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Intraventricular foramen of Monro cavernous malformation Nolan Winslow c, Kingsley Abode-Iyamah a,⇑, Oliver Flouty a, Brian Park c, Patricia Kirby b, Matthew Howard III a a b c

Department of Neurosurgery, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, IA 52245, USA Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, IA, USA Carver College of Medicine, University of Iowa, Iowa City, IA, USA

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Article history: Received 16 February 2015 Accepted 3 March 2015

Keywords: Cavernoma Cavernous angioma Cavernous malformation Hydrocephalus

a b s t r a c t We present a 64-year-old woman who was evaluated after being found unresponsive. Imaging revealed a foramen of Monro cavernoma resulting in hydrocephalus. Supratentorial cavernomas are most frequently found in the cerebral cortex, and although ventricular cavernomas do occur, they are rarely located in the foramen of Monro. Foramen of Monro cavernomas are extremely dangerous, requiring aggressive management when identified. Ó 2015 Elsevier Ltd. All rights reserved.

1. Introduction Cerebral cavernous malformations, also known as cavernous angiomas, cavernomas, or cryptic vascular malformations, are rare venous capillary bed abnormalities [1]. Histologically, they consist of thin walled sinusoid blood vessels lined by a single layer of endothelium associated with evidence of previous hemorrhage and gliosis of the surrounding brain parenchyma [2,3]. These lesions are present in 0.4–0.9% of the population and account for 5–10% of all central nervous system vascular abnormalities [4]. Intraventricular cavernomas are rare and account for 2.5–10.8% of cerebral cavernomas [5–7]. Rarer still are the foramen of Monro cavernomas, which have been reported only 15 times in the literature [4,8,9]. We present a 64-year-old woman with a large, partly calcified third ventricular mass at the foramen of Monro and resultant obstructive hydrocephalus. 2. Case report 2.1. History A 64-year-old woman with a history of hypertension, hyperlipidemia and bladder dysfunction was found unresponsive on the kitchen floor by her husband. She was transferred to the emergency department for evaluation. 2.2. Examination On examination, she was intubated and off sedation. Her pupils were 4 mm and non-reactive on the left, and 3 mm and non-reactive on the right. She had minimal cough and gag reflexes with decorticate posturing to noxious stimuli. A CT scan revealed a calcified mass in the foramen of Monro with associated hydrocephalus (Fig. 1A–C). 2.3. Post-operative course Her neurologic state did not improve post-ventriculostomy. An MRI revealed bilateral subacute infarcts of the thalami, splenium of the corpus callosum and right temporal lobe. Due to her poor ⇑ Corresponding author. Tel.: +1 319 356 1616. E-mail address: [email protected] (K. Abode-Iyamah).

neurological prognosis, the family elected for comfort care. She was extubated 8 days after admission and died 5 days later. An autopsy of her brain was performed and revealed that the hydrocephalus was due to a well circumscribed heterogenous and hemorrhagic mass with dystrophic calcification in the third ventricle. Microscopic examination revealed a cavernous hemangioma with evidence of previous hemorrhages and gliosis of the surrounding periventricular parenchyma (Fig. 2). 3. Discussion Cerebral cavernomas are vascular malformations that occur in 0.4–0.9% of the population and compose 5–10% of all central nervous system vascular malformations [4,10]. They are frequently found as intra-axial lesions in the supratentorial compartment [4,11,12] and present between the third and fourth decades of life with equivalent incidences amongst males and females [13]. Although the mechanism of cerebral cavernoma formation is not well understood, multiple associations have been noted. Greater than 90% of cerebral cavernomas develop spontaneously and typically present as solitary lesions. Approximately 6% of cerebral cavernomas are hereditary, often presenting with multiple lesions [1,14,15]. Three genes have been identified associated with hereditary cavernomas, the most common being the Krev interaction trapped 1 (KRIT1) gene located on chromosome 7 [1,16]. Additionally, pediatric patients who have undergone radiotherapy have also been reported to develop de novo cavernomas [2]. Radiographic diagnosis of a cerebral cavernoma is best accomplished with MRI; on T2-weighted imaging they appear popcorn-like (Fig. 1D) [17]. MRI with the gradient-echo sequence is especially sensitive and is suggested for detection of smaller lesions along with the diagnosis of familial cerebral cavernoma [15,18]. CT scans are less sensitive with up to 50% of patients having undetectable lesions [14]. The lesions can appear ovoid on CT scan and often enhance with contrast (70–94%), although they are not well visualized on angiography given their low flow nature [14,18]. Cavernomas typically show a well circumscribed lesion with various stages of hemorrhage (Fig. 3) [12]. Histopathologically, cavernomas consist of thin walled sinusoidal blood vessels with underdeveloped subendothelial interstitium and interstitial junctions [5]. Hemosiderin deposits and reactive gliosis are typically present, with some reports of amyloid deposition (Fig. 2) [2].

