Combined aortic root replacement and pectus excavatum correction in Marfan's syndrome

A 53-year-old man with Marfan's syndrome was admitted for repair of annulo-aortic ectasia (58 mm). He had also severe pectus excavatum. The skin was incised along the sternal midline. The pectoral muscles were detached laterally. After the perichondrium and costal cartilages were resected bilaterally. the left-sided intercostal muscles and perichondrial sheaths were divided 3 cm lateral to the sternum. To place the retractor in parasternal position, excellent exposure of the heart and aortic root was enabled. The aortic root was replaced with a Carboseal graft. Chest wall reconstructions was completed by modified Ravitch procedure with Gore-tex sheet The patient was discharged after an uneventful recovery on postoperative day 14.     

Combined severe pectus excavatum correction and aortic root replacement in Marfan's syndrome

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.     

Simultaneous repair of severe pectus excavatum and aortic valve replacement following previous open-heart surgery

A 60-year-old man with a severe degree of pectus excavatum and previous coronary artery surgery required aortic valve replacement. At operation the sternal wires were found to be densely adherent to the aortic wall. We describe the surgical technique, which was carried out uneventfully.     

Surgical repair of pectus excavatum

From 1958 to March 1987 we corrected 704 patients with pectus excavatum. The condition occurred more frequently in boys (544 patients) than girls (160 patients). In the majority of patients (86%), the defect was evident at birth or within the first year of life. Musculoskeletal abnormalities were identified in 133 patients (scoliosis, 107; kyphosis, 4; myopathy, 3; Poland's syndrome, 3; Marfan's syndrome, 2; Pierre Robin syndrome, 2; prune belly syndrome, 2; neurofibromatosis, 3; cerebral palsy, 4; tuberous sclerosis, 1; and congenital diaphragmatic hernia, 2). Sixteen patients had associated congenital heart disease. A family history of chest wall deformity was present in 37% of the cases and a history of scoliosis in 11%. Surgical correction was performed using a uniform technique for bilateral subperichondrial resection of the deformed costal cartilages and sternal osteotomy resecting a wedge of the anterior cortex and fracturing the posterior cortex. Anterior displacement was maintained with silk sutures closing the osteotomy defect. In 28 early cases, the sternum was secured by intramedullary fixation with a Steinman pin. All repairs were completed with a low complication rate (4.4%; pneumothorax, 11; wound infection, 5; wound hematoma, 3; wound dehiscence, 5; pneumonia, 3; seroma, 1; hemoptysis, 1; hemopericardium, 1). Six complications were associated with Steinman pin fixation (hemoptysis, seroma, hemopericardium, pneumothorax, 3). Major recurrence occurred in 17 patients (2.7%) and led to revision in 12. Satisfactory long-term results were achieved in the remaining 687 patients, with follow-up ranging from 2 weeks to 27 years. Mean follow-up was 4.3 years.(ABSTRACT TRUNCATED AT 250 WORDS)     

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

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.     

Surgical repair of pectus excavatum

Pectus excavatum is the most common chest deformity. Children with severe deformities suffer physical complaints such as frequent respiratory infections and decreased endurance. Patients with even mild deformities may complain of physical and psychological symptoms after puberty. In most patients, cardiac and respiratory function deteriorates, meaning that surgical correction is important for alleviation of symptoms and improving cardiopulmonary function and quality of life. The methods of surgical repair remain controversial. The traditional method, first described by Ravitch, comprises resection of deformed cartilages and correction of the sternum by wedge osteotomy in the upper sternal cortex. Ravitch’s methods have been modified using autologous or exogenous materials to fix the lower sternum. Nuss reported a novel method in which neither an anterior wound nor the cutting of cartilage or sternum is required; instead, a convex metal bar is placed behind the sternum. We have reported sternocostal elevation, in which a section of costal cartilage is resected, and all of the cartilage stumps are resutured to the sternum. The secured ribs pull the sternum bilaterally, such that the resultant force causes the sternum to rise anteriorly. Because most pectus excavatum patients are young and maintain an acceptable quality of life preoperatively, we believe that the morbidity rate is one of the most important factors in selecting the method for corrective surgery. Repair can be performed safely through the use of skilled techniques and a deep understanding of the anatomy and physiology of the thorax.     

