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[胸壁先天性畸形。外科治疗]

[Congenital deformities of the chest wall. Surgical treatment].

作者信息

Jasonni V, Lelli-Chiesa P L, Repetto P, Torre M, Nobili F, Mazzola C, Martucciello G

机构信息

Divisione e Cattedra di Chirurgia Pediatrica, Istituto G. Gaslini, Genova.

出版信息

Minerva Pediatr. 1997 Sep;49(9):407-13.

PMID:9471544
Abstract

BACKGROUND AND AIMS

Malformations of the front chest wall are congenital defects that have been reported since the seventeenth century and which include the clinical syndromes of funnel chest, pigeon chest and Poland's syndrome. Although they have been reported for such a long time, their pathogenesis is in many ways still unknown and the theories proposed up until now reveal uncertain and unsatisfactory findings. Attempts to gather precise information regarding their real incidence are equally ineffective given that frequently those cases which are not particularly severe are not referred for specialist care. These malformations usually involve severe psychological problems caused by the deformity and, in more severe cases, may lead to alterations in normal cardiac and respiratory function which are above all evident during intense physical effort.

METHODS

The authors critically review the cases of chest malformation corrected by the Department of Pediatric Surgery at the G. Gaslini Institute during the period from 1986 to date. They describe the surgical techniques used, justifying their choice and discussing any postoperative complications.

RESULTS

This experience shows that there is a clear preference for less invasive surgery for the correction of funnel chest compared to the technique proposed by Ravitch in which the sternum was totally mobilised following its extensive detachment from the other bone and muscular structures of the thoracic cage, with a high risk of damaging the internal mammary artery. The operation that was felt to be most suitable for correcting this defect was that described by Wesselhoett and De Luca in 1982. It is easier to perform and less invasive: it eliminates the detachment phase by inserting a support in titanium alloy through the sternal frame. Autologous grafts taken from the bottom contralateral ribs to the defect were used to treat Poland's syndrome in line with the technique suggested by Ravitch, thus achieving good stability of the whole chest; in view of the young age of the patients undergoing correction, it was not thought appropriate to proceed with the cosmetic reconstruction of the pectoral muscles using a peduncled flap of latissimus dorsi. A total of 70 patients were operated in this way; the cosmetic results were very satisfactory, whereas complications were limited to 5 cases of intraoperative pneumothorax, 5 cases of serohematic subcutaneous collection which were treated conservatively, and 1 case of hypertrophic scarring.

摘要

背景与目的

前胸壁畸形是先天性缺陷,自17世纪以来就有相关报道,包括漏斗胸、鸡胸和波兰综合征等临床综合征。尽管它们已被报道了很长时间,但其发病机制在很多方面仍然未知,迄今为止提出的理论结果也不确定且不尽人意。鉴于通常不太严重的病例不会转诊至专科治疗,因此试图收集有关其实际发病率的精确信息同样没有成效。这些畸形通常会导致由畸形引起的严重心理问题,在更严重的情况下,可能会导致正常心脏和呼吸功能的改变,这在剧烈体力活动时最为明显。

方法

作者对1986年至今在G.加斯利尼研究所小儿外科接受矫正的胸壁畸形病例进行了批判性回顾。他们描述了所使用的手术技术,说明了选择的理由并讨论了任何术后并发症。

结果

该经验表明,与拉维奇提出的技术相比,矫正漏斗胸时明显更倾向于采用侵入性较小的手术。在拉维奇的技术中,胸骨从胸廓的其他骨骼和肌肉结构广泛分离后被完全游离,有损伤胸廓内动脉的高风险。被认为最适合矫正此缺陷的手术是1982年韦塞尔霍特和德卢卡描述的手术。该手术更容易实施且侵入性更小:通过胸骨框架插入钛合金支撑物,省去了游离阶段。按照拉维奇建议的技术,取自缺损对侧底部肋骨的自体移植物用于治疗波兰综合征,从而实现了整个胸部的良好稳定性;鉴于接受矫正的患者年龄较小,认为使用背阔肌带蒂皮瓣进行胸肌的美容重建并不合适。共有70例患者接受了这种手术;美容效果非常令人满意,而并发症仅限于5例术中气胸、5例血清血性皮下积液(采用保守治疗)和1例肥厚性瘢痕。

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