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治疗腰椎和胸内脑膜膨出:生物伦理学意义。

Treatment of lumbar and intrathoracic meningocele: bioethical implications.

机构信息

Department of Clinical and Experimental Medicine, University of Catania, Italy.

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出版信息

Acta Biomed. 2021 Jul 1;92(3):e2021211. doi: 10.23750/abm.v92i3.11600.

Abstract

Myelomeningocele is  a  congenital malformation caused by a developmental defect of the spinal cord structures. The exactcause is unknown, but different factors have been involved includingradiation, malnutrition, drugs. Myelomeningocele can develop at any point in the spine, but the lumbosacral region is affected in over 75% of cases. Chest X-raysand computed tomography study are mandatory to reveal tracheal malformations or associatedanomaliesof the ribs.  Treatment of myelomeningocele must be multidisciplinary  and involve at the same time neurologists, radiologists, neurosurgeons, thoracic surgeons, bioethical experts and take care of the childand also of the family. Some experiences concern the possibility of a in-utero correction of myelomeningocele, in order to avoiding serious and progressive damages to the nervoussystem.  Given the improvement of myelomeningocele management, the quality of life is nowadays more acceptable than in the past; however, some severe forms of myelomeningocele cannot still be corrected: in this cases, a "non-interventional" approach may require a form of passive euthanasia that should be discussed and approved with and by parents and  Any dissent of the parents must be respected and considered reasonable. The choice of a "non-intervention", which should be guaranteed to all the people capable of self-determination, is not however so immediate and direct in the case of the minor: the dissent expressed on his behalf by the parents or legal representative may be ethically difficult to be accepted.In this case, the best interest of the child must prevail as the goal of any therapeutic choice.

摘要

脊髓脊膜膨出是一种由脊髓结构发育缺陷引起的先天性畸形。确切的病因尚不清楚,但涉及到多种因素,包括辐射、营养不良、药物等。脊髓脊膜膨出可以在脊柱的任何部位发展,但在超过 75%的病例中,腰骶部受到影响。胸部 X 光和计算机断层扫描研究是揭示气管畸形或肋骨相关异常的必要手段。脊髓脊膜膨出的治疗必须是多学科的,同时涉及神经科医生、放射科医生、神经外科医生、胸外科医生、生物伦理专家,并照顾孩子及其家庭。一些经验涉及到对脊髓脊膜膨出进行宫内矫正的可能性,以避免神经系统的严重和进行性损伤。由于脊髓脊膜膨出管理的改善,现在的生活质量比过去更容易接受;然而,一些严重形式的脊髓脊膜膨出仍然无法得到纠正:在这种情况下,一种“非干预”的方法可能需要一种被动安乐死的形式,应该与父母讨论和批准,任何父母的反对都必须得到尊重和认为是合理的。对于有能力自我决定的人来说,选择“不干预”的权利并不是那么直接和明确,而未成年人的情况则不同:父母或法定代表人代表他表示的反对意见在伦理上可能难以接受。在这种情况下,儿童的最佳利益必须作为任何治疗选择的目标。

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