This document describes the case of a 13-year-old boy who presented with a one month history of progressive headaches, left-sided weakness, and mild bilateral papilledema. Imaging revealed a cystic lesion in the right parieto-occipital region. The patient underwent a neurosurgical procedure to remove the intact cyst, which was confirmed to be an isolated intracranial hydatid cyst based on pathology. Hydatid cysts are caused by the tapeworm Echinococcus and most commonly involve the liver and lungs. Intracranial involvement is rare but can cause signs of increased intracranial pressure like this case.

1. Isolated intracranial Hydatid
Cyst – Multidisplinary Approach
sa•Ahmed Altibi
•Heba Daradkeh
•Ibrahim Nour

2. Case Presentation
saJordan University Hospital
Department of Neurosurgery

3. History
A 13 year old male patient, previously healthy, living in Madaba.
Presented to our pediatrics neurology clinic on the 22nd
of October, 2014.
Complaining of
HEADACHE OF ONE MONTH DURATION

4. History
The headache was :
- Gradual in onset.
- Initially right-sided then later became diffuse.
- Dull in nature.
He had that headache almost daily throughout the month and
it was more severe upon waking up in the morning.

5. History
 It was partially relieved with simple analgesia (Paracetamol
and Iboprufen) but did not have specific exacerbating factors.
 The severity of the headache was progressively increasing,
reaching its peak a couple of days prior to his presentation.

6. History
 The headache was sometimes associated with:
-Nausea without vomiting
-Blurring of vision
-weakness in his left upper and lower limbs, associated with numbness in the
upper limb, he described difficulty carrying objects with his left upper limb. Those
developed only a couple of days before his presentation.

7. History
NO history of :
-Fever
-Seizures
-Loss of consciousness
-trauma
-significant weight loss, poor appetite, or night sweats.

8. History
Past medical history: free
Past surgical history: free
No significant relevant family history
Social history:
He is the youngest in 6 brothers, lives in a rural area.

9. Physical Examination
General :
-In a good general condition
-Fully awake and oriented to place, time & person.
-He did look in pain !

10. Physical Examination
Neurological examination
-No meningeal signs
-Examination of the cranial nerves was normal
-The visual filed by confrontation test was normal
-Fundoscopic examination at the clinic showed
MILD BILATERAL PAPILLEDEMA

11. Physical Examination
Neurological examination ctd.
- He had an obvious left-sided PRONATOR DRIFT
- WEAKNESS in the left upper and lower limbs (4/5)
- SENSORY ATAXIA with a clear Positive Romberg’s Sign
- Deep tendon reflexes were normal
- Babinski & Hoffman’s signs were absent

12. Summary
a13 year old boy who presented with signs and symptoms of increased ICP :
progressive headache, postive pronator drift and mild bilateral papillaedema and
left sided weakness

14. Labs: CBC, Electrolytes
 Electrolytes were normal
 CBC:
 Hb :12.8
 WBC: 7.1 x 109
 Neutrophils: 35%
 lymphocytes: 40%
 Eosinophils : 11%
 Basophils: 1.92%
 Monocytes: 5.68%

15. CT

16. MRI T1
without contrast

17. MRI T2

18. MRI with
contrast

19. Diffusion
Weighted
Image

20. Quick Summary
 So this is an Intra axial supratentorial parieto-occipital cystic lesion with focal
area of enhancement within its wall, no restricted diffusion, and with some
vasogenic edema.

21. List of differentials

22. Is it a Pilocytic astrocytoma?

23. Is it an abscess?
 1. Not an abscess because the DWI was free from any restricted diffusion.

24. These are the images of the child’s liver and lungs:

25. Neurosurgery
We were consulted by pediatrics team
Physical findings were consistent with their findings
Ordered imaging were reviewed

26. Neurosurgery

27. Preparation for surgery
 Patients was started on Mebendizole
3 days before the operation.
Hypertonic saline was prepared to
continuously irrigate and possibly
inject the cyst if needed.

29. Anesthesia - Preoperative
 ASA score: I
 Malampati class 1
 GA
 ASA Standard Monitoring:
 Oxygenation: Pulse Oximetery
 Ventilation
 Circulation: ECG & BP
 Temperature

30. Anesthesia
 Precaution
 Canulae (Gauge 16)
 Central line and arterial line
 Adrenaline 50 mic
 Hydrocortisone
 Antihistamine

31. Imaging:

32. Imaging:

36. After surgery
 Transfer to ICU for observation.
 No postoperative complications.
 Complete resolution of signs
and symptoms
 No Ataxia
 No Pronator drift
 No Weakness

38. Post OP CT

39. Post OP MRI with CM T2

40. POST OP MRI with CM

41.  I recommend an MRI for follow up, especially that this is a child and I would want
to decrease the amount of radiation he would be exposed to, also an MRI is
superior in providing better characterization of the soft tissue.

42. Pathology

43. Macroscopically

44. Microscopically

47. Hydatid Disease
Overview

48. Definition
“Hydatid Disease Is a parasitic infection by the tapeworm echinococcus.
Four species of Echinococcus produce infection in humans;
1. E. granulosus causing cystic echinococcosis (CE)
2.E. multilocularis causing alveolar echinococcosis (AE),
3. E. vogeli causing polycystic echinococcosis
4.E. oligarthrus being an extremely rare cause of human echinococcosis.
1&2 being the most common”. 8

49. Reproduced from: Centers for Disease Control and Prevention. DPDx: Echinococcosis. Available at:http://www.cdc.gov/dpdx/echinococcosis/index.html.

