The efficacy of resection of an intradural extramedullary foramen magnum cavernous malformation presenting with repeated subarachnoid hemorrhage: a case report
© The Author(s). 2017
Received: 20 July 2016
Accepted: 29 January 2017
Published: 9 March 2017
Intradural extramedullary cavernous angiomas of the central nervous system are a rare type of cavernous angioma, but they can cause fatal subarachnoid hemorrhage. The efficacy of resection for this type of cavernous malformations remains uncertain. This is the first report to recommend surgical resection of these types of lesions regardless of the fatal condition.
Our patient was a 70-year-old Japanese man who experienced a sudden onset of an occipital headache, followed by bilateral abducens nerve palsy. Magnetic resonance imaging revealed a small amount of hemorrhage in both of the lateral ventricles and an intradural extramedullary mass lesion in the left side of his foramen magnum. Two weeks after the appearance of initial symptoms, he became comatose. A computed tomography scan showed an increase in the subarachnoid intraventricular hemorrhaging and of the acute hydrocephalus. Following ventricular drainage, total tumor resection was performed using the lateral suboccipital transcondylar approach in conjunction with a first cervical hemilaminectomy. We observed a grape-like vascular-rich tumor with calcification that was adhering tightly to the wall of his left vertebral artery. A histopathological examination of the surgery specimen identified it as a cavernous angioma. After placement of a ventriculoperitoneal shunt and 2 months of rehabilitation, he recovered completely.
An intradural extramedullary foramen magnum cavernous malformation is quite rare. The fragile surface of our patient’s lesion was causing repeated subarachnoid hemorrhage and consequently progressive fatal neurological deterioration. Surgical resection of the lesion to prevent repeated hemorrhage was performed and he recovered fully. Therefore, we recommend surgical resection of the lesion regardless of the potentially fatal condition.
KeywordsIntradural extramedullary cavernous angioma Cavernous hemangioma Foramen magnum Craniocervical junction Abducens nerve palsy Subarachnoid hemorrhage
Cavernous angiomas are benign vascular lesions and can occur anywhere in the central nervous system (CNS). The majority manifest as intra-axial lesions [1, 2]. The damage caused by the hemorrhaging is usually not fatal; however, cavernous angiomas cause recurrent hemorrhages and hemostasis. Intradural extramedullary cavernous angiomas of the CNS are a rare form of cavernous angiomas. They can cause neurological deficits due to mass effect, cranial nerve palsy, and hemorrhages [1, 3–5]. There are no clinical reviews on extramedullary foramen magnum cavernous malformations. Here we report a case of intradural extramedullary foramen magnum cavernous malformation presenting with repeated subarachnoid hemorrhage (SAH) and progressive neurological deterioration. This is the first report to recommend surgical resection of the lesion regardless of the potentially fatal condition.
His postoperative course was uneventful. He underwent placement of a ventriculoperitoneal shunt for hydrocephalus. Moreover, he showed gradual improvement in his state of consciousness as well as in his bilateral abducens nerve palsy and tetraparesis. After 2 months of rehabilitation, he had fully recovered.
Summary of four cases of extramedullary foramen magnum cavernous malformation
Mocco et al., 2005 
Occipital H/A, photophobia,
Palani, 2012 
Occipital H/A, subtle bilateral corticospinal tract sign
Eicker et al., 2015 
Sudden occipital H/A, bilateral abducens nerve palsy, slight right motor deficit
Previous reports have not described a cavernous malformation originating from the foramen magnum. According to a literature review on spinal cavernous malformations , extramedullary cavernous malformations arise from the nerve roots, dura mater, pia mater, and dentate ligament. In this case, we could not determine the origin of the malformation. We found the dark red surface of the lesion through the thin dentate ligament. The lesion was adjacent to the other cerebral structures, and we were able to detach it without cutting the pial surface, the accessory nerve, or the first cervical root. The lesion adhered tightly to the wall of the VA with organized tissue. So, we should take into consideration the wall of the VA as the potential origin of the lesion.
The annual hemorrhage risk rate was determined to range from 0.25 to 3.1 % per patient [3, 4, 6]. The risk of hemorrhage is higher in female patients or in patients with a prior history of hemorrhage . The mechanisms leading to major hemorrhaging remain controversial, given that cavernous hemangiomas are low-flow and low-pressure lesions . In this case, we propose that the lesions’ physical characteristics and its location were contributing factors to inducing hemorrhaging. Operative and histopathological findings confirmed that the surface of the lesion consisted of fragile vessel walls, which can easily cause a hemorrhage. In some cases of spinal extramedullary cavernous malformations [7, 8], SAH may result from the restricted mobility of the lesion. Consequently, the lesion is squeezed between its central calcification and its dynamic surroundings at the foramen magnum.
Although an intradural extramedullary foramen magnum cavernous malformation is very rare, the fragile surface of this lesion was causing repeated SAH. In spite of the potentially fatal condition, our patient recovered fully because recurrence of SAH was prevented; therefore, surgical resection is strongly recommended for intradural extramedullary cavernous malformations.
Intradural extramedullary foramen magnum cavernous malformation causes repeated SAH and progressive fatal neurological deterioration. We recommend surgical resection of the lesion to prevent repeated hemorrhage regardless of the potentially fatal condition.
Central nervous system
Magnetic resonance imaging
The authors thank Dr Satoshi Baba of the Department of Diagnostic Pathology, Hamamatsu University Hospital for his assistance in the pathological diagnosis in this case.
There were no funding sources for this report.
TO, NS, and TS devised the study design, obtained the data, created the figures, drafted the manuscript, and carried out a critical review of the literature. HK and HN revised the manuscript and added comments for the discussion section. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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