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一名重度成骨不全患者行腹腔镜胆囊切除术的管理

Management of a Patient with Severe Osteogenesis Imperfecta Undergoing Laparoscopic Cholecystectomy.

作者信息

Scott Sarah, Jordan Helen, Gill Laura, Luhmann Andreas, Abbott Jamie, Ralston Stuart H

机构信息

Department of Anaesthesia and Intensive Care, Victoria Hospital, Kirkcaldy, KY2 5AH, UK.

Department of General Surgery, Victoria Hospital, Kirkcaldy, KY2 5AH, UK.

出版信息

Calcif Tissue Int. 2025 May 8;116(1):72. doi: 10.1007/s00223-025-01375-9.

Abstract

Individuals with severe osteogenesis imperfecta who require surgery often present a difficult management problem due to limb deformity and shortening, kyphoscoliosis, and deformity of the rib cage. All of these features may be associated with respiratory problems and impaired cardiovascular reserve. Surgical procedures and anaesthetic management represent a substantial challenge in these individuals. Here, we describe the clinical outcome of laparoscopic surgery to remove multiple gallstones in an individual with severe osteogenesis imperfecta. Meticulous pre-operative planning, combined with careful anaesthetic management resulted in a favourable outcome with a beneficial effect on quality of life. We provide a detailed account of the challenges faced and how these were surmounted in the hope that this may be of benefit to other clinicians faced with similar problems. Our experience demonstrates that laparoscopic surgery can be successfully performed in people with severe osteogenesis imperfecta with a favourable outcome.

摘要

患有严重成骨不全症且需要手术的患者,由于肢体畸形和缩短、脊柱侧弯以及胸廓畸形,常常会带来棘手的管理难题。所有这些特征都可能与呼吸问题和心血管储备功能受损有关。手术操作和麻醉管理对这些患者而言是巨大的挑战。在此,我们描述了为一名患有严重成骨不全症的患者进行腹腔镜手术以摘除多发胆结石的临床结果。精心的术前规划,结合细致的麻醉管理,带来了良好的结果,并对生活质量产生了有益影响。我们详细阐述了所面临的挑战以及如何克服这些挑战,希望这可能对其他面临类似问题的临床医生有所帮助。我们的经验表明,腹腔镜手术能够成功应用于患有严重成骨不全症的患者,并取得良好的效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d5a9/12062041/19830ad524f6/223_2025_1375_Fig1_HTML.jpg

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