Presentation, Surgical Management, and Postoperative Outcome of a Fourth Ventricular Cavernous Malformation: Case Report and Review of Literature

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Please cite this article as: Kaye J, Zeller S, Patel NV, Herschman Y, Jumah F, Nanda A, Presentation, Surgical Management, and Postoperative Outcome of a Fourth Ventricular Cavernous Malformation: Case Report and Review of Literature, World Neurosurgery (2020), doi: https://doi.org/10.1016/ j.wneu.2020.01.185. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. 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. © 2020 Elsevier Inc. All rights reserved.

World Neurosurgery

Article Type

Presentation, Surgical Management, and Postoperative Outcome of a Fourth Ventricular Cavernous Malformation: Case Report and Review of Literature Case report

Order of Authors

Joel Kaye, BS1

Title

Sabrina Zeller, BA1 Nitesh V Patel, MD2 Yehuda Herschman, MD2 Fareed Jumah, MD2 Anil Nanda, MD, MPH1,2 Affiliations

1

Robert Wood Johnson Medical School-Rutgers, New Brunswick, New Jersey, USA 2

Department of Neurosurgery, Rutgers- Robert Wood Johnson Medical School & University Hospital, New Brunswick, New Jersey, USA

Corresponding Author

Corresponding Author

Key words

Anil Nanda MD, MPH, FACS Professor and Chairman, Department of Neurosurgery Rutgers- Robert Wood Johnson Medical School Rutgers- New Jersey Medical School. Ph: 973-972-3444 Fax: 973-972-8122 e-mail: [email protected] Cavernous Malformation, Intracranial Vascular Lesion, Cavernoma

Short title

Brainstem Cavernous Malformation: Case report

Manuscript Region of

United States

Origin Disclosure of interest

None

Acknowledgments

None

1

Presentation, Surgical Management, and Postoperative Outcome of a

2

Fourth Ventricular Cavernous Malformation: Case Report and Review

3

of Literature

4

Joel Kaye, BS, Sabrina Zeller, BA, Nitesh V Patel, MD, Yehuda Herschman, MD, Fareed

5

Jumah, MD, Anil Nanda, MD, MPH

6 7

Department of Neurosurgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ

8 9

Keywords: Cavernous Malformation, Intracranial Vascular Lesion, Cavernoma

10 11

Disclosures: None

12 13

Conflicts of Interest: None

14 15 16

Corresponding Author:

17

Anil Nanda MD, MPH, FACS

18

Professor and Chairman, Department of Neurosurgery

19

Rutgers- Robert Wood Johnson Medical School

20

Rutgers- New Jersey Medical School

21

Ph: 732-235-7756 Fax: 973-972-8122

22

e-mail: [email protected]

23 24

25

ABSTRACT

26

Background and Importance

27

Brainstem cavernous malformations (CMs) represent dangerous clinical entities associated with

28

high rates of rebleeding and morbidity compared to those in other locations. Particularly rare are

29

those located within the 4th ventricle. Although 4th ventricular CMs are favorable from a surgical

30

standpoint, there are no defined guidelines on definitive indications and optimal timing of

31

surgery. In addition, the surgical approaches, anatomical considerations, and general

32

observations regarding these lesions are not well reported in the literature.

33

Clinical presentation

34

A 27-year-old male with a known history of a CM on the floor of the 4th ventricle presented with

35

new cranial nerve deficits and signs of increased intracranial pressure. Imaging revealed acute

36

bleeding from a 4th ventricular CM. The patient was urgently taken to surgery for resection.

37

Despite a non-eventful surgery which resulted in gross-total resection, the patient developed a

38

unique constellation of cranial nerve deficits post-operatively, most notably of which was 8.5

39

syndrome.

40

Conclusion

41

CMs of the fourth ventricle are rare clinical entities that can be treated successfully with surgery.

42

The indications for surgery may not always be clear-cut, thus the neurosurgeon’s decision to

43

proceed with surgery must reside on a case-by-case basis using a multifactorial approach. The

44

location of these lesions presents unique challenges given their proximity to vital structures and

45

the technical difficulty required. For these reasons, the resection of these lesions often results in

46

new or persistent neurological deficits. However, despite the associated risks, the potential

47

benefits of surgery oftentimes outweigh the risks of the alternative.

