The Juxtaposition of a Capillary Telangiectasia, Cavernous Malformation, and Developmental Venous Anomaly in the Brainstem of a Single Patient: Case Report

Neurosurgery ◽  
2001 ◽  
Vol 49 (5) ◽  
pp. 1246-1250 ◽  
Author(s):  
Richard E. Clatterbuck ◽  
İlhan Elmacı ◽  
Daniele Rigamonti

ABSTRACT OBJECTIVE AND IMPORTANCE Capillary telangiectasias, cavernous malformations, and developmental venous anomalies are all vascular malformations that occur on the capillary-venous side of the cerebral circulation. The associations of capillary telangiectasias with venous malformations, cavernous malformations with venous malformations, and capillary telangiectasias with cavernous malformations have all been described; however, the association of all three lesions in a single patient is extremely rare. CLINICAL PRESENTATION A 52 year-old Caucasian woman presented to our clinic with an extended history of confusion, distorted visual perceptions, photophobia, neck pain, swallowing problems, and poor balance. The patient's examination was remarkable for difficulty concentrating, mild rotatory nystagmus, subtle decreased sensation over the left side of the face and body, and brisk reflexes. Review of the patient's magnetic resonance imaging examination demonstrated a cavernous malformation, a capillary telangiectasia, and a developmental venous anomaly located adjacent to one another in the brainstem. INTERVENTION Given the patient's complex constellation of symptoms and relatively mild neurological findings, it was difficult to ascribe any one of them to a specific vascular malformation. Conservative management of this patient's vascular malformations was decided upon. CONCLUSION Juxtaposition of these three different vascular lesions in the brainstem of an otherwise normal individual suggests a relationship among them. Although there are several theories that link similar associations through physiological mechanisms such as venous hypertension, we propose that a developmental event disrupting local capillary-venous pattern formation is a plausible alternative.

2021 ◽  
pp. 197140092110428
Author(s):  
Nimisha Parikh ◽  
Richard Williamson ◽  
Matthew Kulzer ◽  
Albert Sohn ◽  
Warren M Chang ◽  
...  

Cavernous malformations are angiographically occult vascular malformations. They are often associated with a developmental venous anomaly through poorly understood mechanisms. We present an unusual case of a gradually enlarging cavernous malformation associated with a developmental venous anomaly with arteriovenous shunting, suggesting venous hypertension or reflux as a potential cause of progressive growth.


Author(s):  
Nazlı Gülsüm Akyel ◽  
Ayşe Gül Alımlı ◽  
Mesut Sivri ◽  
Havva Akmaz Ünlü ◽  
Mehmet Tiftik

Abstract Purpose The main objective of this study is to demonstrate the diagnostic accuracy of susceptibility-weighted angiography (SWAN) in the diagnosis of slow-flow cerebral vascular malformations, especially developmental venous anomaly (DVA). We also aimed to determine the prevalence of DVAs identified by SWAN at 1.5 T. Methods We retrospectively evaluated 1,760 axial SWAN images for the diagnosis of low-flow vascular anomaly. Among them were 305 patients who underwent contrast-enhanced examination due to different indications. Postcontrast images were analyzed by different radiologists who were blinded to patients. The presence of DVA and other features such as location, length, depth, and direction of drainage vein was evaluated. Results Twenty-six patients with DVA had both SWAN and postcontrast images. There were four false-negative patients with SWAN. The sensitivity of the SWAN sequence was 84.6%. In addition, totally 77 DVA (4.36%), 2 capillary telangiectasia (0.11%), and 2 cavernous malformations (0.11%) were detected in 1,760 patients. Conclusion SWAN is an effective method for the diagnosis of developmental venous anomalies and other low-flow cerebral vascular malformations. Especially in the pediatric age, susceptibility-weighted imaging sequences are useful to limit contrast use.


Author(s):  
Hiren Patel ◽  
Janneke van Beijnum

Cerebral cavernous malformations (CCMs), known in addition as cavernomata or cavernous (haem)angiomata, are vascular malformations consisting of abnormally enlarged capillary cavities without intervening parenchyma (see section on capillary telangiectasia, this chapter) or arterial feeders with venous drainage (see arteriovenous malformations, Chapter 50). About 10–20% of CCMs are associated with a developmental venous anomaly (DVA), which is an extreme variation of the normal venous anatomy. CCMs can occur as result of an underlying genetic disorder, and genetic analysis is recommended in patients with a positive family history or multiple lesions. A conservative approach for asymptomatic CCMs seems justified in the large majority of cases and follow-up imaging is only needed if patients become symptomatic. Indications for surgical treatment are multiples haemorrhages, CCMs with mass effect causing symptoms, and patients with intractable epilepsy with an identified epileptogenic focus. Stereotactic radiosurgery can be considered in exceptional cases with a very high surgical risk.


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