Coronary artery bypass via resternotomy after pectus excavatum repair

Pectus excavatum is a chest wall deformity that commonly warrants pediatric surgical correction for cosmesis or respiratory impairment via sternotomy. The repair typically consists of sternal wedge osteotomy and subsequent placement of a Steinman pin across the sternum with fixation to the ribs bilaterally. Coronary artery bypass grafting (CABG) after surgical repair of the sternum with a metal implant poses an intriguing surgical challenge. Literature review reveals only one such previously described case. We present a case of coronary revascularization in an adult who previously underwent pectus excavatum repair with ligation of the internal mammary arteries. Our coronary revascularization was accessed through a resternotomy after surgical removal of the metal implant previously placed during the pectus excavatum repair. Autologous greater saphenous vein was used as a conduit for bypass. The patient did well postoperatively and was discharged on postoperative day 4. The pectus repair remained intact even after the median sternotomy was performed. This was confirmed at the 1-year follow-up for the patient. Resternotomy after pectus excavatum repair with a prosthetic implant poses a challenge to cardiothoracic surgeons. Many such repairs have been described in the pediatric population. As our society ages and coronary artery disease becomes more prevalent, this unique situation may be more commonly encountered. We present an approach to coronary artery bypass grafting via median resternotomy after pectus excavatum repair.     

Postpericardiotomy syndrome after minimally invasive repair of pectus excavatum

Minimally invasive repair of pectus excavatum (MIRPE) was first reported in 1998 and has gained wide acceptance since then. A 17-year-old girl who had undergone thoracotomy and cardiac surgery for transposition of great vessels at the age of 18 months presented with a deep, long pectus excavatum with asymmetry. After initial uneventful postoperative clinical course after MIRPE, the patient had bilateral pleural and pericardial effusion on the sixth postoperative day. Suspecting postpericardiotomy syndrome, systemic steroids were administered, and the symptoms resolved without affecting wound healing. Manifestation of a pericardial effusion combined with bilateral pleural effusion after MIRPE, especially in patients after cardiac surgery, may indicate a postpericardiotomy syndrome that can be treated successfully by intravenous steroids.     

Management of a floating sternum after repair of pectus excavatum

PURPOSE: The aim of this study was to examine the authors' experience with patients who have floating sternum after correction of pectus excavatum via the classical Ravitch procedure. A floating sternum is defined as a sternum in which the only attachment to the chest wall is its superior (cranial) border, and in which the body is secured only by the manubrium and whatever lateral and inferior fibrous bands are present. Typically, a floating sternum is caused by either extensive resection of the costal cartilages and perichondrium during correction of pectus excavatum or failure of proper regrowth of these cartilages. METHODS: The authors retrospectively assessed the charts of all patients diagnosed with a floating sternum noting age at original correction of pectus excavatum, time from original correction of pectus excavatum to diagnosis of floating sternum, age at correction of floating sternum, complaints before stabilization of the sternum, methods of repair, and postoperative complications. RESULTS: Between July 1993 and June 1999, floating sternum was diagnosed in 7 patients. The mean age of patients who underwent operative correction of a floating sternum was 28.9 years (range, 16 to 42 years). The mean time interval between original correction of pectus excavatum, or "redo," and diagnosis of a floating sternum was 9.9 years (range, 2 to 20 years). Complaints before correction of the floating sternum included sternal pain and instability, exercise intolerance, and difficulty breathing. Operative repair consisted of mobilizing the lateral and inferior edges of the sternum, detaching the fibrous perichondrium, performing anterior sternal osteotomies, and finally supporting the sternum with substernal Adkins struts. All 7 patients had successful stabilization of the sternum. Two of 7 patients underwent 2 procedures to successfully stabilize the sternum. One patient has Adkins struts still in place because of hematopoetic malignancy. Six of 7 patients are now without symptoms. CONCLUSIONS: A floating sternum is a morbid phenomenon that may manifest many years after the original procedure. It can cause significant sternal pain, chest wall instability, and respiratory dysfunction, which are the hallmark indications for correction. Repair of a floating sternum can be accomplished successfully.     

