Echinococcal Infections of the Central Nervous System in Children Homepage

Authors

Hakan Karabagli, M.D.

Gokmen Kahilogullari, M.D., Ph.D.

Pinar Karabagli, M.D.

Section Editors

Adrian Caceres, M.D.

Sandip Chatterjee, MBBS, DNB(Surg), FRCS(SN), NAMS(Neurosurg)

Editor in Chief

Rick Abbott, M.D.

Introduction

Echinococcosis (hydatidosis) is caused by infestation with the tapeworm Echinococcus granulosus and, much less frequently, E. multilocularis. Because of the inefficiency of environmental health and protective care, and the raising of animals, the prevalence of the disease is high in South America, Africa, Australia, the Mediterranean countries, and the Middle East. Cerebral manifestations (such as epilepsy, hemiparesis, mass effect, increased ICP) of the disease are rare and present only in about 2–3% of all E. granulosus infestations (1,10,12,26,28). This condition is more prevalent during childhood, with children encompassing up to 80% of patients described in reports (49). Alveolar echinococcosis is an important zoonotic infection caused by the larval stage of E. multilocularis (25).

Key Points

• Childhood disease in developing countries: Hydatid disease of the CNS is a childhood disease most commonly seen in developing regions, such as South America, Africa, Australia, the Mediterranean, and the Middle East.
• Primary cysts single and fertile: Cerebral hydatid cysts may be classified as primary or secondary. Primary cysts almost always appear as single lesions. These cysts are fertile due to their content of scolices and brood capsules. Secondary hydatid cysts result from surgical, spontaneous, or traumatic rupture of a primary cyst in other organs. These cysts are usually multiple and infertile due to their lack of brood capsules and scolices. Liver, lung, heart, spleen, and kidney are the most involved organs and must be carefully investigated in patients, particularly children.
• CSF density cyst +/- calcified walls in MCA territory: For surgical planning and good prognosis, the preoperative diagnosis is important. On CT imaging, cerebral hydatid cysts with a sharply defined border and absorption value similar to that of CSF, with no perilesional edema, are observed as round, well-defined, smooth, thin-walled, and spherical. Calcification of the cyst wall occurs in 20–40% of cases. Most commonly, the cysts occur supratentorially in the MCA perfusion territory.
• Management surgical: The current management of hydatid disease has remained unchanged since the description of the surgical approach described by Dowling (9).