Start Submission Become a Reviewer

Reading: A Spinal Cavernous Malformation in Multiple Cerebral Cavernous Venous Malformations Syndrome

Download

A- A+
Alt. Display

Images in Clinical Radiology

A Spinal Cavernous Malformation in Multiple Cerebral Cavernous Venous Malformations Syndrome

Authors:

Guillaume Vangrinsven ,

University Hospital Antwerp, BE
X close

Phyllis Vanwalleghem,

Monica Hospitals, BE
X close

Özkan Özsarlak

Monica Hospitals, BE
X close

Abstract

Teaching Point: In patients with familial multiple cavernous malformation syndrome with acute focal neurological deficit, a symptomatic spinal cavernous malformation must be included in the differential diagnosis.

How to Cite: Vangrinsven G, Vanwalleghem P, Özsarlak Ö. A Spinal Cavernous Malformation in Multiple Cerebral Cavernous Venous Malformations Syndrome. Journal of the Belgian Society of Radiology. 2023;107(1):1. DOI: http://doi.org/10.5334/jbsr.2986
67
Views
6
Downloads
  Published on 02 Jan 2023
 Accepted on 03 Dec 2022            Submitted on 20 Oct 2022

Case History

A 29-year-old male patient with a known history of familial multiple cavernous malformation syndrome (FCMS) presented with new-onset pyramidal signs during routing follow-up. Initial brain computed tomography (CT) showed multiple hyperdense cerebral lesions corresponding to known cerebral cavernous malformations (CCM), without apparent acute hemorrhage. Because of persisting symptoms, magnetic resonance imaging (MRI) of the thoracic spine was performed 10 days later. T2-weighted images (T2-WI) showed a intramedullary round hyperintense focus with a hypo-intense rim at the level of the 4th thoracic vertebral body (Th4) (Figure 1, white arrow), surrounded by hyperintense signal extending from Th2 to Th5 and medullary swelling (Figure 1, blue arrows). Intramedullary susceptibility artifacts (blooming artifacts) were encountered on axial T2*-WI (Figure 2, white arrow). In this clinical setting, findings are suggestive of a spinal cavernous malformation (SCM).

Sagittal T2-weighted image of the thoracic spine
Figure 1 
Axial T2*-weighted image at the level of Th4
Figure 2 

Patient was treated conservatively and recovered partially. Follow-up MR examinations after 4 and 10 months showed regression of the intramedullary edema with persistence of a central T2-hyperintense focus and blooming artifact on T2*-WI (Figure 3, white arrow).

Sagittal T2-weighted image of the thoracic spine
Figure 3 

Comments

Cerebral and spinal cavernous malformations represent clusters of enlarged capillaries in a ‘mulberry-like’ formation without intervening brain or medullary tissue, often surrounded by hemosiderin due to recurrent microhemorrhages. These malformations can either be sporadic or inherited. FCMS is more likely when multiple cavernomas are present. Different causative genetic mutations have been identified.

SCMs are rare and make up 5% of all spinal lesions. Clinical presentation can be variable with acute or insidious neurological deficits, and SCMs are associated with significant morbidity. Most lesions are located at the level of the thoracic spine, as is the case in our patient [1].

Unenhanced CT may show larger CCM as spontaneous hyperdense foci, but smaller lesions are often overlooked. MRI is the modality of choice for evaluating disease extension and associated hemorrhages. T2*-WI and susceptibility weighted imaging are superior compared to T2-WI for detecting blood breakdown products and one of these sequences should always be performed. Cavernous malformations are radiologically subdivided into 4 categories (Zabramski calssification). Typical (type 2) lesions have a mixed T2-signal centrally with a hypo-intense hemosiderin rim. Bi-directional T2- and T1-hypo-intense extension in the spinal cord representing intramedullary hemorrhage is often seen in SCM. Unlike in CCM, popcorn-like appearance is uncommon. Appearance on T1-WI is variable [1].

Most patients are treated conservatively but surgical resection may be necessary in cases with severe or recurrent symptoms. Patients with FCMS may benefit from follow-up using brain and spinal MRI although no widely used guidelines exist.

Competing interests

The authors have no competing interests to declare.

References

  1. Panda, A, Diehn, FE, Kim, DK, et al. Spinal cord cavernous malformations: MRI commonly shows adjacent intramedullary hemorrhage. J Neuroimaging. 2020; 30(5): 690–6. Epub 2020 May 28. DOI: https://doi.org/10.1111/jon.12738 

comments powered by Disqus