Weber Thomas R
Division of Pediatric Surgery, Department of Surgery, Cardinal Glennon Children's Hospital, St. Louis, MO 63104, USA.
J Pediatr Surg. 2005 Jan;40(1):170-3. doi: 10.1016/j.jpedsurg.2004.09.039.
BACKGROUND/PURPOSE: Asphyxiating thoracic dystrophy (ATD) can occur years after a Ravitch-type repair of pectus excavatum, resulting in debilitating alteration in pulmonary function (PFT). An operation was devised to attempt repair of this deformity.
After institutional review board approval, the records of 10 children (ages 9-18 years) with ATD that developed 4 to 12 years postpectus operation who underwent attempted repair of ATD were reviewed. Data obtained before ATD operation and at 6, 12, and 24 months afterward included chest computed tomography, pulmonary functions (PFT), and a quality of life questionnaire. The operation consisted of sternal split with rib graft placement to permanently hold the sternum apart.
All children survived and the bone grafts healed solidly. Computed tomography showed a change from a flat to a round chest contour on cross section, with increased anteroposterior dimension. Two patients had no change in PFT at 24 months whereas the other 8 had 21% to 30% improvement in PFT parameters. All patients reported improved exercise tolerance, and 3 began sports activities who were previously unable to do so. Two patients on oxygen, essentially bedridden, are now active, breathing only room air. Seven of 10 patients continue to have cosmetic concerns.
A small population of patients who had postoperative pectus repair developed severe, debilitating ATD. The repair described improves most patients, some dramatically, but does not significantly improve cosmetic appearance. The operation is undergoing further refinement to address these issues.
背景/目的：漏斗胸综合征（ATD）可在漏斗胸行Ravitch式修复术后数年出现，导致肺功能（PFT）出现使人衰弱的改变。设计了一种手术来尝试修复这种畸形。
经机构审查委员会批准，对10例（年龄9 - 18岁）漏斗胸综合征患者的记录进行回顾，这些患者在漏斗胸手术后4至12年出现该综合征并接受了ATD修复尝试。在ATD手术前以及术后6、12和24个月获得的数据包括胸部计算机断层扫描、肺功能（PFT）和生活质量问卷。手术包括胸骨劈开并植入肋骨移植物以永久撑开胸骨。
所有患儿均存活，骨移植物牢固愈合。计算机断层扫描显示，胸部横断面轮廓从扁平变为圆形，前后径增加。2例患者在24个月时肺功能无变化，而其他8例患者的肺功能参数改善了21%至30%。所有患者均报告运动耐量有所改善，3例之前无法进行体育活动的患者开始参加体育活动。2例原本依赖氧气且基本卧床的患者现在活动自如，仅呼吸室内空气。10例患者中有7例仍存在美观方面的顾虑。
一小部分接受漏斗胸修复术后的患者出现了严重的、使人衰弱的漏斗胸综合征。所描述的修复方法使大多数患者得到改善，有些改善显著，但并未显著改善美观。该手术正在进一步改进以解决这些问题。
