CASE REPORT Large vein of Galen malformation associated with an apical ventricular septal defect and a patent ductus • •arteriosus In an infant - a case report Abstract PSzkup MD A four month old male infant who had a transarterial embol ization of a large vein of Galen malformation attempted is reported. The infant presented in cardiac failure and a hydrocephalus. In add ition the rare association of an apical ventricular septal defect and a patent ductus arteriosus with pulmonary hypertension was diagnosed. A staged transarterial coil embolisation using AM Cilliers MBBCh, FCPaed (SA) Divisions of Radl%gy and Paediatric Cardl%gy, Baragwanath Hospita/, Johannesburg. 28 SA JOURNAL OF RADIOLOGY. June 1997 microcoils was commenced. The first attempt was uneventful. This was fol lowed by the unfortunate demise of the patient from an intercurrent nosocomial pneumonia. Introduction Vein of Galen malformations (VGM) are rare vascular anomalies in which a primary feature is the dilata- tion of the vein of Galen which be- comes dilated as a result of direct cer- ebral arterial connections bypassing the capillary network.' The cardiac output and venous return to the heart increases resulting in progressive heart failure and pulmonary hypertension. Hydrocephalus may occur as a result of direct obstruction of the Sylvian aq- ueduct by the malformation or a resorptive block of cerebrospinal fluid due to increased pressure within the sagittal sinus.' Cranial bruits are audi- ble in only a third of cases.' Fewer than 300 cases of this anomaly have been reported between 1937 and 1994.2 Case report A male infant presented to our in- stitution at the age of four months with a history of cough and shortness of breath for eight days duration. Clini- cal examination revealed a wasted in- fant in cardiac failure. In addition the patient had a large head (beyond the 97th centile for age) and a loud cra- nial bruit. The blood pressure was 70/50. Biventricular hypertrophy was present clinically and a 3/6 pansystolic to page 29 Large vein of Galon nl;1lfoll11.ltion assoc.iarod ~ith an dpic<'ll vcruriculnr soptal defect arul (l patont ductus arteriosus ill é:Hl infant - <1 C;:lse report from page 28 murmur over the apex was audible. The liver measured 4 em below the right costal margin and the pulmo- nary component of the 2nd sound was loud indicating the presence of pulmonary hypertension. The chest radiograph showed a cardiothoracic ratio of 70% with biventricular en- largement and plethoric lung fields. Right ventricular hypertrophy was the main feature electrographically. Two dimensional echocardiography showed a 3 mm apical ventricular septal defect (VSO), a 3 mm patent ductus arteriosus (PDA) and severe pulmonary hypertension with an es- timated pulmonary artery pressure of 70 mmHg. Cardiac catheterization confirmed balanced pulmonary and systemic pressures with poor response of the pulmonary vasculature to the administration of oxygen. Cranial ultrasonography (Figure 1) and Doppler flow studies revealed Figure 1: Sagittal view of the cranial ultrasound showing the vein of Galen malformation and a dilated straight sinus. a VGM measuring 2.5 x 2.8 em drain- ing into a dilated straight sinus. Dila- tation of the lateral and third ventri- cles was evident. Pre- and post- con- trast axial CT (Figure 2) confirmed a VGM and mild hydrocephalus. Cerebral angiography (Figures 3 and 4) was performed via the transfemoral arterial route. There were major feeder arteries arising Figure 2: Contrast enhanced axial computerised tomography scan of the head showing the large vein of Galen malformation, dilated straight sinus and mild hydrocephalus. from both the posterior cerebral ar- teries, both left and right middle cer- ebral arteries and the right anterior cerebral artery. Other lesser arterial feeder arteries were apparent. Despite medical treatment in the form of oxygen, digoxin and diuretics the patient remained in cardiac failure and failed to gain weight over the next month. In view of the major contribu- tion of the cerebral pathology to the patient's morbidity it was decided to Figure 3: Lateral view of the cerebral angiogram showing multiple feeder vessels to the vein of Galen malformation arising from the right middle cerebral artery and the anterior cerebral artery. 29 SA JOURNAL OF RADIOLOGY. June 1997 Figure 4: Lateral view of the cerebral angiogram showing muit/pie feeder vessels to the vein of Galen malformation arising from the left middle cerebral artery. perform a staged transarterial emboli- zation of the VGM. The first stage was performed un- der general anaesthesia and involved selective catheterization of a feeder artery to the malformation originat- ing from the left middle cerebral ar- tery. Access to the feeder artery was accomplished using a FasTracker 18MX micro catheter, together with Dasher 14 and Mach 16FIX Nitinol guidewires. The feeder artery was embolised (Figure 5) with the place- ment of ten microcoils . The patient tolerated this first procedure well and suffered no complications. Unfortu- nately before the second staged proce- dure could be attempted the patient demised from a nosocomially acquired pneumonia in the intensive care unit. Permission for a post mortem exami- nation was not granted. Discussion Congestive cardiac failure unre- sponsive to medical management in the infant is the primary indication for treatment.P The s vere cardiac failure and failure to thrive in our pa- tient were the major motivational fa - tors in attempting to treat the VGM. topage30 Llrge vein of Gellen rnalforrnation associated with dn apical ventricular septal defect and a palent duclus arteriosus in all infant - a case report from page 29 Figure 5: Lsteral view of the left internal carotid artery angiogram following the embolisstion procedure showing microcails occluding the arterial feeder vessels to the vein of Galen malformation. The cardiac lesions were deemed to be of minor significance not requir- ing treatment at the time. An endovascular approach was under- taken because of the lower anticipated mortality rate of 13%4, compared to a surgical approach.v" A staged transarterial occlusion was initiated to gradually reduce the flow through the fistula to control the cardiac failure, A partial occlusion fre- quently reverses the cardiovascular symptoms so that a complete occlu- sion may not be required.' A further rationale for a staged endovascular occlusion is to avoid "normal perfusion pressure break- through" which may occur with ei- ther a transveneus- or transarterial approach in a large VGM Le, the pre- viously hypoperfused peripheral brain after embolisation of a large VGM may develop a sudden increase in blood flow associated with regional brain swelling and haemorrhage.' Severe neurological damage may result. Associated structural cardiac de- fects are rarely reported. PDA,6,7,8 transposition of the great vessels," a VSD, and coarctation of the aorta? have been described. Coarctation of the aorta may have a physiological explanation in that the developing foetus with an intracranial fistula has reduction of blood flow down the descending aorta which induces de- velopment of hypoplasia of the isth- mus of the aorta." Conclusion Management of vein of Galen aneurysmal malformations is a rare and challenging therapeutic problem for the neuroradiological inter- ventionalist. While there is a higher risk of morbidity and mortality asso- ciated with a direct surgical approach, an endovascular approach has poten- tial complications. Cardiac lesions are rare associations and if significant may contribute substantially to the thera- peutic dilemma. Acknowledgements The authors wish to thank Prof P Fourie (Pretoria Heart Hospital), Prof RW Urwin and Prof Van V Hallbach (both from the Department ofNeuro- interventional Radiology, University of San Francisco) for their advice in the management of the patient. 30 SA JOURNAL OF RADIOLOGY· June 1997 References I. 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