Ann Thorac Surg 2009;88:1627-1631. doi:10.1016/j.athoracsur.2009.06.008
© 2009 The Society of Thoracic Surgeons
Original Articles: General Thoracic
Surgery for Recurrent Pectus Deformities
Theresa D. Luu, MD,
Brian E. Kogon, MD,
Seth D. Force, MD,
Kamal A. Mansour, MD,
Daniel L. Miller, MD*
Department of Surgery, Section of General Thoracic Surgery, Emory University School of Medicine, Atlanta, Georgia
Accepted for publication June 4, 2009.
* Address correspondence to Dr Miller, General Thoracic Surgery, Emory University Clinic, 1365 Clifton Rd NE, Atlanta, GA 30322 (Email: daniel.miller{at}emoryhealthcare.org).
Presented at the Poster Session of the Forty-fourth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 28–30, 2008.
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Abstract
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Background: Pectus repair in adults can be challenging. Standard repair has been the modified Ravitch procedure. More recently the minimally invasive Nuss procedure, used exclusively in children, has been introduced for correction of pectus deformities in adults. There is a paucity of data on which procedure is most appropriate for adults and even less information on the most appropriate operation for pectus recurrence in adults. The purpose of this study is to determine if any specific patient characteristic exists that places patients at an increased risk for recurrence and describe our management of recurrent pectus defects in adults.
Methods: We retrospectively reviewed the records of all patients (>16 years of age) who underwent primary or recurrent repair of pectus deformities from April 1999 through December 2006.
Results: Forty-eight patients, 37 (77%) men and 11 women, underwent pectus repair with a median age of 28 years (range, 16 to 54 years). Indication for initial repair was pectus excavatum in 39 (81%) and pectus carinatum in 9. The primary procedure was a modified Ravitch repair in 40 patients and a Nuss procedure in 8. Thirteen patients (27%) underwent reoperation for recurrence; 8 (62%) patients had undergone a previous Nuss procedure and 5 had a modified Ravitch repair. All reoperative patients had a primary pectus index (PI) greater than 4.0, while 8 (62%) also had an asymmetrical defect. All failed Nuss procedure patients underwent a modified Ravitch repair for correction, while the recurrent open repair patients required complex reconstructions. Results were good or excellent in greater than 90% of patients undergoing a reoperative procedure.
Conclusions: Adults with severe pectus deformities (PI > 4.0) and asymmetric defects are at a greater risk of recurrence after a Nuss procedure. These patients may better be served with a modified Ravitch repair initially. Reoperation for failed pectus repair in adults can be performed safely with outstanding results.
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Introduction
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Surgical repair of pectus deformities is most commonly performed during childhood and early adolescence in order to alleviate symptoms of pain, to minimize cardiac or respiratory compromise, as well as to diminish significant psychosocial consequences [1]. There are an increasing number of adult patients who did not undergo repair during childhood and are now consulting thoracic surgeons for correction of their chest deformities [2]. Pectus repair in adults is a challenge because there is a paucity of studies comparing which type of surgical repair is most appropriate for adults [2–5]. The standard open repair has been modified from the original techniques described by Ravitch in 1949, which involves bilateral subperichondrial resection of abnormal costal cartilages and stabilization of the sternum [2, 3, 6]. Excellent results have been achieved with this open technique [3]. The Nuss procedure was introduced in 1998 [7] for pectus repair in children and has gained popularity because of its minimally invasive approach using a convex metal bar placed behind the sternum with long-term success [8]. Recently, the Nuss procedure has been introduced for repair in adults but the long-term results are unknown [9]. Therefore, controversy exists which technique is most appropriate for adults with primary and recurrent pectus deformities.
Over one third of patients who underwent a pectus repair during childhood, present in adulthood with a recurrence of their pectus deformity. Factors associated with recurrence include the operative technique used, failure to use a metal support bar, younger age, local infection, and the presence of Marfan syndrome [10]. Unfortunately, there is a paucity of published reports describing repair of recurrent pectus deformities and these studies include mostly children and few adults [10–13]. This present study summarizes our clinical experience in the surgical management of adults who underwent reoperation for recurrent pectus deformity. In addition, we investigated if there were any specific patient characteristics that may increase the patient's risk of recurrence of their pectus deformity.
