Bronchoscopy for management and identification of etiology of right middle lobe syndrome: Analysis of 66 cases.

BACKGROUND: Right middle lobe (RML) syndrome is a recurrent or chronic obstruction of the RML causing atelectasis of the right middle lobe due to mechanical and nonmechanical etiologies. The consequences of untreated RML syndrome range from chronic cough to post-obstructive pneumonia and bronchiectasis. We report here our bronchoscopy experience in patients with RML syndrome. METHODS: We conducted a retrospective study of adult patients who underwent bronchoscopy for RML syndrome at Rabin Medical Center from 2008 through 2022. Demographic data and medical history, bronchoscopy findings and procedures, and follow-up results were collected. RESULTS: A total of 66 patients (57.6% male, mean age 63 ± 13 years) underwent bronchoscopy for RML syndrome during the study period. Bronchoscopy revealed a mechanical etiology in 49 (74.2%) cases, including endobronchial mass (21, 31.8%) and external compression (7, 10.6%). Malignancy was identified in 20 (30.3%) cases. In 62 patients (93.9%), the bronchoscopy resulted in partial or complete reopening of the RML bronchus. The therapeutic bronchoscopic procedures were balloon dilatation (19), laser ablation (17), mechanical debridement (12), endobronchial stent insertion (11), and cryoablation (6). CONCLUSIONS: Malignancy was identified as the etiology of RML syndrome in approximately 25% of cases, suggesting bronchoscopy should be performed in every case of RML atelectasis. To our knowledge, this is the first reported series of endobronchial stenting of the RML bronchus in the context of RML syndrome.

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.

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.

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.

[Two cases of chronic atelectasis that improved through use of nasal continuous positive pressure].

We observed improvements in two cases of chronic atelectasis through use of nasal continuous positive airway pressure (nCPAP). Case 1 suffered from middle lobe syndrome accompanied by chronic atelectasis resistant to medical treatment. Case 2 suffered from respiratory failure caused by chronic atelectasis and airway infection complications thereof following a total pneumonectomy and post-pneumonectomy syndrome. The patient was placed on artificial ventilation, and atelectasis was improved by maintaining PEEP and airflow to the atelectatic region. Following extubation we obtained good pneumatization using nCPAP. nCPAP has been reported as effective not only in cases of sleep apnea, but also for cardiogenic pulmonary edema and post-operative atelectasis; we believe it holds great promise for chronic atelectasis as well.

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.

The role of timely intervention in middle lobe syndrome in children.

STUDY OBJECTIVES: Middle lobe syndrome (MLS) in children is characterized by a spectrum of clinical and radiographic presentations, from persistent or recurrent atelectasis to pneumonitis and bronchiectasis of the right middle lobe (RML) and/or lingula. This study was undertaken to evaluate the effect of early intervention, including fiberoptic bronchoscopy (FOB), in the development of bronchiectasis in MLS. DESIGN: Children with atelectasis of the RML and/or lingula persisting for > 1 month or recurring two or more times despite conventional treatment underwent high-resolution CT (HRCT) scanning and FOB. Appropriate treatment and follow-up were provided, and the effect of the duration of symptoms on clinical outcome and the development of bronchiectasis was investigated. The patient cohort was retrospectively reviewed. PATIENTS: We evaluated 55 children with MLS. The median age at diagnosis, duration of symptoms, and duration of clinical deterioration before diagnosis were 5.5 years (range, 3 months to 12 years), 14.5 months (range, 3 to 48 months), and 8 months (range, 3 to 36 months), respectively. MEASUREMENTS AND RESULTS: FOB revealed marked obstruction in two children (ie, a foreign body and an endobronchial tumor) and positive findings for a culture of BAL fluid in 49.1% of patients. The remaining 53 patients were followed up for a median duration of 24 months (range, 5 to 96 months). The clinical outcome was "cure" in 60.4% of patients, "improvement" in 32.1% of patients, and "no change" in the remaining patients. Bronchiectasis was documented prior to FOB by HRCT scan in 15 patients (27.3%). The duration of the deterioration of symptoms prior to presentation positively correlated with the development of bronchiectasis (p = 0.03) and an unfavorable clinical outcome (ie, improvement or no change) [p = 0.02]; a positive correlation was also found between the duration of symptoms and the development of bronchiectasis (p = 0.04). CONCLUSIONS: Timely medical intervention in patients with MLS that includes FOB with BAL prevents bronchiectasis that may be responsible for an ultimately unfavorable outcome.

