David P. Breen

A Multicenter Retrospective Study Investigating the Role of Interventional Bronchoscopic Techniques in the Management of Endobronchial Lipomas

Background: Pulmonary lipomas are rare benign tumors that are usually found endobronchially. Undiagnosed, they can lead to the serious late sequelae associated with endobronchial obstruction. In the majority of cases, they are located in the first three subdivisions of the tracheobronchial tree, and therefore, they are amenable to endoscopic techniques for diagnostic and therapeutic purposes. Objectives: It was our aim to retrospectively study the bronchoscopic management and follow-up of a large series of endobronchial lipomas, as well as defining the demographic and endoscopic characteristics of patients. Methods: A retrospective multicenter study was performed to identify all cases of lipomas that were treated endoscopically in 4 institutions in the period from 1981 to 2002. Demographic, radiological, endoscopic and histological data were collected. Results: Thirty-eight patients were included in the study; 81.6% of cases were males and the average age was 63.5 ± 15.2 years. The majority of the patients were symptomatic (63.2%). Lipomas were located proximally in 18 cases (47.4%) and distally in 20 subjects (52.6%). Specimens obtained by rigid bronchoscopy were diagnostic in all cases. Thirty-six out of 38 patients underwent therapeutic rigid bronchoscopy. Laser and mechanical debulking was performed in 29 cases (76.3%), cryotherapy and mechanical debulking in 7 subjects (18.4%), and mechanical debulking alone in 2 cases (5.3%). No cases of recurrence occurred during the follow-up period. Conclusions: This study demonstrates that endoscopic techniques are effective for the diagnosis and treatment of endobronchial lipomas when there is no evidence of severe distal bronchiectasis. This should be the treatment of choice after a full clinicoradiological evaluation.

The integrated place of tracheobronchial stents in the multidisciplinary management of large post-pneumonectomy fistulas: our experience using a novel customised conical self-expandable metallic stent

BACKGROUND Stump dehiscence after pneumonectomy is a cause of morbidity and mortality in patients treated for non-small-cell lung carcinoma. Surgical repair remains the treatment of choice but can be postponed or contraindicated. Bronchoscopic techniques may be an option with curative intent or as a bridge towards definitive surgery. The aim of the study is to evaluate the efficacy and the outcome of a new customised covered conical self-expandable metallic stent in the management of large bronchopleural fistulas complicating pneumonectomies. METHODS A case series using chart review of non-operable patients presenting with large bronchopleural fistulas (>6mm) post-pneumonectomies as a definitive treatment with curative intent for non-small-cell lung carcinomas and requiring the use of a dedicated conical shaped stent in two tertiary referral centres. RESULTS Seven patients presenting large post-pneumonectomy fistulas (between 6 and 12 mm) were included. Cessation of the air leak and clinical improvement was achieved in all the patients after stent placement. Stent-related complications (two migrations and one stent rupture) were successfully managed using bronchoscopic techniques in two patients and surgery in one. Mortality, mainly related to overwhelming sepsis, was 57%. Delayed definitive surgery was achieved successfully in three patients (43%). CONCLUSIONS This case series assesses the short-term clinical efficacy of a new customised covered conical self-expandable metallic stent in the multidisciplinary management of large bronchopleural fistulas complicating pneumonectomies in patients deemed non-operable. Long-term benefits are jeopardised by infectious complications.

Molecular Testing and Personalized Treatment of Lung Cancer

Lung malignancy is a leading cause of cancer related morbidity and mortality worldwide. The majority (85%) of cases are histologically proven non-small cell lung carcinomas (NSCLC). More than 55% of lung carcinomas harbor at least one genetic alteration, most of them being histologic subtype specific. This review summarizes the progress in personalized care of lung cancer by reviewing the literature on EGFR, ALK and KRAS molecular alterations, currently used in clinical practice, to direct the decision making process for lung cancer therapy. In addition, we will discuss some recently characterized molecular alterations whose targeting is being tested in clinical trials and holds promise for future therapeutic targeting. The role of minimally invasive procedures in lung cancer diagnosis and staging is also discussed as these techniques now play a central role in lung cancer management by providing the bulk of material for modern molecular diagnostics.

On-site customization of silicone stents: towards optimal palliation of complex airway conditions.