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Fig. 1. Non-contrast brain CT scans, (A) axial, (B) coronal and (C) sagittal, showing a large foramen of Monro cavernoma with areas of calcification causing hydrocephalus. (D) Axial T2-weighted MRI showing the foramen of Monro cavernoma and its popcorn-like appearance with hydrocephalus and transependymal edema. (E) Coronal and (F) sagittal T1-weighted post-contrast MRI showing the foramen of Monro mass without evidence of enhancement.

Fig. 2. (A) Post-mortem microscopic section showing numerous thin walled vascular spaces with hyalinized walls (broken arrow) and focal dystrophic calcification (black arrows; 40 magnification; hematoxylin and eosin [H&E]). (B) Post-mortem microscopic sections showing islands of gliosis (thin arrows) between vascular spaces (100 magnification; H&E), (C) Glial fibrillary acidic protein immunostaining of gliosis throughout the cavernoma (40 magnification; glial fibrillary acidic protein staining), and (D) gliotic brain with hemosiderin deposition adjacent to cavernoma (40 magnification; H&E). This figure is available in colour at www.sciencedirect.com.

Depending on their location, cavernomas are categorized as being supratentorial or infratentorial. Intraventricular cavernomas account for 2.5–10.8% of cerebral cavernomas [5–7]. Those located in the third ventricle are classified as suprachiasmatic, foramen of Monro, lateral wall or floor region depending on their origin and attachment to the ventricle [19]. Most lesions are asymptomatic and discovered at autopsy or during imaging for unrelated concerns [12]. When symptoms do occur, they vary

from headaches, seizures, memory deficit, pituitary dysfunction, nausea, vomiting, cranial nerve deficit and focal mass effect causing neurologic deficits [4,19]. The most concerning of these symptoms are those resulting from acute hydrocephalus with foramen of Monro cavernomas. There have been 15 previous case reports on foramen of Monro cavernomas, of which 13 patients presented with symptoms of hydrocephalus, as occurred in our patient (Fig. 1).

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Fig. 3. Coronal post-mortem gross specimen cuts of the brain showing the foramen of Monro cavernoma. There is an area of hemosiderin (black arrow) and also multiple areas of calcification (white arrow). This figure is available in colour at www.sciencedirect.com.

Management of cerebral cavernomas is based, in part, on the occurrence of hemorrhage and the presence of neurological symptoms. Watchful waiting is appropriate for patients in whom the cavernoma is an incidental finding on imaging [20,21]. However, surgical resection of cavernomas should be offered to patients with progressive neurological decline, recurrence of hemorrhage, or seizures [13]. For third ventricular cavernomas, surgical resection of the mass should be considered early, given the potential consequence of hemorrhage in this location [19]. In previous reports, resection has been accomplished via a transcortical and transcallosal approach, with and without the use of an endoscope [2,4,9,11,19,22–28]. Given the lack of neurologic improvement following emergent bilateral ventriculostomies and likely poor neurological outcome, the family of our patient reached the decision for compassionate extubation and she died following this. 4. Conclusion Our patient illustrates the dangers of these rare cavernoma at the foramen of Monro. While most foramen of Monro cavernomas present with symptoms of hydrocephalus, only three of the 15 previously reported patients presented after a hemorrhagic event. Additionally, in the reported third ventricular cavernomas there is a mortality rate of 8.5% [2,8,9,11,19,22–41]. This high mortality rate (compared to the mortality rates for other central nervous system cavernomas) supports the need for aggressive surgical management of lesions at this location. Although emergent bilateral ventriculostomies were performed, the reported patient’s neurological status did not improve. This case report emphasizes the dangers of foramen of Monro cavernomas and the need for urgent surgical resection when possible. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Fischer A, Zalvide J, Faurobert E, et al. Cerebral cavernous malformations: from CCM genes to endothelial cell homeostasis. Trends Mol Med 2013;19:302–8. [2] Kivelev J, Niemela M, Kivisaari R, et al. Intraventricular cerebral cavernomas: a series of 12 patients and review of the literature. J Neurosurg 2010;112:140–9. [3] McCormick WF, Nofzinger JD. ‘‘Cryptic’’ vascular malformations of the central nervous system. J Neurosurg 1966;24:865–75.

