|Year : 2007 | Volume
| Issue : 2 | Page : 72-74
Giant intracranial hydatid cyst
A Trivedi, S Shukla, K Singh, V Sharma
Department of Neurosurgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005, India
Department of Neurosurgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Hydatid disease is caused by the infestation of the larvae of Taenia echinococcus. The definite hosts of echinococcus are various carnivores, the common being the dog. All mammals (more often being sheep and cattle) are hosts. Humans are infected through the feco-oral route by the ingestion of food or milk contaminated by dog feces that contain the ova of the parasite or by direct contact with dogs. The eggs lose their enveloping layer in the stomach, releasing the embryos. The embryos pass through the wall of the gut into the portal system and are carried to the liver where most of the larvae are entrapped and encysted. Some may reach the lungs, and occasionally, some may pass through the capillary filter of the liver and lungs and enter into the systemic circulation. We report a case of giant intracranial left temporoparietooccipital extra-axial hydatid cyst with a size of 12.5 x 8.5 cm in a 5-year-old male presented with holocranial headache with the diminution of vision and papilledema with right hemiperesis successfully managed in our department surgically.
Keywords: Echinococcus, extra-axial, hydatid cyst
|How to cite this article:|
Trivedi A, Shukla S, Singh K, Sharma V. Giant intracranial hydatid cyst. J Pediatr Neurosci 2007;2:72-4
| Case Report|| |
A right-handed 5-year-old male child presented with complaints of holocranial headache since 1½ months with recurrent vomiting. The patient was apparently asymptomatic 1½ months ago and he developed headache, predominantly on morning hours, which was associated with vomiting. Vomiting was projectile in nature, and it relieved the headache. The parents of the patient noticed that the child becomes dull and did not take interest in day-to-day activities such as playing and study. Fifteen days later, the patient complained of diminution of vision, which started from the left eye and subsequently from both the eyes. The patient also had a single episode of right focal convulsion in right upper limb and leg and subsequently generalized tonic-clonic in nature and become unconscious after fits. There was no history of previous fits, prolonged fever, trauma, weakness of limbs and right and left orientation and dressing sense. The patient belonged to a low socioeconomic status.
On examination, the patient was conscious, dull, recognizing relatives, but confused and disoriented to the time and place. Vitals were stable except that he was anemic . All the cranial nerves were normal except the optic nerve. Patient had counting finger on left side. On the right side, vision was 6/36. Pupil bilateral equal and reacting to light. Fundus examination revealed papilledema grade III on the left side and grade I on the right side. Motor examination revealed right hemiparesis with a power grade of 4/5 in all joints with hypertonia and exaggerated deep tendon reflexes and plantar extensor. Sensory system was intact. Other systems were normal.
Noncontrast CT scan revealed large, hypodense, cystic SOL without the surrounding edema and this involved the left temporoparietooccipital region with a midline shift of 1.92 cm. The intracystic density is of CSF. On contrast CT scan, the cyst wall was not enhanced [Figure - 1]. There was no evidence of recent bleeding inside the cyst. X-ray chest and USG abdomen was normal. Other relevant investigations were normal. Patient was taken up for standard left temporoparietooccipital osteoplastic flap craniotomy. The dura was tense; on opening the dura, there was large cyst, which was extra-axial and the cyst wall was transparent with clear plane between the arachnoid and the cyst wall. A 6-Fr infant feeding tube was passed under the cyst between the brain and the cyst wall, and normal saline was injected by a 10 cc syringe in infant feeding tube. Gradually, the whole cyst delivered from the cranium without rupture, as shown in intraoperative picture. The dura was closed and osteoplastic flap reposited and the wound closed in layers. Patient was administered with broad-spectrum antibiotics, anticonvulsants, steroids, and albendazole. The postoperative period was uneventful. At the time of discharge, the patient improved symptomatically and his vision also improved. The patient came with complaints of severe headache on left side with the osteoplastic flap raised after 2 months; the CT scan revealed subdural hypodense collection in the operated area with a midline shift of 5 mm. At once, subdural peritoneal shunt was performed. After that, the patient was doing well at the follow-up at 6 months and the vision improved markedly (6/36 on left side and 6/18 on right side without focal neurological deficit).
| Discussion|| |
Intracranial hydatid disease is rare, with a reported incidence of 1-2% in all the cases with hydatid disease.  Cerebral hydatid disease is more common in the pediatric population. , The history of direct contact with dogs is not available in all the cases as the infection can be acquired by eating contaminated food and milk.
