Childs Nerv Syst DOI 10.1007/s00381-014-2397-4

CASE REPORT

Spinal epidural venous angioma: a case report and review of the literature Liang Wu & Tao Yang & Xiaofeng Deng & Chenlong Yang & Yulun Xu

Received: 1 February 2014 / Accepted: 26 February 2014 # Springer-Verlag Berlin Heidelberg 2014

Abstract Background As a vascular malformation, venous angioma in the spinal cord is extremely rare. To our knowledge, there are only five case reports in the literature, and it has not been previously reported in the pediatric age group. Case report In this paper, we report on a 3-year-old patient who presented with progressive weakness in his left upper limb. Spinal magnetic resonance imaging (MRI) revealed an epidural cystic mass at the C6–T2 level. The lesion was diagnosed as venous angioma after total removal with laminectomy. Postoperatively, the patient remained symptom free, and no tumor recurrence was confirmed based on MRI at the time of the 18-month follow-up. The clinical, radiological, surgical, and pathological features of this abnormality are discussed, and all six reported cases were reviewed. Conclusion Venous angiomas should be included in the differential diagnosis of spinal cystic lesions in children. A definitive diagnosis is difficult based on MRI alone. This rare lesion is amenable to surgery, and gross total removal (GTR) is usually achievable due to a well-demarcated dissection plane. A good clinical outcome after GTR can be expected. Keywords Venous angioma . Epidural . Spinal cord . Vascular malformation

Introduction Vascular malformations are common lesions in the central nervous system and can be histopathologically classified into capillary, cavernous, arteriovenous, and venous types [2, 4, 5]. L. Wu : T. Yang : X. Deng : C. Yang : Y. Xu (*) Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, No.6 Tiantan Xili, Dongcheng District 100050 Beijing, China e-mail: [email protected]

Of these, venous angiomas are the most frequently encountered cerebral vascular malformations, which are characterized by clusters of venous radicles that converge into collecting veins [3, 10]. Vascular lesions involving the spinal cord are uncommon, which are mostly located in the intradural space. They account for about 6–7 % of all spinal intradural tumors [2, 7, 8]. Extradural vascular malformations are rare, and most of them are cavernous angiomas. However, spinal extradural venous angiomas are extremely rare. We report an epidural spinal venous angioma in a 3-year-old boy. The clinical manifestation, imaging and pathological findings, and treatment of the patient are discussed with a review of the literature.

Case report A 3-year-old boy presented to our outpatient clinic with a 2month history of progressive worsening weakness in his left upper limb. No sensory change and sphincter symptoms were noted. A neurological examination revealed that muscle tone was decreased in the left upper limb, and the muscle power was grade 4/5 (classified by the Medical Research Council grading system). Deep and superficial sensations were normal, and no amyotrophy was found. A positive Babinski sign was noted in his left side. Preoperative magnetic resonance imaging (MRI) of the patient’s cervical spine demonstrated an extradural lesion in the C6 to T2 spinal segments (Fig. 1). The mass was iso-/hyperintense on T1-weighted images and mixed iso-/hypo-intense on T2-weighted images compressing the cord laterally. A contrastenhanced MRI of the lesion showed heterogeneously marked enhancement after gadolinium administration. Abnormal flow void signals were not observed. According to the clinical findings and MRI features of the tumor, the preoperative diagnosis was considered to be epidural tumor with apoplexy.

