European Respiratory Society
Surgery for Non-Neoplastic Disorders of the Chest: a Clinical Update

Diseases of the chest are numerous and a multidisciplinary approach by the chest physician and thoracic surgeon is often required, not only in the management of chest malignancies, but also in the treatment of non-neoplastic disorders of the chest. Furthermore, many advances and improvements are put forward in the overall management of patients with advanced lung disease; besides lung transplantation, volume reduction surgery is reported as an alternative surgical option for emphysema patients which may delay, or even preclude the need for transplant. Management of suppurative lung and pleural infections is a challenging problem in clinical practice; classical surgery stands besides thoracoscopic treatment. The frequency of thoracic injuries in trauma victims forms another challenge for the chest physician and thoracic surgeon considering that trauma ranks, at present, only behind cardiovascular disease and cancer. Although disorders of the trachea are relatively uncommon problems that rarely present in routine practice, successful outcomes require judgment in selecting suitable patients and correct timing of surgery, as well as careful determination of the extent of surgery required. The role of surgery in many of these non-neoplastic disorders is extensively discussed in this issue of the European Respiratory Monograph

  • European Respiratory Society Monographs
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    Correspondence: G.M. Verleden, University Hospital Gasthuisberg, Dept Respir Med, 49 Herestraat, B‐3000 Leuven, Belgium.

    Lung and heart-lung transplantations are now considered as effective treatment for highly selected patients with end-stage lung or heart-lung disease. Although the survival rates have improved dramatically in recent years, the development of chronic rejection is still the major cause of late death after transplantation. Histologically chronic rejection is manifested by bronchiolitis obliterans (BO), with a gradual decline in pulmonary function tests and quality of life, eventually leading to respiratory insufficiency and recurrent infections with Gram-negative bacteria and fungi. A lot of research efforts have been put into the detection of possible risk factors for BO and unravelling the pathophysiology of BO. Several key cytokines and growth factors have now been identified. Since the histological diagnosis of chronic rejection remains difficult, a clinical grading system has been proposed, determined as bronchiolitis obliterans syndrome, divided into four, and more recently, five categories, depending on the severity of airflow obstruction. This chapter aims to review the current knowledge on the pathology, the aetiology (e.g. risk factors) and the pathophysiology of chronic rejection after heart-lung and lung transplantation.

  4. Page 19
    Correspondence: G.M. Verleden, University Hospital Gasthuisberg, Dept Respiratory Medicine, 49, Herestraat, B‐3000, Leuven, Belgium.

    Bronchiolitis obliterans (BO) or bronchiolitis obliterans syndrome (BOS) is now regarded as a manifestation of chronic allograft rejection or dysfunction. The patient with BO/BOS presents with increasing symptoms of functional limitation, cough, dyspnoea and recurrent infections. Transbronchial biopsies are not very sensitive for the diagnosis of chronic rejection, that is why the diagnosis is usually based on the flow-volume curve which shows a typical progressive air flow obstruction, for which no other cause can be found.. Recently, several so called surrogate markers for early diagnosis of BO/BOS have been investigated and will be discussed in this chapter. Finally, an overview of the existing treatment modalities for this debilitating condition is given, with specific emphasis on the shift in immunosuppressive drug regimen, which may lead to an arrest in the decline of the pulmonary function parameters. Also some newer (renewed) treatment modalities such as extracorporeal photochemotherapy and total lymphoid irradiation will be discussed.

  5. Page 44
    Correspondence: J.J. Egan, National Heart and Lung Transplant Program, Mater Misericordiae Hospital, Eccles St , Dublin 7, Ireland.

    Infection and chronic rejection are the major causes of mortality and morbidity in lung allografts. The host for lung transplantation is frequently colonised with multiresistant bacterial organisms as a consequence of their primary disease; therefore, irrespective of immunosuppression, the host is at risk of infection. The cytomegolavirus appears to be an important risk factor for fungal and bacterial super infection, and possibly for acute vascular rejection, which in themselves may all contribute to the risk of bronchiolitis obliterans syndrome (BOS). The precise role of bacterial infection in the development of BOS remains unclear; much of this relates to a current lack of standardisation of the definition of infection.

  6. Page 55
    Correspondence: E.K. Verbeken, Dept of Pathology, University Hospital St. Rafaël, Minderbroedersstraat 12 , B-3000, Leuven, Belgium.

