Division of Plastic Surgery, Department of Surgery, Texas Children's Hospital, Houston, TX, USA.
Division of Plastic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA.
Cleft Palate Craniofac J. 2024 Aug;61(8):1383-1388. doi: 10.1177/10556656231163722. Epub 2023 Mar 21.
Children born with Trisomy 13 or 18 (T13/18) often have multiple congenital anomalies, many of which drastically shorten their lifespan. Among these defects are cleft lip and palate, the repair of which presents an ethical dilemma to the surgeon given the underlying comorbidities associated with T13/18. The authors present an ethical discussion and institutional experience in navigating this dilemma.
The authors analyzed existing literature on T13 and T18 surgery and mortality. A retrospective study over ten years was also conducted to identify pediatric patients who underwent surgical correction of cleft lip and/or palate secondary to a confirmed diagnosis of T13/18. The authors identified two patients and examined their treatment course.
The authors' review of literature coupled with their institution's experience builds on the published successes of correcting cleft lip and palate in the setting of T13/18. It was found that both patients identified in the case series underwent successful correction with no surgical complications.
A careful balance must be struck between improved quality of life, benefits of treatment, and risks of surgery in children with T13/T18. Careful consideration should be given to the medical status of these complex patients. If the remaining medical comorbidities are well managed and under control, there is an ethical precedent for performing cleft lip and palate surgeries on these children. A diagnosis of T13/T18 alone is not enough to disqualify patients from cleft lip/palate surgery.
患有三体 13 或 18 号染色体综合征（T13/18）的儿童通常存在多种先天性畸形，其中许多畸形会大大缩短其寿命。这些缺陷包括唇腭裂，由于 T13/18 相关的合并症，修复这些畸形对外科医生来说存在伦理困境。作者提出了一个伦理讨论，并分享了在处理这种困境方面的机构经验。
作者分析了现有的关于 T13 和 T18 手术和死亡率的文献。还进行了一项为期十年的回顾性研究，以确定因确诊为 T13/18 而接受唇裂和/或腭裂手术矫正的儿科患者。作者确定了两名患者，并检查了他们的治疗过程。
作者对文献的回顾以及他们机构的经验，建立在 T13/18 背景下纠正唇腭裂的成功发表成果的基础上。发现病例系列中的两名患者都成功接受了手术矫正，没有手术并发症。
在 T13/T18 儿童中，必须在提高生活质量、治疗益处和手术风险之间取得平衡。应仔细考虑这些复杂患者的医疗状况。如果剩余的合并症得到良好的管理和控制，那么对这些儿童进行唇腭裂手术就有伦理上的先例。仅仅诊断为 T13/T18 并不能使患者丧失接受唇腭裂手术的资格。
