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Pulmonary Hydatid Disease - Radiology
Source: Grainger & Allison's Diagnostic Radiology
Aetiology & Background
Hydatid disease (echinococcosis) is caused by the larval forms of Echinococcus granulosus (most common - unilocular cystic echinococcosis), E. multilocularis, and E. vogeli. It is endemic in the Mediterranean, Eastern Europe, Africa, South America, the Middle East, Australia, and New Zealand. Humans are accidental hosts, acquiring infection by ingesting ova from fomites, contaminated water, or direct contact with dogs.
The lung is the second most commonly affected organ after the liver. The right lung and lower lobes are slightly more often involved.
Cyst Structure
A hydatid cyst has two layers:
- Pericyst (outer host-derived fibrous layer)
- Laminated (ectocyst) membrane with inner germinal (endocyst) layer
This layered structure underlies all the classical radiological signs.
Epidemiology
- Usually solitary
- Multiple and/or bilateral in 10% of cases
- At the time of presentation: two-thirds are ruptured, one-third unruptured
- Size varies from very small to very large
Radiological Features - Classified by Stage
Stage 1 - Intact / Unruptured Cyst
Plain CXR:
- Well-defined, rounded or oval homogeneous opacity - often with completely smooth margins
- The intact cyst appears as a simple mass indistinguishable from a solid tumour or benign mass on CXR
- No calcification (unlike hepatic hydatid which may calcify)
- No satellite lesions
- May cause adjacent compression of lung and diaphragm elevation with large cysts
CT:
- Well-defined, round or oval cystic lesion with a thin, smooth wall
- Water-density (0 HU) content - pure fluid inside the cyst
- The pericyst wall may be visible as a thin rim
- Internal daughter cysts may be seen with E. multilocularis (alveolar type - more complex, infiltrative)
Stage 2 - Early Communication (Pericyst Erosion / Impending Rupture)
When bronchioles erode the outer pericyst, allowing air to enter the space between the pericyst and the laminated membrane:
Plain CXR:
- "Meniscus sign" (also called the "crescent sign"): a fine radiolucent crescent of air appears at the upper pole of the cyst between the outer pericyst and the inner laminated membrane
- This is considered a sign of impending rupture
Figure 6.15 - Hydatid cysts of the lung: intact cyst (solid arrow), ruptured cyst (hollow arrow) with lamellar membrane floating like a water lily (solid arrowhead).
Stage 3 - Ruptured Cyst (Endocyst Rupture into Bronchus)
When both the pericyst and the inner endocyst (laminated membrane) rupture and communicate with the airway, multiple classical signs appear:
(A) Water-Lily Sign (Camalote Sign / Nymphaea Sign)
- The crumpled, collapsed endocyst floats on residual fluid at the bottom of the cyst cavity
- On CXR/CT it resembles a water lily (lotus leaf) or floating membrane above an air-fluid level
- Best demonstrated on CT where the floating membrane is clearly visible
- Pathognomonic of a collapsed (ruptured) pulmonary hydatid cyst
Figure 6.16 - CT showing the "water-lily sign" (arrow) in a ruptured pulmonary hydatid cyst - Bailey & Love's Short Practice of Surgery
(B) Air-Fluid Level
- A horizontal air-fluid level within the now-communicating cyst cavity
- Indicates bronchial communication has occurred and cyst contents are being partially expectorated
(C) Double Wall Sign
- Both the pericyst (outer) and the laminated membrane (inner) are visible as two distinct concentric rings on CT
- The space between them filled with air
(D) Rising Sun Sign / Serpent Sign
- When most fluid has been expectorated, the crumpled membranes lie at the bottom of the cavity
- Rising sun sign: the membrane appears along the lower arc of the cyst
- Serpent sign: the floating membrane has a wavy, snake-like configuration on CT
(E) Empty Cyst Sign
- After complete expectoration of all cyst contents, only an empty collapsed cavity remains
- This is the "empty cyst" or "ghost cyst" appearance - the wall may be visible as a thin ring
Stage 4 - Complications
1. Secondary Infection
- Secondary bacterial infection of a ruptured or partially evacuated hydatid cyst produces a lung abscess
- Appears as a thick-walled cavity with surrounding consolidation and air-fluid level, indistinguishable from a primary pyogenic abscess without clinical context
2. Pleural Rupture
- Rupture into the pleural space causes:
- Pleural effusion (hydatid fluid + daughter cysts in pleural space)
- If there is also airway communication: hydropneumothorax
- Risk of anaphylaxis from spillage of hydatid fluid
3. Massive Haemoptysis / Haemorrhage
- Aggressive invasion of bronchial or pulmonary arteries can result in massive haemoptysis
Classic Radiological Signs - Summary Table
| Sign | Mechanism | Modality | Significance |
|---|
| Rounded opacity | Intact cyst | CXR/CT | Unruptured; may mimic tumour |
| Meniscus / Crescent sign | Air between pericyst and laminated membrane | CXR | Impending rupture |
| Air-fluid level | Bronchial communication, partial expectoration | CXR/CT | Ruptured cyst |
| Water-lily sign (Camalote sign) | Collapsed endocyst floating on fluid | CXR/CT | Pathognomonic of ruptured cyst |
| Double wall sign | Air separating pericyst from endocyst | CT | Rupture into pericyst space |
| Serpent / Rising sun sign | Membranes lying at cyst base after fluid expectorated | CT | Largely evacuated ruptured cyst |
| Empty cyst sign | Complete expectoration of contents | CXR/CT | All contents expectorated |
Imaging Modality Comparison
| Modality | Role |
|---|
| CXR | Initial detection; shows rounded opacity, crescent sign, air-fluid level |
| CT | Modality of choice - best defines the floating membrane, double wall, confirms water-lily sign, demonstrates pleural involvement and secondary complications |
| MRI | Rarely used; may help characterise complex cysts or when CT is inconclusive |
| Ultrasound | Not applicable to lung parenchyma; useful for associated hepatic hydatid |
Source: Grainger & Allison's Diagnostic Radiology - A Textbook of Medical Imaging, Chapter 5 (Echinococcosis / Hydatid Disease), p. 139-140