    Bronchiolitis obliterans (BOS) syndrome is a severe clinical syndrome in lung transplanted patients. The corresponding morphological lesion is bronchiolitis obliterans (BO). The correlation between BOS and BO is far from clear.

    The presented chapter gives an overview of BO in open lung biopsies and autopsy specimens of lung allografts, both with regard to their anatomical localisation and their histology. Anatomically, BO may present either in a pure type, involving mainly membranous and proximal respiratory bronchioles (RBs), in a “proximal” type, involving also bronchi, or in a distal type, involving mainly distal RBs and alveolar ducts. The latter type overlaps with bronchiolitis obliterans organising pneumonia or cryptogenic organising pneumonia. In a biopsy, one of these types prevails with a wide range, though.

    Histologically, BO lesions consist of a spectrum between a predominantly inflammatory and a predominantly fibrotic type. In a single biopsy, one type prevails with only a very narrow range. Asynchronous lesions are not observed.

    Finally, the pathology is briefly discussed in view of some basic pathogenetic paradigms recently formulated regarding inflammation and fibroproliferation. Parallels are drawn between inflammatory and fibrotic and other idiopathic interstitial pneumonias such as non-specific interstitial pneumonia and usual interstitial pneumonia.

    The aim of this chapter is to promote a good understanding between Physician and Pathologist, and between bronchiolitis obliterans syndrome and bronchiolitis obliterans.

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    Correspondence: D. Van Raemdonck, Dept of Thoracic Surgery, University Hospital Gasthuisberg, Herestraat 49 B-3000, Leuven, Belgium.

    Ischaemia-reperfusion-induced lung Injury (IRI) remains an important problem after lung transplantation occurring in 10–20% of the recipients with a significant impact on clinical outcome. IRI has been identified as the main cause of primary graft failure resulting in a post-transplant mortality of 2–5%. This syndrome occurs within 72 h after transplantation and is characterised by impaired gas exchange, fluid loss via endotracheal tube and chest drains, pulmonary infiltrates on chest radiographs, elevated pulmonary vascular resistance, decreased pulmonary compliance, and diffuse alveolar damage on biopsy. Risk factors that have been identified are related to the donor, the preservation, the transplant procedure, and the recipient. Better understanding of the complex pathophysiological mechanism of this insult has marked the last decade. A myriad of molecular and cellular events occurs during ischaemia and reperfusion resulting in endothelial and epithelial cell dysfunction. This knowledge has resulted in new strategies to prevent and to treat IRI although randomised clinical trials to demonstrate an improvement are often lacking. Most publications are limited to personal experience and case series.

    After more than 10 yrs of research, extra-cellular type preservation solutions such as low-potassium dextran glucose or Perfadex® and Celsior® have now been introduced, clinically replacing the intra-cellular type solutions such as modified EC and UW. This has resulted in an improved initial graft function and an improved early outcome. The technique of retrograde flush, where the preservation solution is administered via the left atrial appendage or via the pulmonary veins and drained through the pulmonary artery, is another strategy applied by many transplant teams nowadays. Most surgeons have adopted a technique of controlled reperfusion by slowly releasing the pulmonary artery clamp in order to gradually increase blood flow through the cold graft over a 10‐min period. Initial reperfusion with leukocyte-filtered blood, mixed with a crystalloid solution, has also been reported to be beneficial. Controlled ventilation with a low tidal volume or ventilation delayed until the cold graft has re-warmed (32°C) after the onset of reperfusion, may also be important.

    Inhaled nitric oxide has been useful, clinically, to treat established IRI because it can improve ventilation/perfusion mismatch and decrease pulmonary artery pressures without affecting systemic pressures. On the basis of experimental evidence, some centres routinely use an infusion of prostaglandin (PG)E1 during the postoperative period after lung transplantation, whereas others reserve PGE1 infusion for the treatment of severe IRI. Experimental studies have found that exogenous surfactant-therapy can improve pulmonary function after lung transplantation, especially when given already in the donor. In cases of life threatening reperfusion oedema, the use of extracorporeal membrane oxygenation (ECMO) has been described as a successful, but ultimate treatment strategy. Weaning of the ECMO support is possible in 60–80% of the patients. In other patients, ECMO may ultimately serve as a bridge to retransplantation. However, this final option for early graft failure is controversial because of the poor results and should,therefore, be very restricted.

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    Correspondence: D. Van Raemdonck, Dept of Thoracic Surgery, University Hospital Gasthuisberg, Herestraat 49, B-3000, Leuven, Belgium.

