- Email: [email protected]
Cavernous Angioma of the Cerebral Aqueduct
Accepted Manuscript Cavernous angioma of the cerebral aqueduct Alberto Feletti, MD, PhD, Stavros Dimitriadis, MD, Giacomo Pavesi, MD PII:
S1878-8750(16)31244-X
DOI:
10.1016/j.wneu.2016.11.096
Reference:
WNEU 4906
To appear in:
World Neurosurgery
Received Date: 28 August 2016 Revised Date:
15 November 2016
Accepted Date: 16 November 2016
Please cite this article as: Feletti A, Dimitriadis S, Pavesi G, Cavernous angioma of the cerebral aqueduct, World Neurosurgery (2016), doi: 10.1016/j.wneu.2016.11.096. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
1
ACCEPTED MANUSCRIPT Cavernous angioma of the cerebral aqueduct Alberto Feletti, MD, PhD, Stavros Dimitriadis, MD, Giacomo Pavesi, MD
Corresponding author:
Dr. Alberto Feletti, MD, PhD
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Department of Neurosurgery, NOCSAE Hospital of Modena, Modena, Italy
SC
Department of Neurosurgery, NOCSAE Modena Hospital Via Giardini 1355, 41126 Baggiovara (MO), Italy
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Email: [email protected] Tel: +390593961471
Fax: +390593962408
ETV
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Abbreviation list
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Key words: cerebral aqueduct, cavernous angioma, cavernoma, intraventricular, hydrocephalus,
central nervous system
CSF
cerebrospinal fluid
ETV IVCs
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CNS
endoscopic third ventriculostomy intraventricular cavernomas
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ACCEPTED MANUSCRIPT Abstract Background. Among the rare intraventricular cavernomas (IVCs), purely intraaqueductal cavernomas are exceptional.
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Case description. We report a unique case of a 62-year-old patient who presented to our clinics complaining of progressive headache, memory loss, gait instability, and urinary incontinence. MR imaging showed the presence of a mass lesion located in the lumen of the cerebral aqueduct,
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associated with triventricular hydrocephalus.
Conclusions. We discuss the rationale that led us to treat hydrocephalus with neuroendoscopy,
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which offered the possibility to directly inspect the intraaqueductal lesion, make the diagnosis of
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cavernoma, and treat symptoms due to hydrocephalus without increasing the risk of bleeding.
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ACCEPTED MANUSCRIPT Introduction Cavernous angiomas (or cavernomas) represent 5-10% of the vascular malformations of the central nervous system (CNS). They are usually diagnosed between the 2nd and the 5th decade of life and
hemorrhages are frequent although often subclinical.1,2
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can occur sporadically or as part of a familial syndrome. Due to their histological structure, small
Intraventricular cavernomas are rare (2.5-10.8% of cerebral cavernomas), with 104 cases reported
SC
in the literature so far.3 Particularly, aqueductal localization is exceptional. Cavernomas growing inside the ventricles pose a therapeutic challenge, and the strategy needs to be tailored according to
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the precise location. Neuroendoscopy offers the possibility to treat hydrocephalus, which might be the primary cause of hospitalization, directly inspect the lesion, and remove the cavernoma in selected cases. We report the case of a patient with a cavernoma located in the cerebral aqueduct lumen presenting with symptoms due to hydrocephalus, along with a review of the literature about
the disease.
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Case report
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intraventricular cavernomas. The issues of aqueductal localization require a specific management of
A 62-year-old female patient was admitted to our neurosurgical unit with an 8-month history of
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progressive headache, cognitive impairment (memory loss), gait instability, and urinary incontinence. MRI showed the presence of a cavernous angioma (9 mm x 11 mm x 15 mm) in the lumen of the cerebral aqueduct, associated with a triventricular hydrocephalus (Figure 1). A neuroendoscopic procedure was scheduled in order to perform a third-ventriculostomy (ETV). A flexible endoscope (Karl Storz GmbH and Co, Tuttlingen, Germany) was introduced into the right lateral ventricle through a precoronal burr hole. Immediately below the adytum, the cerebral aqueduct was completely obstructed by a brown lobular mass, which was blocking cerebrospinal fluid (CSF) flow (Figure 1). The vascular malformation was visually identified as a cavernous
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ACCEPTED MANUSCRIPT angioma. In order to prevent any damage to the midbrain with consequent neurological deficits we avoided any attempt of radical removal of the lesion. A standard ETV was performed. The postoperative course was uneventful, and CT scan ruled out any complication. Immediately after
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surgery, urinary incontinence was no longer present. Three and twelve-month follow-up MRI confirmed the ventricle reduction (Figure 1), while physical examination revealed a significant
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recovery of cognitive functions and improvement of postural instability after 6 months.
Discussion
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Although cavernomas are relatively common, those growing inside the ventricular system need to be considered separately because of their peculiar characteristics. Their natural history remains undefined to some extent, although selected studies and reviews defined some features of this uncommon pathological entity. Intraventricular cavernomas (IVCs) have shown more rapid growth than intraparenchimal cavernomas, probably because they are not surrounded by brain tissue,
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although symptoms onset is usually delayed.4 It is not known whether waiting after acute hemorrhage before operating an IVC is the correct strategy, or if it unnecessarily increases the risk
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of harmful bleeding. Although intralesional bleeding can frequently occur, intraventricular bleeding is rare and is shown in 14-27% of the cases as described in literature.3,5,6 Nevertheless, in a recent
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series Kivelev et al found a high tendency of IVCs for rehemorrhage, thus advocating for surgery when hemorrhages are frequent and the mass effect causes progressive neurological deficits.3 They reported a 19% rate of intraventricular bleeding risk. Similarly, our review of the published cases revealed that intraventricular hemorrhage occurs in about 21.4% of cases (Table 1). The symptoms related to their growth and the management of these lesions are different based on the specific location. It has been showed that cavernomas in the third ventricle are different in biological nature and need more aggressive therapy. They grow very rapidly, leading to significant morbidity.7 Particularly, cavernomas occurring at the narrowest passages of the ventricular system as the
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ACCEPTED MANUSCRIPT foramen of Monro and the cerebral aqueduct are of great interest because of their rarity and because they can lead to hydrocephalus, which may sometimes develop acutely. Although microsurgical removal of IVCs is usually safe, in the fourth ventricle it can carry
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increased risk. The 63 patients surgically treated with either total or partial IVC resection showed neurological improvement, while mortality was seen in only 9 cases while the remaining patients presented various post operative symptoms such as hemianopsia, memory loss and hemiparesis.