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Material and Methods
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A retrospective review was performed of 48 patients who underwent primary or recurrent repair of pectus deformities within the Emory Healthcare System (excluding Children's Hospital of Atlanta) from April 1999 through December 2006. Approval was obtained from the Institutional Review Board of Emory University for this retrospective study and consents were waived. There were 37 (77%) men and 11 women with a median age of 28 years (range, 16 to 54 years). Primary indication for repair was pectus excavatum in 39 (81%) patients and pectus carinatum in 9. Evaluation before surgical repair included plain chest radiographs and electrocardiogram in all patients. A computed tomography of the chest was performed in all patients with an excavatum defect in order to evaluate the extent and nature of the deformity as well as calculation of the pectus index, defined as a ratio of the transverse diameter of the chest to anteroposterior diameter between the sternum and spine. In those patients with associated chest pain or shortness of breath, noninvasive cardiac and pulmonary evaluations were performed. Patient's medical records were reviewed for demographics, medical history, symptoms, degree of anatomic defect identified at the time of the surgical repair, the type of surgical correction, hospital course, and patient satisfaction.
Surgical technique for repair of the pectus defect was based on the modified Ravitch repair [6, 13, 14], which was further modified and described in detail by Mansour and colleagues in 2003 [3]. In brief, the Mansour repair is the following: (1) bilateral removal of the deformed costal cartilages (usually 3 through 7) subperichondrially; (2) elevation of the sternum by separating it from the xiphoid, which remains attached to the rectus muscles, and by dividing the attachments of the perichondrial beds; (3) anterior wedge osteotomy of the sternum at the superior portion of the defect and secured with 2-0 ethibond periosteal sutures (Ethicon Inc, Somerville, NJ) to stabilize the position; (4) placement of two No. 1 Prolene sutures (Ethicon Inc) through the right and left sides of the xiphoid (still attached to the rectus abdominis muscles) and around the second rib bilaterally; (5) tightening of the Prolene sutures and securing the xiphoid-rectus muscle complex under the sternum as a posterior buttress to prevent sternal collapse; (6) the perichondrial beds are reattached to the newly positioned sternum; and (7) reapproximation of the bilateral pectoralis major muscles. Detailed figures describing this anatomic repair are shown in Mansour and colleagues [3].
This technique was performed in all patients undergoing primary and secondary repair of their pectus deformities, except for the 5 patients who had recurrent anterior chest wall defect and (or) associated infectious complications which required a complex reconstruction with steel plates, pectus bars, and Prolene mesh or marlex mesh methylacrylate sandwich.
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Results
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The primary procedure was a modified Ravitch procedure in 40 patients and a Nuss procedure in 8 patients. Thirteen (27%) patients required a reoperation for recurrence. Associated skeletal abnormalities included Marfan syndrome and severe scoliosis in 2 patients each. Pectus index in the excavatum patients varied from 2.6 to 9.0 (mean, 4.3); the normal chest has an index of 2.5 [9]. The most common preoperative symptoms (Table 1) were dyspnea on exertion in 28 (58%) and chest pain in 25 (52%) patients. In addition, physiologic alterations were identified as pulmonary impairment in 15% (4 impaired pulmonary function tests, 3 restrictive lung disease) and cardiac problems in 19% (4 electrocardiogram [ECG] changes, 2 LV diastolic dysfunction, 2 mitral valve prolapse and 1 right heart failure with liver congestion). In those patients with mild or moderate pectus deformities, coexisting aesthetics and psychologic reasons played a greater part in the decision to proceed with surgery.
The median hospital stay was 5 days in the primary pectus repair group and 6 days in the reoperative group. Postoperative complication rate (Table 2) for the patients undergoing primary pectus repair was 11% (4 patients), 3 due to a pneumothorax requiring chest tube placement and 1 with a pleural effusion. There were no postoperative complications in the reoperation group. Eight (17%) patients were discharged home with one to two Jackson-Pratt drains until drainage decreased to less than 50 mL per day, which was then pulled as an outpatient. There was no incidence of wound seroma or recurrent infection. No patients required a blood transfusion and there were no perioperative deaths. Physical activity was restricted for 8 to 12 weeks and all patients were discharged from the hospital with a prosthetic protective shield.
