Multiple spinal intramedullary cavernous angiomas with bleeding episode mimicking an intramedullary tumor

Abstract

Intramedullary cavernous angioma is a rare vascular malformation compared to cerebral cavernous malformation. The incidence of cavernous angioma is about 3 - 5% of all central nervous system lesions, 5 - 12% of all spinal vascular lesions, and 1% of all intramedullary lesions in pediatric patients. Although intramedullary cavernous angioma has the same histological picture as cerebral cavernous angioma, the natural history, and surgical approach are different from cerebral cavernous angioma. Due to its location in the eloquent area of the spinal medulla, a slight change in the size of the lesion can affect the neurological function of the patient. We describe a case of an intramedullary cavernous angioma with hemosiderin post bleeding, located in the cervical cord which was initially misdiagnosed as hemorrhagic ependymoma. On whole spine MR imaging we also found an intramedullary cavernous angioma in the lower thoracal cord at the T12 level with mild hemorrhage. Abdominal MRI showed cavernous angiomas in both kidneys. The patient underwent surgical treatment with removal of the lesion in the cervical cord and T12 level, with histopathologic findings consistent with cavernous angioma. No malignancy was detected. Post-surgery, the symptoms gradually improved. Symptomatic intramedullary cavernous angioma tends to bleed repeatedly and being unstable. Early diagnosis and surgical treatment can prevent rebleeding and more severe symptoms.

Keywords: bleeding episode; cavernoma; cavernous angioma; intramedullary; magnetic resonance imaging.

Figure 1

6-year-old boy with spinal intramedullary cavernous angiomas. Findings: a) T1 TSE b) T2 TSE and c) T1 TSE Dixon post-contrast sagittal spinal MRI demonstrating two intramedullary lesions located on cervical level (orange arrow) and thoracic level (blue arrow). They show heterogeneous signal intensity at T1 TSE and T2 TSE and minimal enhancement in T1 TSE Dixon post-contrast. There is hypointense rim suggestive of hemosiderin indicating the presence of chronic bleeding (yellow triangle). Technique: (a–c) Contrast-enhanced whole spine MRI. GE Optima 360 1.5T GE. Sagittal T1 TSE (TE: 25 TR: 2000 Thickness: 3mm). Sagittal T2 TSE (TE: 81 TR: 4000 Thickness: 3mm). Sagittal T1 TSE Dixon post-contrast (TE: 10 TR: 700 Thickness: 3mm). intravenous contrast: 5ml Gadoteric acid (Dotarem)

Figure 2

6-year-old boy with spinal intramedullary cavernous angiomas. Findings: a) T1 TSE b) T2 TSE sagittal spinal MRI demonstrating varying age of blood product with late subacute hemorrhage (blue arrow) is shown as hyperintense signal in T1 TSE and T2 TSE, chronic bleeding (red arrow) shows as hypointense signal in T1 TSE and T2 TSE, and adjacent cord edema (orange triangle). Technique: (a–b) Whole spine MRI. GE Optima 360 1.5T GE. Sagittal T1 TSE (TE: 25 TR: 2000 Thickness: 3mm). Sagittal T2 TSE (TE: 81 TR: 4000 Thickness: 3mm).

Figure 3

6-year-old boy with spinal intramedullary cavernous angiomas. Findings: a) Sagittal T2 TSE, b) axial T2 TSE spinal MRI in cervical level, c) Sagittal T2 TSE and d) axial T2 TSE spinal MRI in thoracic level demonstrating an eccentric lesion with popcorn appearance and hypointense rim suggestive of hemosiderin (red arrow) at the cervical and (yellow arrow) thoracic levels. Technique: (a–d) Whole spine MRI. GE Optima 360 1.5T GE. Sagittal T2 TSE (TE: 81 TR: 4000 Thickness: 3mm). Axial T2 TSE (TE: 106 TR: 5830 Thickness: 3mm).

Figure 4

6-year-old boy with spinal intramedullary cavernous angiomas. Findings: a) Coronal T2 TSE b) axial T2 TSE and c) coronal T2 TSE abdominal MRI demonstrating cavernous angiomas in upper and lower pole of the right kidney (purple arrow), hypoplasia of the left kidney with a cavernous angioma in the upper pole (orange arrow). Technique: (a–c) Abdominal MRI. GE Optima 360 1.5T GE. Coronal T2 TSE (TE: 81 TR: 3200 Thickness: 3mm). Axial T2 TSE (TE: 106 TR: 5830 Thickness: 3mm).

Figure 5

6-year-old boy with spinal intramedullary cavernous angiomas. Intraoperative photograph revealing an intramedullary lesion with chronic blood.

Figure 6

6-year-old boy with spinal intramedullary cavernous angiomas. Histopathology of the surgical exited spinal intramedullary lesion. a) Hematoxylin and Eosin stain (magnification 200x) and b) (magnification 400x) Cervical part lesion show fibrin and macrophage with hemosiderin, no malignancy was detected. c) Hematoxylin and Eosin stain (magnification 200x) and d) (magnification 400x) Distal XII thoracic part lesion show macrophage with hemosiderin pigment and some capillary vessels, no malignancy was detected.