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Bilateral Lung Transplantation and Simultaneous Pectus Excavatum Correction Using the Nuss Technique

2018; Elsevier BV; Volume: 107; Issue: 4 Linguagem: Inglês

10.1016/j.athoracsur.2018.08.066

1552-6259

Daniel Valdivia, Markus Kamler, Clemens Aigner,

Shoulder and Clavicle Injuries

Severe chest wall deformities are considered a contraindication for lung transplantation. A 38-year-old man with idiopathic pulmonary fibrosis and severe pectus excavatum with a Haller index of 4.3 was considered eligible and listed for lung transplantation. Bilateral sequential transplantation and simultaneous correction of the pectus excavatum were performed via anterolateral thoracotomies and Nuss bar insertion with peripheral femorofemoral venoarterial extracorporeal membrane oxygenation support. Total lung capacity increased from 4.1 L preoperative to 5.8 L postoperative. This case demonstrates that a combined approach is feasible with good functional outcome. Severe chest wall deformities are considered a contraindication for lung transplantation. A 38-year-old man with idiopathic pulmonary fibrosis and severe pectus excavatum with a Haller index of 4.3 was considered eligible and listed for lung transplantation. Bilateral sequential transplantation and simultaneous correction of the pectus excavatum were performed via anterolateral thoracotomies and Nuss bar insertion with peripheral femorofemoral venoarterial extracorporeal membrane oxygenation support. Total lung capacity increased from 4.1 L preoperative to 5.8 L postoperative. This case demonstrates that a combined approach is feasible with good functional outcome. Severe chest wall deformities are considered a relative contraindication to lung transplantation; however if an intraoperative correction is feasible, a simultaneous repair might be considered. Pectus excavatum is a relatively rare chest wall deformity and occurs in approximately 0.25% to 2% of the population. Successful repair is possible in 90% to 97% of patients [1Ramzy D. Rao V. Delgado D.H. Ross H.J. Cusimano R.J. Orthotopic cardiac transplantation in a patient with pectus excavatum: approach via left anterior thoracotomy.J Card Surg. 2007; 22: 145-147Google Scholar, 2Nuss D. Minimally invasive surgical repair of pectus excavatum.Semin Pediatr Surg. 2008; 17: 209-217Crossref PubMed Scopus (150) Google Scholar]. Although patients with a flat chest are commonly transplanted and even a series of living donor lobar transplantation is described [3Miyoshi R. Chen-Yoshikawa T.F. Takahagi A. et al.Pulmonary function and exercise capacity in patients with flat chests after lung transplantation.Ann Thorac Surg. 2017; 104: 1695-1701Abstract Full Text Full Text PDF Scopus (6) Google Scholar], the likelihood of a patient with severe uncorrected pectus excavatum presenting for lung transplantation is low. To the best of our knowledge no case of lung transplantation with simultaneous pectus correction has been published in the literature before. The main concern in this situation is limited chest wall compliance together with technical details of the transplant procedure [1Ramzy D. Rao V. Delgado D.H. Ross H.J. Cusimano R.J. Orthotopic cardiac transplantation in a patient with pectus excavatum: approach via left anterior thoracotomy.J Card Surg. 2007; 22: 145-147Google Scholar, 4Jaroszewski D. Notrica D. McMahon L. Steidley D.E. Deschamps C. Current management of pectus excavatum: a review and update of therapy and treatment recommendations.J Am Board Fam Med. 2010; 23: 230-239Crossref PubMed Scopus (155) Google Scholar]. A 38-year-old man with idiopathic pulmonary fibrosis and severe pectus excavatum (Haller index, 4.3) was listed for lung transplantation. Preoperative x-ray and computed tomography revealed a severe symmetric pectus excavatum. Furthermore, a shift of the mediastinum toward the left chest cavity was observed because of previous surgery and chronic shrinking of the left chest cavity as remnants of a video-assisted thoracic surgical procedure with several wedge resections and pleurectomy because of persistent spontaneous pneumothorax (Fig 1). Secondary pulmonary hypertension Dana Point Group 3.2 with a main pulmonary artery pressure of 29 mm Hg was present. Lung function test showed a forced expiratory volume in 1 second of 39%, forced vital capacity of 39%, and a total lung capacity (TLC) of 4.1 L (52%). Blood gas values were 101 mm Hg Pao2 and 43 mm Hg Paco2. No other extrapulmonary organ dysfunctions were present. After admission to the waiting list with a lung allocation score of 37 he developed a left-sided recurrent spontaneous pneumothorax with prolonged drainage treatment. After a waiting list time of 60 days an organ from a 64-year-old female brain-dead donor with cerebral ischemia after 5 days ventilation time was allocated. Chest x-ray and bronchoscopy of the donor showed regular findings. The Pao2-to-fraction of inspired oxygen ratio at fraction of inspired oxygen 1.0/positive end-expiratory pressure 5 was 372 mm Hg. Calculated donor TLC was 6.09 L. The procedure was planned through bilateral small anterior thoracotomies in the fourth intercostal spaces to avoid transection of the sternum. The procedure was performed with femorofemoral venoarterial extracorporeal membrane oxygenation (ECMO) support using a 19F arterial and 21F venous cannula (Fig 2) because of secondary pulmonary hypertension. Because of the previous operation severe adhesions were present in the left chest cavity. The lungs were implanted sequentially according to our routine protocol. Ischemic times were 365 (left lung) and 420 minutes (right lung). After reperfusion of the second implanted lung and meticulous hemostasis a titanium bar was placed using the introducer according to the Nuss technique and fixed bilaterally with one stabilizer on each side. After placement of the bar the ECMO support