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Middle lobe syndrome is a clinical term used to describe right middle lobe atelectasis with or without bronchial compression. Fungal disease has been implicated rarely as a cause of middle lobe syndrome. This is a patient with Blastomyces dermatitidis infection who presented with right middle lobe syndrome.
Middle lobe syndrome (MLS) or atelectasis syndrome can affect any pulmonary lobe but occurs more frequently in the right middle lobe. Only 15 per cent of the reported cases are children. We report two cases of MLS in two boys of 4 and 12 years of age who were referred to our institution because they had suffered from recurrent pneumonies of the right middle lobe for ten months and six years respectively. We performed chest radiographs, computed tomography scanning, ventilation-perfusion scintigraphy and flexible bronchoscopy. The evolution in both cases to an atelectasic middle lobe with pulmonary shrinkage and lack of perfusion was the indication to lobectomy. Histological examination showed a bronchial obstructive process in the young patient and a chronic non specific inflammation of the pulmonary parenchyma in both. The resection brought about a cure. The identification of MLS is usually a late diagnosis. Thus, if atelectasis persists after an adequate medical therapy, resection of the lobe is indicated.
Middle lobe syndrome is defined as recurrent or chronic collapse of the middle lobe of the right lung. It occurs in all age groups and is divided into an obstructive type, with a demonstrable airway occlusion, and a nonobstructive type, with a patent right middle lobe bronchus apparent on bronchoscopy. Middle lobe collapse has specific radiographic findings. Malignancy is the most common cause of the obstructive type, and infections are the second leading etiology. The nonobstructive type is associated with inflammatory conditions and bronchiectasis. In all cases, treatment is directed at the underlying cause.
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Classically, the middle lobe syndrome has been described as being caused by a central obstruction. Clinical records and radiologic findings were reviewed in 129 patients examined between 1955-1981 who had chronic disease in the right middle lobe and/or lingula. Fifty-eight patients (45%) had no evidence of a central obstructive lesion. The majority were middle-aged women with histories of chronic cough and chest pain. Bronchoscopic and radiologic evaluation served to exclude central lesions. Surgical confirmation was available in 38 patients. Pathologic study showed varying degrees of chronic inflammation, pneumonia, and bronchiectasis. Surgical results were excellent in isolated disease. Chronic atelectasis and pneumonitis of the right middle lobe and/or lingula do not always imply central obstruction. A lack of collateral ventilation is a plausible theory to explain the pathophysiology in such patients.
236 cases with middle lobe syndrome were examined with fibrobronchoscope. The total rate of positive finding was 92.8% (219/236). Inflammation (167 cases), lung cancer (47 cases), tuberculosis (17 cases) accounted for most of the cases. The diagnostic rate for the three above-mentioned diseases were 97.6%, 87.2% and 58.8% respectively. Fibrobronchoscopy was also of significant diagnostic value in determining other uncommon cases such as broncholithiasis, lipoid pneumonia, lung infiltration in leukemia, etc. The etiology, chest X-ray (including CT) presentations of this middle lobe syndrome and the diagnostic value of fibrobronchoscopy were discussed. Our experience suggests that this procedure is both safe and effective in the diagnosis of patients with middle lobe syndrome.
Right middle lobe syndrome (RMLS) is characterized by a spectrum of diseases from recurrent atelectasis and pneumonitis to bronchiectasis of the middle lobe. It has been described among all age groups, although the diagnosis in pediatrics may be delayed or missed because of non-specific symptoms or findings. Twenty-one children with RMLS were evaluated during the past 10 years with particular attention to the history, bronchoscopic and pathologic findings. Most of these patients had asthma or a family history of atopic disorders; 3 patients had a family history of RMLS. Only two of the 21 patients had sufficient obstruction on bronchoscopy to account for their disease. Four had evidence of concomitant laryngeal pathology. The various theories of pathogenesis are discussed. In this series, the non-obstructive (impaired collateral ventilation) theory appeared to be most plausible. Bronchoscopy was performed in all instances to rule out obstruction due to foreign body or tumor. It was therapeutic in two-thirds of the cases. Resolution occurred promptly in one-third, and eventually in another third. Of the remaining patients, 4 required lobectomy and were cured; two have decreased but persistent symptoms. An aggressive medical management following bronchoscopy is warranted in all cases, especially when there is a possibility of asthma.
Isolated atelectasis of the middle lobe has been known for many years as the "middle lobe syndrome". Several clinical studies have shown that it may bae caused by malignant tumours. A 10-year study of 135 patients with isolated middle lobe atelectasis is presented. Fifty-eight patients (43%) had malignant tumours. Of 38 who had a thoracotomy, lung resection was possible in 25. In 20 patients regional or systemic dissemination of the tumour had been diagnosed before operation. Seventy-seven patients had benign diseases, of which 74 were non-specific infections. Bronchography was performed in 46 of these cases, and all had abnormal findings in the middle lobe, eight revealing definite bronchiectasis. In three cases tuberculosis was found. In 16 cases the benign diagnosis was established at thoracotomy. Only three patients out of 58 with malignant tumours lived more than five years. Atelectasis of the middle lobe is always a sign of potential malignancy especially in patients with a previously normal chest radiograph.
