{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T02:38:12Z","timestamp":1792809492025,"version":"4.5.0"},"reference-count":25,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,10]]},"abstract":"<jats:sec>\n                    <jats:title>OBJECTIVE<\/jats:title>\n                    <jats:p>Crouzon syndrome with acanthosis nigricans (CAN) is a rare and clinically complex subtype of Crouzon syndrome. At three craniofacial centers, this multicenter study was undertaken to assess clinical signs in relation to the required interventions and treatment course in patients with CAN.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>A retrospective cohort study of CAN was performed to obtain information about the clinical treatment course of these patients. Three centers participated: Erasmus Medical Centre, Rotterdam, the Netherlands; John Radcliffe Hospital, Oxford, United Kingdom; and H\u00f4pital Necker-Enfants Malades, Paris, France.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>Nineteen patients (5 males, 14 females) were included in the study. All children were operated on, with a mean of 2.2 surgeries per patient (range 1\u20136). Overall, the following procedures were performed: 23 vault expansions, 10 monobloc corrections, 6 midface surgeries, 11 foramen magnum decompressions, 29 CSF-diverting surgeries, 23 shunt-related interventions, and 6 endoscopic third ventriculostomies, 3 of which subsequently required a shunt.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>\n                      This study demonstrates that patients with the mutation c.1172C&gt;A (p.Ala391Glu) in the\n                      <jats:italic>FGFR3<\/jats:italic>\n                      gene have a severe disease trajectory, requiring multiple surgical procedures. The timing and order of interventions have changed among patients and centers. It was not possible to differentiate the effect of a more severe clinical presentation from the effect of treatment order on outcome.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/2021.2.peds20933","type":"journal-article","created":{"date-parts":[[2021,8,30]],"date-time":"2021-08-30T10:52:09Z","timestamp":1630320729000},"page":"425-431","source":"Crossref","is-referenced-by-count":8,"title":["Clinical signs, interventions, and treatment course of three different treatment protocols in patients with Crouzon syndrome with acanthosis nigricans"],"prefix":"10.3171","volume":"28","author":[{"given":"Catherine A.","family":"de Planque","sequence":"first","affiliation":[{"name":"Dutch Craniofacial Centre, Department of Plastic and Reconstructive Surgery and Hand Surgery, Erasmus Medical Centre Sophia Children\u2019s Hospital, University Medical Centre Rotterdam, Rotterdam, The Netherlands;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven A.","family":"Wall","sequence":"additional","affiliation":[{"name":"Craniofacial Unit, Department of Plastic and Reconstructive Surgery, John Radcliffe Hospital, Oxford, United Kingdom; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louise","family":"Dalton","sequence":"additional","affiliation":[{"name":"Craniofacial Unit, Department of Plastic and Reconstructive Surgery, John Radcliffe Hospital, Oxford, United Kingdom; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanna","family":"Paternoster","sequence":"additional","affiliation":[{"name":"Craniofacial Unit, Department of Pediatric Neurosurgery, H\u00f4pital Necker-Enfants Malades, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c9ric","family":"Arnaud","sequence":"additional","affiliation":[{"name":"Craniofacial Unit, Department of Pediatric Neurosurgery, H\u00f4pital Necker-Enfants Malades, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie-Lise C.","family":"van Veelen","sequence":"additional","affiliation":[{"name":"Dutch Craniofacial Centre, Department of Plastic and Reconstructive Surgery and Hand Surgery, Erasmus Medical Centre Sophia Children\u2019s Hospital, University Medical Centre Rotterdam, Rotterdam, The Netherlands;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah L.","family":"Versnel","sequence":"additional","affiliation":[{"name":"Dutch Craniofacial Centre, Department of Plastic and Reconstructive Surgery and Hand Surgery, Erasmus Medical Centre Sophia Children\u2019s Hospital, University Medical Centre Rotterdam, Rotterdam, The Netherlands;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Johnson","sequence":"additional","affiliation":[{"name":"Craniofacial Unit, Department of Plastic and Reconstructive Surgery, John Radcliffe Hospital, Oxford, United Kingdom; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jayaratnam","family":"Jayamohan","sequence":"additional","affiliation":[{"name":"Craniofacial Unit, Department of Plastic and Reconstructive Surgery, John Radcliffe Hospital, Oxford, United Kingdom; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene M. 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