membranes, in the pericardial cavity adjacent to the lateral wall of left ventricle (arrow). lymphatics ... hydatid cysts are 'meniscus sign', 'spin sign' and the. 'water lilly sign'. ... extrusion of smaller constituents of the ruptured cyst, rather than the ...
24 Kumar et al Cardiac hydatidosis
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Recurrent cardiac hydatidosis in a child presenting as acute stroke Sandeep Kumar, MD, Roumina Hasan, MBBS Department of Radiodiagnosis, Sanjeevni Mri Centre, Moradabad, UP Abstract Cardiac echinococcosis is a rare but potentially life threatening condition, with surgery being the definitive treatment. We present the case of a 4 year boy who was diagnosed with concurrent pulmonary and left intraventricular hydatid cysts. He was operated for the same and advised prophylactic albendazole. After a symptom free interval of 8 months the child developed acute onset right sided hemiplegia, which was confirmed on magnetic resonance imaging (MRI) brain as acute left basal ganglia nonhaemorrhagic infarct. Also a recurrent pericardial hydatid cyst was visualized, which was assumed to be the embolic source for the stroke. We report this exceptional case of recurrent cardiac hydatidosis presenting as acute stroke. (Ind J Thorac Cardiovasc Surg 2008; 24: 24-27) Key words: Magnetic resonance imaging, Hydatid cyst, Cardiac. Introduction Echinococcosis is a widely prevalent zoonosis in the sheep herding areas of world like Mediterranean coast, the Middle East, Australia, New Zealand and south America. It can virtually involve any organ system in the human body with the most common site being Liver ( 75%), and Lungs (15-20%)1, which can be explained by its feco-oral route of transmission. Simultaneous liver & lung hydatid cysts have been reported in 5-13% of these patients2. Cardiac involvement is rare and has been reported in literature as accounting for 0.05-2 % of all human hydatid cases1-3. Surgery is the mainstay in treatment of cardiac hydatidosis with proven excellent prognosis. We report a case of recurrent cardiac hydatidosis presenting as acute stroke in a follow up patient of surgically treated simultaneous cardiopulmonary hydatidosis. To the best of our knowledge this association has not been reported in the literature as yet. Case report A 4-year-old boy with complaints of fever and cough Address for correspondence: S. Kumar Flat Number 388, Block E, DDA, SFS Flats, Rohini, Sector 18, New Delhi, India 110085. © IJTCVS 097091342410308/43 Received - 15/05/07; Review Completed - 16/08/07; Accepted - 28/11/07.
was referred to us for Chest X ray. Chest radiograph revealed a large, well defined, smooth marginated mass lesion involving the left upper-middle lung zones, silhouetting the left upper mediastinum with borderline cardiomegaly (Fig. 1). Computed tomography (CT) Thorax was performed and it revealed a large well defined, non enhancing unilocular cystic mass lesion almost completely occupying the left upper lobe, abutting the chest wall and the pericardium. Another well defined unilocular cystic lesion of size 3.2x 2.9 cm was seen within the left ventricle ( Fig. 2A & 2B). There was no evidence of any pericardial effusion seen. A diagnosis of concurrent cardiopulmonary hydatidosis was made. Enzyme-Linked Immuno Sorbent Assay (ELISA) with Immunoglobulin G (IgG) for hydatid was negative, however indirect haemagglutinin test was positive. The patient was subsequently operated upon and underwent left posterolateral thoracotomy with pulmonary cystectomy. A single 10 x 8 cm hydatid cyst containing approximately 90 ml of clear fluid was excised from upper lobe of left lung with adhesionolysis of pericyst from pericardium and chest wall done. In the same sitting median sternotomy under cardiopulmonary bypass was done and the left ventricular cyst, which was found to be deeply attached to the ventricular wall, was removed intact from the surrounding tissue. There was no communication seen between the cyst lumen and ventricular cavity. The ventriculotomy was sutured with Teflon strips. The post op recovery was uneventful. The histopathological reports were consistent with hydatid cyst and the
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Kumar et al 25 Cardiac hydatidosis
Fig. 1. X-ray chest (PA view) : A large well defined smooth marginated mass lesion is seen almost completely occupying the left upper-middle herithomax, with borderline cardiomegaly.
Fig. 2B. CECT Thorax (axial section at the level of left ventricle): reveals a well defined cystic mass lesion (arrow) in the left ventricle.
Fig. 2A. CT scan Thorax Left paramedian Sagittal reconstrcution: Shows concurrent unilocular cysts in the upper lobe of left lung and in the ventricle (arrows).
patient was discharged with prophylactic medication on albendazole. 8 months later the patient had an acute onset right sided hemiplegia, which was not associated with any fever or vomitting and was referred to us for Magnetic resonance Imaging (MRI) head and CT Thorax. MRI Brain revealed a non hemorrhagic acute infarct in left basal ganglia region, involving the caudate nucleus, putamen & anterior limb of internal capsule, corresponding to the deep branches of Left Middle cerebral artery territory (Fig. 3).