Síndrome do lobo médio: estudo de 10 casos atendidos no Hospital Universitário Lauro Wanderley / Middle lob disease: 10 cases assisted in the University Hospital Lauro Wanderley

Os autores fazem um estudo de 10 casos de síndrome do lobo médio (SLM), atendidos no Hospital Universitário Lauro Wanderley da Universidade Federal da Paraíba (HULW/UFPB), no período de 1986 a 1999. Enfatizam os aspectos clínicos, epidemiológicos e laboratoriais de diagnóstico, inclusive por imagem, comentando cada caso isoladamente. Ressaltam os aspectos radiográficos mais sugestivos dessa enfermidade, visando a instituição de um protocolo terapêutico cirúrgico para posterior seguimento e evolução. Chamam ainda a atenção para a instituição do tratamento tuberculostático precoce, no intuito de evitar seqüelas irreparáqveis no lobo médio, sobretudo sua forma mais temível - a síndrome do lobo médio. Concluem que o tratamento cirúrgico da SLM é considerado imprescindível no sentido não apenas de corrigir as graves deformidades da arquitetura pulmonar do lobo médio, mas também de acelerar a cura.

[The middle lobe syndrome in pediatrics. A study of 27 cases]. / Síndrome del lóbulo medio en pediatría: estudio de 27 casos.

OBJECTIVE: The purpose of this study was to analyze the features, treatment and evolution of middle lobe syndrome (MLS) or atelectasis syndrome in children. PATIENTS AND METHODS: Between 1992 and 1996, all cases of MLS seen in our center by our staff were followed by using a prospective study protocol. RESULTS: Twenty-seven patients were studied. The average age at the onset of MLS was 3 years 1 month +/- 2 years 4 months. Recurrent pneumonia was seen in 66.7% and 44.4% had asthma and/or elevated IgE. Acute symptoms at the onset were seen in 74% of the cases, with persistent cough being the most common symptom. Complementary procedures (chest X-rays, computed tomography scans, bronchoscopy, V/P scintigraphy) demonstrated 16 cases of pneumonia, 13 of atelectasis, 7 of bronchiectasia and 4 cases with other abnormalities. Infectious etiology was the most predominant (66.7%) Bronchoscopy was performed in 8 cases and 5 of them improved immediately. Pulmonary scintigraphy was normal in those patients with radiographic residual images. All patients were initially medically treated. Three were lobectomized. Twenty-three patients healed. The younger the patient at the onset of MLS the better the cure rate. CONCLUSIONS: Infectious causes were dominant in our series. Early and aggressive medical treatment may cure the majority of cases. Bronchoscopy is helpful in the diagnosis and as a therapeutic procedure. Scintigraphy could be helpful as a resolution criterion when there are residual radiographic images.

Middle lobe syndrome.

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.

[Middle lobe syndrome]. / Sindrome del lobo medio.

The authors, based on their actual experience with four cases and using previously published articles, state the pathological and clinical features of the middle lobe syndrome. Considering the physio-pathological processes, the authors determine the therapeutic program: the first approach would be pharmacological, second, if necessary, surgical-seeking to be as conservative as possible.

Right middle lobe syndrome in children.

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.

Middle lobe syndrome.

A review of the major literature dealing with the middle lobe syndrome shows that benign inflammatory disease is the most common etiological factor (62%), with bronchiectasis responsible for at least a quarter of the patients in these series. Early workers indicated that carcinoma rarely originates in the right middle lobe; however, 22% of patients reviewed had malignant tumors as a cause of the syndrome. The original view that bronchial compression was the pathophysiological abnormality leading to development of the syndrome has been rejected by more recent authors. The focus has now turned to the relative isolation of the middle lobe, especially when a complete minor fissure is present. This isolation prevents the aerating effects of collateral ventilation of the upper lobe from reaching the middle lobe and thus impairs the clearing of secretions from the middle lobe bronchus. Bronchoscopy and bronchography are vital in the rational approach to this syndrome. Severe stenosis of the bronchus or tumor can be seen endoscopically in about 40% of patients, and bronchography will demonstrate an anatomical abnormality more than 70% of the time. Both the surgical and the medical approaches to therapy have been endorsed strongly by various authors in the 30 years since the syndrome was described. It now appears that bronchoscopy and, if need be, bronchography should be undertaken to rule out an endobronchial lesion. Timing of these studies will depend on the patient's age, with early examination advocated for the older patient at high risk for lung cancer. If there is reasonable evidence that the process is benign, medical management should be attempted. Lobectomy is performed if malignancy is suspected or if medical therapy fails.

Middle lobe syndrome: diagnosis and management.

Ninety-eight patients with middle lobe syndrome were reviewed. There were 31 male and 67 female patients ranging in age from 4 to 80 years. The most common presenting symptoms were cough, dyspnea, fever, hemoptysis, and chest pain. Diagnostic studies included chest roentgenogram, bronchoscopy, and bronchography. Sixty-five patients had full recovery following intensive medical therapy. Thirty-one patients underwent surgical resection for persistent disease. There were five post-operative complications and 1 death in this series.

Isolated middle lobe atelectasis: aetiology, pathogenesis, and treatment of the so-called middle lobe syndrome.

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.