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Bailey and Woodard in 1959, and Courville in 1961 reported on two cases of cavernoma
obstructing the upper end of the cerebral aqueduct.8,9 To our knowledge, our case is the first
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reported cavernoma of the cerebral aqueduct lumen. The aqueduct location carries the main risk of CSF obstruction similarly to the foramen of Monro.10 Actually our patient, as well as Courville’s patient, presented with symptoms related to hydrocephalus. It is not clear if the bleeding risk of aqueduct cavernomas is high as those in the third ventricle. However, as the only symptoms of our
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patient were those related to intracranial hypertension, and the microsurgical removal of the lesion would have certainly be possible but would have carried significant morbidity risks, we decided to treat hydrocephalus with an ETV and to follow up the cavernoma with MRI. We did not leave an
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external ventricular drain in place, because the patient could be awaken immediately after the procedure, and clinical monitoring is preferable in these cases. IVC’s location is crucial when
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assessing intraoperative risks.3 The role of neuroendoscopy in IVCs has been well established. In all cases it is possible to directly inspect the lesion, even in deep locations as the aqueduct or the fourth ventricle, using rigid or flexible endoscopes. This can give precious information about the final diagnosis and possible signs of previous bleeding, as hemosiderin traces. Actually, in the majority of cases MRI gives only non-specific imaging leading to doubtful diagnosis.11 Moreover, it is possible to treat the associated hydrocephalus with an ETV. Although endoscopic removal of aqueductal tumors has been shown possible, and a complete endoscopic removal of cavernomas has been reported in 2 cases, it should not be the first option.12-14 Bleeding can be copious and difficult
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ACCEPTED MANUSCRIPT to manage through such a small approach, and walls of the cavernoma may collapse resulting in a more challenging resection. These complications have been reported in 3 cases, when a purely endoscopic removal was attempted but soon abandoned.14,15 It has been hypothesized that IVCs can
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rapidly grow also because there is no brain tissue surrounding them to counteract their expansion.4 To a certain extent, the cerebral aqueduct is a very peculiar location in this perspective, as its walls can act as a counteracting force when the IVC reaches a volume that balances the aqueduct
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compliance. This was another consideration leading us to treat hydrocephalus first, avoiding the immediate attempt to microsurgically remove the cavernoma. When symptoms are only related to
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hydrocephalus, and the site of cavernoma is difficult to reach with significant risks of morbidity (aqueduct, fourth ventricle), the treatment of hydrocephalus alone must be considered.16 Some authors claim that the placement of a VP shunt can contribute to a further growth of the lesion by lowering intraventricular pressure.17 Actually, ETV is the best choice in such cases because it minimizes the risks related to quick, substantial reduction of CSF pressure with consequent rise in
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Conclusions
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differential pressure between the ventricle and the vascular system and higher risk of rupture.18
Aqueductal cavernomas are an exceedingly rare finding, usually leading to obstructive
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hydrocephalus. Their position makes their removal difficult, with potential high risk of morbidity. We presented a case of aqueductal cavernoma, showing that flexible neuroendoscopy allows for direct visual inspection of the lesion growing in the aqueduct lumen, for confirmation of the diagnosis, and for ETV to treat the associated hydrocephalus. ETV can lead to complete resolution of symptoms due to CSF blockage. It is the treatment of choice when a cavernoma blocks the aqueduct without any sign of previous bleeding.
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ACCEPTED MANUSCRIPT Funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
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Acknowledgements: none.
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ACCEPTED MANUSCRIPT References 1) Han JH, Kim DG. Stereotactic radiosurgery for brainstem cavernous malformations. World Neurosurg. 2013;80:e187-189
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2) Patibandla MR, Thotakura AK, Panigrahi MK. Third ventricular cavernous malformation: an unusual lesion. Br J Neurosurg. 2014;28:110-112
3) Kivelev J, Niemelä M, Kivisaari R, Hernesniemi J. Intraventricular cerebral cavernomas: a series
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of 12 patients and review of the literature. J Neurosurg. 2010;112:140-149
4) Katayama Y, Tsubokawa T, Maeda T, Yamamoto T. Surgical management of cavernous
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malformations of the third ventricle. J Neurosurg. 1994;80:64–72
5) Ogawa A, Katakura R, Yoshimoto T. Third ventricle cavernous angioma: report of two cases. Surg Neurol. 1990;34:414–420
6) Ohbuchi H, Osaka Y, Ogawa T, Nanto M, Nakahara Y, Katsura K, Tenjin H, Kasuya H. Trigonal
Med Invest. 2012;59:275-279
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cavernous malformation with intraventricular hemorrhage: a case report and literature review. J
7) Han MS, Moon KS, Lee KH, Kim SK, Jung S. Cavernous hemangioma of the third ventricle: a
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case report and review of the literature. World J Surg Oncol. 2014;12:237 8) Bailey OT, Woodard JS. Small vascular malformations of the brain: their relationship to
108
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unexpected death, hydrocephalus and mental deficiency. J Neuropathol Exp Neurol. 1959;18:98-
9) Courville CB. Obstructive internal hydrocephalus incident to small vascular anomaly of midbrain. Report of case. Bull Los Angel Neuro Soc. 1961;26:41-45 10) Lee BJ, Choi CY, Lee CH. Intraventricular cavernous hemangiomas located at the foramen of monro. J Korean Neurosurg Soc. 2012;52:144-147 11) Chadduck WM, Binet EF, Farrell FW Jr, Araoz CA, Reding DL. Intraventricular cavernous hemangioma: report of three cases and review of the literature. Neurosurgery. 1985;16:189–197
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ACCEPTED MANUSCRIPT 12) Feletti A, Denaro L, Marton E, d'Avella D, Longatti P. Endoscopic treatment of hydrocephalus due to aneurysm of the vein of Galen: case report and literature review. Minim Invasive Neurosurg. 2007;50:285-291
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13) Gaab MR, Schroeder HW. Neuroendoscopic approach to intraventricular lesions. Neurosurg Focus. 1999;6:e5
14) Prat R, Galeano I. Endoscopic resection of cavernoma of foramen of Monro in a patient with
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familial multiple cavernomatosis. Clin Neurol Neurosurg. 2008;110:834–837
15) Sato K, Oka H, Utsuki S, Shimizu S, Suzuki S, Fujii K. Neuroendoscopic appearance of an
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intraventricular cavernous angioma blocking the foramen of Monro—case report. Neurol Med Chir (Tokyo). 2006;46:548–551
16) Darwish B, Boet R, Finnis N, Smith N. Third ventricular cavernous haemangioma. J Clin Neurosci. 2005;12:601–603
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17) Voigt K, Yasargil MG. Cerebral cavernous haemangiomas or cavernomas. incidence, pathology, localization, diagnosis, clinical features and treatment. Review of the literature and report of an unusual case. Neurochirurgia (Stuttg). 1976;19:59–68
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18) Feletti A, Marton E, Fiorindi A, Longatti P. Neuroendoscopic aspiration of tumors in the posterior third ventricle and aqueduct lumen: a technical update. Acta Neurochir (Wien).