In the recurrent repair patients, 10 were men and 3 were women, with a median age of 14 years (range, 5 to 19 years) at the time of the initial repair and a median age of 26 years (range, 16 to 46 years) at time of the reoperation. All 13 failed pectus patients were classified with moderate or severe pectus excavatum deformity with a pectus index at the time of repeat surgery of greater than 4.0 (mean 4.3), while 8 (62%) of these patients also had moderate to severe asymmetric defects. In the 8 previous Nuss patients the bars were removed at a median of 18 months (range, 14 to 36 months) prior to their recurrence surgery.
In the 13 patients (27%) who required a reoperation, 8 (62%) patients had undergone a previous Nuss procedure (all performed at outside institutions) and 5 patients had a prior Ravitch repair, which was complicated by a local infection in 3 and a limited chest wall defect in 2 (Table 3). All of the failed Nuss procedure patients underwent a subsequent modified Ravitch repair; 2 had pectus bars placed for additional support. In the 2 patients who had substernal bars placed, the bars were subsequently removed at 6 and 24 months after the recurrent procedure. In the 5 recurrent patients who had undergone an open repair, all required complex reconstructions which included a modified Ravitch and chest wall reconstruction in 3 and chest wall reconstruction only in 2.
Relief of symptoms was good or excellent in 96% of patients who underwent primary repair and in 92% patients who underwent reoperation (Table 4). Only one patient who underwent a modified Ravitch repair for a failed Nuss procedure required excision of a protuberant costal cartilage two years later. No patient indicated that the reoperation made the condition worse. In greater than 90% of patients, the patients experienced resolved chest pain, less dyspnea on exertion, and moderate to marked improvement in exercise tolerance. Repeat cardiac and pulmonary studies were not performed postoperatively.
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Comment
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Pectus deformities are the most common congenital chest deformity, occurring in approximately 1:400 births, and affecting males five times more often than females [5]. Increasing evidence indicates that both pectus excavatum and carinatum deformities worsen with age with varied degrees of symptoms and physiologic limitations, as well as aesthetic and psychosocial impairments [5]. Consequently, the majority of these patients undergo surgical repair during childhood and early adolescence. However, there are significant numbers of patients who progress to adulthood without surgical repair with persistent and often worsening symptoms because of loss of flexibility of their previous pliable pediatric chest wall [2]. Studies have shown that the indications for surgical repair of pectus defects in adults are similar to those reported for pediatric patients. Most patients have psychosocial reasons for seeking medical attention in addition to progressive symptoms of chest pain, dyspnea on exertion, palpitations, and reduced stamina, but not infrequently other pulmonary or cardiac problems identified [4]. In our series, approximately two-thirds of patients had some component of respiratory compromise such as dyspnea on exertion (58%), frequent upper respiratory infections (4%), and asthma (4%). Objective pulmonary impairment was seen in 15% (usually a restrictive pattern). Similarly, approximately 70% of patients had cardiac-related symptoms such as chest pain (52%), decreased endurance (10%), and palpitations (8%) in addition to objective abnormal cardiac findings in 19% (most commonly ECG changes and right ventricular dysfunction). Furthermore, all patients with pectus excavatum had varying degrees of displacement of the heart into the left chest and most patients had a pectus index 3.2 or greater.
Surgical techniques vary, but most pediatric surgeons today favor the minimally invasive repair, as previously described by Nuss and colleagues in 1998 [7], over the standard open repair which involves subperichondrial resection of abnormal costal cartilages as described by Ravitch in 1949 [6]. The feasibility of the open repair using the modified Ravitch technique for adult patients has been well documented [1–3, 5, 10]. In the current series, the majority of our patients were repaired using a modified Ravitch anatomic repair, in which a metal support bar is not used (except in 2 patients in the recurrence group), utilizing the xiphoid-rectus muscles complex translocated underneath the sternum for posterior support. As demonstrated in our study, 96% of patients undergoing primary pectus repair and 92% undergoing reoperation by the modified Ravitch anatomic procedure had good or excellent results with resolution of their preoperative symptoms. No patient had recurrence of their deformity except for one patient in the reoperation group who had a minor protuberance requiring correction at the time of pectus bar removal.