was gradually weaned, and the patient was decannulated in the operating room. The patient was extubated after 23 hours and remained in the intensive care unit for 5 days. Postoperative pictures, chest radiography, and computed tomography showed an adequate correction of the pectus (Haller index, 2.4) (Fig 3). His postoperative course was uncomplicated. Chest radiography demonstrated the remnants of the severe apical adhesions with a local minor hematoma without the necessity for an intervention. Postoperative lung function showed an increased TLC of 5.8 L (84%) together with a forced expiratory volumne in 1 second of 72% and a forced vital capacity of 63%. The patient was doing fine after a follow-up of 210 days. Severe chest wall deformities are considered an absolute contraindication to lung transplantation [5Weill D. Benden C. Corris P.A. et al.A consensus document for the selection of lung transplant candidates: 2014—an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation.J Heart Lung Transplant. 2015; 34: 1-15Abstract Full Text Full Text PDF PubMed Scopus (884) Google Scholar]. One of the main concerns is limited chest wall compliance, subsequently leading to impaired ventilation mechanics together with potential problems in postoperative mobilization and pain control. Conditions like severe scoliosis and asymmetry of the chest are difficult to handle, and these patients are usually declined for transplantation [6Orens J.B. Estenne M. Arcasoy S. et al.International guidelines for the selection of lung transplant candidates: 2006 update—a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation.J Heart Lung Transplant. 2006; 25: 745-755Abstract Full Text Full Text PDF PubMed Scopus (921) Google Scholar]. Patients with a flattened chest, as is commonly observed in those with pleuroparenchymal fibroelastosis and in pediatric patients with developmental shortcomings due to the underlying disease, can however be successfully transplanted [3Miyoshi R. Chen-Yoshikawa T.F. Takahagi A. et al.Pulmonary function and exercise capacity in patients with flat chests after lung transplantation.Ann Thorac Surg. 2017; 104: 1695-1701Abstract Full Text Full Text PDF Scopus (6) Google Scholar]. Even though postoperative lung function is reduced in these patients, a remodeling of the chest cavity takes place, leading to acceptable outcomes without any corrective chest wall procedures. Severe pectus excavatum is a relatively rare condition, and in symmetric cases minimal invasive correction with the Nuss technique is the established standard method. Flexibility of the chest wall is a prerequisite to allow this type of repair [2Nuss D. Minimally invasive surgical repair of pectus excavatum.Semin Pediatr Surg. 2008; 17: 209-217Crossref PubMed Scopus (150) Google Scholar]. If the Nuss technique is not feasible, a modified Ravitch approach is required. In the setting of lung transplantation several issues need to be considered when dealing with patients with chest wall deformities. A certain deformation of the chest wall is usually induced by the underlying disease such as a hyperinflation caused by emphysema and a chronic shrinking cause by pulmonary fibrosis [7Lutfi M.F. The physiological basis and clinical significance of lung volume measurements.Multidiscip Respir Med. 2017; 12: 3Crossref PubMed Scopus (67) Google Scholar]. In our patient the severe shrinking of the chest cavity caused by the pulmonary fibrosis and the previous operation on the left side was even further aggravated by the presence of the pectus excavatum demonstrated by the TLC of only 52% of predicted values. If the pectus remained uncorrected, implantation of an adequately sized donor lung would not be feasible and the risk of an insufficient postoperative respiratory situation would be unacceptably high. Size matching was performed to implant a lung with a TLC between the patients predicted and real TLC. Pretransplant correction of the chest wall deformation in the situation of an underlying parenchymal disease bears the risk of deterioration of the patient and is not indicated. Standard approach for lung transplantation is either a clamshell incision or bilateral anterior thoracotomies. To provide optimal postoperative stability for the Nuss bar a bilateral thoracotomy approach was chosen. Because of the secondary pulmonary hypertension intraoperative extracorporeal support was indispensable, and a femorofemoral approach was chosen to avoid positioning of the cannulas in the already severely limited space in the chest cavity. The transplant procedure was completed as the first step, and only thereafter was the Nuss bar placed. Because of the limited thoracotomies stabilization of the bar at the chest wall underneath the thoracotomy was feasible without problems. Meticulous hemostasis is particularly important in this situation as a revision operation is substantially more problematic because of the necessity to remove the bar to reenter the chest cavity. Follow-up was performed according to our routine protocol. Although removal of the bar is generally performed 2 or 3 years after initial surgery [2Nuss D. Minimally invasive surgical repair of pectus excavatum.Semin Pediatr Surg. 2008; 17: 209-217Crossref PubMed Scopus (150) Google Scholar], in this particular case of an adult patient we do not plan to remove the bar to avoid possible related complications [8Henry B. Lacroix V. Pirotte T. Docquier P.L. Lung middle lobe laceration needing lobectomy as complication of Nuss bar removal.Case Rep Orthop. 2018; 2018: 8965641Google Scholar]. In conclusion this case report demonstrates that bilateral lung transplantation and pectus excavatum correction using the Nuss technique can safely be performed simultaneously through bilateral thoracotomies, avoiding sternal division using femorofemoral venoarterial ECMO support. This should be considered in future patient selection guidelines.