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2013;155:1467-1473
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ACCEPTED MANUSCRIPT Figure legend
Figure 1: Preoperative axial (a) and sagittal (b) T2-weighted magnetic resonance imaging (MRI)
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showing the cavernous angioma located in the lumen of the cerebral aqueduct. Intraoperative neuroendoscopic view of the lesion (c). Postoperative sagittal T2-weighted MRI follow up scan
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after 1 year, showing ventricle reduction (d).
ACCEPTED MANUSCRIPT Table 1. Intraventricular cavernomas: literature review Age (yrs), Sex 2, M
Clinical Presentation mass effect
Location
Treatment
Outcome
4th ventricle
died
31, M 16, F 2 days, M
mass effect mass effect mass effect
4th ventricle lat ventricle lat ventricle
partial resection total removal total removal no op
68, F
mass effect
3rd ventricle
no op
died
33, F
mass effect
lat ventricle
total removal
44, F
hydrocephalus
VC shunt
15, M 36, F
mass effect seizures
Cerebral acqueduct lat ventricle lat ventricle
total removal total removal
improved hemianopsia
43, M
mass effect
lat ventricle
total removal
27, M
mass effect
4th ventricle
29, F
IVH
4th ventricle
partial resection total removal
hemiplegia & hemianopsia died
56, ND
IVH
lat ventricle
no op
died
45, F
IVH
lat ventricle
improved
18, F
mass effect
partial resection total removal
31, F
mass effect
3rd ventricle
total removal
improved
8 days, F
mass effect
lat ventricle
total removal
improved
60, F
mass effect
4th ventricle
48, F
mass effect
3rd ventricle
partial resection partial resection
symptom recurrence hydrocephalus, bleeding
mass effect
3rd ventricle
total removal
improved
Harbaugh et al., 1984 Chadduck et al., 1985
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improved comatose died
homonymous hemianopsia died
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40, M
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Authors & Year Finkelburg, 1905 Dandy, 1928 Merritt, 1940 Arnstein et al., 1951 Latterman, 1952 Schneider & Liss, 1958 Courville 1961 Jain, 1966 Coin et al., 1977 Numaguchi et al., 1977 Giombini & Morello, 1978 Terao et al., 1979 Pau & Orunesu, 1979 Namba et al., 1979 Vaquero et al., 1980 Pozzati et al., 1981 Iwasa et al., 1983 Kendall et al., 1983 Lavyne & Patterson, 1983 Amagasa et al., 1984
improved
improved
44, F
IVH
3rd ventricle
total removal
improved
Simard et al., 1986 Yamasaki et al., 1986
21, F 29, F 4 mos, F 22, M 13, F 73, M 9, M
seizures mass effect seizures mass effect mass effect mass effect mass effect
lat ventricle lat ventricle lat ventricle lat ventricle lat ventricle lat ventricle 3rd ventricle
hemianopsia improved improved not registered not registered improved improved
Suzuki, 1988
36, M 47, M 40, M
mass effect mass effect mass effect
3rd ventricle 4th ventricle lat ventricle
total removal total removal total removal not registered not registered total removal partial resection total removal total removal total removal
improved improved improved
ACCEPTED MANUSCRIPT Sabatier et al., 1989
9 mos, M
IVH
lat ventricle
no op
cerebellar dysfunction
Voci et al., 1989
19, F
IVH
3rd ventricle
total removal
improved
Ogawa et al., 1990
16, M
mass effect
3rd ventricle
improved
40, M
mass effect
3rd ventricle
total removal, VP shunt total removal
Andoh et al., 1990
62, F
mass effect
lat ventricle
total removal
Tatagiba et al., 1991
33, M 35, M 24, F 44, F
IVH seizures mass effect IVH, mass effect
lat ventricle lat ventricle lat ventricle 4th ventricle
total removal total removal total removal total removal
improved died improved improved
3, F
IVH, mass effect
lat ventricle
total removal
39, F
seizures
mild hemiparesis improved
5, M
seizures
10, F 9, F
mass effect seizures
50, F 45, F 49, M 49, M 43, F 36, F
mass effect IVH mass effect mass effect mass effect mass effect
52, F 32, F
mass effect mass effect
3rd ventricle 3rd ventricle
mass effect
lat ventricle
Sinson et al., 1995
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Katayama et al., 1994
lat ventricle
lat ventricle
lat ventricle 3rd ventricle
3rd ventricle 3rd ventricle 3rd ventricle 3rd ventricle 3rd ventricle 3rd ventricle
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Itoh & Usui, 1991 Miyagi et al., 1993 Lynch et al., 1994
transient DI, homonymous hemianopsia homonymous quadrantanopia
partial resection partial resection total removal partial resection, VP shunt not registered not registered not registered total removal total removal total removal
improved improved died
not registered not registered not registered transient DI died hemiparesis, hypothyroidism, hydrocephalus improved improved
mass effect
3rd ventricle
44, F
mass effect
lat ventricle
total removal
permanent memory loss
16, F 36, M
mass effect seizures
lat ventricle lat ventricle
total removal total removal
42, M
asymptomatic
3rd ventricle
FagundesPereyra et al., 2000 Suess et al., 2002
15, F
mass effect
lat ventricle
partial resection total removal
improved rt hemihypertonia improved
36, F
mass effect
3rd ventricle
total removal
improved
Crivelli et al., 2002
38, M
mass effect
3rd ventricle
total removal
improved
2 days, M
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Hashimoto et al., 1997 Kaim et al., 1997 Gaab & Shroeder, 1999 Reyns et al., 1999
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64, M
total removal total removal, VP shunt total removal, VP shunt total removal
mild mental retardation not registered
improved
ACCEPTED MANUSCRIPT seizures
lat ventricle
total removal
17, F 52, M 62, F
seizures IVH mass effect
lat ventricle lat ventricle 3rd ventricle
total removal total removal total removal
homonymous hemianopsia improved improved improved
Anderson et al., 2003
45, F
mass effect
total removal
improved
Darwish et al., 2005 Milenkovic, 2005
47, F
incidental
septum pellucidum, lat ventricle 3rd ventricle
56, M
mass effect
3rd ventricle
total removal, shunt total removal
Chen et al., 2006 Kumar et al., 2006
51, F
mass effect
3rd ventricle
total removal
improved
8, M
mass effect
total removal
improved