Background: Stents may be indicated as treatment of benign and malignant conditions of the central airways. Occasionally, aberrant and/or distorted airways secondary to therapy or surgery may preclude the use of commercially manufactured stents. The customization of stents is well described for nonpulmonary diseases, but to date there are only limited data in the literature on prosthesis customization for airway disease. Objectives: To review all the different techniques and indications of on-site silicone stent customizations and their related complications. Methods: A retrospective study was undertaken to identify all patients who underwent treatment with a silicone stent which was customized on site by the physician. The study included subjects treated during an 8-year period. Results: Forty-nine on-site customizations were performed in 43 patients (34 males, mean age 63 ± 5.2 years). Stent customization was performed in 5.4% of the cases. Stent customization was performed for malignant and benign disease in 35 (81.3%) and 8 (18.7%) cases, respectively. In all cases, the stent deployed successfully and performed well while in situ. Stent manipulation did not increase complications. Conclusions: Customization of airway stents by the physician can lead to successful stenting of difficult airway lesions with a good short-term safety profile. Standard commercially available stents may have resulted in suboptimal management of complex airway pathologies. A prospective study is warranted to further investigate the safety profile and complications associated with stent customization.

Endoscopic successful management of tracheobronchomalacia with laser: apropos of a Mounier-Kuhn syndrome

Mounier-Kuhn syndrome is a rare condition that combines tracheobronchomegaly (TBM) and severe tracheobronchomalacia. Symptoms can be severe with recurrent bronchopulmonary infections and cough-induced syncope. Therapeutic management is non-specific and limited to chest physiotherapy and antibiotics during infectious exacerbations. We report a case of Mounier-Kuhn syndrome that was successfully managed by treating the posterior collapse of the central airway with yttrium aluminum pevroskyte laser. Endoscopic aspects, respiratory symptoms, and lung function tests all improved and remained stable with a follow-up of 8 years. Laser, at low power settings, could be a new therapeutic option in selected cases of tracheobronchomalacia.

Hiatal hernias are correlated with increased severity of non-cystic fibrosis bronchiectasis.

Hiatal hernias (HH) are associated with gastro‐oesophageal reflux and may contribute to lung disease severity. We aimed to evaluate the prevalence of HH among stable non‐cystic fibrosis bronchiectasis (NCFB) patients and determine associations with disease severity.

Malignant pleural effusion.

Malignant pleural effusion (MPE) refers to the presence of neoplastic cells in the pleural fluid. Approximately 40 000 people per year in the UK are affected by MPE and it is associated with significant morbidity and an overall poor prognosis. Management should be prompt and care plans should be individualized and involve a multidisciplinary team of healthcare professionals. This article reviews the pathophysiology of MPE along with available investigations and management strategies for these patients.

Tracheal Compression in a Patient with Marfan’s Syndrome-Associated Tracheomegaly Treated by an XXL Stent: The Largest Diameter Airway Stent Ever Placed in a Previously Undescribed Airway Condition

A 43-year-old man was referred to our institution with severe extrinsic compression of the trachea at the level of the main carina secondary to an aortic aneurysm, causing respiratory distress and requiring mechanical ventilation. The patient had a past history of Marfan’s syndrome and tracheomegaly (the estimated tracheal diameter bronchoscopically was 28 mm). Palliation of the compression was successfully achieved by a custom-made, self-expandable, fully covered metallic stent with a diameter of 28 mm and a length of 60 mm. The patient was weaned off ventilation. The stent stayed in place for 2 years without major complications. This case represents the first stent ever inserted in a very rare condition combining tracheomegaly and extrinsic compression in Marfan’s syndrome. It is also the first report of successful placement of the largest tracheal stent which was manufactured exclusively for the airway.

A Rare Cause of an Endobronchial Tumour in Children : The Role of Interventional Bronchoscopy in the Diagnosis and Treatment of Tumours while Preserving Anatomy and Lung Function

An 11-year-old girl was referred to our institution with complete obstruction of the left main stem bronchus by an endoluminal mass and was successfully treated by a combination of laser and mechanical debulking. Coring of the mass both allowed histological diagnosis (inflammatory myofibroblastic tumour) and definitive treatment with no recurrence after 3 years of follow-up. This case report emphasizes the major role of interventional bronchoscopy in the management of tumoural obstruction in children in whom benign conditions are frequent. Interventional bronchoscopy, which is minimally invasive when compared to surgery, in the vast majority of cases allows a precise diagnosis as it provides large samples of tissue removed during coring; in some cases it can be curative by itself and preserves lung parenchyma and function.

“Dancing Eye Syndrome” Secondary to Opsoclonus-Myoclonus Syndrome in Small-Cell Lung Cancer

Among paraneoplastic neurologic disorders (PND), opsoclonus-myoclonus syndrome, so-called “dancing eye syndrome,” is a rare disorder combining multivectorial eye movements, involuntary multifocal myoclonus, and cerebellar ataxia. Although several paraneoplastic antibodies against postsynaptic or cell-surface antigens have been reported, usually most patients are serum antibody negative. We report a 65-year-old patient with opsoclonus-myoclonus syndrome revealing a small-cell lung carcinoma. If serologic antineuronal anti-body screening was negative, autoantibodies against glutamic acid decarboxylase (anti-GAD) were positive. Despite the specific anticancer treatment and high dose corticosteroids, the patient developed a severe and progressive encephalopathy and died 10 days later.