Epidemiology
History
- In 1948, Graham Described “Middle Lobe Syndrome” in a Patient with Right Middle Lobe Atelectasis Associated with Nontuberculous Mycobacterial Infection (Postgrad Med, 1948) [MEDLINE]
- In 1955, Effler and Ervin Published a Review on Anatomic and Clinical Features of the Middle Lobe Syndrome (Am Rev Tuberc, 1955) [MEDLINE]
Definition
- Middle Lobe Syndrome is Generally Defined as Recurrent or Chronic Atelectasis or Infection of the Right Middle Lobe of the Lung
- An Analogous Lingula Syndrome Has Also Been Described for the Lingula of the Left Lung (Chest, 2004) [MEDLINE]
Demographics
- Incidence
- National Study of Middle Lobe Syndrome Requiring Surgical Resection from Iceland (Clin Respir J, 2009) [MEDLINE]
- Male: 1.43 milion males per year
- Female: 2.94 milion females per year
- Age
- Middle Lobe Syndrome Can Occur at Any Age
- Familial Clustering
- Familial Clustering Has Been Reported
Etiology
Obstructive
- General Comments
- Characterized by Intrinsic Obstruction or Extrinsic Compression of Right Middle Lobe Bronchus by Bronchoscopy
- Endobronchial Infection
- Types
- Actinomycosis (see Actinomycosis, [[Actinomycosis]])
- Mycobacterium Avium Complex (MAC) (see Mycobacterium Avium Complex, [[Mycobacterium Avium Complex]])
- Most Cases of Middle Lobe Syndrome Associated with Nontuberculous Mycobacteria Have a Patent Right Middle Lobe Bronchus (Dis Chest, 1966) [MEDLINE]
- Endobronchial Submucosal “Pearls” May Be Seen in HIV Patients (Biopsy of These is Usually Positive for AFB)
- Mycobacterium Tuberculosis (see Tuberculosis, [[Tuberculosis]])
- Nocardiosis (see Nocardiosis, [[Nocardiosis]])
- Endobronchial Benign Tumor
- Endobronchial Malignancy
- Endobronchial Mass/Nodule/Anatomic Distortion
- Types
- Amyloidosis (see Amyloidosis, [[Amyloidosis]]): due to endobronchial amyloidosis
- Aspirated Foreign Body (see Airway Foreign Body, [[Airway Foreign Body]]): more commonly occurs in children
- Broncholithiasis (see Broncholithiasis, [[Broncholithiasis]]): due to erosion of broncholith from adjacent lymph nodes into the right middle lobe bronchus
- Cardiovascular Anomaly
- Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]]): due to endobronchial granuloma
- Silicosis (see Silicosis, [[Silicosis]]): due to endobronchial silicosis
- Situs Inversus (see Situs Inversus, [[Situs Inversus]])
- Tracheobronchopathia Osteochondroplastica (see Tracheobronchopathia Osteochondroplastica, [[Tracheobronchopathia Osteochondroplastica]]): due to xxxx
- Esophageal Traction Diverticula (see xxxx, [[xxxx]])
- Inspissated Secretions Obstructing the Right Middle Lobe Bronchus
- Peribronchial Lymphadenopathy Extrinsically Compressing the Right Middle Lobe Bronchus (see Mediastinal Mass, [[Mediastinal Mass]])
- General Comments: peribronchial lymphadenopathy is the most common etiology of extrinsic compression of the right middle lobe bronchus
- Infection
- Neoplasm
- Metastasis to Peribronchial Lymph Nodes
- Other
- Anthrasilicosis (see xxxx, [[xxxx]])
- Asbestos (see Asbestos, [[Asbestos]])
- Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]])
- Physiology: extrinsic compression of right middle lobe bronchus
Non-Obstructive (aka “Peripheral Middle Lobe Syndrome”)
- General Comments
- Characterized by Absence of Demonstrable Right Middle Lobe Bronchus Obstruction by Bronchoscopy
- Non-Obstructive Cases May Also Manifest Pathology in the Lingula on the Left Side (“Lingula Syndrome”) (Chest, 2004) [MEDLINE]
- Commonly Manifests as Recurrent Pneumonia in Association with Asthma, Bronchitis, or Cystic Fibrosis ( JAMA, 1966) [MEDLINE] (J Maine Med Assoc, 1972) [MEDLINE] (Arch Dis Child, 1992) [MEDLINE]