19, F
seizures, mass effect
lat ventricle trigonum lat ventricle trigonum
20, M
mass effect
total removal
35, M
mass effect
lat ventricle trigonum 3rd ventricle
Improved, seizures remained improved
total removal
improved
8, M
mass effect
3rd ventricle
total removal
improved
47, F
mass effect
3rd ventricle
total removal
improved
25, M
mass effect
lat ventricle trigonum
total removal
improved
56, ND
mass effect
3rd ventricle
total removal
improved
lat ventricle trigonum lat ventricle
total removal
improved
VP shunt
improved
4th ventricle
total removal
improved
lat ventricle
total removal
improved
4th ventricle
total removal
CN VI & VII peripheral paresis the same as preop
lat ventricle
stereotactic biopsy, partial
improved
52, F 66, M
43, F
65, M
58, F
20, M
gait disturbances, mild headaches, hydrocephalus mild headaches, nausea/vomiting IVH, severe headaches, nausea/vomiting, hydrocephalus IVH, mild headaches, nausea/vomiting, CN deficit
IVH, mild headaches
improved
improved
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mass effect
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Longatti et al., 2006 Zakaria et al., 2006 Sato et al., 2006 GonzalezDarder et al., 2007 Prat & Galeano, 2008 Stavrinou et al, 2008 Kivelev et al, 2010
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11, F
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Nieto et al., 2003 Tatsui et al., 2003 Wang et al., 2003
total removal
ACCEPTED MANUSCRIPT 15, M
IVH, CN deficit
4th ventricle
resection total removal
52, M
headache, nausea/vomiting CN deficit
3rd ventricle
total removal
4th ventricle
total removal
lat ventricle
conservative
4th ventricle
total removal
improved
lat ventricle
conservative
improved
lat ventricle
total removal
53, M
Prada et al, 2010
32, F
Sabat, 2010
60, F
Ohbuchi et al, 2012 Byung-Jou et al, 2012
67, F
Bhatia et al, 2013
29, F
Giannetti, 2013
56, M
Headache, short term memory loss, vomit Headache, vomiting, Hydrocephalus gait disturbances, visual acuity disturbances, confusional state Headache, vomiting Headache
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30, F
Patibandla et al, 2014 Faropoulos et al, 2015
35, M 56, M
25, F 61, F 36, F
31, M
Deteriotating conscious state Seizures Headache, Neck stiffness, confusion Headache, loss of consciousness
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CN VI & VII paresis improved
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65, M
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49, F
IVH, acute severe headaches, nausea/vomiting IVH, acute severe headaches, hydrocephalus IVH, acute headaches, nausea/vomiting, hydrocephalus IVH & ICH, headaches, hemiparesis headaches, gait disturbances, nausea/vomiting IVH, headaches, imbalance, gait disturbances, hydrocephalus IVH
minor memory disturbances
lat ventricle trigonum
total removal
improved
lat ventricle
total removal
improved
lat ventricle trigonum 3rd ventricle
total removal
improved
total removal
improved
3rd ventricle
total removal
improved
lat ventricle
total removal, ETV
3rd ventricle
total removal
improved
septum pellucidum, lat ventricle lat ventricle
total removal
improved
total removal
improved
lat ventricle lat ventricle
total removal total removal
improved improved
septum pellucidum, lat ventricle
total removal
improved
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35, M
EP
49, F
persistent CN VII peripheral paresis improved
ACCEPTED MANUSCRIPT 64, F
Present case
62, F
loss of consciousness, Hydrocephalus Headache, gait disturbances , memory loss, urinary incontinence, Hydrocephalus
lat ventricle
ETV
died
Cerebral aqueduct
ETV
improved
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Winslow et al, 2015
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EP
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ETV: endoscopic third ventrculostomy, VP-shunt: ventriculoperitoneal shunt, IVH: intraventricular hemorrhage
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SC
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ACCEPTED MANUSCRIPT
ACCEPTED MANUSCRIPT Highlights 1) Cavernomas are rare in the ventricles and exceptional in the aqueduct 2) Some intraventricular cavernomas may lead to hydrocephalus
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3) Neuroendoscopy can treat hydrocephalus and make visual diagnosis
ACCEPTED MANUSCRIPT Abbreviation list central nervous system
CSF
cerebrospinal fluid
ETV
endoscopic third ventriculostomy
IVCs
intraventricular cavernomas
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CNS
S1878-8750(16)31244-X
DOI:
10.1016/j.wneu.2016.11.096
Reference:
WNEU 4906
To appear in:
World Neurosurgery
Received Date: 28 August 2016 Revised Date:
15 November 2016
Accepted Date: 16 November 2016
Please cite this article as: Feletti A, Dimitriadis S, Pavesi G, Cavernous angioma of the cerebral aqueduct, World Neurosurgery (2016), doi: 10.1016/j.wneu.2016.11.096. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
1
ACCEPTED MANUSCRIPT Cavernous angioma of the cerebral aqueduct Alberto Feletti, MD, PhD, Stavros Dimitriadis, MD, Giacomo Pavesi, MD
Corresponding author:
Dr. Alberto Feletti, MD, PhD
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Department of Neurosurgery, NOCSAE Hospital of Modena, Modena, Italy
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Department of Neurosurgery, NOCSAE Modena Hospital Via Giardini 1355, 41126 Baggiovara (MO), Italy
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Email: [email protected] Tel: +390593961471
Fax: +390593962408
ETV
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Abbreviation list
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Key words: cerebral aqueduct, cavernous angioma, cavernoma, intraventricular, hydrocephalus,
central nervous system
CSF
cerebrospinal fluid
ETV IVCs
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CNS
endoscopic third ventriculostomy intraventricular cavernomas
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ACCEPTED MANUSCRIPT Abstract Background. Among the rare intraventricular cavernomas (IVCs), purely intraaqueductal cavernomas are exceptional.
