Crowned dens syndrome: A case series of 72 patients at eight teaching hospitals in Japan

Abstract Background Crowned dens syndrome (CDS) is characterized by calcification around the odontoid process, accompanied by neck pain. Although CDS is supposedly rare, we regularly diagnose and manage this condition, indicating a perception gap between previous studies and our experience. The purp...

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Main Authors: Hiroki Isono, Haruka Kuno, Takunori Hozumi, Ken Emoto, Sho Nishiguchi, Masahiro Sakai, Madoka Ito, Koichi Kitamura, Kazuhito Hirose, Eiji Hiraoka, Naoto Ishimaru, Hiroyuki Kobayashi, Yasuharu Tokuda
Format: Article
Language:English
Published: Wiley 2023-05-01
Series:Journal of General and Family Medicine
Subjects:
Online Access:https://doi.org/10.1002/jgf2.616
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author Hiroki Isono
Haruka Kuno
Takunori Hozumi
Ken Emoto
Sho Nishiguchi
Masahiro Sakai
Madoka Ito
Koichi Kitamura
Kazuhito Hirose
Eiji Hiraoka
Naoto Ishimaru
Hiroyuki Kobayashi
Yasuharu Tokuda
author_facet Hiroki Isono
Haruka Kuno
Takunori Hozumi
Ken Emoto
Sho Nishiguchi
Masahiro Sakai
Madoka Ito
Koichi Kitamura
Kazuhito Hirose
Eiji Hiraoka
Naoto Ishimaru
Hiroyuki Kobayashi
Yasuharu Tokuda
author_sort Hiroki Isono
collection DOAJ
description Abstract Background Crowned dens syndrome (CDS) is characterized by calcification around the odontoid process, accompanied by neck pain. Although CDS is supposedly rare, we regularly diagnose and manage this condition, indicating a perception gap between previous studies and our experience. The purpose of this study was to determine the annual incidence of CDS, time to diagnosis in CDS, as well as the features of CDS. Methods The study design was a retrospective case series study conducted at eight teaching hospitals in Japan. We identified CDS cases from April 2013–March 2015. CDS was diagnosed when patients had acute onset of neck pain and CT showed calcification around the dens and when other diagnoses were unlikely. Results Seventy‐two CDS cases were identified. Mean annual incidence was 4.6 ± 2.3 cases at each hospital. Among those with available data, 57 of 64 had limited rotation (89.1%). The diagnosis of CDS was made in general internal medicine or the emergency medicine department in 61 cases (84.7%). A total of 62 cases (86.1%) were diagnosed within 1 day of presentation, and the median time from initial presentation at the hospital to diagnosis was 0.0 days (25th–75th percentiles, 0.0–1.0). For treatment, NSAIDs were used in 56cases (77.8%) and acetaminophen in 20 cases (27.8%). Conclusion CDS might be more common than has been reported to date. Time to diagnosis of CDS was within 1 day of visiting a teaching hospital. Cervical motion restriction is common in CDS and may be useful in establishing the diagnosis.
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spelling doaj.art-cdb3d9473b6c4783bf4493169ba50dd22023-05-30T08:56:11ZengWileyJournal of General and Family Medicine2189-79482023-05-0124317117710.1002/jgf2.616Crowned dens syndrome: A case series of 72 patients at eight teaching hospitals in JapanHiroki Isono0Haruka Kuno1Takunori Hozumi2Ken Emoto3Sho Nishiguchi4Masahiro Sakai5Madoka Ito6Koichi Kitamura7Kazuhito Hirose8Eiji Hiraoka9Naoto Ishimaru10Hiroyuki Kobayashi11Yasuharu Tokuda12Department of General Medicine HITO Medical Center Ehime JapanDepartment of Family Medicine, General Practice and Community Health, Faculty of Medicine University of Tsukuba Ibaraki JapanDepartment of General Internal Medicine Kameda Medical Center Chiba JapanDepartment of General Internal Medicine Aso Iizuka Hospital Fukuoka JapanDepartment of General Internal Medicine Shonan Kamakura General Hospital Kanagawa JapanDepartment of Nephrology Endocrinology and Diabetes, Tokyo Bay Urayasu Ichikawa Medical Center Chiba JapanDepartment of Palliative Medicine Kobe University Graduate School of Medicine Hyogo JapanDepartment of Nephrology Endocrinology and Diabetes, Tokyo Bay Urayasu Ichikawa Medical Center Chiba JapanDepartment of General Medicine Tsukuba Medical Center Hospital Ibaraki JapanDepartment of Internal Medicine Tokyo Bay Urayasu Ichikawa Medical Center Chiba JapanDepartment of General Internal Medicine Akashi Medical Center Hyogo JapanDepartment of General Medicine Tsukuba University Hospital Ibaraki JapanDepartment of Medicine, Muribushi Okinawa Center for Teaching Hospitals Okinawa JapanAbstract Background Crowned dens syndrome (CDS) is characterized by calcification around the odontoid process, accompanied by neck pain. Although CDS is supposedly rare, we regularly diagnose and manage this condition, indicating a perception gap between previous studies and our experience. The purpose of this study was to determine the annual incidence of CDS, time to diagnosis in CDS, as well as the features of CDS. Methods The study design was a retrospective case series study conducted at eight teaching hospitals in Japan. We identified CDS cases from April 2013–March 2015. CDS was diagnosed when patients had acute onset of neck pain and CT showed calcification around the dens and when other diagnoses were unlikely. Results Seventy‐two CDS cases were identified. Mean annual incidence was 4.6 ± 2.3 cases at each hospital. Among those with available data, 57 of 64 had limited rotation (89.1%). The diagnosis of CDS was made in general internal medicine or the emergency medicine department in 61 cases (84.7%). A total of 62 cases (86.1%) were diagnosed within 1 day of presentation, and the median time from initial presentation at the hospital to diagnosis was 0.0 days (25th–75th percentiles, 0.0–1.0). For treatment, NSAIDs were used in 56cases (77.8%) and acetaminophen in 20 cases (27.8%). Conclusion CDS might be more common than has been reported to date. Time to diagnosis of CDS was within 1 day of visiting a teaching hospital. Cervical motion restriction is common in CDS and may be useful in establishing the diagnosis.https://doi.org/10.1002/jgf2.616calcium pyrophosphate deposition diseasecrowned dens syndromegeneral internal medicine
spellingShingle Hiroki Isono
Haruka Kuno
Takunori Hozumi
Ken Emoto
Sho Nishiguchi
Masahiro Sakai
Madoka Ito
Koichi Kitamura
Kazuhito Hirose
Eiji Hiraoka
Naoto Ishimaru
Hiroyuki Kobayashi
Yasuharu Tokuda
Crowned dens syndrome: A case series of 72 patients at eight teaching hospitals in Japan
Journal of General and Family Medicine
calcium pyrophosphate deposition disease
crowned dens syndrome
general internal medicine
title Crowned dens syndrome: A case series of 72 patients at eight teaching hospitals in Japan
title_full Crowned dens syndrome: A case series of 72 patients at eight teaching hospitals in Japan
title_fullStr Crowned dens syndrome: A case series of 72 patients at eight teaching hospitals in Japan
title_full_unstemmed Crowned dens syndrome: A case series of 72 patients at eight teaching hospitals in Japan
title_short Crowned dens syndrome: A case series of 72 patients at eight teaching hospitals in Japan
title_sort crowned dens syndrome a case series of 72 patients at eight teaching hospitals in japan
topic calcium pyrophosphate deposition disease
crowned dens syndrome
general internal medicine
url https://doi.org/10.1002/jgf2.616
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