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Pectus excavatum from a pediatric surgeon’s perspective

机译:从小儿外科医师的角度看直肠切除

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摘要

Historically, pectus excavatum (PE) was reported to be congenital, but in our experience only 22% are noticed in the first decade of life. Thus far, genetic studies support an autosomal recessive heritability, which coincides with only 40% of our patients having some positive family history, but is also contradictory given a constant sex ratio of 4:1 in favor of males. This inconsistency may be explained by the effect of more than one pectus disease-associated allele. Once the deformity is noticed, it tends to progress slowly until puberty, when rapid progression is often seen. We recommend surgical repair at around 12–14 years of age since the chest wall is still typically flexible and because this allows us to keep the bar in place as the patient progresses through puberty which may help decrease growth-related recurrences. Patients with mild to moderate PE are treated with therapeutic deep breathing, posturing, and aerobic exercises, and in appropriately selected patients, the vacuum bell may also be offered. Patients that have severe symptomatic PE are offered Minimally Invasive Repair of Pectus Excavatum (MIRPE). The surgical technique in children is similar to that of adults, except for the higher forces involved that often necessitate sternal elevation and more involved stabilization strategies. Postoperative management includes pain control, deep breathing, and early ambulation. Exercise restriction is mandatory for the first six weeks with slow resumption of normal activity after 12 weeks.
机译:从历史上来说,据称胸腔瘘(PE)是先天性的,但根据我们的经验,在生命的头十年中仅发现22%。迄今为止,遗传学研究支持常染色体隐性遗传,这与我们只有40%的患者有一定家族史的患者相吻合,但鉴于男性的性别比恒定为4:1,这也是矛盾的。这种不一致可能是由一种以上与眼部疾病相关的等位基因的影响所解释。一旦发现畸形,它往往会缓慢发展直至青春期,此时经常可以看到快速发展。我们建议在大约12-14岁的年龄进行手术修复,因为胸壁通常仍很柔软,因为这可以使我们在患者进入青春期时将其固定在适当的位置,这可能有助于减少与生长相关的复发。对中度至中度PE的患者进行深呼吸,姿势和有氧运动的治疗,在适当选择的患者中,也可提供真空罩。对患有严重症状性PE的患者提供微创切除的果肉切除术(MIRPE)。儿童的外科手术技术与成人的外科手术技术相似,不同之处在于所涉及的较高的力量通常需要胸骨抬高并且需要更多的稳定策略。术后管理包括疼痛控制,深呼吸和早期移动。在开始的六周内必须限制运动,而在十二周后缓慢恢复正常活动。

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