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从儿科外科医生角度看漏斗胸

Pectus excavatum from a pediatric surgeon's perspective.

作者信息

Nuss Donald, Obermeyer Robert J, Kelly Robert E

机构信息

Department of Surgery, Eastern Virginia Medical School, 601 Children'S Lane, Norfolk, Virginia 23507, USA.

出版信息

Ann Cardiothorac Surg. 2016 Sep;5(5):493-500. doi: 10.21037/acs.2016.06.04.

Abstract

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.

摘要

历史上,漏斗胸(PE)被认为是先天性的,但根据我们的经验,只有22%在生命的第一个十年被发现。到目前为止,基因研究支持常染色体隐性遗传,这与我们40%的患者有一些阳性家族史相吻合,但考虑到男女比例恒定为4:1且男性占优,这也存在矛盾。这种不一致可能由多种漏斗胸疾病相关等位基因的作用来解释。一旦发现畸形,它往往会缓慢进展直到青春期,此时常可见快速进展。我们建议在12 - 14岁左右进行手术修复,因为胸壁通常仍具有柔韧性,并且这使我们能够在患者度过青春期时将固定棒留在原位,这可能有助于减少与生长相关的复发。轻度至中度PE患者采用治疗性深呼吸、姿势矫正和有氧运动进行治疗,对于适当选择的患者,也可使用负压胸壁矫正器。有严重症状的PE患者可接受漏斗胸微创修复术(MIRPE)。儿童的手术技术与成人相似,只是所涉及的力量更大,这通常需要抬高胸骨并采用更复杂的稳定策略。术后管理包括疼痛控制、深呼吸和早期活动。术后前六周必须限制运动,12周后缓慢恢复正常活动。

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