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.

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.

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.

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: 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.

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.

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.

[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.

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.

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.

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.

Atelectasis in childhood.

The clinician who identifies atelectasis in a child must determine the mechanism by which atelectasis developed, the functional significance of the collapsed lung, and the etiology responsible for its presence. This article provides a framework to help physicians address these issues and thereby elect the most rational diagnostic and therapeutic interventions at the most appropriate time.

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]. / 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.

[Fibrobronchoscopy in the diagnosis of middle lobe syndrome].

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.

[Middle lobe syndrome]. / Síndrome del lóbulo medio.

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.

[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.