Developmental venous anomaly [Neurosurgery Wiki]

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====== Developmental venous anomaly ======

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DVAs are abnormalities in the veins that drain blood from the brain. While DVAs are usually considered benign, they can be associated with other cerebrovascular malformations. In most cases, DVAs do not cause symptoms but may be identified incidentally during imaging studies.

===== Key concepts =====

● a [[vascular malformation]] that is part of the venous drainage of the involved area with intervening brain present. Therefore direct treatment is rarely indicated

● low-flow, low-pressure

● usually demonstrable on [[angiography]] as a starburst pattern

● rarely symptomatic: seizures rare, hemorrhage even more uncommon. Venous infarcts may occur
(controversial)

● may have an associated [[cavernous malformation]] which is more likely to be symptomatic

Developmental venous anomaly (DVA) AKA [[venous malformation]] or (developmental) [[venous angioma]]. A tuft of medullary veins that converge into an enlarged central trunk that drains either to the deep or superficial venous system. The veins lack large amounts of [[smooth muscle]] and elastic. No abnormal arteries are found. There is neural parenchyma between the vessels. Most common in regions supplied by the [[MCA]] or in the region of the [[vein of Galen]]. They may be associated with a [[cavernous malformation]].

Non hereditary. These are low-flow and low-pressure.

Most are clinically silent, but rarely seizures and even less frequently hemorrhage may occur. Venous infarcts have been described, but maybe coincidental. If symptoms are present, look for an associated [[cavernous malformation]] ([[GRASS]] MRI images may reveal some cavernous malformations that might otherwise be occult).

----

They have mostly supplanted the more pedestrian [[venous angioma]]
((Lasjaunias P, Burrows P, Planet C, et al. Developmental
venous anomalies (DVA): the so-called venous
angioma. Neurosurg Rev 1986;9:233–42.)).

[[Congenital malformation]] of [[vein]]s which drain normal [[brain]]. They were thought to be rare before cross-sectional imaging but are now recognised as being the most common cerebral [[vascular malformation]], accounting for ~55% of all such lesions.

A [[venous anomaly]] is characterized by the caput medusae sign of veins draining into a single larger collecting vein, which in turn drains into either a [[dural sinus]] or into a deep ependymal vein. The appearance has also been likened to a palm tree.

====Pathology====

The aetiology of VAs remains uncertain, but may relate to arrested development of venous structures

Histologically they consist of a number of abnormally thickened veins with normal feeding arteries and capillaries

====Classification====

The most common locations are:

frontoparietal region (36-64%) , usually draining towards the frontal horn of the lateral ventricle
    
cerebellar hemisphere (14-27%) draining towards the fourth ventricle

However, DVAs can be seen anywhere, draining either superficially or deep.

see [[Spinal cord venous angioma]].

see [[Mesencephalic developmental venous anomaly]]
====Associations====
Lesions are usually solitary (75%), except in blue rubber bleb naevus syndrome
    
~20% (range 8-33%) of cases 2 are associated with cavernous malformations and are referred to as mixed vascular malformations (MVM)
there is an association with venous malformations of the head and neck.

===== Diagnosis =====

[[Developmental venous anomaly diagnosis]].

===== Treatment =====

In general, these should not be treated, as they are the venous drainage of the brain in that vicinity. If surgery is indicated for associated [[cavernous malformation]]s, the [[angioma]] should be left alone. Surgery for the angioma itself is reserved only for documented bleeding or for intractable [[seizure]]s that can be definitely attributed to the lesion.


===== Retrospective observational studies =====

Pei et al. in a retrospective observational study based on a pathetically small [[sample size]] (n=14 over 10 years)
((Pei J, Chen Q, Zhang H, Wei L, Wang S. Clinical characteristics and management of cerebral developmental venous anomalies accompanied with cavernous malformation. Neurosurg Rev. 2025 Jun 12;48(1):505. doi: 10.1007/s10143-025-03652-5. PMID: 40504280.))
, which in itself annihilates any potential for [[generalizability]]. The authors do not justify why such a long period yielded such a low number of cases, raising serious concerns about either poor case identification or a highly selective, possibly biased, inclusion process.

🧂 2. No Control, No Comparison, No Context

There is no control group, no comparative statistical analysis, and not even a basic attempt to correlate DVA characteristics with outcomes in a meaningful way. It reads like a long anecdote pretending to be science. Without a matched cohort or any stratification, this paper adds no value beyond what a decent case series from the 1990s might have contributed.

🪫 3. Absurdly Descriptive, Fatally Inconclusive

The “findings” are little more than clinical rehashes: some patients bled, some didn’t. Two had surgery. Some developed new CMs. So what? There’s no pathophysiological insight, no predictive factor exploration, no imaging correlation beyond general MR mention, and no meaningful discussion on when to operate. The core conclusion — that posterior fossa DVA hemorrhage may signal de novo CM formation — is speculative fluff without mechanistic grounding or statistical weight.

🧻 4. “Follow-up” ≠ “Evidence”

Follow-up periods ranging from 10 to 150 months with no standardization of imaging intervals, clinical assessments, or decision-making criteria render the data nearly useless. There's no structured follow-up protocol, which makes temporal associations (e.g. bleeding → new CM formation) unreliable and subject to retrospective reconstruction bias.

🎭 5. Underwhelming Insight, Overstated Significance

The conclusion claims novelty where there is none. The notion that hemorrhage may precede CM formation in DVAs is not new, and without histopathological confirmation or molecular investigation, the article offers no contribution to our understanding of venous angiogenesis, microenvironmental stress, or susceptibility to CM formation.

