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De novo arteriovenous malformations: case report and review of the literature

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Abstract

Intracerebral arteriovenous malformations (AVMs) are traditionally recognized as congenital lesions. However, with the advent of frequent, noninvasive imaging of the brain, that notion has been challenged. We describe another patient with a de novo cerebral arteriovenous malformation and evaluate the reported literature for trends in the development of these lesions. Cases were selected from the English literature using the PUBMED database using the search term “acquired or de novo cerebrovascular arteriovenous malformations”. A total of seven patients (including the one reported in this study) with de novo arteriovenous malformations are reported. Majority of patients were female, and mostly diagnosed as children. Their mean age at diagnosis was 18 years (6–32), and the mean time from the initial intracranial study to the diagnosis of an AVM was 8 years (3–17). De novo formation of AVMs is being increasingly reported, especially in young females. We present only the seventh such case reported in the literature and challenge the traditional view that all arteriovenous malformations are congenital in nature.

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Authors and Affiliations

  1. Section of Neuroradiology, Department of Diagnostic Radiology, Yale University School of Medicine, New Haven, CT, USA

    Amit Mahajan

  2. Neurosurgery and Neuroendovascular and Skull Base Surgery Programs, Department of Neurosurgery, Yale University School of Medicine, P. O. Box 208082, New Haven, CT, 06520-8082, USA

    Tejas C. Manchandia, Grahame Gould & Ketan R. Bulsara

Authors
  1. Amit Mahajan
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  2. Tejas C. Manchandia
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  3. Grahame Gould
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  4. Ketan R. Bulsara
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Correspondence to Ketan R. Bulsara.

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Comments

Ulrich Sure, Essen, Germany

Mahajan et al. contribute another case of a de novo arteriovenous malformation (AVM). Their case is very well documented and nicely illustrated. It is the seventh case in literature, and it is noteworthy that four of these previously reported individuals were children.

The authors eloquently discuss the assumption that AVMs might not be congenital rather than acquired lesions. Their biology might well be dynamic, which has been shown in a number of clinical and clinicopathological reports. It remains questionable whether the pathobiology of the actual case (similarly as two previously reported cases) might be attributed to inflammatory insult as the authors discuss.

Discussing about the possible biology and embryology of AVMs should always include the quotation of a highly interesting question that was first discussed in the excellent review of Sean Mullan et al. (1) in 1996: “Why does the intrauterine and neonatal ultrasound fail to monitor pial AVMs?”... although it frequently diagnoses vein of Galen malformations, whose incidence should be definitely rarer than the incidence of pial AVMs.

Thus, in my eyes, it is important to report on cases of de novo AVMs in order to shift our neurosurgeons’ understanding of the biology of AVMs toward a new paradigm: “AVMs should not be considered as congenital pathologies”.

Reference

1. Mullan S, Mojtahedi S, Johnson DL, Macdonald RL (1996) Embryological basis of some aspects of cerebral vascular fistulas and malformations. J Neurosurg 85:1–8

Anton Valavanis, Gerasimos Baltsavias, Zürich, Switzerland

This case report presents the case of a brain AVM that was detected in a patient many years after a previous MRI did not show such a vascular abnormality. The case is interesting because it further corroborates the concept that cerebral arteriovenous shunts, representing an heterogeneous group of vascular lesions, constitute the manifestation of a complex process between several interacting components such as a causative trigger (genetic and/or environmental factors that produce a permanent change to the), a target (artery or vein), a revealing trigger (various mechanical, hormonal, pharmaceutical, hemodynamic, thermal, infectious, metabolic factors, or radiation that reveal the underlying, not yet expressed change), and the timing (early or late effect and expression) in the course of the ongoing vascular remodeling (1). Since “congenital” means dating from birth, it refers to the component of time and ignores the issue of whether an underlying latent flaw was present at birth but not expressed yet as a visible morphological alteration. Therefore, the so-called traditional view that brain AVMs are congenital has been certainly challenged since many years as a result of important evidence; but, this is only related to their morphological identification and not to a potential quiescent and otherwise congenital defect.

Two distinct entities that apparently result from a prenatal revealing trigger are the vein of Galen aneurysmal malformations that develop during embryonic period and dural sinus malformations that occur during the fetal period. The pial arteriovenous shunts most likely are presented as a result of a perinatal revealing trigger. Most other intracranial AV lesions, including brain AVMs, develop in the postnatal period and most likely after infancy and adolescence.

Reference

1. Lasjaunias P, ter Brugge KG, Berenstein A (2006) Surgical neuroangiography 3, clinical and interventional aspects in children. Second edition. Springer, Heidelberg, pp. 93–104

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Mahajan, A., Manchandia, T.C., Gould, G. et al. De novo arteriovenous malformations: case report and review of the literature. Neurosurg Rev 33, 115–119 (2010). https://doi.org/10.1007/s10143-009-0227-z

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  • DOI: https://doi.org/10.1007/s10143-009-0227-z

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