Book contents
- Frontmatter
- Contents
- List of abbreviations
- Part I Fundamentals of CXR interpretation – ‘the basics’
- Part II A–Z Chest Radiology
- Abscess
- Achalasia
- Alveolar microlithiasis
- Aneurysm of the pulmonary artery
- Aortic arch aneurysm
- Aortic rupture
- Asbestos plaques
- Asthma
- Bochdalek hernia
- Bronchiectasis
- Bronchocele
- Calcified granulomata
- Carcinoma
- Cardiac aneurysm
- Chronic obstructive pulmonary disease
- Coarctation of the aorta
- Collapsed lung
- Consolidated lung
- Diaphragmatic hernia – acquired
- Diaphragmatic hernia – congenital
- Embolic disease
- Emphysematous bulla
- Extrinsic allergic alveolitis
- Flail chest
- Foregut duplication cyst
- Foreign body – inhaled
- Foreign body – swallowed
- Goitre
- Haemothorax
- Heart failure
- Hiatus hernia
- Idiopathic pulmonary fibrosis
- Incorrectly sited central venous line
- Kartagener syndrome
- Lymphangioleiomyomatosis
- Lymphoma
- Macleod's syndrome
- Mastectomy
- Mesothelioma
- Metastases
- Neuroenteric cyst
- Neurofibromatosis
- Pancoast tumour
- Pectus excavatum
- Pericardial cyst
- Pleural effusion
- Pleural mass
- Pneumoconiosis
- Pneumoperitoneum
- Pneumothorax
- Poland's syndrome
- Post lobectomy/post pneumonectomy
- Progressive massive fibrosis
- Pulmonary arterial hypertension
- Pulmonary arteriovenous malformation
- Sarcoidosis
- Silicosis
- Subphrenic abscess
- Thoracoplasty
- Thymus – malignant thymoma
- Thymus – normal
- Tuberculosis
- Varicella pneumonia
- Wegener's granulomatosis
Lymphangioleiomyomatosis
Published online by Cambridge University Press: 25 February 2010
- Frontmatter
- Contents
- List of abbreviations
- Part I Fundamentals of CXR interpretation – ‘the basics’
- Part II A–Z Chest Radiology
- Abscess
- Achalasia
- Alveolar microlithiasis
- Aneurysm of the pulmonary artery
- Aortic arch aneurysm
- Aortic rupture
- Asbestos plaques
- Asthma
- Bochdalek hernia
- Bronchiectasis
- Bronchocele
- Calcified granulomata
- Carcinoma
- Cardiac aneurysm
- Chronic obstructive pulmonary disease
- Coarctation of the aorta
- Collapsed lung
- Consolidated lung
- Diaphragmatic hernia – acquired
- Diaphragmatic hernia – congenital
- Embolic disease
- Emphysematous bulla
- Extrinsic allergic alveolitis
- Flail chest
- Foregut duplication cyst
- Foreign body – inhaled
- Foreign body – swallowed
- Goitre
- Haemothorax
- Heart failure
- Hiatus hernia
- Idiopathic pulmonary fibrosis
- Incorrectly sited central venous line
- Kartagener syndrome
- Lymphangioleiomyomatosis
- Lymphoma
- Macleod's syndrome
- Mastectomy
- Mesothelioma
- Metastases
- Neuroenteric cyst
- Neurofibromatosis
- Pancoast tumour
- Pectus excavatum
- Pericardial cyst
- Pleural effusion
- Pleural mass
- Pneumoconiosis
- Pneumoperitoneum
- Pneumothorax
- Poland's syndrome
- Post lobectomy/post pneumonectomy
- Progressive massive fibrosis
- Pulmonary arterial hypertension
- Pulmonary arteriovenous malformation
- Sarcoidosis
- Silicosis
- Subphrenic abscess
- Thoracoplasty
- Thymus – malignant thymoma
- Thymus – normal
- Tuberculosis
- Varicella pneumonia
- Wegener's granulomatosis
Summary
Characteristics
This is a rare disease of unknown cause characterised by the presence of abnormal smooth muscle proliferation of the pulmonary interstitium, particularly in the bronchioles, pulmonary vessels and lymphatics.
Exclusively affects women of a child-bearing age.
Associated with chylous ascites, fatty liver and renal angiomyolipomas.
Clinical features
Progressive exertional dyspnoea.
Haemoptysis.
Restrictive lung function and hypoxia.
Increase in symptoms during pregnancy and on the oral contraceptive pill.
Radiological features
CXR – coarse reticular interstitial pattern with cyst formation on the background of large volume lungs. There is a common association with chylous pleural effusions (70%), pneumothoraces (40%) and mediastinal lymphadenopathy.
HRCT – numerous random thin-walled cysts of varying size and relatively regular shape. Bronchovascular bundles at edge of cyst. Lymphadenopathy, pleural effusions and pneumothoraces. Dilated thoracic duct.
Long-standing cases may develop pulmonary arterial hypertension with enlargement of the central pulmonary arteries.
Differential diagnosis
Langerhan's cell histiocytosis – male smokers, with upper zone predisposition and more cyst irregularity.
Tuberous sclerosis – rare. Other characteristic abnormalities present.
Emphysema – smokers. No wall to the emphysematous bullae.
Neurofibromatosis – rare with musculoskeletal and neurogenic abnormalities.
Lung fibrosis (e.g. UIP). Basal fibrosis and honeycombing.
Management
Lung biopsy may be required to confirm the diagnosis.
No specific treatment.
10-year survival is 75%.
Progression to pulmonary insufficiency and arterial hypertension.
- Type
- Chapter
- Information
- A-Z of Chest Radiology , pp. 126 - 127Publisher: Cambridge University PressPrint publication year: 2007