Tuberculosis and Other Granulomatous Diseases of the Airway.

Granulomatous diseases of the airway are challenging lesions to diagnose and effectively manage not only because they are uncommon but also because they can occur in different forms, each with unique clinical and radiological characteristics. Most such lesions can be effectively managed conservatively with repeated airway dilatation, use of intraluminal stents, and specific antimicrobial treatment. The only exception is those lesions presenting with localized airway obstruction wherein surgical resection may be indicated and beneficial.

Tracheobronchopathia osteochondroplastica - a 61-year-old female with middle lobe syndrome.

The authors present a case of a 61-year-old female patient suffering from tracheobronchopathia osteochondroplastica (TBO). Etiology of this unit is uncertain. TBO is characterised by presence of multiple nodules of various size, situated in the submucosa of the trachea and major bronchi. The course is usually benign, until lesion obstructs the bronchial lumen. In the presented case, occluded middle lobe bronchi resulted in peripheral atelectasis of the lung.

Middle lobe syndrome: an intriguing presentation of tracheobronchial amyloidosis.

Pulmonary involvement in amyloidosis is a distinct rarity. This clinical entity usually presents as tracheobronchial amyloidosis (TBA). A 32-year-old, never-smoker man presented with episodic dyspnoea and wheezing along with cough and mucoid sputum. The chest radiograph was suggestive of a middle lobe syndrome (MLS). High-resolution CT (HRCT) of the chest confirmed the presence of MLS. In addition, HRCT showed circumferential thickening of the trachea and the main bronchi, with thickening of the posterior membranous wall of trachea. Fibrebronchoscopy, done to evaluate MLS, visualised multiple small polypoidal lesions in the lower part of trachea and carina. Endobronchial biopsies showed homogeneous, acellular amorphous deposit in the subepithelial region, which was congophilic in nature. A diagnosis of TBA presenting as MLS was made. To the best of our knowledge, this is the first detailed report of MLS as a presentation of TBA in the English literature.

Middle lobe syndrome: an extraordinary presentation of endobronchial tuberculosis.

Tuberculous infection of the tracheobronchial tree, termed as endobronchial tuberculosis (EBTB), is more common in young adults and females. This clinical entity is poorly understood and the diagnosis is frequently delayed as sputum smears are often negative for acid fast bacilli and the chest radiograph can be normal, resulting in diagnostic confusion. Bronchoscopy continues to play a key role in its diagnosis. Though atelectasis is not uncommon in these patients, EBTB presenting as a middle lobe syndrome (MLS) has rarely been documented. MLS refers to chronic or recurrent collapse of the right middle lobe and has a myriad of causes. The pathogenesis of this entity too is not fully established. We report this exceptional clinical manifestation in a 19-year-old male, who presented for evaluation of respiratory symptoms for 6 months along with constitutional complaints. Imaging suggested the presence of MLS and bronchoscopy established the diagnosis of endobronchial tuberculosis. GeneXpert evaluation of bronchial aspirate detected Mycobacterium tuberculosis. Histopathology confirmed the presence of granulomatous lesions. Subsequently, the cultures of bronchial aspirate and post-bronchoscopy sputum grew M. tuberculosis. Appropriate therapy with anti-tuberculosis drugs resulted in a remarkable symptomatic and radiological improvement. EBTB presenting as a MLS is a distinct rarity.

Ten years' experience in surgical treatment of right middle lobe syndrome.

PURPOSE: In this study we present the clinical, radiological, pathological, bronchoscopic and surgical results of 40 patients with diagnosis of middle lobe syndrome who were referred to our thoracic surgery unit for surgical intervention in a 10 years period. METHODS: Forty patients with obstructive and non-obstructive causes of middle lobe syndrome referred to our thoracic surgery unit. Clinical data were collected from the patients' records in a ten years period. This study evaluates diagnostic approaches and surgical treatments in right middle lobe syndrome. RESULTS: We studied 23 females (57.5%) and 17 males (42.5%) with a mean age of 31.7. Clinical findings were cough 95%, sputum 80% and intermittent hemoptysis in 50% of patients. Middle lobe collapse was seen in CT scan of all patients. Bronchiectasis was the most common pathologic finding (55%). Tuberculosis was not rare and was final pathology in 20% of patients. In three patients ruptured hydatid cyst was final finding. Surgery was done without mortality and with only minor complications. CONCLUSION: Lobectomy of right middle lobe is a good therapeutic option in these patients. Due to high prevalence of tuberculosis and hydatid cyst in Middle Eastern countries these two must be considered as causes of middle lobe syndrome.

Síndrome de Lady Windermere: afectación del lóbulo medio y língula por Mycobacterium avium complex / Lady Windermere syndrome: involvement of the middle lobe and lingula by Mycobacterium avium complex

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Middle lobe syndrome in children today.