Results of treatment of 242 patients with middle-lobe syndrome are analyzed. Indications for surgical treatment of middle-lobe syndrome must be determined very individually. Organo-saving surgeries are the methods of choice for benign processes in the middle lobe. In malignant tumors, destructive and cirrhotic processes of the middle lobe the resections are indicated.
Middle lobe syndrome (MLS) is an uncommon lung disorder involving the right middle lobe and/or lingula and is characterized by a spectrum of clinical and pathological lesions ranging from recurrent atelectasis or pneumonias to bronchiectasis. Despite several series reporting the clinical features of MLS, histopathological descriptions are rare. We reviewed the clinical characteristics and pathological findings in 21 patients with MLS who underwent surgical resections. Six male and 15 female patients between the ages of 5 and 80 years (mean, 47 years) were studied. All patients were symptomatic and complained of chronic cough (8), hemoptysis (6), chest pain (4), dyspnea (3), or fever (2). The right middle lobe was involved in 11 patients, the lingula in four patients, and both right middle lobe and lingula in six patients. Chest radiographs, bronchograms, and/or computed tomography scans were available for review in 19 patients and showed consolidation (8), bronchiectasis (9), patchy infiltrates (5), and atelectasis (4) in various combinations. Pathological findings included bronchiectasis in 10 patients, chronic bronchitis/bronchiolitis with lymphoid hyperplasia in seven, patchy organizing pneumonia in six, atelectasis in five, granulomatous inflammation in five, and abscess formation in four. Three patients with granulomatous inflammation had associated atypical mycobacterial infection. Broncholithiasis was confirmed by pathological examination in one patient. No pathological cause for bronchial obstruction was identified in the remaining 20 patients, although one was thought to have had broncholithiasis on the basis of preoperative bronchoscopy. The presence of bronchiectasis, bronchitis or bronchiolitis, organizing pneumonia, or atelectasis in specimens from the right middle lobe or of lingula in the absence of an identifiable cause of bronchial obstruction should suggest a diagnosis of MLS.
Two different processes have been proposed for pathogenesis of Mycobacterium avium complex (MAC) disease which show the middle lobe syndrome: 1) middle lobe bronchiectasis followed by MAC infection and 2) MAC disease resulted in secondary bronchiectasis. Two surgical specimen from MAC cases showing middle lobe syndrome were studied histo-pathologically. The first case was a 60 year-old female with frequent bloody sputum, who had been diagnosed as bronchiectasis in her childhood. Pathological examination of the resected middle lobe showed prominent cylindric bronchiectasis in the indurated middle lobe, and epithelioid cell granulomas were scattered limited to the fibrous bronchial walls, without any granulomas in the lung parenchyma. These findings suggested a secondary infection of MAC to the non-specific pre-existed bronchiectasis. The second case of a 55 year-old female having repeated bloody sputum, who was diagnosed to be tuberculosis but no improvement with anti-tuberculosis drugs. Pathological examination of the middle lobe showed scattered epithelioid cell granulomas with lymphocytic infiltration in the lung parenchyma. A few epithelioid cell granulomas were also found in the mucosa of middle lobe bronchi. In this case, pulmonary MAC lesions seemed to precede the central bronchial lesion with later development of bronchiectasis. Summarizing above findings two different mode of pathogenesis ways may be considered; one is non-specific bronchiectasis followed by middle lobe MAC disease and the other is pulmonary MAC lesion in the middle lobe as a primary change.
Middle lobe syndrome is a distinct clinical entity characterized by right middle lobe atelectasis. Prompt diagnosis and initiation of medical therapy including the administration of antibiotics and the avoidance of irritating agents may be effective. However, abnormal shadows on chest radiography remain unchanged even when acute symptoms have disappeared, suggesting latent lesional inflammation or recurrence. We describe 2 cases of atelectasis in the middle lobe of the right lung which resolved completely after administration of low-dose roxithromycin. Thus, low-dose therapy with a macrolide could be of interest or some value in these cases, but it is too early to draw a definite conclusion.
We evaluated the incidence of middle lobe syndrome in the Haibara area, and its relationship to atypical mycobacterial infection. Of the 30,588 persons who underwent annual mini-chest roentgenography in 1992 or 1993 or both, 51 (0.17%) had middle lobe syndrome, diagnosed from posteroanterior and lateral chest X-ray films. The incidence was significantly higher in persons over 50 years old than in persons under 50 years old (0.26% vs 0.02%: p < 0.001), and was higher in femals than in males (0.20% vs 0.11%: p = 0.527). Of 16 patients examined by bronchoscopy and computed tomography, 7 showed evidence of cylindrical bronchiectasis, and four had mycobacterium avium complex pulmonary disease presenting as middle lobe syndrome. All four were women who were 51 years of age or older and none had predisposing pulmonary disorders. Computed tomography showed multiple nodular shadows with or without bronchiectasis located in the middle lobe or the lingula. Cavitary lesions were not seen. These results indicate that middle lobe syndrome is not rare, and that infection with mycobacterium avium complex should be considered when multiple nodular shadows are seen in the middle lobe or the lingula.
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