Blood metal levels after minimally invasive repair of pectus excavatum

 Open in a separate windowOBJECTIVESMinimally invasive repair of pectus excavatum (MIRPE) is the most popular surgical approach for paediatric patients with pectus excavatum. A substernal stainless still bar is inserted and left in place for 3 years and then removed. Our goal was to investigate blood metal levels after MIRPE and to correlate them with surgical details, such as the numbers of bars and stabilizers and the length of time the bar was in place.METHODSBlood levels of iron, chromium, manganese, molybdenum and nickel were analysed in 130 teenagers (108 boys and 22 girls) who had MIRPE using inductively coupled plasma mass spectrometry. A total of 62 patients were operated on using MIRPE (study group) and 68 patients were evaluated at implant time (control group). Differences between the numbers of bars implanted and the presence or absence of stabilizers were also considered.RESULTSSignificant increases in the levels of abnormal chromium were found in patients in the study group compared with the controls (P = 0.02). When we compared the group of patients with 2 or more bars with the group with 1 bar, the percentage of patients with a value above the threshold increased by 29 (P = 0.05). A significant increase in chromium levels was observed in patients with stabilizers (P = 0.03). Above-threshold levels of molybdenum were found in 5.1% of patients in the control group, but the number was not statistically significant (P = 0.09).CONCLUSIONSWe demonstrated that stainless steel devices used in MIRPE can elevate blood metal levels in paediatric patients. Moreover, we demonstrated that the use of metal stabilizers is associated with higher metal levels, probably due to increased dispersion.     

Serum metal levels after minimally invasive repair of pectus excavatum

BackgroundMetal implants may wear and corrode, resulting in systemic dissemination of metallic debris that is measurable in serum. Concern exists regarding adverse health effects related to implant-derived debris. Minimally invasive repair of pectus excavatum (MIRPE) is a popular technique in which a stainless steel substernal bar is implanted to achieve deformity correction. Serum metal levels have not previously been investigated after MIRPE.MethodsSerum chromium, molybdenum, and nickel levels were measured in this cross-sectional study of 11 children implanted with pectus bars after MIRPE. Samples were analyzed using high-resolution inductively coupled plasma mass spectrometry.ResultsMedian serum chromium and nickel values were elevated 3.3-fold (P = .0003) and 2.3-fold (P = .25), respectively, compared with age-matched controls. Serum chromium and nickel levels were abnormally elevated in 6 (55%) of 11 and 5 (45%) of 11, respectively. In patients whom postexplantation metal levels were measured, previously elevated levels were lowered. Serum chromium levels in children after MIRPE are comparable with adult cohorts with hip arthroplasty implants measured 1-year postoperatively. No acute metal toxicity was observed.ConclusionsAbnormally elevated levels of serum metal levels are measurable in children implanted with pectus bars. These findings warrant further investigation to assess the biocompatibility of this surgical implant in children.     

Comparison of aortic root replacement in patients with Marfan syndrome

Objectives: Although the aortic-valve-sparing (AVS) reimplantation technique according to David has shown favorable durability results in mid-term and long-term studies, composite valve grafting (CVG) according to Bentall is still considered the standard procedure. Methods: Retrospectively, we evaluated the results of aortic root replacement of patients with Marfan syndrome (MFS) who underwent surgery between January 1995 and January 2010. MFS was diagnosed using the Ghent criteria. AVS was used in 58 patients and CVG in 30 patients with MFS. AVS was done for aortic-root aneurysm (n = 48) or aortic dissection type A (n = 10). CVG was used for aortic-root aneurysm in 14 patients or aortic dissection type A in 16 patients. The mean follow-up was 3.2 (95% CI: 2.4–4.2) years. Results: In both groups, 30-day mortality was 0%. Three patients (10.0%) in the CVG group required resternotomy for postoperative bleeding versus two patients (3.4%) in the AVS group (p = 0.3). At follow-up, mortality was 10% in the CVG group versus 3.4% in the AVS group (p = 0.3). Re-operation was required in two patients (3.4%) after AVS and in three patients after CVG (10%) (p = 0.3). Three patients (10.0%) who underwent CVG had endocarditis and two patients (6.7%) had a stroke during follow-up, whereas no endocarditis and stroke occurred after AVS. After 14 years, stratified event-free survival was better in the AVS group (event-free survival was 82.3% vs 58.6%, log-rank test p = 0.086), especially after aneurysm (p = 0.057). After 10 years, freedom from aortic regurgitation ≥II° in the AVS group was 80% for aneurysm and 50% after dissection (p = 0.524). Conclusion: The reimplantation technique according to David was associated with excellent survival, good valve function and a low rate of re-operation, endocarditis, and stroke. There was a trend to better event-free survival for AVS patients making it the procedure of choice in MFS patients.