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Case description. We report a unique case of a 62-year-old patient who presented to our clinics complaining of progressive headache, memory loss, gait instability, and urinary incontinence. MR imaging showed the presence of a mass lesion located in the lumen of the cerebral aqueduct,
SC
associated with triventricular hydrocephalus.
Conclusions. We discuss the rationale that led us to treat hydrocephalus with neuroendoscopy,
M AN U
which offered the possibility to directly inspect the intraaqueductal lesion, make the diagnosis of
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cavernoma, and treat symptoms due to hydrocephalus without increasing the risk of bleeding.
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ACCEPTED MANUSCRIPT Introduction Cavernous angiomas (or cavernomas) represent 5-10% of the vascular malformations of the central nervous system (CNS). They are usually diagnosed between the 2nd and the 5th decade of life and
hemorrhages are frequent although often subclinical.1,2
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can occur sporadically or as part of a familial syndrome. Due to their histological structure, small
Intraventricular cavernomas are rare (2.5-10.8% of cerebral cavernomas), with 104 cases reported
SC
in the literature so far.3 Particularly, aqueductal localization is exceptional. Cavernomas growing inside the ventricles pose a therapeutic challenge, and the strategy needs to be tailored according to
M AN U
the precise location. Neuroendoscopy offers the possibility to treat hydrocephalus, which might be the primary cause of hospitalization, directly inspect the lesion, and remove the cavernoma in selected cases. We report the case of a patient with a cavernoma located in the cerebral aqueduct lumen presenting with symptoms due to hydrocephalus, along with a review of the literature about
the disease.
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Case report
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intraventricular cavernomas. The issues of aqueductal localization require a specific management of
A 62-year-old female patient was admitted to our neurosurgical unit with an 8-month history of
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progressive headache, cognitive impairment (memory loss), gait instability, and urinary incontinence. MRI showed the presence of a cavernous angioma (9 mm x 11 mm x 15 mm) in the lumen of the cerebral aqueduct, associated with a triventricular hydrocephalus (Figure 1). A neuroendoscopic procedure was scheduled in order to perform a third-ventriculostomy (ETV). A flexible endoscope (Karl Storz GmbH and Co, Tuttlingen, Germany) was introduced into the right lateral ventricle through a precoronal burr hole. Immediately below the adytum, the cerebral aqueduct was completely obstructed by a brown lobular mass, which was blocking cerebrospinal fluid (CSF) flow (Figure 1). The vascular malformation was visually identified as a cavernous
4
ACCEPTED MANUSCRIPT angioma. In order to prevent any damage to the midbrain with consequent neurological deficits we avoided any attempt of radical removal of the lesion. A standard ETV was performed. The postoperative course was uneventful, and CT scan ruled out any complication. Immediately after
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surgery, urinary incontinence was no longer present. Three and twelve-month follow-up MRI confirmed the ventricle reduction (Figure 1), while physical examination revealed a significant
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recovery of cognitive functions and improvement of postural instability after 6 months.
Discussion
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Although cavernomas are relatively common, those growing inside the ventricular system need to be considered separately because of their peculiar characteristics. Their natural history remains undefined to some extent, although selected studies and reviews defined some features of this uncommon pathological entity. Intraventricular cavernomas (IVCs) have shown more rapid growth than intraparenchimal cavernomas, probably because they are not surrounded by brain tissue,
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although symptoms onset is usually delayed.4 It is not known whether waiting after acute hemorrhage before operating an IVC is the correct strategy, or if it unnecessarily increases the risk
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of harmful bleeding. Although intralesional bleeding can frequently occur, intraventricular bleeding is rare and is shown in 14-27% of the cases as described in literature.3,5,6 Nevertheless, in a recent
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series Kivelev et al found a high tendency of IVCs for rehemorrhage, thus advocating for surgery when hemorrhages are frequent and the mass effect causes progressive neurological deficits.3 They reported a 19% rate of intraventricular bleeding risk. Similarly, our review of the published cases revealed that intraventricular hemorrhage occurs in about 21.4% of cases (Table 1). The symptoms related to their growth and the management of these lesions are different based on the specific location. It has been showed that cavernomas in the third ventricle are different in biological nature and need more aggressive therapy. They grow very rapidly, leading to significant morbidity.7 Particularly, cavernomas occurring at the narrowest passages of the ventricular system as the
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ACCEPTED MANUSCRIPT foramen of Monro and the cerebral aqueduct are of great interest because of their rarity and because they can lead to hydrocephalus, which may sometimes develop acutely. Although microsurgical removal of IVCs is usually safe, in the fourth ventricle it can carry
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increased risk. The 63 patients surgically treated with either total or partial IVC resection showed neurological improvement, while mortality was seen in only 9 cases while the remaining patients presented various post operative symptoms such as hemianopsia, memory loss and hemiparesis.