50. Notes on Lifecycle
 Definitive host  dogs or related species
 Intermediate host  sheep, goats, camels, horses, cattle, and swine
 Humans are incidental hosts; they do not play a role in the transmission cycle. 8

51.  Eggs are highly resistant and can remain infective for a year in a moist
environment at low temperature. 8
 Infectious eggs re passed to humans via fecal-oral transmission. This may occur
via environmental contamination of water and cultivated vegetables or contact
between infected domestic dogs and humans (often in children). 8

52.  The same life cycle occurs with E. multilocularis (1.2 to 3.7 mm), with the
following differences: the definitive hosts are foxes, and to a lesser extent dogs,
cats, coyotes, and wolves; the intermediate host are small rodents; and larval
growth (in the liver) remains indefinitely in the proliferative stage, resulting in
invasion of the surrounding tissues. 8

53. Epidemiology
 Cystic hydatidosis is a significant public health problem in South America, the
Middle East and eastern Mediterranean, some sub-Saharan African countries,
western China, and the former Soviet Union. 8
 Echinococcosis is one of the major zoonotic parasitic diseases in the Middle East
and Arabic North Africa from Morocco to Egypt.
 It is hyper endemic in Iran, Turkey, Iraq, Jordan, Morocco, Libya, Tunisia, and
Algeria, and endemic in Egypt. 10
 Both cystic and alveolar echinococcosis has been reported from these areas.
However, cystic echinococcosis is more prevalent. 10

54. Clinical Presentation
 Initially asymptomatic and may remain so for many years.
 The clinical presentation depends upon the site of the cysts and their size.
 Hydatid cysts may be found in almost any site of the body, either from primary
inoculation or via secondary spread.
 the liver (63%), lungs (25%), muscles (5%), bones (3%), kidneys (2%), brain (1%),
and spleen (1%). 9

55. Cerebral Hydatid disease (Neurohydatidosis)
 Epidemiology
 Cerebral hydatid disease is a rare parasitic infestation and accounts for 1-2 % of
all cystic echinococcosis. 2
 “Intracranial involvement is more commonly seen in pediatric population, and
80% of patients in the recent series were children.” 7
 “This high incidence in children is probably related to patent ductus arteriosus.” 7

56. Clinical Presentation of Neurohydatidosis
Symptoms and signs include:
 focal neurological deficits
 headaches
 increased intracranial pressure
 hydrocephalus
 papilledema and loss of vision
 altered mental status
 seizures (rare)

57. Clinical Presentation, ctd
 Intracranial cysts are more frequently settled in the supratentorial compartment
in the vascular territory of middle cerebral artery 1
and the parietal lobe is the
most preferable site.
 The other less common sites reported are skull, cavernous sinus, eyeball, pons,
skull, extradural, cerebellum and ventricles.
 Intracranial hydatid cysts are commonly solitary. Multiple cysts are rare. 7

58. Types of intracranial hydatid cysts
 Primary hydatid cysts
 occur as direct invasion of larva that managed and filtered via liver and lung to the
brain
 usually solitary but may be multiple
 is fertile
 Secondary hydatid cysts
 occur as a result of rupture of primary cysts in others organs then reaching by
embolization to the brain
 usually multiple
 infertile
 do not have brood capsule or scolices

59. Treatment and prognosis of neurohydatidosis
 Management is surgical, with removal of the entire cyst without rupture using
Dowling’s maneuver (instilling warm saline between the cyst wall and the
brain) 5
.
 In some cases where it is felt that removing the cyst intact (without cyst rupture
during surgery) is unlikely to succeed, the cyst can be removed after puncture
and aspiration of its contents.
 Serology and histopathology of the excised cyst will confirm the diagnosis
of neurohydatidosis.
 Rupture of the cyst can result in recurrence in the subarachnoid space, both
intracranially and of the spine 6
.

60. References
 1. Arora SK, Aggarwal A, Datta V. Giant primary cerebral hydatid cyst: A rare cause of childhood seizure. J Pediatr Neurosci. 2014;9 (1): 73-5.
 2. Polat P, Kantarci M, Alper F et-al. Hydatid disease from head to toe. Radiographics. 2003;23 (2): 475-94.
 3. Senturk S, Oguz KK, Soylemezoglu F et-al. Cerebral alveolar echinoccosis mimicking primary brain tumor. AJNR Am J Neuroradiol. 2006;27 (2):
 4. Binesh F, Mehrabanian M, Navabii H. Primary brain hydatosis. BMJ Case Rep. 2011;2011 (mar05 1): .
 5. Ulutas M, Cinar K, Secer M. Removal of large hydatid cysts with balloon-assisted modification of Dowling's method: technical report. Acta Neurochir (Wien). 2015;157 (7): 1221-
4.
 6. Izci Y, Tüzün Y, Seçer HI et-al. Cerebral hydatid cysts: technique and pitfalls of surgical management. Neurosurg Focus. 2008;24 (6): E15.
 7. Basarslan, S. K., Gocmez, C., Kamasak, K., & Ceviz, A. (2015). The Gigant primary cerebral hydatid cyst with no marked manifestation: a case report and review of literature.
European Review for Medical and Pharmacological Sciences, 19(8), 1327–9. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/25967703

8. Pedro L Moro, MD, MPH (2016). Epidemiology and control of echinococcosis. In: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2016.
 9. Pedro L Moro, MD, MPH (2016). Clinical manifestations and diagnosis of echinococcosis. In: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2016.
 10. Sadjjadi, S. M. (2006). Present situation of echinococcosis in the Middle East and Arabic North Africa. Parasitology International, 55 Suppl, S197–202.