- Bronchogenic Cyst (see Bronchogenic Cyst, [[Bronchogenic Cyst]])
- Idiopathic Middle Lobe Syndrome
- Physiology: unclear pathogenesis
- Infection/Inflammation of Right Middle Lobe
- Types
- Actinomycosis (see Actinomycosis, [[Actinomycosis]])
- Aspergillus (see Aspergillus, [[Aspergillus]])
- Blastomycosis (see Blastomycosis, [[Blastomycosis]])
- Bordetella Pertussis (see Bordetella Pertussis, [[Bordetella Pertussis]])
- Chlamydophila Psittaci (Psittacosis) (see Psittacosis, [[Psittacosis]])
- Echinococcosis (see Echinococcosis, [[Echinococcosis]])
- Haemophilus Influenza (see Haemophilus Influenza, [[Haemophilus Influenza]])
- Histoplasmosis (see Histoplasmosis, [[Histoplasmosis]])
- Moraxella Catarrhalis (see Moraxella Catarrhalis, [[Moraxella Catarrhalis]])
- Mycobacterium Avium Complex (MAC) (see Mycobacterium Avium Complex, [[Mycobacterium Avium Complex]])
- Most Cases of Middle Lobe Syndrome Associated with Nontuberculous Mycobacteria Have a Patent Right Middle Lobe Bronchus (Dis Chest, 1966) [MEDLINE]
- in 1992, the “Lady Windermere Syndrome” (from the Fastidious Female Character in the Victorian-Era Play, Lady Windermere’s Fan) was First Described in a Group of Female Patients with MAC Infection Initially in Middle Lobe or Lingular Distributions (in the Absence of Airway Obstruction or Predisposing Pulmonary Disease) (Chest, 1992) [MEDLINE]
- Mycobacterium Fortuitum (see Mycobacterium Fortuitum, [[Mycobacterium Fortuitum]])
- Most Cases of Middle Lobe Syndrome Associated with Nontuberculous Mycobacteria Have a Patent Right Middle Lobe Bronchus (Dis Chest, 1966) [MEDLINE]
- Mycobacterium Tuberculosis (see Tuberculosis, [[Tuberculosis]])
- Nocardiosis (see Nocardiosis, [[Nocardiosis]])
- Staphylococcus Aureus (see Staphylococcus Aureus, [[Staphylococcus Aureus]])
- Streptococcus Pneumoniae (Pneumococcus) (see Streptococcus Pneumoniae, [[Streptococcus Pneumoniae]])
- Primary Ciliary Dyskinesia (see Primary Ciliary Dyskinesia, [[Primary Ciliary Dyskinesia]])
- Pulmonary Infarction (see Pulmonary Infarction, [[Pulmonary Infarction]])
Physiology
Factors Predisposing to Right Middle Lobe Syndrome
- Right Middle Has Relatively Poor Collateral Ventilation (as Compared to the Upper Lobes)
- Right Middle Lobe is Anatomically Surrounded by Two Fissures
- Right Middle Lobe Has a Greater Ratio of Pleural Surface to Nonpleural Surface, as Compared to the Upper Lobes
- Study of Middle Lobe Collateral Ventilation (Am Rev Respir Dis, 1978) [MEDLINE]
- Collateral Ventilation in the Right Middle Lobe of Young Normal Subjects is Characterized by High Resistance and a Long-Time Constant, Relative to the Upper Lobes
- Right Middle Lobe Bronchus is Encircled by a Ring of Lymph Nodes
- Encircling Lymph Nodes Can Become Enlarged by Various Processes (Tuberculosis. etc)
- Right Middle Lobe Bronchus is Long, Narrow, and Has an Acute Takeoff Angle
- Right Middle Lobe Bronchus May Have a “Fish-Mouthed” Appearance on Bronchoscopy
Diagnosis
Bronchoscopy (see Bronchoscopy, [[Bronchoscopy]])
- Required to Rule Out Endobronchial Pathology and Collect Specimens for Microbiologic Processing
- Bronchoscopy is Abnormal in Approximately 40% of Cases
- The Most Common Abnormalities Found are Right Middle Lobe Bronchial Stenosis or an Endobronchial Tumor
- Endobronchial Ultrasound (EBUS): helpful to detect lymphadenopathy and calcification
Chest X-Ray/Chest CT (see Chest X-Ray, [[Chest X-Ray]] and Chest Computed Tomography, [[Chest Computed Tomography]])
- Findings
- Normal: chest imaging may be normal in cases with intermittent or recurrent atelectasis
- Right Middle Lobe Infiltrate
- Right Middle Lobe Atelectasis
- CXR-Lateral: Anterior Wedge-Shaped Density with Base Toward Pleura and Apex Pointing Toward the Hilum