48

49

Introduction

50

Cavernous malformations (CM), also known as cavernomas or cavernous angiomas, are vascular

51

lesions of the brain and spinal cord composed of dilated, thin-walled sinusoids covered by a

52

single layer of endothelium. CMs account for 10-15% of all neurovascular malformations most

53

commonly present in the third and fourth decades of life.1 The abnormal architecture of CMs and

54

associated loss of normal vascular structural integrity predisposes these lesions to repeated bouts

55

of bleeding. Asymptomatic CMs can traditionally be watched; surgical resection is usually

56

reserved for those that produce symptoms or re-bleed multiple times and are operable.2

57

Stereotactic radiosurgery (SRS) and minimally invasive thermal ablation have also been used

58

with varying success.2-4

59

While nearly one-third of CMs are found incidentally, symptomatic patients most commonly

60

present with intracerebral hemorrhage, seizure, headache, or focal neurologic deficits.

61

Symptomatology is highly dependent on the anatomical location of the CM; while lobar CMs

62

most commonly present with seizures, brainstem CMs most commonly present with intracerebral

63

hemorrhage, focal neurologic deficits, and signs/symptoms of obstructive hydrocephalus.5

64

Brainstem CMs comprise 8-35% of all intracerebral CMs, and are particularly worrisome

65

because of their critical location and increased risk of bleeding.5,6 Multiple meta-analyses have

66

reported significantly higher rates of both initial bleeding and re-bleeding with brainstem CMs

67

compared to non-brainstem CMs.5,7 Their increased risk of bleeding along with the brainstem’s

68

critical and delicate anatomy combine for an especially dangerous clinical entity — one in which

69

relatively minor changes in the lesion can result in rapid and devastating morbidity. Furthermore,

70

the inherent risks associated with operating within the brainstem may create a particularly

71

precarious scenario for neurosurgeons.

72

We present a case of a 27-year-old male who presented with acute neurologic decline from a

73

bleeding cavernous malformation of the 4th ventricle for which he was treated surgically. To our

74

knowledge, only 18 cases of 4th ventricular CMs have previously been reported.8-19 Furthermore,

75

we review the natural history, treatment, and outcome of this exceptionally rare clinical entity

76

and discuss its anatomic and surgical considerations.

77 78

Case Presentation

79

History of Present Illness

80

A 27-year-old year-old male with a known history of a brainstem cavernous malformation on the

81

4th ventricle floor presented with diplopia when looking to the left. The lesion was initially

82

discovered three years prior when he presented with similar symptoms. His symptoms resolved

83

at that time with steroids. He was noted to have some radiographic signs of repeat hemorrhage

84

on serial imaging but the decision to continue surveillance was made. In the summer of 2019, he

85

was noted to wake up with sudden diplopia, nausea, and vomiting. Once again, his diplopia was

86

noted on leftward gaze. He also had paresthesias in the left V1/V2 distribution and an unsteady

87

gait. Non-contrast CT head revealed a round hyperdense lesion on the left side of the 4th

88

ventricular floor, juxtaposed to the region of the facial colliculus. A mixed intensity lesion with a

89

hypointense rim at the left side of the floor of the 4th ventricle was seen on MRI (Figure 1). As

90

this was his third hemorrhage and the lesion was felt to be safely accessible, the patient and

91

family were extensively counseled regarding the risks of surgical intervention. After thorough

92

discussion, the patient and family were amenable to surgical resection.

93 94

Surgical Approach

95

The patient was positioned prone and stereotactic navigation was utilized. Neuromonitoring was

96

also used. A suboccipital craniotomy was performed extending down to the foramen magnum

97

(Supplemental video). A Y-shaped durotomy was performed and care was taken to keep the

98

arachnoid intact. Exposure then continued and a small arachnoidotomy was performed to let off

99

cerebrospinal fluid. The cerebellar tonsils were exposed and careful intertonsillar sharp

100

dissection was performed using an arachnoid knife and microscissors. A fixed retractor was

101

gently applied to the left tonsilouvular junction and the rhomboid fossa was visualized. The

102

lesion was readily identified, and stereotactic navigation confirmed it (Figure 2A). Bipolar

103

cautery was used on the lesion surface within the infrafacial triangle and a 3 mm corticectomy

104

was performed. Dark venous blood was immediately noted, and careful suction and further

105

dissection identified the lesion nidus. Using bipolar cautery and suction, the lesion was carefully

106

resected (Figure 2B). Margins were extended until white matter was noted circumferentially.

