British Journal of Neurosurgery, December 2014; 28(6): 808–810 © 2014 The Neurosurgical Foundation ISSN: 0268-8697 print / ISSN 1360-046X online DOI: 10.3109/02688697.2014.922529

SHORT REPORT

Upper thoracic intradural-extramedullary cavernous malformation presenting as subarachnoid hemorrhage without spinal dysfunction: A case report and review of the literature Chuan-Yuan Tao, Min He, Yue-Kang Zhang & Chao You Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China

limb numbness, or weakness. He was admitted to a local hospital immediately after ictus. An urgent cranial computed tomography (CT) scan disclosed nothing abnormal, but lumbar puncture (LP) yielded bloody CSF, so SAH was confirmed. Diagnostic workup in our department including cerebral pan-angiography and magnetic resonance imaging (MRI) of brain revealed no evidence of aneurysm, vascular malformation, or tumorous lesion. Further whole spinal MRI scans were obtained, which showed an intraduralextramedullary mass lesion measuring 1.5 ⫻ 1.5 ⫻ 1 cm3 in size at the level of thoracic level (T2), and exhibited isointense to slightly hyperintense signal on T1-weighted images (Fig. 1a), heterogeneously hypo-intense signal surrounded by a black hemosiderin ring on T2-weighted images (Fig. 1b); contrast enhancement could not be documented (Fig. 1c). Spinal angiography vealed no vascular malformations.In view of the characteristic appearance on MRI, cavernous malformation (CM) was suspected. A T2 laminectomy was performed under microscope. A dark bluish mulberry-shaped lesion was identified ventrolaterally to the spinal cord on the left side, adherent to the T2 dorsal entry zone in the inferior pole (Fig. 2a). The mass was totally removed after sacrificing the T1 nerve root in its superior pole. Histopathology of excised specimen was consistent with a CM (Fig. 2b). The postoperative course was uneventful and the headache resolved completely; no sensorimotor deficit developed. The follow-up spinal MRI obtained 3 months after surgery demonstrated the total removal of the mass lesion (Fig. 1d).

Abstract A 45-year-old man had subarachnoid hemorrhage (SAH) which was confirmed by lumbar puncture, since it was negative on head computed tomography. The result of neurological examination was normal. Following pan-cerebral angiography and cranial magnetic resonance imaging (MRI) failed to find out the cause of bleeding. The whole spinal MRI revealed an intradural-extramedullary mass lesion at the upper thoracic level which was consistent with cavernous malformation after surgery. When patients presented with SAH of no spinal symptoms, the diagnosis of an intradural-extramedullary cavernous malformation is challenging. A whole spinal workup should be considered in a patient with spontaneous SAH when bleeding from intracranial origin is carefully excluded. Keywords: intradural-extramedullary cavernous malformation; spinal dysfunction; subarachnoid hemorrhage

Introduction Intradural-extramedullary cavernous malformation (IECM) is extremely rare. To date, only 30 cases have been reported. Most of these lesions are located in the lower spinal region, and only a few of them presented with subarachnoid hemorrhage (SAH). There are 3 cases of IECM of upper thoracic location causing SAH in the literature, in which sensorimotor deficit or sphincter change was observed right after the ictus of the first hemorrhage or repeated hemorrhage.1–3 These spinal dysfunctions lead to the work-up of spinal column to clarify the bleeding source from IECM. Herein, we report an exceptional case of upper thoracic IECM manifesting SAH without any spinal symptoms or signs, and discuss the diagnostic procedure to avoid misdiagnosis and prevent recurrent bleedings.

