Department of Surgery, Eastern Virginia Medical School, Norfolk, VA 23507, USA.
J Pediatr Surg. 2010 Jan;45(1):193-9. doi: 10.1016/j.jpedsurg.2009.10.037.
The presence of a pectus excavatum (PE) requiring surgical repair is a major skeletal feature of Marfan syndrome. Marfanoid patients have phenotypic findings but do not meet all diagnostic criteria. We sought to examine the clinical and management differences between Marfan syndrome patients and those who are marfanoid compared with all other patients undergoing minimally invasive PE repair.
A retrospective institutional review board-approved review was conducted of a prospectively gathered database of all patients who underwent minimally invasive repair of PE. Patients were grouped according to diagnosis of Marfan syndrome (MAR), Marfanoid appearance (OID), and all others (ALL). Patient demographics, preoperative imaging and testing, operative strategy, complications, and postoperative surveys were evaluated. Fisher's Exact test and chi(2) were applied for statistical analysis.
From June 1987 to September 2008, 1192 patients underwent minimally invasive PE repair (MAR = 33, OID = 212, ALL = 947). There was a significantly higher proportion of females with either MAR or OID who underwent repair (21.5%vs 15.5%, P = .04). The MAR patients had significantly more severe PE determined by computed tomography index (MAR = 8.75, OID = 5.82, ALL = 4.94, P < .0001) and required multiple pectus bars (> or =2) to be placed during operation (MAR = 58%, OID = 36%, ALL = 29%, P = .001). There was a trend toward higher wound infection rates in MAR patients (MAR = 6%, OID = 1.4%, ALL = 1.3%, P = .07). The recurrence rate was similar among all groups (MAR = 0%, OID = 2%, ALL = 0.7%, P = .12). Successful outcome from surgeon perspective in either MAR or OID patients was similar to ALL (98%vs 98%, P = .88) and correlated well with patient satisfaction after repair (96%vs 95%, P = .43).
Minimally invasive PE repair is safe in patients with Marfan syndrome or marfanoid features with equally good results. Patients with Marfan syndrome have clinically more severe PE requiring multiple bars for chest repair and may have slightly higher wound infection rates. Patients are satisfied with minimally invasive repair despite a phenotypically more severe chest wall defect.
漏斗胸（PE）需要手术修复是马凡综合征的主要骨骼特征。马凡样患者具有表型发现，但不符合所有诊断标准。我们试图研究马凡综合征患者与马凡样患者以及所有接受微创 PE 修复的其他患者之间的临床和管理差异。
对前瞻性收集的所有微创修复 PE 患者数据库进行回顾性机构审查委员会审查。根据马凡综合征（MAR）、马凡样外观（OID）和所有其他（ALL）的诊断对患者进行分组。评估患者的人口统计学、术前影像学和检查、手术策略、并发症和术后调查。应用 Fisher 精确检验和卡方检验进行统计学分析。
1987 年 6 月至 2008 年 9 月，1192 例患者接受微创 PE 修复（MAR = 33，OID = 212，ALL = 947）。MAR 或 OID 修复的女性比例明显较高（21.5%vs 15.5%，P =.04）。MAR 患者的 CT 指数（MAR = 8.75，OID = 5.82，ALL = 4.94，P <.0001）确定的漏斗胸严重程度明显更高，并且在手术中需要放置多个胸骨支撑条（>或=2 个）（MAR = 58%，OID = 36%，ALL = 29%，P =.001）。MAR 患者的伤口感染率呈上升趋势（MAR = 6%，OID = 1.4%，ALL = 1.3%，P =.07）。所有组的复发率相似（MAR = 0%，OID = 2%，ALL = 0.7%，P =.12）。MAR 或 OID 患者的手术医师从手术角度看，手术结果与 ALL 相似（98%vs 98%，P =.88），与修复后患者满意度也相关良好（96%vs 95%，P =.43）。
微创 PE 修复在马凡综合征或马凡样特征的患者中是安全的，结果同样良好。马凡综合征患者的漏斗胸临床更严重，需要多个胸骨支撑条进行胸部修复，并且伤口感染率可能略高。尽管胸廓壁缺陷的表型更严重，但患者对微创修复仍感到满意。