    The widespread application of lung transplantation is limited by the shortage of suitable donor organs resulting in longer waiting times for listed patients with a substantial risk of dying prior to transplantation. To overcome this critical organ shortage, many transplant programmes have begun to explore the use of alternative donors.

    Several groups have shown that lungs from donors not matching the ideal criteria for lung donation, so called marginal or extended donors, can still be transplanted with no differences in outcome. Nevertheless caution needs to be exercised when transplanting less optimal lungs in recipients with an increased operative risk.

    In small or paediatric recipients, successful downsizing techniques by peripheral (segmental) resection, lobar transplantation, and pulmonary bipartitioning have been developed in order to allow the use of larger grafts for smaller recipients facing longer waiting times or requiring urgent transplantation.

    The use of lobes from living donors is an option in small recipients, mainly in patients with cystic fibrosis receiving the lower lobes of two family members or even unrelated individuals. Improved long-term outcome with a lower incidence of bronchiolitis obliterans syndrome has been reported.

    A few cases of successful transplantation of a lung, retrieved from a donor after cardiac arrest, a so called non-heart-beating donor (NHBD), were recently reported. In the controlled NHBD, the warm ischaemic period of the graft is limited. In the uncontrolled NHBD, topical cooling of the lung with a cold preservation solution inside the cadaver is advocated to minimise the warm ischaemic damage prior to cold flush preservation. Functional evaluation of the graft is mandatory thereafter to assess its viability so that only NHBD lungs of high quality will be transplanted.

    Finally, ex vivo reperfusion for medical intervention opens the prospect to resuscitate lungs by that have previously been rejected for transplantation because of inferior quality.

    Xenotransplantation and the use of an implantable artificial lung are currently still under investigation.

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    Correspondence: E. Pompeo, Division of Thoracic Surgery, Policlinico Tor Vergata, Tor Vergata University, V.le Oxford 81, 00133 Rome, Italy.

    Surgical treatment of emphysema was designed to improve subjective dyspnoea, respiratory function and quality of life in severely disabled patients. Three surgical options are currently employed, namely bullectomy, lung volume reduction surgery (LVRS) and lung transplantation.

    Bullectomy is indicated in presence of giant bullae occupying more than one half of the hemithorax with compression of relatively healthy lung tissue.

    LVRS entails nonanatomical resections of severely diseased emphysematous areas of the lungs and is performed in severely disabled patients with hyperinflation, heterogeneous distribution of destruction prevailing in the upper lobes, a forced expiratory volume in one second (FEV1) >20% predicted and a normal carbon dioxide arterial tension. LVRS, can be performed by open or thoracoscopic approaches, in unilateral or bilateral fashion and have been shown to offer prolonged functional benefit with acceptable mortality.

    Patients with lower FEV1, hypercapnia, and associated pulmonary hypertension are candidates for lung transplantation. Emphysema, especially in the context of α1-antitrypsin deficiency, is currently one of the most common indications for lung transplantation, accounting for ∼45% of cases worldwide. Single-lung transplantation is simpler and shorter than the bilateral transplantation, and there is less frequent need for cardiopulmonary bypass. Yet, it could potentially extend the availability of donor organs. These advantages have to be weighed against bilateral sequential lung transplantation procedure characteristics, which include an improved pulmonary function and the ability to use marginal lung donors as compared with single-lung transplantation.

    In conclusion, in properly selected patients, bullectomy, LVRS and lung transplantation can offer prolonged functional benefit and satisfactory long-term survival. Nonetheless, since they are invasive options with a risk of both mortality and morbidity, they are directed only at patients who remain symptomatic despite optimal medical treatment.

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    Correspondence: E.W. Russi, Pulmonary Division, University Hospital Zurich, Raemistr. 100, CH-8091, Zürich, Switzerland.

    Emphysema is usually a major feature in severe Chronic obstructive pulmonary disease. Besides lung function abnormalities, especially pulmonary hyperinflation and decreased diffusion capacity, the imaging patterns on conventional chest radiography and computed tomography are also used to estimate its extent and distribution. The selection of patients for lung volume reduction surgery (LVRS) is based on sound pathophysiological concepts. LVRS is particularly beneficial in heterogeneous distribution of emphysematous destruction. However, many questions concerning LVRS remain unanswered. The benefits of pulmonary rehabilitation programmes have been stressed during the last decade, especially on health related, quality of life and functional exercise capacity, rather than on survival.