- Right Middle Lobe Bronchiectasis
Clinical
General Comments
- Asymptomatic: some cases
- Asymptomatic Cases May Be Incidentally Detected on Chest Imaging
Pulmonary Manifestations
- Bronchiectasis (see Bronchiectasis, [[Bronchiectasis]])
- Physiology: due to poor secretion clearance and chronic/recurrent infection
- Cough (see Cough, [[Cough]])
- Epidemiology: common
- Clinical
- Cough May Be Chronic or Recurrent
- Decreased Breath Sounds Over the Right Middle Lobe
- Clinical: may be auscultated over the right middle lobe
- Hemoptysis (see Hemoptysis, [[Hemoptysis]])
- Epidemiology: occurs in cases with complicating infection
- History of Atopy, Asthma, or Chronic Obstructive Pulmonary Disease (COPD)
- Epidemiology: present in up to 50% of cases (Thorax, 1980) [MEDLINE] (Clin Respir J, 2009) [MEDLINE]
- Recurrent Pneumonia (see Pneumonia, [[Pneumonia]])
- Vocal Fremitus (E->A Changes) Over the Right Middle Lobe
- Clinical: may be auscultated over the right middle lobe
Other Manifestations
- Fatigue (see Fatigue, [[Fatigue]])
- Epidemiology: occurs in cases with complicating infection
- Fever (see Fever, [[Fever]])
- Epidemiology: occurs in cases with complicating infection
- Weight Loss (see Weight Loss, [[Weight Loss]])
- Epidemiology: occurs in cases with complicating infection
Treatment
- Avoidance of Airway Irritants
- Tobacco Cessation (see Tobacco, [[Tobacco]])
- Asthma Therapy: indicated for cases associated with associated asthma
- Bronchodilators
- Inhaled Corticosteroids (see Corticosteroids, [[Corticosteroids]])
- Mucolytics
- Mechanical Secretion Clearance Modalities: not well-studied in the treatment of middle lobe syndrome
- Antibiotics
- Antibiotics Should Be Targeted Toward Organisms Recovered by Bronchoscopic Sampling
- Use of Nebulized Antibiotics Has Not Been Well Studied in Middle Lobe Syndrome
- Low-Dose Roxithromycin Therapy (Respiration, 2001) [MEDLINE]: may be useful, especially if bronchiectasis is also present
- Bronchoscopy (see Bronchoscopy, [[Bronchoscopy]])
- Secretion Clearance
- Intrabronchial Air Insufflation: to treat lobar collapse
- Balloon Dilation/Argon Plasma Coagulation/Electrocautery/Cryosurgery/Laser Therapy/Stent Placement: may be used in some cases
- Surgery
- Indications
- Resistant/Complex Cases (Which are Unresponsive to Medical Therapy) and Who Have Proven Right Middle Lobe Bronchial Obstruction (Clin Respir J, 2009) [MEDLINE]
- Resistant/Complex Cases with Chronic Atelectasis (>6 Months) Despite Medical Therapy: especially if patient has associated debilitating symptoms (such as persistent cough, fever, failure to thrive, etc)
- Hemoptysis Not Amenable to Interventional Radiology Angioembolization
- Concern Regarding the Presence of Underlying Malignancy
References
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- The middle lobe syndrome; a review of the anatomic and clinical features. Am Rev Tuberc 1955;71:775–784 [MEDLINE]
- Chronic middle lobe infection. Factors responsible for its development. Ann Thorac Surg 1966;2:612–616 [MEDLINE]
- Right middle lobe syndrome in children. JAMA 1966;197:8–14 [MEDLINE]
- The right middle lobe syndrome, a non-obstructive complex. Dis Chest 1966;50:57–66 [MEDLINE]
- Middle lobe disease. South Med J 1967;60:1029–1032 [MEDLINE]
- Middle lobe syndrome in asthmatic children. J Maine Med Assoc 1972;63:46–48 [MEDLINE]
- Collateral ventilation and the middle lobe syndrome. Am Rev Respir Dis 1978;118: 305–310 [MEDLINE]
- Isolated middle lobe atelectasis: aetiology, pathogenesis, and treatment of the so-called middle lobe syndrome. Thorax 1980;35:449–452 [MEDLINE]
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