Middle lobe syndrome in children is a distinct clinical and radiographic entity that has been well described in the pediatric literature. However, issues regarding its etiology, clinical presentation, and management continue to puzzle the clinical practitioner. Pathophysiologically, there are two forms of middle lobe syndrome, namely obstructive and nonobstructive. Middle lobe syndrome may present as symptomatic or asymptomatic, as persistent or recurrent atelectasis, or as pneumonitis or bronchiectasis of the middle lobe and/or lingula. A lower threshold of performing a chest radiograph is warranted in children with persistent or recurrent nonspecific respiratory symptoms, particularly if there is clinical deterioration, in order to detect middle lobe syndrome and to initiate a further diagnostic and therapeutic workup.

Síndrome del lóbulo medio y tuberculosis endobronquial. Aportación de la fibrobroncoscopia / Middle lobe syndrome and endobronchial tuberculosis. Contribution of fiberoptic bronchoscopy

El síndrome del lóbulo medio es una patología frecuente en el niño, sobre todo en relación con el asma y/o la hiperreactividad bronquial, aunque existen otras muchas causas, como los cuerpos extraños, la tuberculosis endobronquial, etc. Presentamos el caso de un niño de 5 años de edad, con atelectasia persistente en la base derecha con participación del lóbulo medio, que se diagnosticó de tuberculosis. La tomografía computarizada confirmó la lesión y, ante una prueba de la tuberculina positiva, se realizó una fibrobroncoscopia para descartar una tuberculosis endobronquial, que demostró la presencia de una lesión polipoidea en el bronquio intermediario a la entrada del lóbulo medio. Se describen las diversas formas de tuberculosis endobronquial, así como el tratamiento con corticoides sistémicos. Se aconseja la realización de una fibrobroncoscopia en los casos de tuberculosis pulmonar con alteraciones radiológicas persistentes (atelectasia, atrapamiento aéreo, etc.) y/o falta de mejoría clínica pese al tratamiento adecuado(AU)

Middle lobe syndrome is a common condition in children, mainly related to asthma and/or bronchial hyperresponsiveness, although there are many other causes, such as foreign bodies, endobronchial tuberculosis, etc. We report a 5-year-old child with persistent atelectasis involving the right middle lobe, and diagnosed with tuberculosis. Computed tomography confirmed the injury and after a positive tuberculin skin test, fiberoptic bronchoscopy is performed to rule out endobronchial tuberculosis showing a polypoid lesion in the bronchus intermedius at the entrance of the middle lobe. It describes the various forms of endobronchial tuberculosis, and treatment with systemic corticosteroids. Fiberoptic bronchoscopy is recommended in cases of pulmonary tuberculosis with persistent radiographic abnormalities (atelectasis, air trapping, etc.) and/or a lack of clinical improvement with an adequate treatment(AU)

Middle lobe syndrome: a review of clinicopathological features, diagnosis and treatment.

Middle lobe syndrome (MLS) is a rare but important clinical entity that has been poorly defined in the literature. It is characterized by recurrent or chronic collapse of the middle lobe of the right lung but can also involve the lingula of the left lung. Pathophysiologically, there are two forms of MLS, namely obstructive and nonobstructive. Obstructive MLS is usually caused by endobronchial lesions or extrinsic compression of the middle lobe bronchus such as from hilar lymphadenopathy or tumors of neoplastic origin, resulting in postobstructive atelectasis and pneumonitis. In the nonobstructive type, no obstruction of the middle lobe bronchus is evident during bronchoscopy or with computerized tomography of the chest. The etiology of the nonobstructive form is not completely understood. Inefficient collateral ventilation, infection and inflammation in the middle lobe or lingula are thought to play a role, and bronchiectasis is the most common histological finding. Patients with proven endobronchial lesions or malignancy are usually offered surgical resection directly. This contrasts with nonobstructive MLS, where most patients respond to medical treatment consisting of bronchodilators, mucolytics and broad-spectrum antibiotics. However, some patients do not respond to conservative treatment and may suffer irreversible damage of the middle lobe or lingula, in addition to having recurrent symptoms of infection or inflammation. These selected patients can be offered surgical resection of the middle lobe or lingula, which is associated with a low mortality rate and favorable outcome.

[A case of a carcinoid tumour presenting as an "upper lobe syndrome"]. / Un cas de tumeur carcinoïde typique diagnostiqué sur un « syndrome du lobe supérieur ¼

A 53-year-old woman presented with progressive cough related to an endobronchial carcinoid tumour. The location of the tumour in the right upper lobe bronchus could be described as an "upper lobe syndrome" by analogy with the "middle lobe syndrome" or Brock's syndrome. Surgical management consisted of lobectomy and lymph node dissection. This established the diagnosis of typical carcinoid tumour. There was no mediastinal nodal invasion. Three months after surgery all symptoms had disappeared.

Middle lobe syndrome in the left lower lobe in chronic obstructive pulmonary disease.