Childs Nerv Syst Fig. 1 a Sagittal T1-weighted MRI showing an iso-/hyperintense signal epidural mass at C6–T2 level. b Sagittal T2weighted MRI showing a mixed iso-/hypo-intense signal epidural mass at the C6–T2 level. c Sagittal contrast-enhanced T1weighted MRI illustrating heterogeneously marked enhancement of the lesion. d Coronal contrast-enhanced T1weighted MRI illustrating the lesion compressing the cord with heterogeneously marked enhancement. e Axial contrastenhanced T1-weighted MRI illustrating the lesion compressing the cord laterally

The patient underwent a C7 to T2 laminectomy using a posterior approach. A cystic, dark red, vascular epidural mass was found on the left side of thecal sac. The intraoperative findings showed that the lesion had complete, tense, and thickened capsule that was well demarcated from the dura mater but dense to T1 nerve root. The cyst contents were found to be dark reddish fluid after suction with a syringe, which appeared to be old hemorrhagic blood. Because of the complete deflation of the cyst and well-defined margins with the dura, total resection and preservation of the T1 nerve root were achieved under spinal evoked-potential monitoring. A histopathological examination revealed that the mass was composed of cystic dilated vessels with multiple layers of smooth muscle cells in the walls. Mitosis and necrosis were absent. The immunohistochemical examinations revealed that the cells of cystic walls were positive for CD34, Vimentin, and smooth muscle actin (SMA), indicating that the blood vessels were the venous type, but negative for S-100, neurofilament, myoglobin, and epithelial membrane antigen (EMA). All of the findings were consistent with a diagnosis of venous angioma (Fig. 2). In the immediate postoperative period, the weakness in the patient’s left upper limb worsened (muscle power of grade 3/5) but improved to a satisfactory level within 4 weeks

postoperatively. During 18 months of follow-up, the boy remained symptom free, and no recurrence was found on MRI examination (Fig. 3).

Discussion Since Decker et al. described the first case in 1978, only five cases of intraspinal venous angiomas have been reported in the English literature [3, 7, 10]. Table 1 summarizes the clinical features of previous cases and our patient. Of the six reported patients, the male/female ratio of intraspinal venous angiomas (1:1) suggests no gender predilection. The tumors tend to arise from the lumbar and cervicothoracic spinal regions. In the reported cases, the lesions in five cases were epidural, and one was intradural and extramedullary. The signs and symptoms of venous angiomas are consistent with those of other common epidural spinal tumors. The clinical presentations of venous angiomas include somatic pain, numbness, and weakness in the limbs. Moreover, radiculopathy is the most common complaint, which suggests that the nerve root is involved. The symptoms usually have sudden or acute onset, which evolve over a period of weeks to months. Regardless of the duration, rapid deterioration

Childs Nerv Syst Fig. 2 a Photomicrographs of the surgical specimens illustrating cystic dilated vessels with multiple layers of smooth muscle cells in the walls. H&E×100. b The epithelial cells of cystic walls showing positivity for CD34. CD34×100. c The cells of cystic walls showing positivity for SMA. SMA×100. d The cells of cystic walls showing negativity for EMA. EMA×100

occurred in a few cases, most likely caused by intracystic hemorrhage [3, 10]. The MRI features of intraspinal venous angiomas are variable and could be misleading, as shown in the previously reported cases [3, 7, 9, 10]. Most of the cases involved epidural lesions, and cystic formation could be found Fig. 3 a Postoperative sagittal contrast-enhanced T1-weighted MRI of the patient showing complete removal of the lesion. b Postoperative axial contrastenhanced T1-weighted MRI of the patient showing complete removal of the lesion. c Sagittal contrast-enhanced T1-weighted MRI of the patient showing no recurrence after 18 months. d Axial contrast-enhanced T1weighted MRI of the patient showing no recurrence after 18 months

commonly. The signals of venous angiomas ranged from iso- to hyper-intensity on both T1-weighted and T2weighted images. The lesions could show no or irregularly heterogeneous enhancement after gadolinium administration, and all the cases had well-defined tumor margins. These MRI findings are similar to those of spinal cystic lesions, such as

C6–T2, ED 2 months 3/M Present case

5 months L4–5, ED 2 weeks L3, ED 4 years; worsening T12–L1, ID 2 weeks before and EM admission 18/M 20/M Nishimura/2013 [10] 54/F

C7–T1, ED 1 month

C cervical, ED epidural, EM extramedullary, GTR gross total removal, Hyper hyper-intensity, Hypo hypo-intensity, ID intradural, Iso iso-intensity, L lumbar, NA not available, T thoracic, WI weighted image

