Paraspinal Arterio-Venous Fistula in Children: Two More Cases of an Exceptional Malformation

Abstract

We report on the case of two toddlers who presented in the last 2 years with heart and vascular murmur, respectively, and in whom the diagnosis of paraspinal arterio-venous fistula was made. Paraspinal arterio-venous fistulae in children are extremely rare congenital or post-traumatic vascular malformations. In the rare case of connection with the spinal venous system, they might affect spinal vascularization due to potential venous congestion. Interventional embolization rather than surgery is the treatment of choice for such lesions. Up to now, there is no consensus about the indication of prophylactic closure of asymptomatic fistulae. However, close clinical follow-up with repeated spinal magnetic resonance imaging to exclude venous congestion is mandatory for young asymptomatic patients until treatment.

Paraspinal arteriovenous fistula: Stuttgart classification based on experience and a review of the literature

“…Paraspinal AV shunts also occur in children. 13,21 Congenital PAVSs with and without a detectable genetic disorder can be distinguished. If diagnosed shortly after birth without history of trauma, a congenital origin of the paraspinal shunt can be assumed.…”

“…28,30 diScuSSion Numerous classifications for spinal AVSs have been published, since the first AVSs around the spinal cord were described in the late 19th century by Hebold and Gaupp. 5,[31][32][33][34][35][36][37][38][39][40] These classifications divide spinal AVSs into subgroups on the basis of morphological criterion which, according to Rodesch and Lasjaunias, create unfortunate confusion due to subjective interpretation of angiographic data or imprecise description. Thus, in 2003 Rodesch and Lasjaunias proposed a classification scheme for spinal AVSs, categorizing them according to (1) their precise localization, (2) the type of shunt involved (fistula or nidus) and (3) their potential association with genetic alterations, vascular biological features, and angiogenesis.…”

“…53 Therefore, the need for treatment in this subgroup of PAVSs is discussed controversial, especially because the natural course of this rare entity is unknown and epidural venous engorgement occurs infrequently. 21 Nevertheless, there seems to be a tendency towards prophylactic treatment in asymptomatic children to avoid the possible development of Figure 4. A 44-year-old male patient with Klippel-Trénaunay syndrome.…”

A rare case of treatment of Giant paravertebral arteriovenous malformation combined with idiopathic scoliosis

“…Farhat el al. [19] описали 2 случая паравертебральной АВМ у детей. Клиническим проявлением в данных случаях являлись лишь систолодиастолические шумы при аускультации в паравертебральной области у одного ребенка и в области 2-го межреберья -у второго.…”

“…Авторы также указали, что, по данным литературы, расположение мальформации спереди от тела позвонка может в дальнейшем стать причиной ортопедических нарушений. Кроме того, авторы отмечают возможность компрессии интраканальных нервных структур расширенными эпидуральными венами [19].…”