Intracranial hydatid cysts are more frequently located in the supratentorial compartment. The parietal lobe is the most common site. All the four cases reported by Dharker et al .  and three out of five cases of intracerebral hydatid cysts as reported by Balasubramaniam et al .  had parietal lobe involvement. The other less common sites are skull,  cavernous sinus,  eye ball,  pons,  , skull,  extradural,  cerebellum and ventricles.  The cerebral hydatid cysts usually grow slowly and present clinical symptoms only when they increase in size and produce mass effect. The growth rate of the hydatid cysts of the brain and has been variably reported between 1.5-10 cm/year. , In our case, the size of the hydatid cyst was 12.5 × 8.5 cm (ovoid); the cyst wall was whitish and translucent.
Solitary intracranial hydatid cysts are common; multiple intracranial cysts are rare. Onal et al .  found only 3 cases of multiple cysts in their series of 33 cases and Lunardi et al .  found 2 cases in their series of 12 cases. Increased intracranial pressure and papilledema were the predominant findings in this group, whereas focal neurological deficits were most prevalent in adults. CT and MRI revealed round cystic lesions that were isodense and iso-intense, respectively, to the cerebrospinal fluid (CSF) with no rim enhancement or perifocal edema. Long-term follow-up confirms that intracranial hydatid cysts should always be surgically removed without rupture; the outcome remains excellent in these cases. Correct preoperative diagnosis is vital for the successful outcome of the surgery. A high index of suspicion is therefore required in endemic areas despite the availability of advanced neuroimaging techniques . Medical treatment with albendazole seems to be beneficial both pre- and postoperatively. Novel diagnostic methodologies such as MR spectroscopy and MR diffusion-weighted imaging might help in the diagnosis of intracranial hydatid cysts. 
The primary cysts are formed as a result of direct infestation of the larvae in the brain without the demonstrable involvement of other organs. In primary multiple cysts, each cyst has a separate pericyst with brood capsule scolices, and they originate from multiple larvae that affect brain after crossing the gastrointestinal tract, liver, lungs and right side of heart without affecting them. The primary cysts are fertile as they contain scolices and brood capsules; hence, the rupture of primary cyst can result in recurrence. The secondary multiple cysts results from spontaneous, traumatic or surgical rupture of the primary intracranial hydatid cyst and they lack brood capsule and scolices. The secondary intracranial hydatid cysts are therefore infertile and the resultant risk of recurrence after their rupture is negligible.
The patients with intracranial hydatid cysts usually present with focal neurological deficit and features of raised intracranial pressure; the latter may be due to the large size or due to interference with pathway of the CSF flow. Erashin et al .  observed that 18 out of 19 cases presented with raised intracranial pressure. Four cases had seizures. All the patients in the present series had focal neurological deficits and the features of raised intracranial pressure; two patients presented with seizures. MR and CT scans characteristically show hydatid cyst as a spherical, well-defined, nonenhancing cystic lesion without peripheral edema. , On CT and MR scan, the fluid density is found to be generally equal to that of CSF. A fine rim of peripheral enhancement with perilesional edema may be seen in the presence of active inflammation.  MR scan may show a low-density cyst wall  and relations with surrounding structures is better delineated than that in CT scan. In our case, the patient presented with headache with focal neurological deficit, and papilledema with the diminution of vision.
Various surgical techniques commonly used for removal of the cysts are summarized by Arana-Iniquez  and include direct puncture and aspiration of the cyst fluid through a small hole in the cyst wall, and expulsion of the cyst through a small cortical incision over cyst using insuffalation of air in the contralateral ventricle. The most popular technique, however, is to use irrigation saline with mild force between the cyst wall and brain interface in order to deliver the cyst intact . This is often possible because the adhesions around the cyst wall are minimal. We performed this procedure in our case, as shown in operative slides.
Only a few reports are available that mention the efficacy of this drug therapy. Isolated case reports , showed complete disappearance of the multiple intracranial hydatid cysts with albendazole therapy with a daily dose of 10 mg/kg, taken three times a day for 4 months. albendazole is a broad spectrum oral antihelminthic drug, which acts by blocking the glucose uptake of the larvae and the adult worm. The glycogen storage is depleted, thereby decreasing the ATP formation that results in the death of the parasite. Golematis et al .  analyzed 44 patients who were treated with albendazole and found that the large cysts decreased in the size, while the smaller ones disappeared. Erashin et al.  reported better effectiveness of the drug therapy in recurrent cases and in cases with rupture at the surgery. We have advised the administration of albendazole 10 mg/kg postoperatively for 1 month.
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[Figure - 1]
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