Middle Lobe Syndrome

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]])
    • Physiology: xxx
  • 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]])
    • Epidemiology: common
  • 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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  • Chronic middle lobe infection. Factors responsible for its development. Ann Thorac Surg 1966;2:612–616 [MEDLINE]
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  • 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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  • Mycobacterium avium complex pulmonary disease presenting as an isolated lingular or middle lobe pattern. The Lady Windermere syndrome. Chest. 1992 Jun;101(6):1605-9 [MEDLINE]
  • Right middle lobe syndrome caused by Mycobacterium fortuitum in a patient with human immunodeficiency virus infection. South Med J 1992; 85:767–769 [MEDLINE]
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