Discussion CMs can occur anywhere along the neuraxis and constitute 5–16% of all spinal angiomatous anomalies. IECM is the rarest type of spinal CM, and only 30 cases have been reported. They have been described most commonly in thoracolumbar

Case report A 43-year-old man experienced a sudden onset of occipital headache, accompanied by nausea without coma, seizure,

Correspondence: Prof. Chao You, Department of Neurosurgery, West China Hospital, Sichuan University, 37 Guoxue Alley, Chengdu, Sichuan 610041, P. R. China. Tel: ⫹ 8613402842965. E-mail: [email protected] Received for publication 12 June 2013; accepted 5 May 2014

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Fig. 1. Preoperative sagittal MRI (a) of cervicothoracic spine showing an oval mass with an iso-intense signal surrounded by a ring-shaped hyperintense signal (arrow). Hemosiderin ring on T2-weighted imaging (arrow, b). No enhancement on T1-weighted imaging (arrow, c). Postoperative sagittal T1-weighted MRI of spine (d) demonstrating that the lesion was completely removed.

and lumbar region, and typically present with symptoms associated with spinal nerve or spinal cord compression. IECM in upper thoracic region is rare, with only 4 cases reported previously.1–3 Of these, 3 patients suffered from SAH and all of them were manifested with spinal syndromes. Our case is the fourth report of upper thoracic location with SAH, the first report of no spinal dysfunction when SAH occurred in that area. SAH from spinal tumor is unusual which may or may not become visible in the cerebral CT image. In the absence of spinal symptoms or signs, bleeding source of SAH of uncertain origin is quite difficult to detect. In such circumstances, the value of spinal MRI in angiogram negative SAH is debatable. On the one hand, as SAHs from spinal canal are uncommon and mostly manifest with spinal dysfunctions, when spine MRI is widely applied in patients without spinal symptoms or signs, a very low diagnostic yield is expected. However, on the other hand, in selected bleeding

patterns including CT-negative but LP-positive SAH rather than the classic aneurysmal type, just like in our case, this investigation would produce a positive rate of 5% as reported by Rogg et al.4 Nonetheless, a large study is necessary to further decide the sensitivity and specificity of such examination. In our opinion, given the noninvasiveness and safety as well as its usefulness in spinal tumor and vascular lesion detection, the spinal MRI should be considered especially in those SAHs with atypical patterns. Furthermore, a delay in diagnosis in some surgically curable conditions will lead to repeated hemorrhage,1 or sometimes may be catastrophic.

Conclusion IECMs are rare, particularly in the upper thoracic region. They can manifest as SAHs without spinal symptoms or

Fig. 2. An intraoperative photograph (a) disclosing a dark bluish mulberry-shaped cavernous malformation ventrolaterally to spinal cord. Histologically (b), the lesion was composed of vascular channels, covered by a single layer of endothelium (hematoxylin and eosin stain, 100⫻ magnified).

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signs which makes their accurate diagnosis very challenging. A whole spinal workup should be considered in a patient with spontaneous SAH when bleeding from intracranial origin is carefully excluded, whether spinal symptoms exist or not.

Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.

References 1. Heimberger K, Schnaberth G, Koos W, Pendl G, Auff E. Spinal cavernous haemangioma (intradural-extramedullary) underlying repeated subarachnoid haemorrhage. J Neurol 1982;226:289–93. 2. Mori K , Ishii H, Tomita Y, et al. Intradural-extramedullary spinal cavernous angioma-case report. Neurol Med Chir (Tokyo) 1991;31:593–6. 3. Sharma R, Rout D, Radhakrishnan VV. Intradural spinal cavernomas. Br J Neurosurg 1992;6:351–6. 4. Rogg JM, Smeaton S, Doberstein C, et al. Assessment of the value of MR imaging for examining patients with angiographically negative subarachnoid hemorrhage. Am J Roentgenol 1999;172:201–6.

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Upper thoracic intradural-extramedullary cavernous malformation presenting as subarachnoid hemorrhage without spinal dysfunction: a case report and review of the literature.

A 45-year-old man had subarachnoid hemorrhage (SAH) which was confirmed by lumbar puncture, since it was negative on head computed tomography. The res...
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