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http://dx.doi.org/10.1016/j.jocn.2015.03.043

Onyx embolization of a ruptured aneurysm in a patient with moyamoya disease Badih Daou, Nohra Chalouhi, Stavropoula Tjoumakaris, Robert H. Rosenwasser, Pascal Jabbour ⇑ Department of Neurosurgery, Thomas Jefferson University, Jefferson Hospital for Neuroscience, Third Floor, 901 Walnut Street, Philadelphia, PA 19107, USA

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Article history: Received 13 May 2015 Accepted 22 May 2015

Keywords: Aneurysm Endovascular treatment Moyamoya Onyx

a b s t r a c t We report a woman who presented with an intraparenchymal hemorrhage. Her cerebral angiogram showed a middle cerebral artery (MCA) M1 occlusion with multiple collaterals supplying the distal MCA territory, compatible with moyamoya disease. Also, an associated 8 mm dysplastic distal aneurysm fed by a left-sided P2 perforator was seen, collateral from the posterior cerebral artery. The aneurysm was successfully occluded with Onyx (ev3 Endovascular, Plymouth, MN, USA) embolization. The woman had an uneventful postoperative course. Aneurysm formation in patients with moyamoya disease represents a major hemorrhagic risk. Several treatment strategies exist including endovascular and surgical approaches. Patients with moyamoya disease who present with aneurysmal intracerebral hemorrhage should be treated to prevent rebleeding. Onyx embolization can be an effective treatment of aneurysms that are associated with moyamoya disease and would otherwise be difficult to treat surgically. Ó 2015 Elsevier Ltd. All rights reserved.

1. Introduction Moyamoya disease is well known to present with intracerebral, intraventricular or subarachnoid hemorrhage in adults [6]. These presentations are the result of an increased propensity for aneurysm development and the fragility of the vessels encountered with this disease [3,7]. The management of patients with hemorrhagic presentations remains controversial [2,7,11]. The incidence of aneurysm formation within the moyamoya adult population is 3–14%, and represents a major potential hemorrhagic risk for these patients [1,3]. Both surgical and endovascular approaches have been used in the management of aneurysms associated with moyamoya disease [7].

2. Case report A 53-year-old woman was brought to our institution after being found unresponsive. On physical exam, she opened her eyes to painful stimuli, was hemiplegic on the right side, and was moving

⇑ Corresponding author. Tel.: +1 215 9557000; fax: +1 215 5037038. E-mail address: [email protected] (P. Jabbour).

her left side spontaneously. Her pupils were equal, 4 mm bilaterally with a sluggish pupillary light reflex. A head CT scan showed an intraparenchymal hemorrhage in the left corona radiata and thalamus with extensive intraventricular hemorrhage. Given her age and lack of risk factors for stroke, a cerebral angiogram was performed which showed a middle cerebral artery (MCA) M1 occlusion with multiple collaterals supplying the distal MCA territory, compatible with moyamoya disease, and more extensive on the right side. Also, an 8 mm dysplastic distal aneurysm fed by a left-sided P2 perforator was seen, collateral from the posterior cerebral artery (PCA) (Fig. 1, 2). 2.1. Treatment Embolization of the perforator and the aneurysm with 0.1 mL Onyx-18 (ev3 Endovascular, Plymouth, MN, USA) was performed. A control angiogram with selective left vertebral artery injection showed 100% occlusion of the aneurysm (Fig. 3). The follow-up CT scans and MRI demonstrated a stable multicompartmental hemorrhage without any new hemorrhage or infarcts. Her postoperative course was uneventful and she was discharged to a rehabilitation facility following commands in all four extremities (3/5 strength on the right) and stable.

Intraventricular foramen of Monro cavernous malformation.

We present a 64-year-old woman who was evaluated after being found unresponsive. Imaging revealed a foramen of Monro cavernoma resulting in hydrocepha...
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