Perioperative Management of the Patient With Chromosome 6p Duplication: A Case Report And a Narrative Review of Literature

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HUDEC Jan PROKOPOVÁ Tereza GALKO Michal PRÝMEK Martin REPKO Martin GÁL Roman

Rok publikování 2025
Druh Článek v odborném periodiku
Časopis / Zdroj Bratislava medical journal
Fakulta / Pracoviště MU

Lékařská fakulta

Citace
www https://link.springer.com/article/10.1007/s44411-024-00012-5
Doi https://doi.org/10.1007/s44411-024-00012-5
Klíčová slova 6p duplication syndrome; Perioperative management; Total intravenous anaesthesia; Scoliosis; Case report
Popis Chromosome 6p duplication syndrome represents a rare genetic disorder. About 50 patients have been described till now. The syndrome is also called trisomy 6p, and it is clinically characterized by short stature with microcephaly, mental retardation, congenital heart defects, or kidney tract anomalies. Epileptic seizures and spine deviations have also been described in a few children. Perioperative management of patients with chromosome 6p duplication can be challenging for all healthcare providers due to the multiorgan disability of the patients. To our knowledge, we report the first case of comprehensive perioperative management of patients with chromosome 6p duplication. Our case report is about a 9-year-old girl scheduled for scoliosis surgery. It discusses individual steps in the preoperative examination, surgery, and postoperative care, including preventing possible complications. This publication aims to show possible options for comprehensive perioperative management of patients with extremely rare syndrome because there are no published studies about perioperative management. Understanding the 6p duplication syndrome may improve the patient's outcome in the perioperative period.

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