Child’s Nerv Syst (2001) 17:743–745 DOI 10.1007/s003810100498 Serdar Kemaloǧlu Umit Ozkan Yasar Bükte Murat Acar Adnan Ceviz Received: 17 March 2001 Revised: 1 June 2001 Published online: 22 September 2001 © Springer-Verlag 2001 S. Kemaloǧlu (✉) · U. Ozkan · A. Ceviz Department of Neurosurgery, School of Medicine, Dicle University, Diyarbakir, Turkey e-mail: mserdarkemaloglu@yahoo.com Tel.: +90-412-2285124 Fax: +90-412-2488440 S. Kemaloǧlu Faik Ali Sok. Rauf Bey Apt, No: 22 Ofis 21280, Diyarbakir, Turkey Y. Bükte · M. Acar Department of Radiology, School of Medicine, Dicle University, Diyarbakir, Turkey C A S E R E P O RT Growth rate of cerebral hydatid cyst, with a review of the literature Abstract This extremely rare case was one of secondary solitary cerebral echinococcosis associated with possible cerebral thromboembolism. A 7-year-old girl living in a rural area was admitted to our hospital with a history of headache, right-sided hemiparesis, and dysphasia. She had been treated 6 months previously for a cerebral infarct, diagnosed from sudden altered consciousness and a myoclonic generalised convulsion. The growth rate determined for the cerebral hydatid cyst was about 4.5 cm during the 6-month period. In children a parasitic cyst can be the source of a cerebral embolus, particularly in areas where hydatid disease Introduction Echinococcosis is an infestation caused by Echinococcus granulosus as a tapeworm. While liver and the lungs are the organs most commonly affected by hydatid disease, brain involvement is relatively rare. In about 2% of cases, hydatid disease involves the central nervous system (CNS) [1, 4, 5]. Cerebral hydatidosis can be divided into two distinct histological types, primary and secondary. Primary CNS echinococcosis is the most common form and is nearly always solitary. The secondary form is usually multiple and usually results either from the rupture of a primary cyst into a vessel or from rupture of the primary cerebral echinococcosis [5, 8]. Primary hydatid disease of the heart is rare, being found in approximately 0.5–2% of all cases of hydatid disease. The left ventricle is the most common localisation in the heart [6]. resulting from cardiac echinococcosis is endemic. Keywords Cerebral hydatid cyst · Growth rate · Embolism · Cardiac echinococcosis The growth rate of the hydatid cysts in the brain is still a subject of debate. Many authors have reported different growth rates for hydatid cysts in the brain [1, 2, 3, 4, 5, 6, 7, 8]. We report the growth rate of an unusual solitary cerebral hydatid cyst with a possible cerebral thromboembolism resulting from cardiac hydatidosis leading to valve dysfunction. Case report A 7-year-old girl living in a rural area was admitted with a history of headache, right-sided hemiparesis and dysphasia. She had been treated for a cerebral infarct 6 months earlier. The onset of illness had been sudden, with altered consciousness and a myoclonic generalised convulsion. Initially, neurological examination demonstrated central facial paresis, right hemiparesis and speech disturbances. Computerised tomography (CT) of the head revealed a non-enhanced irregular hypodense area in the left parieto-occipital region (Fig. 1). She 744 Fig. 1 The CT study taken during the first hospital stay revealed an infarct in the distribution region of the left middle cerebral artery Fig. 2 Follow-up CT scan taken 6 months later, showing both the right occipital hydatid cyst and the left frontoparietal infarct area This cyst was assumed to be a hydatid cerebral cyst. A surgical intervention was planned for correction of the cardiac lesion after removal of cerebral cyst. Mebendazole treatment was started prior to surgery. A unilateral occipitoparietal craniotomy flap was turned. The dura and arachnoid were opened. A cortical incision was made over the most superficial part of the cyst. The cyst was then removed by Dowling's technique. Histopathological examinations of the cyst showed a characteristic cyst capsule, and the fluid contained scolices. Three weeks after this operation, the patient underwent left atriotomy and a cystic mass was removed from the lateral surface of the left ventricle in the department of cardiovascular surgery. Histopathological examination of the specimen confirmed the diagnosis of cardiac hidatidosis for this lesion. Three months after the operation, right hemiparesis was still present, but the child was conscious and her speech was moderately improved. She is now 8 years old and is still receiving antiparasitic and anticonvulsive therapy. Discussion Fig. 3a, b Follow-up MRI of the head 6 1/2 months later, showing both the right occipital hydatid cyst and the left frontoparietal infarct area on T1- and T2-weighted images had been treated with medication on that occasion and discharged markedly improved. Six months later, she was again seen in the outpatient clinic because of a mild deterioration of consciousness, right central facial paresis and hemiparesis. Laboratory tests were normal, but mild eosinophilia was present. Chest radiography and abdominal ultrasound were normal. Echocardiography showed a mass lesion measuring 19×21 mm on the left ventricular surface of the anterior mitral valve. Cranial CT showed a single, non-enhancing cystic lesion with a well-defined contrast administration, occupying the right occipital region and an irregular hypodense area in the left parieto-occipital region (Fig. 2). Brain magnetic