📉 6. Academic Contribution: Zero

This paper does not challenge existing paradigms, does not offer new diagnostic algorithms, does not clarify surgical indications, and does not provide imaging criteria for risk stratification. It is, at best, a passive catalog of incidental findings, and at worst, a missed opportunity to synthesize a meaningful review of a rare yet increasingly discussed phenomenon.

🧨 Final Verdict:

This paper is a classic example of academic inertia: a low-yield, low-impact descriptive report inflated by vague speculation and unearned conclusions.

It neither advances clinical management nor adds scientific clarity. Neurosurgical journals must uphold higher standards than allowing decade-long mini-cohorts to masquerade as evidence.

Recommendation: Should not have passed peer review without major revision.
Scientific Contribution Score: 2/10
Clinical Utility Score: 1/10



===== Case series =====

Fifty-two children (20 boys and 32 girls [median age: 6 years] were identified with DVAs. Their age distribution was as follows: 1.9% neonates (< 1 month), 11.5% infants (1 month to 1 year), 30.8% 1-5 years, 30.8% 5-12 years and 25% 12-16 years. The majority (92.3%) presented with asymptomatic DVAs identified incidentally. Overall, anatomical distribution revealed predilection for frontal region (42.3%) with other common sites being posterior fossa (17.3%) and basal ganglia (13.5%). Temporal (11.5%), parietal (9.6%) and occipital (5.8%) were the remainder. Associated cavernous malformations (CMs) were present in 3/52 (5.8%), and no DVAs were associated with aneurysms or arteriovenous malformations (AVMs). Three patients had more than one DVA. There were three deaths unrelated to DVAs over median follow-up of 3.8 years. Four patients (7.7%) suffered DVA-related intracranial haemorrhage presenting with neurological deficits. The ages of the children with DVA-related haemorrhages were 21 days, 2 years and 6 months, 7 years and 1 month and 11 years and 7 months. Left-sided DVA haemorrhages predominated (3/4, 75%). The relative risk of a cerebellar DVA haemorrhage compared to its supratentorial counterpart was 5.35 (OR 6.8, 95% CI 0.8-58).

DVA-related haemorrhage is sevenfold greater in the paediatric cohort compared to adults and is significantly associated with a cerebellar location and [[cavernous malformation]]s. There were no haemorrhages over a median period of 3.8 years of prospective follow-up
((Silva AHD, Wijesinghe H, Lo WB, Walsh AR, Rodrigues D, Solanki GA. Paediatric 
developmental venous anomalies (DVAs): how often do they bleed and where? Childs 
Nerv Syst. 2020 Jan 3. doi: 10.1007/s00381-019-04460-1. [Epub ahead of print]
PubMed PMID: 31900628.
)).

===== Case reports =====

A 27-year-old woman who presented with a sensorineural hearing loss followed by facial paresis. Magnetic resonance imaging (MRI) and computed tomography (CT) angiography revealed hematoma with adjacent converging veins showing a typical "caput medusa" sign in the left middle cerebellar peduncle, in favor of DVA. Due to the compression effect of hematoma, she underwent surgery. Hearing loss and facial paresis improved significantly during the postoperative follow-up.

Although DVA is mostly benign and asymptomatic, complications such as hemorrhage rarely occur. Hearing loss is an extremely rare presentation that can be attributable to the compression effect on the cranial nerve VII to VIII complex. In the case of compression effect or progression of symptoms, surgical intervention is necessary. A good clinical outcome could be expected postoperatively
((Ebrahimzadeh K, Tavassol HH, Mousavinejad SA, Ansari M, Kazemi R, Bahrami-Motlagh H, Jalili Khoshnoud R, Sharifi G, Samadian M, Rezaei O. The Sensorineural Hearing Loss Related to a Rare Infratentorial Developmental [[Venous Angioma]]: A Case Report and Review of Literature. J Neurol Surg A Cent Eur Neurosurg. 2021 Jun 14. doi: 10.1055/s-0041-1725960. Epub ahead of print. PMID: 34126638.)).

----

Although the association of developmental venous anomalies (DVAs) with [[cavernous malformation]]s is well documented, the association with [[arteriovenous malformation]]s (AVM) is unusual. The aim is to report an additional case and to review the concepts associated with these mixed malformations in order to guide patient management.

A case of AVM associated with a DVA was identified and a literature review was performed according to [[PRISMA]] guidelines.

Case report: In an 18-year-old man presented with sub-acute headache but with a normal neurological examination, the MRI-scan showed a right occipital DVA associated with hemosiderin spots evocative of earlier asymptomatic bleedings. The Digital Subtraction Angiography revealed a right parieto-occipital Spetzler-Martin Grade III AVM, fed by branches from the right middle and posterior cerebral arteries, with a superficial drainage flowing into a DVA that then joined the superior sagittal sinus. Multistep embolization was performed, leading to a partial reduction of the nidus, but preserving the DVA permeability. After a six-year follow-up. bleeding did not recur and the MRI aspect of the malformation was perfectly stable.

The co-occurrence of a [[DVA]] and an [[AVM]] is rare but has a higher bleeding risk than AVM alone (69% vs 38%) and must consequently be suspected when a DVA is revealed by a [[hemorrhage]], in the absence of associated [[cavernoma]]. These mixed malformations represent a therapeutic challenge that has to be tailored to the venous anatomy and to the malformation [[Spetzler]]-Martin grade. DVA permeability should be preserved to avoid deleterious venous infarction
((Picart T, Dumot C, Guyotat J, Eker O, Berhouma M, Pelissou-Guyotat I. Arteriovenous malformation drained into a developmental venous anomaly: a case report and up-dated literature review. Neurochirurgie. 2020 Oct 10:S0028-3770(20)30404-5. doi: 10.1016/j.neuchi.2020.08.003. Epub ahead of print. PMID: 33049289.))