Interventional neuroradiology (INR) has evolved from a hybrid mixture of daring radiologists and iconoclastic neurosurgeons into a multidisciplinary specialty, which has become indispensable for cerebrovascular and neurological centers worldwide. This manuscript traces the origins of INR and describes its evolution to the present day. The focus will be on cerebrovascular disorders including aneurysms, stroke, brain arteriovenous malformations, dural arteriovenous fistulae, and atherosclerotic disease, both intra- and extracranial. Also discussed are cerebral vasospasm, venolymphatic malformations of the head and neck, tumor embolization, idiopathic intracranial hypertension, inferior petrosal venous sinus sampling for Cushing’s disease, and spinal interventions. Pediatric INR has not been included and deserves a separate, dedicated review.

In diffuse forms of arteriovenous malformation following Fontan procedure, “classical” medical therapy, inhaled nitric oxide and sildenafil, may play a role, until re-direction of hepatic flow to pulmonary circulation cures it. However, in refractory cases, as reported in our 2-year-old patient, unusual medications such as calcium channel blockers can be tried and continued if patients respond adequately.

The development of pulmonary arteriovenous malformations is a well-known complication after Fontan operations, and may result in significant morbidity due to increasing arterial desaturation. We compared the use of bubble contrast echocardiography and pulmonary angiography in detecting such malformations. We also examined which anatomical and haemodynamic variables were associated with their development. Our study includes 20 patients who had undergone modified Fontan procedures, 10 with atriopulmonary and 10 with total cavopulmonary connections, in Gothenburg between 1980 and 1991. All patients underwent cardiac catheterisation and pulmonary angiography. Bubble contrast echocardiography was performed at the same time, with injection of agitated polygelin colloid solution (Haemaccel, Hoechst) into the right and left pulmonary arteries, respectively. Transoesophageal echocardiography was used to detect the appearance of bubble contrast in the pulmonary venous atrium. The aim was also to evaluate the role of hepatic venous blood. Of the 20 patients, 9 (45%) had a positive contrast echocardiography study, compared with only 2 (10%) detected by pulmonary angiography. Patients with positive contrast echocardiography had a significantly lower arterial oxygen saturation than those with negative studies, both at rest (88% vs 95%, p < 0.01) and during exercise testing (78% vs 89%, p = 0.01). Bubble contrast echocardiography is much more sensitive in detecting pulmonary arteriovenous malformations than pulmonary angiography. By injecting echo contrast into the right and left pulmonary arteries, the method can be made highly selective. Pulmonary arteriovenous malformations develop much more frequently in patients with the Fontan circulation than previously reported.

A report of a case with a vascular malformation of the internal auditory canal (IAC) is presented. A review of the literature of this rather rare lesion is also made in an attempt to outline its clinical features, radiological diagnosis and management. The differential diagnosis and distinction between vascular malformations and other internal auditory canal tumours are discussed.