[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"detail-sidebar-cat-0-en-105":3,"doc-seo-408003-105":59,"doc-detail-408003-en":130},{"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":123,"head_meta":125,"extra_data":127,"updated_unix":129},105,"en","defying-the-odds-survival-in-severe-prenatal-caffeys-disease","Defying the Odds - Survival in Severe Prenatal Caffey's Disease","","Caffey’s disease (infantile cortical hyperostosis) is a rare infant disorder marked by subperiosteal new bone formation and diffuse soft-tissue inflammation, with an even rarer prenatal form presenting before birth. This severe prenatal variant causes symmetric cortical thickening and often leads to perinatal death, especially early in gestation, with risks including pulmonary hypoplasia and hepatic dysfunction. A premature infant born at 27 weeks survived after prolonged NICU care, then showed near-complete skeletal remodeling at one year and complete radiographic resolution by two years.",{"@graph":69,"@context":122},[70,84,105],{"@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/defying-the-odds-survival-in-severe-prenatal-caffeys-disease/408003/",{"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/defying-the-odds-survival-in-severe-prenatal-caffeys-disease/408003.png","ImageObject",300,407,{"name":92,"@type":93},"Kurz","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-29","2026-09-28",true,{"@type":102,"interactionType":103,"userInteractionCount":44},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What distinguishes prenatal Caffey’s disease from infantile cortical hyperostosis?","Question",{"text":112,"@type":113},"Prenatal cortical hyperostosis presents before birth with diffuse, symmetric cortical thickening and is more non-inflammatory; its pathogenetic mechanism is less defined than infantile cortical hyperostosis.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How was the diagnosis of severe prenatal Caffey’s disease supported in the case?",{"text":117,"@type":113},"Prenatal ultrasound showed polyhydramnios, fetal hydrops, and abnormal long-bone ossification. Postnatal radiographs demonstrated diffuse “cotton-wool” periosteal reactions across all long bones and ribs, while lab and genetic testing excluded conditions such as osteogenesis imperfecta, congenital infection, and skeletal dysplasia.",{"name":119,"@type":110,"acceptedAnswer":120},"What outcome did the infant have after treatment and follow-up?",{"text":121,"@type":113},"The infant required prolonged ventilatory support but gradually improved and was discharged after a 19-week NICU stay. Follow-up imaging showed near-complete remodeling at one year and normal growth and complete radiographic resolution by two years.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},408003,1791100544,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":44,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":52,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":144,"preview_page_count":4},2336478945635,"https://ap-avatar.wpscdn.com/davatar_6f874abed73319feea01a86fa6f0fab8","Review began 10/14/2025  \nReview ended 11/21/2025  \nPublished 11/30/2025  \n© Copyright 2025  \nMcIlwain et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CCBY 4.0. , which permits unrestricted use , distribution , and reproduction in any medium , provided the original author and source are credited.  \nDOI: 10.7759/cureus.98173  \nOpen Access Case Report  \nDefying the Odds: Survival in Severe Prenatal Caffey's Disease  \nRyan N. McIlwain 1, Andrew J. Heflin 2, Sharon Maina 2, William A. Cutchen 2 , Tyler C. McDonald 2  \n1. Department of Medicine, Frederick P. Whiddon College of Medicine, University of South Alabama, Mobile, USA 2. Department of Orthopaedic Surgery, University of South Alabama, Mobile, USA  \nCorresponding author: Tyler C. McDonald, [tylermcdonaldmd@gmail.com](tylermcdonaldmd@gmail.com)  \nAbstract  \nCaffey’s disease, or infantile cortical hyperostosis, is a rare disorder characterized by subperiosteal new bone formation and diffuse soft tissue inflammation in infants younger than six months. A much rarer prenatal form presents before birth with diffuse, symmetric cortical thickening and often results in perinatal death, especially when it develops early in gestation. The severe form may be associated with pulmonary hypoplasia, hepatic dysfunction, and high neonatal mortality. We report the case of a premature infant born at 27 weeks’ gestation to a 36-year-old mother with chronic hypertension. Prenatal ultrasound demonstrated polyhydramnios, fetal hydrops, and abnormal long-bone ossification. Postnatal radiographs showed diffuse “cotton-wool” periosteal reactions involving all long bones and ribs, consistent with prenatal Caffey’s disease. Differential diagnoses such as osteogenesis imperfecta, congenital infection, and skeletal dysplasia were excluded through laboratory and genetic testing. The infant required prolonged ventilatory support but gradually improved and was discharged after a 19-week neonatal intensive care stay. At oneyear follow-up, imaging showed near-complete remodeling of affected bones with only mild humeral deformity, and by two years, the child demonstrated normal growth, ambulation, and complete radiographic resolution. With diagnosis at 27 weeks, this case highlights one of the earliest documented survivors of severe prenatal Caffey 's disease and suggests that once complications of prematurity are managed, the skeletal manifestations can resolve spontaneously. Awareness of this condition may help avoid misdiagnosis and guide appropriate counseling and follow-up.  \nCategories: Radiology, Pediatrics, Orthopedics  \nKeywords: caffey 's disease, fetal bone disease, infantile cortical hyperostosis, neonatal bone disorders, periosteal reaction, prenatal cortical hyperostosis  \nIntroduction  \nCaffey’s disease, or infantile cortical hyperostosis (ICH), is a disorder characterized by spontaneous subperiosteal bone formation and diffuse inflammation [1] . An autosomal-dominant inheritance associated with a Type I collagen mutation (COL1A 1) has been described [2] . It is hypothesized that alterations in Type I collagen impair extracellular matrix components and alter cell signaling, propagating a proliferative cell response [3] . It typically manifests in newborns before six months of age with global soft tissue swelling and is identified by the characteristic “patchy” areas of bone growth on radiographs, which are prone to spontaneous dissolution and recurrence. ICH is a self-limiting condition with typical resolution by three years [4,5] .  \nThere is a much rarer form, prenatal cortical hyperostosis (PCH), which is a distinct entity. Compared to ICH, this form is more diffuse, non-inflammatory, and the pathogenetic mechanism is less defined [2] . Osseous involvement is symmetric, manifesting as cortical bone thickening, or “cotton-wool” appearance, and angulated long bones are more common [6] . Most cases are lethal in the neonatal period (e","cbCain4GqvWtSpHL","https://ap.wps.com/l/cbCain4GqvWtSpHL","pdf",6103312,"English","# Abstract\n# Introduction\n# Case Presentation","[{\"question\":\"What distinguishes prenatal Caffey’s disease from infantile cortical hyperostosis?\",\"answer\":\"Prenatal cortical hyperostosis presents before birth with diffuse, symmetric cortical thickening and is more non-inflammatory; its pathogenetic mechanism is less defined than infantile cortical hyperostosis.\"},{\"question\":\"How was the diagnosis of severe prenatal Caffey’s disease supported in the case?\",\"answer\":\"Prenatal ultrasound showed polyhydramnios, fetal hydrops, and abnormal long-bone ossification. Postnatal radiographs demonstrated diffuse “cotton-wool” periosteal reactions across all long bones and ribs, while lab and genetic testing excluded conditions such as osteogenesis imperfecta, congenital infection, and skeletal dysplasia.\"},{\"question\":\"What outcome did the infant have after treatment and follow-up?\",\"answer\":\"The infant required prolonged ventilatory support but gradually improved and was discharged after a 19-week NICU stay. Follow-up imaging showed near-complete remodeling at one year and normal growth and complete radiographic resolution by two years.\"}]","Defying the Odds - Survival in Severe Prenatal Caffey's Disease | PDF",1790577629,25]