Minimally invasive repair of pectus excavatum in patients with Marfan syndrome and marfanoid features

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• 作者：Richard E. Redlinger Jr. ; Gregory D. Rushing ; Alan D. Moskowitz ; Robert E. Kelly Jr. ; Donald Nuss ; Ann Kuhn ; Robert J. Obermeyer ; Michael J. Goretsky
• 关键词：Pectus excavatum ; Marfan syndrome ; Minimally invasive ; Connective tissue disorder
• 刊名：Journal of Pediatric Surgery
• 出版年：2010
• 出版时间：January 2010
• 年：2010
• 卷：45
• 期：1
• 页码：193-199
• 全文大小：300 K

文摘

Purpose

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.

Methods

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 χ² were applied for statistical analysis.

Results

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 (≥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).

Conclusions

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.