Report",3,{"item":32,"name":10,"@type":21,"position":33},"https://docshare.wps.com/document/pediatric-triad-of-craniofacial-fibrous-dysplasia-chiari-malformation-type-i-and-syringomyelia-case-report/440967/",4,{"url":32,"name":10,"@type":35,"image":36,"author":41,"headline":10,"publisher":44,"fileFormat":47,"inLanguage":8,"description":12,"dateModified":48,"datePublished":49,"encodingFormat":47,"isAccessibleForFree":50,"interactionStatistic":51},"DigitalDocument",{"url":37,"@type":38,"width":39,"height":40},"https://docshare.wps.com/thumbnails/pediatric-triad-of-craniofacial-fibrous-dysplasia-chiari-malformation-type-i-and-syringomyelia-case-report/440967.png","ImageObject",300,407,{"name":42,"@type":43},"Hazel","Person",{"url":19,"name":45,"@type":46},"DocShare","Organization","application/pdf","2026-10-01","2026-09-29",true,{"@type":52,"interactionType":53,"userInteractionCount":26},"InteractionCounter",{"@type":54},"ViewAction",{"@type":56,"mainEntity":57},"FAQPage",[58,64,68],{"name":59,"@type":60,"acceptedAnswer":61},"What makes this pediatric triad clinically notable?","Question",{"text":62,"@type":63},"Craniofacial fibrous dysplasia associated with Chiari malformation type I and syringomyelia is described as extremely rare in children, and the case highlights their combined clinical manifestations.","Answer",{"name":65,"@type":60,"acceptedAnswer":66},"How did surgery affect syringomyelia in the reported patient?",{"text":67,"@type":63},"Postoperative MRI showed a significant decrease in syringomyelia, and by six months there was nearly complete resolution.",{"name":69,"@type":60,"acceptedAnswer":70},"What diagnostic findings supported the final diagnosis?",{"text":71,"@type":63},"Histopathological examination of the resected cranial bone confirmed fibrous dysplasia, while cervical MRI demonstrated tonsillar herniation and syringomyelia extending below T5.","https://schema.org",{"og:url":32,"og:type":74,"og:title":10,"og:site_name":45,"og:description":12},"article",{"robots":76,"canonical":32},"index,follow",{"doc_id":78,"site_id":7},440967,1790834883,{"code":4,"msg":81,"data":82},"success",[83,87,91,95,100,105,110,114,119,122,126],{"id":22,"doc_module":4,"doc_module_name":25,"category_name":84,"show_sort_weight":85,"slug":86},"Story & Novel",90,"story-novel",{"id":26,"doc_module":4,"doc_module_name":25,"category_name":88,"show_sort_weight":89,"slug":90},"Literature",80,"literature",{"id":33,"doc_module":4,"doc_module_name":25,"category_name":92,"show_sort_weight":93,"slug":94},"Exam",70,"exam",{"id":96,"doc_module":4,"doc_module_name":25,"category_name":97,"show_sort_weight":98,"slug":99},5,"Comic",60,"comic",{"id":101,"doc_module":4,"doc_module_name":25,"category_name":102,"show_sort_weight":103,"slug":104},6,"Technology",50,"technology",{"id":106,"doc_module":4,"doc_module_name":25,"category_name":107,"show_sort_weight":108,"slug":109},7,"Healthcare",40,"healthcare",{"id":111,"doc_module":4,"doc_module_name":25,"category_name":29,"show_sort_weight":112,"slug":113},8,30,"research-report",{"id":115,"doc_module":4,"doc_module_name":25,"category_name":116,"show_sort_weight":117,"slug":118},9,"Religion & Spirituality",20,"religion-spirituality",{"id":117,"doc_module":4,"doc_module_name":25,"category_name":120,"show_sort_weight":117,"slug":121},"World Cup","world-cup",{"id":123,"doc_module":4,"doc_module_name":25,"category_name":124,"show_sort_weight":123,"slug":125},10,"Lifestyle","lifestyle",{"id":127,"doc_module":4,"doc_module_name":25,"category_name":128,"show_sort_weight":96,"slug":129},19,"General","general",{"code":4,"msg":81,"data":131},{"doc_id":78,"user_id":132,"nickname":42,"user_avatar":133,"doc_module":4,"category_id":111,"category_name":29,"doc_title":10,"doc_description":12,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":26,"is_deleted":4,"is_public":22,"is_downloadable":22,"audit_status":22,"page_count":96,"language":139,"language_code":8,"site_id":7,"html_lang":8,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":12,"update_tm":143,"read_time":144},137441390410,"https://ap-avatar.wpscdn.com/avatar/2000252f4ab5702993?_k=1776741390130283984","Acta Neurochirurgica (2026) 168:2  \n[https://doi.org/10.1007/s00701-025-06756-5](https://doi.org/10.1007/s00701-025-06756-5)  \nPediatric triad of craniofacial fibrous dysplasia, Chiari malformation type I and syringomyelia: a case report  \nYaxiong Li1 · Jianfeng Liu1 · Jian Guan2 · Conghui Li1  \nReceived: 14 December 2025 / Accepted: 18 December 2025 © The Author(s) 2026  \nAbstract  \nFibrous dysplasia is a benign bone disease characterized by the replacement of normal bone tissue with fibrous tissue, resulting in irregular bone structure. Cases of craniofacial fibrous dysplasia in children associated with Chiari type I malformation and syringomyelia are extremely rare. This case illustrates the complex clinical manifestations of craniofacial fibrous dysplasia along with Chiari type I malformation and syringomyelia, in which surgical intervention significantly improved the prognosis, and follow-up revealed near-complete resolution of the syringomyelia. It offers valuable insights for managing similar cases in the future.  \nKeywords Fibrous dysplasia · Craniofacial · Chiari type I malformation · Syringomyelia · Cerebrospinal fluid  \nAbbreviations  \nFD  \nCFD  \nCM1  \nSM  \nCSF  \nCT  \nMRI  \nASIA  \nT  \nP-VEPQ-F-VEPPCFV  \nFibrous dysplasia Craniofacial fibrous dysplasia Chiari type I malformation Syringomyelia Cerebrospinal fluid Computed tomography Magnetic resonance imaging  \nAmerican Spinal Injury Association Impairment Scale  \nThoracic  \nPattern visual evoked potential Flash visual evoked potential Posterior cranial fossa volume  \n* Conghui Li[liyaxiong414@163.com](liyaxiong414@163.com)  \n1 Department of Neurosurgery, The First Hospital of Hebei Medical University, 89\\# Dong-Gang Road, Shijiazhuang, Hebei Provence 050000, China  \n2 Department of Neurosurgery, China International Neurological Institute, Xuanwu Hospital, Capital Medical University, Beijing, China  \nIntroduction  \nFibrous dysplasia (FD) is a rare benign fibrous bone lesion characterized by the abnormal growth of fibrous tissue within the marrow cavity, replacing normal bone structure and leading to skeletal deformities, fractures and compression of adjacent neurovascular structures [8] . When the lesion involves the craniofacial region (Craniofacial fibrous dysplasia, CFD), it can cause osseous remodeling and volume changes. Such structural alterations may predispose patients to Chiari type I malformation (CM1), which is defined by the herniation of the cerebellar tonsils below the foramen magnum (≥ 5 mm), and is frequently associated with syringomyelia (SM) . SM involves fluid-filled cavities within the spinal cord due to abnormal accumulation of cerebrospinal fluid, which can cause spinal cord damage and functional deficits [1, 13] . Literature indicates that the incidence of cranial FD is approximately 1 in 15,000–30,000 [8], and cases complicated by CM1 and SM are extremely rare. This article reports a clinical case of a child with CFD, CM1 and SM, looking to explore how these three conditions are related and how they can be treated.  \nCase report  \nA 16-year-old girl was hospitalized due to unsteady gait and intermittent dizziness for 2 years. Five years ago, after experiencing vision loss, she was diagnosed with FD at a local  \nhospital and underwent bilateral optic canal decompression. However, her vision showed no significant improvement after the surgery. Upon admission, the physical examination revealed a left pupil of 4.0 mm with absent light reflex and a right pupil of 3.0 mm with brisk light reflex. Muscle strength was grade 5 in all extremities, and muscle tone was normal. Bilateral Babinski signs were negative, and the Romberg sign was positive. Alkaline phosphatase (ALP) was 3167.5 U/L (reference range, 35.0–135.0), and human growth hormone (HGH) was 2.42 μg/L (reference range, 1–5); the remaining blood tests were within normal limits. There were no skin plaques, precocious puberty, hyperthyroidism and other signs of McCune-Albright syndrome (MAS). Cr","cbCaiaCrb7BdApUe","https://ap.wps.com/l/cbCaiaCrb7BdApUe","pdf",978740,"English","# Abstract\n# Introduction\n# Case report\n## Preoperative presentation and investigations\n## Surgical procedure\n## Histopathology and postoperative outcome","[{\"question\":\"What makes this pediatric triad clinically notable?\",\"answer\":\"Craniofacial fibrous dysplasia associated with Chiari malformation type I and syringomyelia is described as extremely rare in children, and the case highlights their combined clinical manifestations.\"},{\"question\":\"How did surgery affect syringomyelia in the reported patient?\",\"answer\":\"Postoperative MRI showed a significant decrease in syringomyelia, and by six months there was nearly complete resolution.\"},{\"question\":\"What diagnostic findings supported the final diagnosis?\",\"answer\":\"Histopathological examination of the resected cranial bone confirmed fibrous dysplasia, while cervical MRI demonstrated tonsillar herniation and syringomyelia extending below T5.\"}]","Pediatric triad of craniofacial fibrous dysplasia, Chiari malformation type I and syringomyelia - case report | PDF",1790694121,13]