{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,13]],"date-time":"2026-09-13T09:05:20Z","timestamp":1789290320148,"version":"build-2803163510"},"reference-count":0,"publisher":"Indian Orthopaedic Research Group","issue":"8","content-domain":{"domain":["jocr.co.in"],"crossmark-restriction":true},"short-container-title":["Jocr"],"published-print":{"date-parts":[[2026]]},"abstract":"<jats:p>Introduction: Crisponi syndrome is a rare autosomal recessive disorder caused by pathogenic variants in the CRLF1 gene and characterised by neonatal autonomic instability, feeding difficulties, recurrent hyperthermia, and persistent thermoregulatory dysfunction. Although survival has improved with advances in supportive care, musculoskeletal manifestations, particularly progressive spinal deformities, remain insufficiently described.\n\nCase Report: We report a 16-year-old male with genetically confirmed Crisponi syndrome who developed severe progressive scoliosis. Recurrent febrile episodes limited brace compliance, and the deformity progressed to a pre-operative Cobb angle of 90.5 degrees. Pre-operative imaging demonstrated a structurally fixed, single C-shaped curve without segmentation anomalies. Following multidisciplinary evaluation addressing autonomic and anaesthetic risks, posterior spinal fusion and instrumentation from T2 to L2 were performed using pedicle screws, hooks, sublaminar bands, and multilevel chevron osteotomies. Perioperative management emphasised temperature regulation and preparation for hyperthermic crises.\n\nConclusion: The postoperative course was uneventful, with preserved neurological function and stable radiographic correction at the 1-month follow-up. This case demonstrates that posterior spinal fusion can be safely undertaken in patients with Crisponi syndrome when meticulous multidisciplinary planning is implemented.\n\nKeywords: Crisponi syndrome, Scoliosis, Spinal deformity, Posterior spinal fusion, Pediatric spine surgery, Multidisciplinary management<\/jats:p>","DOI":"10.13107\/jocr.2026.v16.i08.7794","type":"journal-article","created":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T12:07:39Z","timestamp":1786104459000},"page":"75-80","update-policy":"https:\/\/doi.org\/10.13107\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Surgical Management of Progressive Scoliosis in Crisponi Syndrome: Multidisciplinary Perioperative Strategy and Technical Considerations"],"prefix":"10.13107","volume":"16","author":[{"given":"Mazlum Veysel","family":"Sili","sequence":"first","affiliation":[{"name":"Department of Orthopedics and Traumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mert","family":"Morkan","sequence":"additional","affiliation":[{"name":"Department of Orthopedics and Traumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00fcseyin Esat","family":"Bulduk","sequence":"additional","affiliation":[{"name":"Department of Orthopedics and Traumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdullah","family":"Serin","sequence":"additional","affiliation":[{"name":"Department of Orthopedics and Traumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elif","family":"Dulkadir","sequence":"additional","affiliation":[{"name":"Department of Orthopedics and Traumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mehmet","family":"Ayvaz","sequence":"additional","affiliation":[{"name":"Department of Orthopedics and Traumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"5271","published-online":{"date-parts":[[2026]]},"container-title":["Journal of Orthopaedic Case Reports"],"original-title":[],"deposited":{"date-parts":[[2026,9,13]],"date-time":"2026-09-13T08:45:28Z","timestamp":1789289128000},"score":1,"resource":{"primary":{"URL":"https:\/\/jocr.co.in\/wp\/2026\/08\/surgical-management-of-progressive-scoliosis-in-crisponi-syndrome-multidisciplinary-perioperative-strategy-and-technical-considerations\/"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026]]},"references-count":0,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2026]]},"published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.13107\/jocr.2026.v16.i08.7794","relation":{},"ISSN":["2321-3817"],"issn-type":[{"value":"2321-3817","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026]]},"assertion":[{"value":"Research Article","name":"content_type","label":"Content Type"},{"value":"Yes","name":"crosschecked","label":"CrossChecked"},{"value":"Yes","name":"peer_reviewed","label":"Peer Reviewed"},{"value":"10.13107\/jocr.2026.v16.i08.7794","name":"published","label":"Published"},{"value":"Creative Commons Attribution","name":"license","label":"License","explanation":{"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},"group":{"name":"rights","label":"Rights"}}]}}