There was no evidence of any intracranial hydatid cyst seen. CT Thorax showed normal post operative status of left lung with no recurrence or any residual pulmonary lesion. However a well defined cystic lesion of size 2.9x1.5 cm was visualized in the pericardial cavity attached to the lateral wall of left ventricle (Fig. 4). The lesion showed undulating thin internal septa, simulating internally collapsed cyst membranes. A diagnosis of recurrent cardiac hydatidosis with non haemorrhagic acute infarct in left basal ganglia region was made. The patient was advised re-operation, for which his parents refused and he is being currently managed conservatively on albendazole & supportive therapy for the cerebrovascular event. Discussion Isolated cardiac hydatid cyst is rare, with synchronous polyvisceral involvement more common. Usually it is secondarily found while searching for a possible embolic source for hydatidosis in other organ systems. The distribution in heart parallels that of coronary blood supply, with the most common site being left ventricle (60%), followed by right ventricle (15%), the interventricular septum ( 9%), the left atrium (8 %), the right atrium (4%), the interatrial septum (2%), and the
26 Kumar et al Cardiac hydatidosis
Fig. 3. T2 WI MRI (axial images) : Show abnormal hyperintense signals in the left basal ganglia region involving the caudate nucleus, putamen and the anterior limb of internal capsule.
Fig. 4. CT scan thorax in follow up after 8 months: Shows a relatively well define cystic mass lesion showing internal undulated membranes, in the pericardial cavity adjacent to the lateral wall of left ventricle (arrow).
pericardium (2-10%) 2,4 . It is understood that echinococcus larva usually reaches the myocardium via the coronary circulation, even though intestinal
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lymphatics & pulmonary circulation have also been implicated as possible routes, with reported cases of involvement by contiguous spread from adjacent organs like liver & lung5,6. The low incidence of cardiac involvement can be explained by the fact that the echinococcus larva has to circumvent the hepatic & pulmonary ‘filters’ in order to reach the heart, where, the contractility of the myocardium further prevents its implantation. Unlike our present case, Cardiac hydatidosis rarely presents in early childhood, because of its reported slow growth rate of 2-3 cm per year making it only possible for the embryo lodged in the heart to attain its full growth in 1-5 years1,3. Cardiac hydatidosis are usually asymptomatic but can present with protean manifestations depending on its size, location and integrity of the cyst. Most common reported symptoms of cardiac hydatidosis are precordial chest pain, cough, arrhythmia, valvular dysfunction, pericardial reactions, pulmonary or systemic embolism, pulmonary hypertension, anaphylactic reaction complications and sudden death2. Most common complication is rupture of the cyst (24% –60%)2. 2-Dimensional Echocardiography is regarded as the best diagnostic procedure for the demonstration of cardiac hydatid cyst6. Characteristic features described are movement of cyst contents (hydatid sand) on changing the patient posture and progressive image modulation (thickening of contents) with medical therapy5.6. CT scan & MRI play an important adjuvant role in the investigative workup of cardiac hydatidosis by providing precise anatomical localisation and confirmation of the cystic nature of the mass and showing its relation to cardiac and extra cardiac structures. Typical radiological signs described for hydatid cysts are ‘meniscus sign’, ‘spin sign’ and the ‘water lilly sign’. The pathognomonic sign for hydatid cyst, the ‘snake sign’ which is seen in the recurrent cardiac cyst in our present case refers to the undulated membranes formed by disruption of parasitic membranes by the high fluid pressure, resulting in seepage of fluid into the space between the pericyst and ectocyst7. A well defined hypointense rim of maximum thickness 4-5 mm on MRI has been reported to be another important characteristic of hydatid cyst, differentiating it from other cystic non parasitic lesions8. Definitive treatment for cardiac echinococcosis is surgical expirtation and it is indicated even in asymptomatic cases in order to prevent catastrophic complications from ruptured cyst3. Surgery has been proved to be curative with excellent prognosis. Surgical
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mortality has been reported to be 0.29-0.6 % 2 . Supplemental Albendazole has been widely advocated post surgery to prevent recurrence, but just as in our case it has been reported to be a failure in some cases9. The most plausible explanation for the pericardial recurrence seen in our case can be attributed to inadvertent spillage during surgery. Pericardial involvement can present as : (a) silent rupture and the appearance of the echinococcus cyst some months later; (b) acute pericarditis with or without cardiac tamponade; (c) constrictive pericarditis10. Presentation of cardiac hydatidosis as acute stroke is exceptionally rare and only few cases have been reported in literature2,3,5. Yaliniz et al report that since intracranial germinative membrane embolism is invariably fatal, the non fatal stroke must result from extrusion of smaller constituents of the ruptured cyst, rather than the germinative membrane itself11,12. This appears to be a reasonable explanation in our case,who presented with acute non fatal stroke & was found to harbour an otherwise asymptomatic recurrent cardiac hydatidosis. In the few reported cases of cardiac hydatidosis presenting as acute stroke5,12,13, secondary hydatid cyst formation was seen in the region of infarct in two instances12. Our case is exceptional on two counts, firstly in reporting a recurrence of cardiac hydatidosis in a child within the same year of surgery even after taking adequate prophylactic albendazole and secondly, release of microemboli from this cardiac recurrence causing acute non fatal stroke. Cardiac hydatidosis with its myriad manifestations constitute a medical emergency requiring an urgent and aggressive surgical treatment with adequate follow up, in lieu of its recurrence with morbid consequences.
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