Despite the excellent results of the modified Ravitch technique [15], many primary care physicians view this procedure as an aggressive radical approach for a fairly benign condition. Thus, many patients are referred for the Nuss procedure. Although there was initial enthusiasm by both patients and surgeons for this procedure in adults, recent reports have demonstrated that the excessive force necessary to elevate the sternum in adults [16, 17] leads to increases in complication rates, in length of hospitalization, and in severity of postoperative pain that is considerably higher than with the Nuss procedure in children [18]. In our series postoperative complications were minimal and no patient experienced prolonged pain after undergoing the modified Ravitch anatomic procedure. Another advantage of our anatomic repair is that the patients do not require subsequent surgery to remove the stabilizing bar. Multiple complications such as displacement, pain, and infection, as well as pleural effusion, pneumothorax, bleeding, and worst of all recurrence of the pectus defect, can occur related to the bar while it is in place and at the time of removal [19, 20].
All of the patients who experienced recurrence of their pectus deformities had excavatum defects, while the majority (62%) developed recurrence after the Nuss procedure. The remaining recurrences occurred after a modified Ravitch procedure in the setting of a local infection or localized failure of cartilaginous regrowth, which subsequently required complex reconstructions. These complex reconstructions are uncommon, but the outcomes are excellent [21]. The majority of the patients with recurrent pectus defects had asymmetric defects with severe pectus indexes greater than 4.0. This observation was also seen by Hebra and colleagues [4] in adult patients undergoing the Nuss procedure who experienced a significant number of residual asymmetries of the chest after repair. In fact, less than 50% of the adult patients achieved 100% correction of their deformity and 2 patients required conversion to a modified Ravitch repair. Hebra and colleagues concluded that the Nuss procedure can only achieve 100% correction of the chest deformity in adult patients with symmetric pectus defects. Therefore, patients with severe asymmetry of their excavatum defect (Fig 1) should undergo open surgical correction as their initial treatment. Our current study concurs with their conclusion.
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Fig 1. Computed tomography of the chest of a 38-year-old patient with severe asymmetric pectus excavatum.
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Reoperation for recurrent pectus excavatum defects is a challenge (Fig 2). Thorough evaluation needs to be performed to determine if surgery is indicated. Evaluation should include a chest computed tomographic scan with pectus index, pulmonary function tests, echocardiogram, and cardiopulmonary exercise testing if necessary. If the patient's recurrence is impairing their normal activities of daily living then reoperation should be entertained. The controversy is what operation should be done and what approach; an open technique versus a closed technique (Nuss procedure). If the defect is minimal but the physiologic effect is significant then a Nuss procedure should be considered, even though, in adults it is associated with prolonged pain in greater than 50% of patients, prolonged bar requirement, and possible persistence of the recurrence. A Nuss procedure should not be performed in patients with asymmetric defects and in patients with significant calcifications in the regrown costal cartilages. Correction of the defect in these patients should not be performed with the Nuss procedure because the chest is too rigid to allow elevation of the sternum to the correct position leading to a life of prolonged complications, distress, and pain.
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Fig 2. Computed tomography of the chest of the 38-year-old patient with recurrent pectus excavatum 30 months after undergoing a Nuss procedure and 6 months after bar removal.
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Surgical correction of recurrent pectus chest deformities in adults can be performed with low morbidity, short hospital stay, and can achieve good to excellent physiologic and cosmetic results. In adults who have a severe pectus excavatum with a pectus index greater than 4.0 and an asymmetric defect or calcified costal cartilages, a Nuss procedure should not be performed because of a high recurrence rate and complications. These patients may be better served with a modified Ravitch anatomic repair as the initial treatment of choice.
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Abstract
Introduction