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Bailey and Woodard in 1959, and Courville in 1961 reported on two cases of cavernoma
obstructing the upper end of the cerebral aqueduct.8,9 To our knowledge, our case is the first
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reported cavernoma of the cerebral aqueduct lumen. The aqueduct location carries the main risk of CSF obstruction similarly to the foramen of Monro.10 Actually our patient, as well as Courville’s patient, presented with symptoms related to hydrocephalus. It is not clear if the bleeding risk of aqueduct cavernomas is high as those in the third ventricle. However, as the only symptoms of our
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patient were those related to intracranial hypertension, and the microsurgical removal of the lesion would have certainly be possible but would have carried significant morbidity risks, we decided to treat hydrocephalus with an ETV and to follow up the cavernoma with MRI. We did not leave an
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external ventricular drain in place, because the patient could be awaken immediately after the procedure, and clinical monitoring is preferable in these cases. IVC’s location is crucial when
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assessing intraoperative risks.3 The role of neuroendoscopy in IVCs has been well established. In all cases it is possible to directly inspect the lesion, even in deep locations as the aqueduct or the fourth ventricle, using rigid or flexible endoscopes. This can give precious information about the final diagnosis and possible signs of previous bleeding, as hemosiderin traces. Actually, in the majority of cases MRI gives only non-specific imaging leading to doubtful diagnosis.11 Moreover, it is possible to treat the associated hydrocephalus with an ETV. Although endoscopic removal of aqueductal tumors has been shown possible, and a complete endoscopic removal of cavernomas has been reported in 2 cases, it should not be the first option.12-14 Bleeding can be copious and difficult
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ACCEPTED MANUSCRIPT to manage through such a small approach, and walls of the cavernoma may collapse resulting in a more challenging resection. These complications have been reported in 3 cases, when a purely endoscopic removal was attempted but soon abandoned.14,15 It has been hypothesized that IVCs can
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rapidly grow also because there is no brain tissue surrounding them to counteract their expansion.4 To a certain extent, the cerebral aqueduct is a very peculiar location in this perspective, as its walls can act as a counteracting force when the IVC reaches a volume that balances the aqueduct
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compliance. This was another consideration leading us to treat hydrocephalus first, avoiding the immediate attempt to microsurgically remove the cavernoma. When symptoms are only related to
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hydrocephalus, and the site of cavernoma is difficult to reach with significant risks of morbidity (aqueduct, fourth ventricle), the treatment of hydrocephalus alone must be considered.16 Some authors claim that the placement of a VP shunt can contribute to a further growth of the lesion by lowering intraventricular pressure.17 Actually, ETV is the best choice in such cases because it minimizes the risks related to quick, substantial reduction of CSF pressure with consequent rise in
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Conclusions
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differential pressure between the ventricle and the vascular system and higher risk of rupture.18
Aqueductal cavernomas are an exceedingly rare finding, usually leading to obstructive
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hydrocephalus. Their position makes their removal difficult, with potential high risk of morbidity. We presented a case of aqueductal cavernoma, showing that flexible neuroendoscopy allows for direct visual inspection of the lesion growing in the aqueduct lumen, for confirmation of the diagnosis, and for ETV to treat the associated hydrocephalus. ETV can lead to complete resolution of symptoms due to CSF blockage. It is the treatment of choice when a cavernoma blocks the aqueduct without any sign of previous bleeding.
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ACCEPTED MANUSCRIPT Funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
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Acknowledgements: none.
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ACCEPTED MANUSCRIPT References 1) Han JH, Kim DG. Stereotactic radiosurgery for brainstem cavernous malformations. World Neurosurg. 2013;80:e187-189
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2) Patibandla MR, Thotakura AK, Panigrahi MK. Third ventricular cavernous malformation: an unusual lesion. Br J Neurosurg. 2014;28:110-112
3) Kivelev J, Niemelä M, Kivisaari R, Hernesniemi J. Intraventricular cerebral cavernomas: a series
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of 12 patients and review of the literature. J Neurosurg. 2010;112:140-149
4) Katayama Y, Tsubokawa T, Maeda T, Yamamoto T. Surgical management of cavernous
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malformations of the third ventricle. J Neurosurg. 1994;80:64–72
5) Ogawa A, Katakura R, Yoshimoto T. Third ventricle cavernous angioma: report of two cases. Surg Neurol. 1990;34:414–420
6) Ohbuchi H, Osaka Y, Ogawa T, Nanto M, Nakahara Y, Katsura K, Tenjin H, Kasuya H. Trigonal
Med Invest. 2012;59:275-279
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cavernous malformation with intraventricular hemorrhage: a case report and literature review. J
7) Han MS, Moon KS, Lee KH, Kim SK, Jung S. Cavernous hemangioma of the third ventricle: a
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case report and review of the literature. World J Surg Oncol. 2014;12:237 8) Bailey OT, Woodard JS. Small vascular malformations of the brain: their relationship to
108
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unexpected death, hydrocephalus and mental deficiency. J Neuropathol Exp Neurol. 1959;18:98-
9) Courville CB. Obstructive internal hydrocephalus incident to small vascular anomaly of midbrain. Report of case. Bull Los Angel Neuro Soc. 1961;26:41-45 10) Lee BJ, Choi CY, Lee CH. Intraventricular cavernous hemangiomas located at the foramen of monro. J Korean Neurosurg Soc. 2012;52:144-147 11) Chadduck WM, Binet EF, Farrell FW Jr, Araoz CA, Reding DL. Intraventricular cavernous hemangioma: report of three cases and review of the literature. Neurosurgery. 1985;16:189–197
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ACCEPTED MANUSCRIPT 12) Feletti A, Denaro L, Marton E, d'Avella D, Longatti P. Endoscopic treatment of hydrocephalus due to aneurysm of the vein of Galen: case report and literature review. Minim Invasive Neurosurg. 2007;50:285-291
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13) Gaab MR, Schroeder HW. Neuroendoscopic approach to intraventricular lesions. Neurosurg Focus. 1999;6:e5
14) Prat R, Galeano I. Endoscopic resection of cavernoma of foramen of Monro in a patient with
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familial multiple cavernomatosis. Clin Neurol Neurosurg. 2008;110:834–837
15) Sato K, Oka H, Utsuki S, Shimizu S, Suzuki S, Fujii K. Neuroendoscopic appearance of an
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intraventricular cavernous angioma blocking the foramen of Monro—case report. Neurol Med Chir (Tokyo). 2006;46:548–551
16) Darwish B, Boet R, Finnis N, Smith N. Third ventricular cavernous haemangioma. J Clin Neurosci. 2005;12:601–603
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17) Voigt K, Yasargil MG. Cerebral cavernous haemangiomas or cavernomas. incidence, pathology, localization, diagnosis, clinical features and treatment. Review of the literature and report of an unusual case. Neurochirurgia (Stuttg). 1976;19:59–68
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18) Feletti A, Marton E, Fiorindi A, Longatti P. Neuroendoscopic aspiration of tumors in the posterior third ventricle and aqueduct lumen: a technical update. Acta Neurochir (Wien).
