Large vein of Galen malformation associated with an apical ventricular septal defect and a patent ductus arteriosus in an infant: A case report

Peter Szkup ,Antoinette Cilliers

doi:10.4102/sajr.v2i2.1586

Peter Szkup , Antoinette Cilliers

Open Access

https://doi.org/10.4102/sajr.v2i2.1586

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Abstract

A four month old male infant who had a transarterial embolization of a large vein of Galen malformation attempted is reported. The infant presented in cardiac failure and a hydrocephalus. In addition 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 microcoils was commenced. The first attempt was uneventful. This was followed by the unfortunate demise of the patient from an intercurrent nosocomial pneumonia.

Highlights

Vein of Galen malformations (VGM) are rare vascular anomalies in which a primary feature is the dilatation of the vein of Galen which becomes dilated as a result of direct cerebral 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 aqueduct by the malformation or a resorptive block of cerebrospinal fluid due to increased pressure within the sagittal sinus.' Cranial bruits are audible in only a third of cases.' Fewer than 300 cases of this anomaly have been reported between 1937 and 1994.2
The first stage was performed under general anaesthesia and involved selective catheterization of a feeder artery to the malformation originating from the left middle cerebral artery

Summary

Introduction

Vein of Galen malformations (VGM) are rare vascular anomalies in which a primary feature is the dilatation of the vein of Galen which becomes dilated as a result of direct cerebral arterial connections bypassing the capillary network.' The cardiac output and venous return to the heart increases resulting in progressive heart failure and pulmonary hypertension. 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 estimated 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. In view of the major contribution of the cerebral pathology to the patient's morbidity it was decided to Figure 1: Sagittal view of the cranial ultrasound showing the vein of Galen malformation and a dilated straight sinus. The first stage was performed under general anaesthesia and involved selective catheterization of a feeder artery to the malformation originating from the left middle cerebral artery. The feeder artery was embolised (Figure 5) with the placement of ten microcoils The patient tolerated this first procedure well and suffered no complications.

Discussion

Conclusion