107

Gentle irrigation was used, and hemostasis was achieved (Figure 2C). Neuromonitoring

108

remained stable throughout the procedure. Dural closure was performed primarily. The patient

109

woke up at his neurological baseline and was transferred to the neurosurgical intensive care unit.

110 111

Postoperative Course

112

On postoperative day one the patient’s pupils were equal and reactive bilaterally; however,

113

deficits were seen with extraocular movements. Right cranial nerves III and VI were intact as the

114

right eye was able to adduct and abduct independently. While the patient was able to adduct the

115

left eye independently, left cranial nerve VI was not intact and the patient was unable to abduct

116

the left eye. He exhibited one-and-a-half syndrome with diplopia while looking to the right.

117

Although his left eye was able to adduct (oculomotor), his medial longitudinal fasciculus (MLF)

118

was unable to trigger his right abducens nerve. The patient’s preoperative cranial nerve V deficit

119

had improved with increased sensation in his V1 and V2 distributions. The patient also exhibited

120

cranial nerve VII deficit with House-Brackmann (HB) grade IV facial droop and inability to fully

121

close his left eye. The remainder of cranial nerves were intact. The patient was able to move all

122

extremities, with 5/5 muscle strength on his right side and slightly decreased 4+/5 strength on his

123

left. He was otherwise neurologically intact with no additional signs or symptoms. He was ready

124

for discharge on postoperative day 3, and his motor strength had improved to 5/5 on his

125

left. Gross total resection was confirmed on MRI (Figure 3).

126 127

Discussion

128

Cavernous malformations (CMs) of the brainstem account for 8-35% of all intracerebral

129

CMs.5,6,20 Compared to CMs in other locations, the natural history of brainstem CMs is marked

130

with a significantly higher risk of bleeding and rebleeding. .7 Some series have reported rates of

131

re-bleeding after an initial bleed as high as 45-60% per year.4,21 Patients typically present with

132

hemorrhage and focal neurologic deficits.5

133 134

Management of these lesions may present a challenge for neurosurgeons, as it’s often unknown

135

whether the inherent risks of operating within the brainstem outweigh the risks of an untreated

136

brainstem CM. While most authors agree that asymptomatic incidental brainstem cavernomas

137

generally can be managed conservatively with expectant observation, there is continued debate

138

regarding precise indications for surgical management of symptomatic cavernomas. In 2017 the

139

Angioma Alliance, led by a multidisciplinary group of expert CM clinicians, recommended that

140

surgical resection of brainstem CMs be offered after a second symptomatic bleed, but that

141

indications for resection after a single disabling bleed were weaker.2 Other authors propose that

142

it may be unwise to wait for a second symptomatic bleed.4,22,23 Samii et al concluded that despite

143

the postoperative morbidity associated with resection of brainstem CMs, the potential for severe

144

neurological deficits as a result of multiple rebleeding episodes often justifies swift surgical

145

management. The authors add that postoperative morbidity is commonly caused by changes in

146

microcirculation and edema in the adjacent structures, thus the neurological deficits seen

147

immediately postoperatively are often transient.24 Ultimately, the decision to proceed with

148

surgery for brainstem CMs should be multifactorial, with consideration given to age,

149

comorbidities, symptomatology, size of the lesion, location of the lesion, goals of treatment, and

150

the experience of the neurosurgeon.