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2013;155:1467-1473
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ACCEPTED MANUSCRIPT Figure legend
Figure 1: Preoperative axial (a) and sagittal (b) T2-weighted magnetic resonance imaging (MRI)
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showing the cavernous angioma located in the lumen of the cerebral aqueduct. Intraoperative neuroendoscopic view of the lesion (c). Postoperative sagittal T2-weighted MRI follow up scan
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after 1 year, showing ventricle reduction (d).
ACCEPTED MANUSCRIPT Table 1. Intraventricular cavernomas: literature review Age (yrs), Sex 2, M
Clinical Presentation mass effect
Location
Treatment
Outcome
4th ventricle
died
31, M 16, F 2 days, M
mass effect mass effect mass effect
4th ventricle lat ventricle lat ventricle
partial resection total removal total removal no op
68, F
mass effect
3rd ventricle
no op
died
33, F
mass effect
lat ventricle
total removal
44, F
hydrocephalus
VC shunt
15, M 36, F
mass effect seizures
Cerebral acqueduct lat ventricle lat ventricle
total removal total removal
improved hemianopsia
43, M
mass effect
lat ventricle
total removal
27, M
mass effect
4th ventricle
29, F
IVH
4th ventricle
partial resection total removal
hemiplegia & hemianopsia died
56, ND
IVH
lat ventricle
no op
died
45, F
IVH
lat ventricle
improved
18, F
mass effect
partial resection total removal
31, F
mass effect
3rd ventricle
total removal
improved
8 days, F
mass effect
lat ventricle
total removal
improved
60, F
mass effect
4th ventricle
48, F
mass effect
3rd ventricle
partial resection partial resection
symptom recurrence hydrocephalus, bleeding
mass effect
3rd ventricle
total removal
improved
Harbaugh et al., 1984 Chadduck et al., 1985
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improved comatose died
homonymous hemianopsia died
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40, M
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Authors & Year Finkelburg, 1905 Dandy, 1928 Merritt, 1940 Arnstein et al., 1951 Latterman, 1952 Schneider & Liss, 1958 Courville 1961 Jain, 1966 Coin et al., 1977 Numaguchi et al., 1977 Giombini & Morello, 1978 Terao et al., 1979 Pau & Orunesu, 1979 Namba et al., 1979 Vaquero et al., 1980 Pozzati et al., 1981 Iwasa et al., 1983 Kendall et al., 1983 Lavyne & Patterson, 1983 Amagasa et al., 1984
improved
improved
44, F
IVH
3rd ventricle
total removal
improved
Simard et al., 1986 Yamasaki et al., 1986
21, F 29, F 4 mos, F 22, M 13, F 73, M 9, M
seizures mass effect seizures mass effect mass effect mass effect mass effect
lat ventricle lat ventricle lat ventricle lat ventricle lat ventricle lat ventricle 3rd ventricle
hemianopsia improved improved not registered not registered improved improved
Suzuki, 1988
36, M 47, M 40, M
mass effect mass effect mass effect
3rd ventricle 4th ventricle lat ventricle
total removal total removal total removal not registered not registered total removal partial resection total removal total removal total removal
improved improved improved
ACCEPTED MANUSCRIPT Sabatier et al., 1989
9 mos, M
IVH
lat ventricle
no op
cerebellar dysfunction
Voci et al., 1989
19, F
IVH
3rd ventricle
total removal
improved
Ogawa et al., 1990
16, M
mass effect
3rd ventricle
improved
40, M
mass effect
3rd ventricle
total removal, VP shunt total removal
Andoh et al., 1990
62, F
mass effect
lat ventricle
total removal
Tatagiba et al., 1991
33, M 35, M 24, F 44, F
IVH seizures mass effect IVH, mass effect
lat ventricle lat ventricle lat ventricle 4th ventricle
total removal total removal total removal total removal
improved died improved improved
3, F
IVH, mass effect
lat ventricle
total removal
39, F
seizures
mild hemiparesis improved
5, M
seizures
10, F 9, F
mass effect seizures
50, F 45, F 49, M 49, M 43, F 36, F
mass effect IVH mass effect mass effect mass effect mass effect
52, F 32, F
mass effect mass effect
3rd ventricle 3rd ventricle
mass effect
lat ventricle
Sinson et al., 1995
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Katayama et al., 1994
lat ventricle
lat ventricle
lat ventricle 3rd ventricle
3rd ventricle 3rd ventricle 3rd ventricle 3rd ventricle 3rd ventricle 3rd ventricle
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Itoh & Usui, 1991 Miyagi et al., 1993 Lynch et al., 1994
transient DI, homonymous hemianopsia homonymous quadrantanopia
partial resection partial resection total removal partial resection, VP shunt not registered not registered not registered total removal total removal total removal
improved improved died
not registered not registered not registered transient DI died hemiparesis, hypothyroidism, hydrocephalus improved improved
mass effect
3rd ventricle
44, F
mass effect
lat ventricle
total removal
permanent memory loss
16, F 36, M
mass effect seizures
lat ventricle lat ventricle
total removal total removal
42, M
asymptomatic
3rd ventricle
FagundesPereyra et al., 2000 Suess et al., 2002
15, F
mass effect
lat ventricle
partial resection total removal
improved rt hemihypertonia improved
36, F
mass effect
3rd ventricle
total removal
improved
Crivelli et al., 2002
38, M
mass effect
3rd ventricle
total removal
improved
2 days, M
