Primary multiple intraventricular hydatid cysts in a child.

Cystic hydatidosis caused by Echinococcus granulosus is a rare disease which mainly involves the liver and lungs. In 1%-2% cases, it may affect the brain. Primary multiple hydatid cysts of the brain are very rare. A 14-year-old girl presented with a history of headache and altered sensorium. Computed tomography scan and magnetic resonance imaging showed multicystic intraventricular lesions and patient was operated by neurosurgeon. Multiple cysts were removed. Antihelminthic medication was started, and patient was discharged with full recovery of neurological function. Hydatid cyst must be considered as a differential diagnosis of cystic space-occupying lesions of the brain. Surgery and antihelminthic drugs will help to get rid of infection.

INTRODUCTION

A hydatid cyst is the larval stage of a small tapeworm, Echinococcus granulosus. This is an emerging zoonotic parasitic disease throughout the world. Hydatid cysts are more prevalent in Australia, New Zealand, South America, Russia, France, China, India, and the Middle East countries.[12] Although hydatid cysts are known commonly to affect the liver and lung, it can also affect the brain in 1%–2% cases. Brain involvement is more commonly seen in children and usually has intraparenchymal localization.[3] Intracranial hydatid cysts are commonly solitary. Multiple intracranial cysts are rare. Intracranial hydatid cyst may be classified as primary or secondary. Primary cysts are formed as a result of direct infestation of larvae in brain without demonstrable involvement in other organs. The secondary multiple cysts result from spontaneous, traumatic, or surgical rupture of primary intracranial hydatid cyst, and they lack brood capsule and scolices. Complete surgical removal of primary hydatid cyst of the brain is often possible. However, complete surgical removal of multiple hydatid cysts of the brain is often very difficult.[45]

In this study, we report a 14-year-old female with primary multiple intraventricular hydatid cysts causing neurological manifestations.

CASE REPORT

A 14-year-old female child, first issue of a nonconsanguineous marriage, was brought with a history of headache for 10 days and multiple episodes of nonbilious projectile vomiting and altered sensorium on the day of admission. History of contact with pet animal was not there. Birth, development, and immunization history was normal. On examination, patient was a febrile and drowsy. Heart rate – 55/min, respiratory rate – 18/min on room air, blood pressure – 110/80 mmHg. Eye examination showed bilateral papilledema. Central nervous system examination showed drossy, Glasgow Coma Scale – E3, V5, M5 Pupils were bilaterally equal and reacting to light. Cranial nerves were normal. Tone was normal, power was 4/5 in all four limbs, reflexes – brisk, plantars-upgoing, no meningeal signs.

Child was admitted in Pediatric Intensive Care Unit with working diagnosis of meningoencephalitis or space-occupying lesion (SOL).

Neuroradiological examination

Computed tomography (CT) brain was suggestive of cystic lesion in left lateral ventricle causing descending transtentorial herniation and midline shift of 12 mm.

Magnetic resonance imaging (MRI) brain showed multicystic intraventricular nonenhancing lesions with trapped lateral ventricles suggesting intraventricular space occupying lesion. Ruptured intraventricular hydatid cyst. It also showed descending transtentorial herniation with compression of midbrain and midline shift of 16 mm to the left.

The child was stabilized with intravenous (IV) fluids, hyperosmolar therapy, prophylactic anticonvulsant, and mechanical ventilation. IV antibiotics, IV dexamethasone, and oral albendazole were started. Decompressive craniectomy and cyst removal were done by neurosurgeon.

Preoperative investigations

Hemogram showed normocytic normochromic picture. Hemoglobin was 10 g/dl, blood bactec - no growth; serum electrolytes, liver function test, and renal function tests were within normal limits; ultrasonography abdomen was normal.

Intraoperative findings

Craniotomy was done – dura was tense. Corticectomy was done – temporal horn reached. Cysts were excised. Cysts appeared thin wall and contained clear fluid. All (total 30) cysts cleared from temporal and occipital region. Cyst and fluid specimen was sent to pathology and microbiology department for confirmation.

Histopathology report of cyst confirmed the diagnosis of hydatid cyst [Figure 1] Cyst fluid wet mount showed plenty of brood capsules and protoscolices [Figure 2].

Figure 1

Histopathological slide showing brood capsule

Figure 2

Wet mount showing brood capsule and protoscolices

However, patient's serum was negative for Echinococcus IgG antibodies.

Postoperative period

Repeat MRI brain confirmed removal of all cysts with minimal periventricular ooze. Intraventricular drain was removed after 3 days. Repeat CT brain after 3 days showed no signs of hydrocephalus or raised intracranial pressure and sensorium improved. Hence, supportive therapy was gradually waned off. IV antibiotics were omitted after 5 days.

Child was shifted to ward and continued on oral eptoin, albendazole, cefuroxime, and prednisolone. She developed left facial nerve palsy which showed spontaneous gradual improvement. Patient was later discharged and was told to continue eptoin for 3 months, albendazole for 6 months, and to omit steroids after 5 weeks.

DISCUSSION

Hydatid cyst is a zoonotic disease caused by E. granulosus. Infection with the larval stage of the tapeworm causes hydatid cysts in humans. Hydatid disease is endemic in the sheep and cattle raising areas of the world.

Although hydatid cysts are known commonly to affect liver and lungs, in 1%–2% of cases, it can also affect brain. Brain involvement is more commonly seen in children (50%–75%).[3] CT scan, MRI is very useful investigations for clinical diagnosis. Brood capsules and protoscolices seen in histopathology slides and wet mount examination of fluids confirm the diagnosis. It also helps to differentiate cystic echinococcosis caused by E. granulosus from alveolar echinococcosis caused by Echinococcus multilocularis infection.[6] Primary cysts in brain are usually solitary and are formed as a result of direct infestation of larvae in brain without the demonstrable involvement of other organs. Lack of effective immune system in brain, special architecture of brain tissue, patent ductus arteriosus, and patent foramen ovale have been proposed risk factors for isolated cerebral hydatid cysts.[7] Secondary cysts are multiple caused by accidental spillage during surgery, trauma, or even spontaneously. Secondary cysts are infertile and lack germinative layer and brood capsules.

Multiple primary cysts in ventricle are extremely rare as multiple larvae directly invade the brain. Boujan and Jalal have reported primary multiple cysts in brain in a 10-year-old child. A total of 73 cysts were removed from his cerebrum.[8] Popli and Khudale have also reported primary multiple hydatid cysts in a child in Australia.[9] Gupta et al. have reported two cases of multiple hydatid cysts in the brain in Mumbai.[10] Yüceer et al. have reported multiple hydatid cysts in brain. However, it was secondary due to surgical rupture of primary cyst.[5]

Intracranial hydatid cysts usually present as papilledema and symptoms due to SOL.[357] Our patient also had papilledema and altered sensorium due to SOL.

The best treatment is removal of hydatid cyst and oral administration of Albendazole. To avoid recurrences, albendazole is given for long duration.[7]

CONCLUSION

The incidence of primary multiple hydatid cysts of brain is very rare. In our case, larvae might have passed through the capillary filter of liver and lungs, entered systemic circulation, and reached brain. The aim is total removal of cyst and prevents recurrences.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.