Middle lobe syndrome is a term that refers to a recurrent collapse of a lung segment, typically the right middle lobe. We discuss a case that middle lobe syndrome occurred in an unusual lung segment and related to an unusual cause.

Massive benign pericardial cyst presenting with simultaneous superior vena cava and middle lobe syndromes.

A 66 year old woman presented in extremis with symptoms and clinical and radiological signs of simultaneous obstruction of superior vena cava and middle lobe of right lung secondary to compression by a massive benign anterior mediastinal cyst. Excision of the cyst at median sternotomy resulted in complete resolution of all symptoms. This report is unusual on account of a) the concomitant presence of superior vena cava and middle lobe syndromes caused by a benign cyst because of its sheer size producing obstruction of these structures and b) the complete resolution of all symptoms and signs after removal of the cyst. Benign anterior mediastinal cysts are unknown to cause either of the two syndromes. To our knowledge, it is the first report of a benign anterior mediastinal cyst causing either superior vena cava syndrome or middle lobe syndrome or both simultaneously. Etiologies of both superior vena cava and middle lobe syndromes are discussed in detail.

Síndrome de Lady Windermere / Lady Windermere syndrome

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Bronchial hyperresponsiveness, atopy, and bronchoalveolar lavage eosinophils in persistent middle lobe syndrome.

Most cases of middle lobe syndrome (MLS) in children are considered to be due to asthma and may recover spontaneously; however, in persistent MLS, repeated episodes of infection often institute a vicious cycle that may lead to persistent symptoms and bronchial hyperresponsiveness (BHR). The present study was undertaken to investigate whether asthma, as an underlying diagnosis, is predictive of a favorable outcome of children with persistent MLS. We evaluated 53 children with MLS who underwent an aggressive management protocol that included fiberoptic bronchoscopy (FOB) and bronchoalveolar lavage (BAL). These patients were compared to two other groups: one consisting of children with current asthma but no evidence of MLS (N = 40) and another of non-asthmatic controls (N = 42), matched for age and sex. Prevalence of sensitization (>or=1 aeroallergen) did not differ between patients with MLS and "non-asthmatics" but was significantly lower than that of "current asthmatics." A positive response to methacholine bronchial challenge was observed with increased frequency among children with MLS when compared to "current asthmatic" and non-asthmatic children. Multivariate logistic regression analysis revealed a positive correlation between an increased number of eosinophils in the BAL fluid (BALF) and a favorable outcome, whereas no correlation was detected between sensitization or BHR and BAL cellular components. In conclusion, children with MLS have an increased prevalence of BHR, even when compared to asthmatics, but exhibit prevalence of atopy similar to that of non-asthmatics. An increased eosinophilic BALF count is predictive of symptomatic but not radiographic improvement of MLS patients after aggressive anti-asthma management.

Mycobacterium avium complex with a distinct clinical and iconographic presentation: the Lady Windermere syndrome.

Clinically significant pulmonary disease caused by non-tuberculous mycobacteria such as Mycobacterium avium Complex (MAC) usually occurs upon pre-existing lung diseases or immune-deficiency. In 1992, a particular presentation of pulmonary MAC, occuring in otherwise healthy middle-aged women, was described with pulmonary consolidations localized in lingula and middle lobe. For this specific, rare condition, the term Lady Windermere syndrome was introduced. We report a particular case of this syndrome, in which an otherwise healthy individual developed clinically significant disease upon MAC (subtype: Mycobacterium avium) infection of the right middle lobe and lingula of the lung. The patient did not have the classical risk factors for developing this syndrome (e.g. habitual cough suppression, long and narrow bronchi) indicating their modest contribution in the pathogenesis. In our case, guideline based therapy was found to be inadequate because of multi-drug resistance, so an alternative treatment regime was given with good clinical result.

Middle lobe syndrome as the pulmonary manifestation of primary Sjögren's syndrome.

Middle lobe syndrome - recurrent atelectasis and/or bronchiectasis involving the right middle lobe and/or lingula - has, up to now, not been reported as the pulmonary manifestation of primary Sjögren's syndrome. We describe a patient in whom lymphocytic bronchiolitis in the atelectatic lobes was proved histologically from two separate transbronchial biopsies. The atelectasis responded well to glucocorticoid treatment, suggesting that the peribronchiolar lymphocytic infiltrates may have played an important role in the development of middle lobe syndrome in this patient.

[Middle lobe syndrome]. / Un cas de syndrome du lobe moyen.

Broncholithiasis is uncommon and usually a late complication of tuberculosis in France. The most common cause of broncholithiasis is erosion by and extrusion of a calcified lymph node into a bronchial lumen. Clinical symptoms are not specific and diagnosis may be an endoscopic or a radiologic surprise. We report a case of broncholithiasis in a patient with chronic cough. Fibroscopy established the diagnosis by showing the broncholith.