GTR; good recovery at 18 months Heterogeneous +/+ Iso/hypo Iso/hyper

GTR; no recurrence GTR; no recurrence GTR; good recovery at 2 years +/NA +/NA +/+ Iso Hyper with rim Peripheral Iso Hyper with rim Peripheral Slight hyper Iso No

GTR; no recurrence +/NA Hyper with rim Peripheral Iso

NA NA

43/F Lee/2007 [7]

T1WI

NA

Right lower abdomen and groin radiating pain Left shoulder and left arm radiating pain Right gluteal to foot dorsum pain Right leg pain Low-back pain; left-sided pain and numbness in the hip, thigh, and inguinal and perianal regions Left upper limb weakness L1–2, ED 6 weeks 65/F Decker/1978 [3]

Age (years)/ Duration of illness Tumor location Presentations sex Author/year

Table 1 Summary of previously reported intraspinal venous angiomas

MRI findings

T2WI

After Gd

NA/+

GTR; good recovery

Cystic/nerve Treatment and outcome root attachment

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arachnoid cysts, neurenteric cysts, ependymal cysts, and ventriculus terminalis. In our case, variations in the preoperative MRI findings led to difficulties with the diagnosis. Furthermore, cystic fluid intensity may be distinct on MRI depending upon the different contents within the cyst (protein or hemorrhage), even within the same pathology. Thus, a definitive diagnosis of venous angiomas was difficult based on MRI alone, and a complete histopathological examination was required to differentiate between other intraspinal cystic lesions. Histologically, spinal vascular lesions can be definitely differentiated by pathological examinations. The classic description of the venous angioma is a cluster of venous radicles that converge into a collecting vein, characterized by many irregular and cystic vessels with thin walls and surface-lining epithelial cells [10]. Venous angiomas are currently accepted as developmental venous anomalies. Moreover, immunohistochemistry is often a necessary adjunct for the differential diagnosis. Immunostaining for CD34 has been recognized as the most suitable and reliable diagnostic marker of endothelial cells. The positivity for SMA but negativity for EMA in the vessel walls favors diagnosing endothelial cells over arachnoidal or ependymal cells. All these features can differentiate from arachnoid cysts, neurenteric cysts, and ependymal cysts. However, the information regarding the incidence, natural history, and pathogenesis for venous angiomas in the CNS is still unknown [1, 6, 13]. Since venous angioma is histologically benign and usually well marginated, gross total removal (GTR) using a microsurgical technique based on protection of spinal function should be attempted during surgical exploration [10–12]. Intraoperatively, the affected nerve root should be preserved as far as possible. However, if the lesion membrane is confirmed as part of the nerve root sheath itself, transecting the entire affected nerve root for complete resection is acceptable. Selective spinal angiography and preoperative embolization may demonstrate vascularity and feeding vessels of venous angiomas and reduce intraoperative bleeding. However, there are few relevant reports in the previous cases, and the effectiveness of these approaches for spinal epidural venous angiomas is still unknown. The postoperative follow-up evaluations showed that good recovery was obtained and no tumor recurrence was noted. Therefore, spinal epidural venous angiomas are amenable to surgery, and a good clinical outcome after GTR can be expected.

Conclusion The present case and review suggest that spinal epidural venous angioma, an extremely rare vascular malformation, should be considered in the differential diagnosis of

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intraspinal cystic lesions in children. Definitive preoperative diagnosis is difficult based only on MRI due to lack of highly specific appearances. Total resection using the standard microneurosurgical techniques is advised for treatment of spinal epidural venous angioma due to a well-demarcated dissection plane, and a good clinical outcome after GTR can be expected. Conflicts of interest The authors have reported no conflicts of interest.

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Spinal epidural venous angioma: a case report and review of the literature.

As a vascular malformation, venous angioma in the spinal cord is extremely rare. To our knowledge, there are only five case reports in the literature,...
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