Our data demonstrate that most treated children survive and undergo normal neurological development; an understanding of the clinical, anatomical, and pathophysiological features of VGAM has, therefore, reversed the former poor prognosis. Our level of understanding about the lesion allows us to predict most situations and remedy them by applying a strict evaluation protocol and working within an optimal therapeutic window. Patient selection and timing remain the keys in the management of this condition. It is more important to restore normal growth conditions than a normal morphological appearance, with the primary therapeutic objective being normal development in a child without neurological deficit.

Forty-three patients with vein of Galen aneurysmal malformations (VGAM) referred to us for endovascular treatment between 1985 and 1990 and 335 additional cases published in the literature were reviewed with particular attention to the presence of ventricular enlargement and outcome after shunting. Hydrocephalus was the second most frequent symptom (46.8%); it is more frequent in infants (73%) than in children, adults (30%) or neonates (15%). Of the patients reported in the literature, 17.9% had undergone shunting. Within the shunted population there was an overall morbidity of 41% and a mortality of 10% (especially in the infant group). In our series 17 patients (39.5%) were shunted and a significant difference in the clinical outcome was noted between the shunted and the nonshunted group. Of the nonshunted patients, 66.6% were free of any neurological deficit or mental retardation and fewer than 5% presented with significant mental retardation. On the other hand, only 33.3% of the shunted patients had a favorable outcome and more than 15% developed significant mental retardation. Among the various causes of hydrocephalus in patients with VGAMs, such as obstruction of the aqueduct, subarachnoid hemorrhage, or ex vacuo hydrocephalus, high venous pressure may be of particular importance. In this article a physiopathological interpretation of the hydrodynamics in VGAMs is developed and a speculative explanation for CSF disorders related to ventricular shunting proposed. Treatment of hydrocephalus in VGAMs can be achieved through obliteration of the malformation or at least diminishing the venous pressure; surgical ventricular shunting does not have to be the first treatment of hydrodynamic disorders associated with VGAMs, especially in infants.

We present four cases of pial arteriovenous fistula (AVF) in children as the presenting manifestation of Rendu-Osler-Weber disease (ROW). The common clinical manifestations of ROW in adults, such as skin telangiectasia and mucosal haemorrhagic complications, seldom occur in children, since telangiectases develop with age. Pial AVF in ROW also conform to the usual age incidence and are therefore present in childhood. Of the four children in this series, three had multiple AVF. Two presented with central nervous system haemorrhage, one with seizures and the other with progressive neurological deficit. There were no clinical or angioarchitectural differences between the AVF associated with ROW and sporadic AVF. The diagnosis was based in all cases on the family history. Transarterial embolisation to obliterate the AVF was carried out in all patients. One patient had early rebleeding after partial embolisation of the AVF, with a fatal outcome. Three patients were cured and one asymptomatic in long-term follow up. No exhaustive search was conducted for multiorgan telangiectases, since there is no indication for treatment of asymptomatic telangiectasia in ROW. No pulmonary fistulae were found. ROW should be suspected in children with multiple pial AVF; they may be the only manifestation of the disease, since epistaxis and telangiectasia are unusual in early life.

Since 1984 we have been involved in the management of 30 children who had cardiac manifestations secondary to cerebrocranial arteriovenous shunts. Aneurysm malformation of the vein of Galen was the most common vascular lesion observed (73% of cases). In 77% of the patients the cardiac symptoms were the main presenting complaint. Medical treatment and/or endovascular therapy were indicated, depending on the age of the patients and the severity of the cardiac manifestations. Following embolization, the cardiac symptoms resolved (73%) or improved (18%) in 1 or 2 sessions. Mortality in the embolized group was 9%, and transient nonneurologic morbidity occurred in one case. Overall mortality, including four patients rejected for embolization, was 20%. These results compare favorably with medical and/or surgical management, alone or combined. The technique, challenges, indications and contraindications of endovascular therapy are discussed. Embolization represents an effective adjunct treatment to control, improve or cure the congestive cardiac manifestations caused by cerebrocranial arteriovenous shunts.

The authors present four consecutive cases of multiple dural arteriovenous (AV) shunts in children. This entity represents a rare but severe clinical situation. The etiology of the shunts is not known. There is clinical and radiological evidence that they are evolutionary lesions. The clinical presentation in this series was usually by cardiac manifestations (one case), cerebrospinal fluid disorders (two cases), neurological symptoms (three cases), including intracranial hemorrhage (two cases), and cranial bruits (four cases). No radical treatment leading to anatomical cure of all dural AV shunts was thought to be possible using current methods, including modern endovascular and surgical techniques, in any of the four cases. Therefore, treatment was only symptomatic and directed at some of the AV shunts. Targeted arterial embolization with permanent embolic agents represents the most rational technique for symptomatic relief in these patients. However, clinical recurrence often happens without evidence of recanalization. Secondary multifocal pial AV shunts opening into the abnormal sinus occurred in two of the cases; they may have been induced by venous sump from the sinus draining the dural AV shunts. Mechanical occlusion (or excision) in multiple dural AV shunts in children does not represent a satisfactory goal, as the shunts can be the expression of a more complex and yet unknown disease.

Objective: To describe the contribution of 3-dimensional computed tomography (3D-CT) in the prenatal diagnosis of skeletal dysplasias (SD) in a cohort of patients with inconclusive diagnosis by ultrasound (US). Methods: Between May 2007 and February 2010, six pregnant women with suspected fetal SD on US examination but with no specific diagnosis were studied with 3D-CT. The images were evaluated by a multidisciplinary team who proposed a likely diagnosis. Further postnatal workup included clinical and radiological evaluation in all cases. Prenatal and postnatal diagnoses were compared. Results: The use of 3D-CT provided a precise diagnosis confirmed postnatally in 5/6 patients. These included osteogenesis imperfecta type II (n = 2), osteogenesis imperfecta type III (n = 1), chondrodysplasia punctata (n = 1) and thanatophoric dysplasia type I (n = 1). A precise diagnosis could not be made in 1 case – either pre- or postnatally. Conclusion: Prenatal 3D-CT contributed to the diagnosis of the specific fetal SD in the majority of these cases. 3D-CT may have a complementary role to US where fetal SD is suspected, but no specific diagnosis can be made using US alone. Further studies on clinical performance and risk-benefit analysis are needed.

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A study of the embryology and the anatomy of the ophthalmic artery shows that the branches to the important sensory structures arise proximal to the second intraorbital segment of the vessel. Damage to vision will be avoided if embolisation is restricted to vessels anterior to this "safety point", which is easily recognised on an angiogram. The ideal point of injection of emboli is even more distal and varies with the extent of the lesion and the material used. Three cases are described with vascular lesions supplied by the ophthalmic artery and embolised with Histoacryl.