resonance imaging (MRI) showed a single well-rounded hypointense cystic lesion in the right occipital region and irregular hypointense area in the left parieto-occipital region on axial SE T1-weighted images. The lesions were hypointense on axial SE T2-weighted images (Fig. 3a, b). This cyst had not been present on the CT 6 months earlier and was obviously growing very fast: the growth rate was 4.5 cm in 6 months. Table 1 Growth rates presented for cerebral hydatid cyst cases in the literature to date (O occiptal, F frontal, T temporal, O.S.I. other system involvement, Sol. solitary, mult. multiple) The brain is affected in only 1–2% of patients with echinococcosis granulosus, and the incidence of hydatid disease of the heart is approximately 1% of all cases reported in the literature [6, 8]. Primary cerebral echinococcosis is caused by the embolism of parasitic embryos that have escaped the lymphomesenteric, hepatic, cardiac and pulmonary filters [1, 8]. Primary cerebral hydatid cysts are usually solitary and occur mostly in children. Secondary cysts stem from scolices caused either by rupture of fertile cysts into the left ventricle or by rupture of a primary cerebral hydatid cyst, and these tend to multiplicity [5, 8]. The size attained by hydatid cysts depends on several factors related to the parasite, the particular tissue affected and the host. There are only five case reports in the literature to date in which the growth rate of a cerebral echinococcosis is specifically discussed [1, 4, 5, 6, 7, 8] (Table 1). Two of these reports deal with cases of primary cerebral echinococcosis [4, 8]. The other cases are secondary and multiple. Vaquera et al. [7] were the first to report a case of a cerebral hydatic cyst in a patient previously operated on for cardiac hydatidosis. The patient had three intracranial cystic lesions, one of which was within the previously established infarct area in the right frontal region. The growth rate determined for the cyst was 1 cm/year in this study. A similar patient reported by Sierra et al. [5] was found to have multiple cere- Reference Age Sex Duration of symptoms Localisation Diagnostic O. S.I. method Sol.- mult. Growth rate [7] [5] [4] [8] [1] Present case 37 14 15 8 24 8 M M M F M F 12 14 7 12 6 7 O-P F-T O F-P O O CT CT CT CT CT CT/MRI Solitary 1 cm/year 5 cm/year 10 cm/year – 1 cm/month 7 cm/year Heart + – Liver Heart Heart Multiple Solitary Multiple Solitary 745 bral echinococcosis, with one cyst located in the previously specified infarct zone, and the parasite growth rate determined by this group was 5 cm/year. The authors assumed that the patient had an embolus at the same time and that it was probably due to the spontaneous rupture of a small hydatid cyst in a left cardiac cavity [4]. Jimenez-Mejias et al. [3] reported that size and growth rate of the parasite were greater in children than in adults, an opinion supported by our CT findings, which included an intracerebral growth that had achieved a diameter of 4 cm in not quite 6 months. Evliyaoglu et al. [1] reported multiple secondary cerebral echinococcosis an adult who had a cardiac hydatid cyst, and its growth rate was 1 cm/month. Pasaoglu et al. [4] reported that the growth rate of the primary multiple cerebral hydatid cyst in their case was 10 cm/year. Cardiac hydatid cysts may be the source of parasitic embolism to different arteries. Hydatid cerebral embolism continues to be repeatedly reported in the literature [1, 5, 6, 7, 8]. In none of the reports has it been pointed out that cardiac valve hydatidosis could cause cerebral thromboembolism. In the present case, cardiac echinococcosis first caused a cerebral infarct resulting from possible thromboembolism and then the parasitic embolism grooved in the opposite hemisphere. The time from the first thromboembolism and recognition of the symptomatic cyst was about 6 months. In the differential diagnosis of cerebral embolism, particularly in the paediatric age group and in areas where hydatid cyst is endemic, parasitic embolism and thromboembolism caused by cardiac echinococcosis should be considered. References 1. Evliyaoglu C, Yuksel M, Gul B, Kaptanoglu E, Yaman M (1998) Growth rate of multiple intracranial hydatid cysts assessed by CT from the time of embolisation. Neuroradiology 40:387–389 2. Heimlich A, Omaha A, Armani F, Assam O (1990) Development of intracerebral hydatid cyst evaluated with x-ray computed tomography. A case report. Neurochirurgie 36:312–314 3. Jimenez-Mejias ME, Castillo OE, Cuello CJA, Garcia GD, Romero AJ, Pachon DJ (1991) Cerebral hydatidosis. Analysis of a series of 23 cases. Med Clin (Barc) 97:125–132 4. Pasaoglu A, Orhon C, Akdemir H (1989) Multiple primary hydatid cysts of the brain. Turk J Pediatr 31:57–61 5. Sierra J, Oviedo J, Berthier M, Leiguarda R (1985) Growth rate of secondary hydatid cysts of the brain. Case report. J Neurosurg 62:781–782 6. Turgut M, Benli K, Eryilmaz M (1997) Secondary multiple hydatid cysts caused by intracerebral embolism of cardiac echinococcosis: an exceptional case of hydatidosis. Case report. J Neurosurg 86:714–718 7. Vaquero J, Jimenez C, Martinez R (1982) Growth rate of hydatid cysts evaluated by CT scanning after presumed cerebral hydatid embolism. Case report. J Neurosurg 57:837–838 8. Vatansever M, Biliciler B, Aladag MA, Colak A (1996) A huge cerebral hydatid cyst associated with small liver cyst: a comparison of growth rates and sizes. Neurosurg Rev 19:123–126