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    Correspondence: Federico Venuta, University of Rome, La Sapienza, Department of Thoracic Surgery, Policlinico Umberto I, Viale del Policlinico, Rome 00161, Italy.

    Chronic obstructive pulmonary disease (COPD) is one of the most common causes of death both in Europe and in the USA. At the most advanced phases of the disease, when symptoms become disabling, the quality of life for these patients is extremely compromised. During the last 50 yrs various surgical procedures have been proposed to relieve dyspnoea and improve quality of life: costochondrectomy, pneumoperitoneum, glomectomy and many others. Early results were often encouraging but no sustained good results were obtained. All of these procedures were eventually abandoned.

    At the present time, only resection of giant bullae and lung transplantation have shown substantial beneficial effect. Surgical bullectomy has been employed successfully to improve lung function in patients with bullous emphysema for more than four decades; single- and bilateral-lung transplantations are now considered a viable therapeutic option for a selected group of patients with end-stage emphysema.

    Lung volume reduction has been recently rediscovered; it results in a significant but temporary functional improvement with palliation of dyspnoea and amelioration of quality of life. Also this surgical procedure can now be safely proposed for a selected group of patients with emphysema, with some limitations that are still debated in the literature.

    More recently, other experimental endoscopic procedures have been described; two of them seem more promising: the airway bypass and the endoscopic lung-volume reduction.

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    Correspondence: N. Konietzko, Ruhrlandklinik, Tueschener Weg 40, 45239, Essen, Germany.

    Most extrapulmonary function-improving surgical techniques were developed some 25–60 yrs ago and the indications, results and complications are well known. They comprise of the correction for funnel chest and severe scoliosis of the spine. However, for some abnormalities such as diaphragm paralysis or thoracoplasty, surgery cannot offer clear extrapulmonary function improving solutions.

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    Correspondence: G. Massard, Cardio-thoracic Surgery/Université Louis Pasteur, Service de Chirurgie Thoracique, Hôpitaux Universitaires de Strasbourg, F‐67091, Strasbourg, France.

    Surgery for suppurative lung disease is often technically demanding and the patient may have an increased operative risk because their general health status has mostly suffered from the infectious disease status. Thoracic aspergillosis is by far the most common fungal disease of the lung and the pleura, requiring surgical care in Western Europe. For a long time, only the saprophytic aspergilloma has received surgical interest, but in the last decade it has become clear that surgical management may also improve the outcome of selected patients with invasive aspergillosis. Patients with bronchiectasis should be considered for surgery when the disease is localised and when they present with haemoptysis and/or recurrent infections with failure of medical treatment and poor compliance to this treatment. The basic principle of surgery in multidrug-resistant tuberculosis is to remove all cavitary disease after the antibiotic regimen has already been given for a 3‐month period; the operation has a curative intent and prevents spreading of these multidrug-resistant mycobacteria in the community. Pneumonectomy for destroyed lung is mostly indicated because of recurrent infections; it remains a distressing problem which may be regarded as one of the most serious challenges in general thoracic surgery.

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    Correspondence: H.B. Ris, Service de Chirurgie Thoracique, Centre Hospitalier Universitaire Vaudois, CH-1011, Lausanne, Switzerland.

    The mainstay of treatment of pleural empyema is the treatment of ongoing infection and the prevention of recurrent infection and late restriction. The choice of the appropriate intervention depends on the nature of the underlying disease, the chronicity of the empyema, and the patient's overall condition. Computed tomography (CT) scans are important in estimating the chronicity of empyema and detecting its underlying cause. The different treatment strategies, with respect to chronicity and underlying cause of empyema, will be discussed on behalf of 282 consecutive patients with surgically treated American Thoracic Society (ATS) stage II and III pleural empyema over a 10‐yr period at the present authors' institution; CHUV, Lausanne, Switzerland.

    ATS stage I parapneumonic effusions are usually treated with antibiotics and thoracocentesis or a chest-tube drainage. However, recent studies have challenged the usefulness of chest-tube drainage alone in complicated parapneumonic effusions and a high failure rate has been reported, especially in multilocolulated effusions. Treatment of stage II empyema consists of fibrinolysis via a chest tube or thoracoscopic (video-assisted thoracoscopic surgery; VATS) debridement. VATS is preferred in good-risk paients whilst fibrinolysis is an excellent option in patients who do not tolerate surgery. Fibrinolysis and VATS are valid options in the treatment of fibrinopurulent empyema but they are not effective in its organising stage. Stage III empyema requires formal decortication by thoracotomy in order to prevent recurrence and restriction. Alterations of lung perfusion and respiration mechanics induced by chronic empyema may be restored if decortication is promptly performed once empyema has been diagnosed.

