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This study reports the clinical experience and outcomes of selecting the substantially touched vertebra (<jats:styled-content style=\"fixed-case\">STV<\/jats:styled-content>) as the lowest instrumented vertebra (<jats:styled-content style=\"fixed-case\">LIV<\/jats:styled-content>) in spinal surgeries for patients with <jats:styled-content style=\"fixed-case\">\n<jats:italic>B3GALT6<\/jats:italic>\n<\/jats:styled-content>\u2010related disorders, a group of extremely rare skeletal and connective tissue disorders.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective study included patients who were molecularly diagnosed with <jats:styled-content style=\"fixed-case\">\n<jats:italic>B3GALT6<\/jats:italic>\n<\/jats:styled-content>\u2010related disorders and received spinal surgeries for (kypho)scoliosis between 2017 and June 2023. Their medical records were reviewed. We also conducted a systematic literature review to identify (kypho)scoliosis management in patients with <jats:styled-content style=\"fixed-case\">\n<jats:italic>B3GALT6<\/jats:italic>\n<\/jats:styled-content>\u2010related disorders.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified a total of four patients. Patient 1 presented with severe kyphoscoliosis and segmentation defects and received a pedicle subtraction osteotomy with short fusion and dual growing rods from <jats:styled-content style=\"fixed-case\">T3<\/jats:styled-content> to <jats:styled-content style=\"fixed-case\">L3<\/jats:styled-content>. However, coronal imbalance was observed at the 18\u2010month follow\u2010up. Genetic testing revealed biallelic disease\u2010causing variants in <jats:styled-content style=\"fixed-case\">\n<jats:italic>B3GALT6<\/jats:italic>\n<\/jats:styled-content>. A revision surgery was successfully performed, with the level of the <jats:styled-content style=\"fixed-case\">LIV<\/jats:styled-content> extended to the <jats:styled-content style=\"fixed-case\">STV<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">L4<\/jats:styled-content>). The <jats:styled-content style=\"fixed-case\">LIV<\/jats:styled-content> was similarly extended to the <jats:styled-content style=\"fixed-case\">STV<\/jats:styled-content> in the index surgery for subsequent Patients 2 and 3 who received preoperative genetic testing results, and no complication has been observed. Patient 4 underwent preoperative Halo\u2010pelvic traction to minimize complications, followed by posterior spinal fusion. The curves were successfully reduced without complications. A systematic\u00a0literature review identified 86 articles reporting (kypho) scoliosis management in 12 of the 63 patients with <jats:styled-content style=\"fixed-case\">\n<jats:italic>B3GALT6<\/jats:italic>\n<\/jats:styled-content>\u2010related disorders. Limited surgical experience has been reported, with an increased rate of complications, including death.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Selecting the <jats:styled-content style=\"fixed-case\">STV<\/jats:styled-content> as the <jats:styled-content style=\"fixed-case\">LIV<\/jats:styled-content> is recommended in spinal surgeries for patients with <jats:styled-content style=\"fixed-case\">\n<jats:italic>B3GALT6<\/jats:italic>\n<\/jats:styled-content>\u2010related disorders, considering the characteristic joint hypermobility associated with the condition. Additionally, preoperative Halo\u2010pelvic traction may also be safe and effective. Furthermore, preoperative molecular diagnosis is essential for enabling precision medicine and minimizing complications.<\/jats:p><\/jats:sec>","DOI":"10.1111\/os.70072","type":"journal-article","created":{"date-parts":[[2025,5,15]],"date-time":"2025-05-15T10:10:46Z","timestamp":1747303846000},"page":"2025-2037","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Selecting the Substantially Touched Vertebra as the Lowest Instrumented Vertebra in Spinal Surgeries for <scp><i>B3GALT6<\/i><\/scp>\u2010Related Disorders: Clinical Experience and Literature Review"],"prefix":"10.1111","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8445-1869","authenticated-orcid":false,"given":"Aoran","family":"Maheshati","sequence":"first","affiliation":[{"name":"Department of Orthopedic Surgery Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &amp; Peking Union Medical College  Beijing China"},{"name":"State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &amp; 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