Ann Thorac Surg 2006;81:1913-1915
© 2006 The Society of Thoracic Surgeons
How to do it
Combined Severe Pectus Excavatum Correction and Aortic Root Replacement in Marfan's Syndrome
Kalyana C. Javangula, FRCS
*
,
Timothy J.P. Batchelor, AFRCS,
Osama Jaber, MD,
Kevin G. Watterson, FRACS,
Kostas Papagiannopoulos, MD (CTH)
Department of Cardiothoracic Surgery, Leeds General Infirmary, Leeds, United Kingdom
Accepted for publication March 8, 2005.
* Address correspondence to Dr Javangula, Department of Cardiothoracic Surgery, Leeds General Infirmary, Great George St, Leeds, LS1 3EX UK (Email: kalyanachakravarthi{at}hotmail.com).
 |
Abstract
|
|---|
A 23-year-old man with Marfan's syndrome was admitted for repair of annulo-aortic ectasia and severe pectus excavatum. A submammary skin incision approach followed by bilateral subperichondrial resection of abnormal costal cartilages was performed. The left intercostal muscles and perichondrial sheaths were divided 2 inches lateral to the sternum in a parasternal fashion to place the retractor. The aortic root was replaced with a 23-mm St. Jude's composite graft (St. Jude Medical, Inc, St. Paul, MN). Chest wall reconstruction was completed with a high sternal osteotomy and support of the sternum was made with Gortex strips (W.L. Gore & Associates, Inc, Flagstaff, AZ). The patient made an uneventful recovery.
|
Introduction
|
|---|
Pectus deformities can coexist with cardiovascular diseases. This association is well known in Marfan's syndrome. Cardiac surgical correction requires adequate exposure, which is particularly difficult with coexisting pectus excavatum. We report a case requiring simultaneous repair of a severe pectus excavatum deformity and annulo-aortic ectasia using a novel approach.
|
Technique
|
|---|
A 23-year-old man with Marfan's syndrome was referred with annulo-aortic ectasia and severe aortic regurgitation. In addition, he had a severe pectus deformity and associated kyphoscoliosis with the lower sternal body and xiphoid process touching the spine (Fig 1). As a result, the contents of the mediastinum were displaced into the left hemithorax. After thorough evaluation and discussion, the patient gave consent for simultaneous repair.
A submammary skin incision was performed midway between the nipples and costal margin with an upward convexity in the midline. Skin flaps were elevated both superiorly and inferiorly. The pectoral muscles were reflected laterally and the rectus abdominis was detached from the sternum, xiphoid process, and costal cartilages. The perichondrium was incised over the third to seventh costal cartilages bilaterally extending from each costochondral junction to the body of the sternum. After completing the subperichondrial resection of the costal cartilages the left-sided intercostal muscles and perichondrial sheaths were divided 2 inches lateral to the sternum. A Finochietto retractor (CGU Manufacturing Co. Ltd, London, England) was positioned with the intact sternum medially and the rib stumps laterally, enabling excellent exposure of the heart and aortic root (Fig 2).
Under hypothermic cardiopulmonary bypass, the aortic root was replaced with a 23-mm St. Jude Medical composite graft (St. Jude Medical, Inc, St. Paul, MN). After weaning from bypass, the chest wall reconstruction was completed. A transverse anterior osteotomy of the sternum was performed at the manubriosternal junction. The posterior sternal table was preserved to maintain adequate blood supply. Additional internal fixation was provided by creating a "sternal bed" with two wide strips of a 2-mm Gortex patch (W.L. Gore & Associates, Inc, Flagstaff, AZ) anchored bilaterally at the edges of the fourth and sixth pairs of ribs using heavy Ethibond sutures (Ethicon Ltd, Edinburgh, UK) (Fig 3). The anterior chest wall was reinforced by placing reefing sutures on the perichondrial sheaths. The pectoral and rectus muscles were reattached to the sternum after positioning mediastinal and pleural drains. The subcutaneous layers and skin were closed over two vacuum drains.
View larger version (133K):
[in this window]
[in a new window]
|
Fig 3. Intraoperative photograph showing the "sternal bed" created by Gortex strips (W.L. Gore & Associates, Inc, Flagstaff, AZ) supporting the sternum in its new position.
|
|
Prophylactic antibiotics were given for 72 hours. All drains were removed by postoperative day 5. The patient made an uneventful recovery with good cosmetic results after being reviewed at the outpatient clinic in 8 postoperatively (Fig 4).
|
Comment
|
|---|
Combined corrections of chest wall deformities and intracardiac lesions have been reported previously [1, 2]. Aortic root replacement combined with pectus repair is uncommon [3]. To the best of our knowledge, we believe that this technique of a transverse skin incision with a left parasternal approach has not been previously described. The key issue in such a technique is to allow adequate exposure for cardiac correction while preserving the sternum and its vascular bed.
A major problem associated with a pectus deformity is the difficulty in performing a midline sternotomy with symmetrical retraction while providing adequate exposure. The left parasternal approach provides excellent access to the heart and ascending aorta without splitting the sternum. Preservation of both internal thoracic arteries ensures appropriate sternal wound healing. In contrast, the reported classic technique of a vertical midline skin incision and midline sternotomy with bilateral costochondral resections carries an obvious risk of transverse multiple sternal fractures with ischemic necrosis [4]. The use of "sternal turnover" should be discouraged due to obvious risks already mentioned, especially with prolonged cardiac procedures [3]. Many surgeons advocate internal support to avoid sternal depression and recurrence. However, the use of metal bars has been proven unnecessary. Furthermore, bars can migrate or become an obstacle to future cardiac interventions.
Historically there have been concerns regarding combined correction. These include an increased risk due to complications such as bleeding and infection and very extended operating times. Indeed, it has been stated that combined pectus and cardiac repair should be avoided [5]. However, successful repairs with good outcomes have been reported from other centers. Our technique could be applicable to other cardiac procedures such as coronary artery bypass grafting or heart valve replacement combined with pectus repair. One should bear in mind the concerns of thoracic dystrophy after extensive pectus correction in young patients. Long-term follow-up and additional experience is necessary to establish its safety and effectiveness.
|
References
|
|---|
- Willekes LC, Backer LC, Mavroudis C. A 26-year review of pectus deformity repairs, including simultaneous intracardiac repair Ann Thorac Surgery 1999;67:511-518.[Abstract/Free Full Text]
- Tomomi H, Masaihiho Y, Yoshihiro O, Naoki Y, Shigeteru O, Yoshio O. Simultaneous repair of pectus excavatum and congenital heart disease over the past 30 years Eur J Cardiothorac Surg 2002;22:874-878.[Abstract/Free Full Text]
- Chien HF, Chu SH. Simultaneous Bentall's procedure and sternal turnover in a patient with Marfan syndrome J Cardiovasc Surg (Torino) 1995;36(6):559-562.[Medline]
- Shin-ichi O, Yoshio M, Katsuo F. A repair of funnel chest without sternal dissection in aortic root replacement Eur J Cardiothorac Surg 2003;23:109-111.[Abstract/Free Full Text]
- Welch KJ, Castenada AR, Keane JF, Fyler DC, Shamberger RC. Anterior chest wall deformities and congenital heart disease J Thorac Cardiovasc Surg 1988;96:427-432.[Abstract]
Top
Abstract
Introduction
