General
- AKA hydatid (cyst) disease
Microbiology
- E. granulosa
- General
- Endemic areas (Uruguay, Australia, New Zealand…).
- The tapeworms are 2 to 5 mm in length
- Contain only three to four proglottids (Eggs).
- Each cyst contains multiple larvae (hydatid sand), and rupture can lead to further cyst formation.
- Primary definitive host (adult worm)
- Dog (A dog tapeworm)
- The intermediate host (larval stage)
- Sheep
- Man
- Life cycle
- Ova (proglottids) are excreted in dog faeces
- Contaminate herbage eaten by sheep.
- Embryos (Oncosphere) hatch and the parasite burrows through the duodenal wall to gain hematogenous access to multiple organs
- Cysts form in the
- Liver (65%)
- Lung (20%)
- Brain (2%)
- Dogs eat these infested organs and the parasite enters the intestine where it remains.
- Man is infected either by
- Eating food contaminated with ova, or
- Direct contact with infected dogs.
- The larva develops into a large cystic lesion
- Containing
- External laminar membrane,
- Germinal layer (the brood capsule),
- Central fluid layer.
- Within the cyst fluid are numerous protoscolexes, which form from the brood capsule (refer to figure).
- Hydatid cyst in lung showing the laminate and proliferative layers. Daughter cysts contain scolices; many of which have degenerated as evidenced by the presence of free hooks. (From the collection of Herman Zaiman, “A Pictorial Presentation of Parasites.”)
- When ingested by the definitive host, the protoscolexes develop into tapeworms.
- If the cyst ruptures, however, the protoscolexes can develop into additional hydatid cysts.
CNS involvement
- Occurs in only ≈ 3%.
- Produces cerebral cysts that are confined to the white matter.
- Cyst
- Types
- Primary cysts
- Solitary,
- Secondary cysts
- Multiple
- Embolization from cardiac cysts that rupture or from iatrogenic rupture of cerebral cysts
- Enlarges slowly (rates of ≈ 1cm per year are quoted, but this is variable and may be higher in children),
- Cyst usually does not present until quite large with findings of
- Increased ICP
- Seizures
- Focal deficit
Evaluation
Bloods
- Patients often have eosinophilia and may have positive serologic tests for hydatid disease.
- Enzyme-linked immunosorbent assay (ELISA) or indirect hemagglutination assays are readily available and can be confirmed by immunoblot assays.
Radiological
- CT
- Density of the cyst is similar to CSF,
- It does not enhance (although rim enhancement may occur if there is an inflammatory reaction),
- Little surrounding edema.
- It contains germinating parasitic particles called “hydatid sand” containing ≈ 400,000 scoleces/ml.
- MRI
- A major cyst with multiple compartments in which smaller cysts
Treatment
Medical
- Albendazole (Zentel®) 400mg PO BID (pediatric dose: 15mg/kg/d) × 28 days, taken with a fatty meal, repeated as necessary.
Surgery
- Removal of the intact cyst.
- Every effort must be made to avoid rupturing these cysts during removal, or else the protoscolexes may contaminate the adjacent tissues with possible recurrence of multiple cysts or allergic reaction.
- The Dowling technique is recommended60:
- The head is positioned so that the cyst points straight up towards the ceiling when the OR table is 30° head up
- Drilling burr holes and performing craniotomy must be done very carefully to avoid rupturing the cyst or tearing the dura, which is thin and under tension
- Do not coagulate with anything but low-power bipolar (to avoid cyst rupture)
- Open the dura circumferentially away from the dome of the cyst as it may be adherent to the dura
- Keep the surface of the cyst moist to prevent desiccation and rupture
- Open the thinned overlying cortex gently, separating it from the cyst with irrigation and cottonoids.
- The cortical opening need only be ≈ 3/4 the cyst diameter but no less
- Insert a soft rubber catheter between the cyst and the brain, and gently irrigate with saline as the head of the OR table is slowly lowered 45° while the surgeon supports the adjacent cortex with his/her fingers
- Continue irrigating more saline and float the cyst out and into a saline filled receptacle
- If the cyst is ruptured during the procedure,
- Immediately place a sucker in the cyst to aspirate the contents, remove the capsule, and wash the cavity with saline for 5minutes.
- Change instruments and gloves.
- Placing 10% formalin soaked cottonoids on the cavity for a few minutes is controversial61
- Drapes and potentially contaminated surfaces should be covered with hypertonic saline.