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Hashimoto et al., 1997 Kaim et al., 1997 Gaab & Shroeder, 1999 Reyns et al., 1999
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64, M
total removal total removal, VP shunt total removal, VP shunt total removal
mild mental retardation not registered
improved
ACCEPTED MANUSCRIPT seizures
lat ventricle
total removal
17, F 52, M 62, F
seizures IVH mass effect
lat ventricle lat ventricle 3rd ventricle
total removal total removal total removal
homonymous hemianopsia improved improved improved
Anderson et al., 2003
45, F
mass effect
total removal
improved
Darwish et al., 2005 Milenkovic, 2005
47, F
incidental
septum pellucidum, lat ventricle 3rd ventricle
56, M
mass effect
3rd ventricle
total removal, shunt total removal
Chen et al., 2006 Kumar et al., 2006
51, F
mass effect
3rd ventricle
total removal
improved
8, M
mass effect
total removal
improved
19, F
seizures, mass effect
lat ventricle trigonum lat ventricle trigonum
20, M
mass effect
total removal
35, M
mass effect
lat ventricle trigonum 3rd ventricle
Improved, seizures remained improved
total removal
improved
8, M
mass effect
3rd ventricle
total removal
improved
47, F
mass effect
3rd ventricle
total removal
improved
25, M
mass effect
lat ventricle trigonum
total removal
improved
56, ND
mass effect
3rd ventricle
total removal
improved
lat ventricle trigonum lat ventricle
total removal
improved
VP shunt
improved
4th ventricle
total removal
improved
lat ventricle
total removal
improved
4th ventricle
total removal
CN VI & VII peripheral paresis the same as preop
lat ventricle
stereotactic biopsy, partial
improved
52, F 66, M
43, F
65, M
58, F
20, M
gait disturbances, mild headaches, hydrocephalus mild headaches, nausea/vomiting IVH, severe headaches, nausea/vomiting, hydrocephalus IVH, mild headaches, nausea/vomiting, CN deficit
IVH, mild headaches
improved
improved
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mass effect
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Longatti et al., 2006 Zakaria et al., 2006 Sato et al., 2006 GonzalezDarder et al., 2007 Prat & Galeano, 2008 Stavrinou et al, 2008 Kivelev et al, 2010
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11, F
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Nieto et al., 2003 Tatsui et al., 2003 Wang et al., 2003
total removal
ACCEPTED MANUSCRIPT 15, M
IVH, CN deficit
4th ventricle
resection total removal
52, M
headache, nausea/vomiting CN deficit
3rd ventricle
total removal
4th ventricle
total removal
lat ventricle
conservative
4th ventricle
total removal
improved
lat ventricle
conservative
improved
lat ventricle
total removal
53, M
Prada et al, 2010
32, F
Sabat, 2010
60, F
Ohbuchi et al, 2012 Byung-Jou et al, 2012
67, F
Bhatia et al, 2013
29, F
Giannetti, 2013
56, M
Headache, short term memory loss, vomit Headache, vomiting, Hydrocephalus gait disturbances, visual acuity disturbances, confusional state Headache, vomiting Headache
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30, F
Patibandla et al, 2014 Faropoulos et al, 2015
35, M 56, M
25, F 61, F 36, F
31, M
Deteriotating conscious state Seizures Headache, Neck stiffness, confusion Headache, loss of consciousness
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CN VI & VII paresis improved
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65, M
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49, F
IVH, acute severe headaches, nausea/vomiting IVH, acute severe headaches, hydrocephalus IVH, acute headaches, nausea/vomiting, hydrocephalus IVH & ICH, headaches, hemiparesis headaches, gait disturbances, nausea/vomiting IVH, headaches, imbalance, gait disturbances, hydrocephalus IVH
minor memory disturbances
lat ventricle trigonum
total removal
improved
lat ventricle
total removal
improved
lat ventricle trigonum 3rd ventricle
total removal
improved
total removal
improved
3rd ventricle
total removal
improved
lat ventricle
total removal, ETV
3rd ventricle
total removal
improved
septum pellucidum, lat ventricle lat ventricle
total removal
improved
total removal
improved
lat ventricle lat ventricle
total removal total removal
improved improved
septum pellucidum, lat ventricle
total removal
improved
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35, M
EP
49, F
persistent CN VII peripheral paresis improved
ACCEPTED MANUSCRIPT 64, F
Present case
62, F
loss of consciousness, Hydrocephalus Headache, gait disturbances , memory loss, urinary incontinence, Hydrocephalus
lat ventricle
ETV
died
Cerebral aqueduct
ETV
improved
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Winslow et al, 2015
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ETV: endoscopic third ventrculostomy, VP-shunt: ventriculoperitoneal shunt, IVH: intraventricular hemorrhage
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ACCEPTED MANUSCRIPT Highlights 1) Cavernomas are rare in the ventricles and exceptional in the aqueduct 2) Some intraventricular cavernomas may lead to hydrocephalus
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3) Neuroendoscopy can treat hydrocephalus and make visual diagnosis
ACCEPTED MANUSCRIPT Abbreviation list central nervous system
CSF
cerebrospinal fluid
ETV
endoscopic third ventriculostomy
IVCs
intraventricular cavernomas
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CNS