    Open window thoracostomy, thoracoplasty and intrathoracic muscle transfer are still valid surgical options in the treatment of special kinds of empyema. These procedures have in fact relived a renaissance in recent years due to an increase of complex intrathoracic diseases observed in a growing number of immunocompromised patients.

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    Correspondence: R. Loddenkemper, Lungenklinik Heckeshorn, Zum Heckeshorn 33, D‐14109, Berlin, Germany.

    The treatment of parapneumonic effusions (PPE) and empyema must always include antibiotics, which is sufficient in the early pleuritis sicca stage and in the exsudative stage. The fibrinopurulent stage also needs pleural space drainage in almost all cases also. The authors'current treatment approach, which has achieved a success rate of ∼90% on >440 patients, consists in drainage via double-lumen trocar catheter allowing simultaneous irrigation. Sometimes insertion of the tube is combined with medical thoracoscopy. Intrapleural fibrinolytic therapy is used over 5–6 days on average. In the authors' experience, the majority of fibrinopurulent effusions/empyemas can be managed successfully by nonsurgical treatment; the latter becomes necessary only when medical treatment fails or in the later organisational stage of empyema.

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    Correspondence: J. Hasse, Abt. Thoraxchirurgie, Chirurgische Universitätsklinik Freiburg, Joachimsacker str. 33, CH4103, , Boltmingen, Switzerland.

    Congenital malformations of the respiratory system are only occasionally the subject of thoracic surgery in adults. Among the spectrum of clinically relevant diseases pulmonary sequestration and bronchogenic cysts are the most frequent. The purpose of this chapter is to present a brief synopsis of such malformations, their clinical symptoms and, eventually, complications as well as treatments and outcomes based on the recent literature and the current author's experiences. It is shown that in the majority of congenital lesions presenting in adulthood surgery is successful and carries a low risk of morbidity and mortality. Therefore, even in the case of incidental diagnosis of asymptomatic malformations, operative correction is usually indicated.

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    Correspondence: J‐F.Velly, Dept of Thoracic Surgery, Hôpital du Haut-Lévêque, 33604 Bordeaux-Pessac, France.

    Management of tracheobronchial ruptures after blunt thoracic trauma requires a good knowledge of original mechanisms and of immediate or secondary physiological consequences of these lesions. An urgent and precise endoscopic diagnosis is mandatory. Immediate surgical treatment is the rule, taking into account, however, the schedule of other surgical repairs in case of polytrauma. The technical problems of surgical repair are nowadays overcome and prognosis mainly depends on the initial control of respiratory failure and complications.

  18. Page 234
    Correspondence: M. Noppen, Head of Clinic, Interventional Endoscopy Clinic, Academic Hospital AZ-VUB, 101, Laarbeeklaan, B-1090, Brussels, Belgium.

    Surgery remains the definitive treatment for the majority of patients suffering from central airway injury of benign origin. Corrective tracheobronchial surgery, however, requires considerable surgical expertise, an operable lesion, and an operable patient. Hence this type of surgery can only rarely be performed immediately after the causative injury has occurred, and preoperative stabilisation of the patient is often necessary. Conservative, endoscopic treatment therefore is indicated for temporary treatment awaiting definitive surgical repair, and even for permanent treatment when surgery remains impossible because of patient-or lesion-related characteristics, or because of patient refusal. Conservative endoscopic treatment includes various techniques which can be applied through rigid or flexible bronchoscopy, such as mechanical and balloon dilation, Nd:YAG‐laser or electrocautery resection, and airway stenting. Lesions candidate for conservative treatment include postintubation stenosis of the trachea, burn lesions of the airways, airway fistulae (bronchopleural, tracheo-oesophageal), and iatrogenic and traumatic airway rupture.

    Indications, techniques and outcome of conservative endoscopic temporary or permanent treatment of these disorders are discussed. Conservative endoscopic techniques constitute a valuable treatment complement while awaiting definitive surgical repair, and can even be proposed as a permanent treatment in inoperable patients.