151 152

We recount the case of a 27-year-old male who presented to our institution with a second

153

symptomatic bleed from a 4th ventricular CM. The clinical indications for surgery in this case

154

were clear, and the proximity of the lesion to the ependymal surface further favored surgery from

155

a technical standpoint.20,24 When approaching lesions located in the floor of the fourth ventricle

156

(also known as the rhomboid fossa), there are two identified safe entry zones, specifically the

157

suprafacial and infrafacial triangles, via a suboccipital approach. The suprafacial triangle is

158

bordered by the medial longitudinal fasciculus medially, the facial nerve caudally, and the

159

cerebellar peduncle laterally. Alternatively, the infrafacial triangle is bordered by the medial

160

longitudinal fasciculus medially, the striae medullaris caudally, and the facial nerve laterally

161

(Figure 4). While the infrafacial triangle is narrower and has more important neighboring

162

structures compared to the suprafacial triangle, we utilized the infrafacial triangle due to the

163

location of the lesion. For this approach, only lateral retraction of the brainstem should be

164

utilized as the facial nucleus is deeper in this region. Medial retraction risks damaging the medial

165

longitudinal fasciculus and rostral retraction risks damage to the abducens (CN VI) nucleus,

166

paramedian pontine reticular formation (PPRF), and the facial nerve (CN VII).25

167

168

The resection itself was uneventful and there were no intra-operative complications. The patient

169

did, however, experience a unique set of postoperative complications, most notably of which was

170

8.5 syndrome. This syndrome is a rare constellation of cranial nerve deficits characterized by the

171

combination of A) ipsilateral conjugate horizontal gaze palsy with ipsilateral internuclear

172

ophthalmoplegia (INO), jointly known as 1.5 syndrome, and B) CN VII palsy.26 In our patient,

173

the only remaining horizontal eye movement was contralateral (right) abduction, along with a

174

HB grade IV CN VII palsy. This discrete neurological finding can be caused by a simultaneous

175

interruption of the CN VII nucleus along with interruption of either A) the ipsilateral medial

176

longitudinal fasciculus (MLF) and ipsilateral PPRF, or B) the ipsilateral MLF and ipsilateral CN

177

VI nuclei. Though unfortunate, it represents an elegant manifestation of disruption of the local

178

anatomy and was likely unavoidable. To our knowledge, the present case is the first to report an

179

8.5 syndrome as a post-operative complication after CM resection.

180 181

The location of the CM in the present case is worth discussing, as its position within the 4th

182

ventricle is exceptionally uncommon. Kivelev et al reported that intraventricular CMs constitute

183

just 2.5-10.8% of all CMs and confirmed only 77 total published cases in their review. Of the 77,

184

only 12 were in the 4th ventricle.8 With the addition of five more cases that we found during our

185

literature review9,10,15,16,19 and including our own, a total of 18 4th ventricular CMs have ever

186

been reported (Table 1).

187 188

Upon reviewing the literature of 4th ventricular CMs, the most common presenting symptoms

189

included mass effect and intracerebral hemorrhage resulting in headache, nausea, and vomiting.

190

Including the present case, cranial nerve deficits were seen in 12 (71%) of the 17 cases,

191

particularly involving cranial nerves V, VI, and VII. All cases were treated surgically; several

192

reports documented previous bleeding episodes prior to the presentation as the indication for

193

surgical intervention. In 14 cases (78%), gross total resection was achieved. Those with partial

194

resection (n=4) had poor outcomes; one patient experienced a recurrence of symptoms, one

195

experienced symptom persistence with regrowth requiring retreatment, and the final two resulted

196

in death. While total resection resulted in resolution of neurological deficits at follow-up in 7,

197

symptoms persisted for the remaining patients. With such a limited sample size, it’s difficult to

198

draw definitive conclusions regarding the costs and benefits of surgery for 4th ventricular

199

cavernomas; however, the extent of the literature supports gross total resection in symptomatic

200

cases when safely achievable. With advancements in imagine-guided lesional mapping, neuro-

201

navigation, microscopic technique, and the cumulative experience of neurosurgeons worldwide,

202

these lesions can be managed successfully.

203 204

Conclusions

205

Cavernous malformations of the brainstem are a rare clinical entity that neurosurgeons must be

206

ready to face. Factors like high rates of hemorrhage and rehemorrhage and proximity to eloquent

207

brain make management decision-making a daunting task for physicians and patients alike.

208

Although many cases have been successfully treated with resection, the indications for surgical

209

intervention remain debated. Therefore, management must be tailored carefully to each the

210

patient based on clinical presentation, lesion’s location, and thorough surgical risk assessment.

211

References

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1.

Stapleton CJ, Barker FG. Cranial Cavernous Malformations. Stroke. 2018;49(4):1029-1035.

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Akers A, Al-Shahi Salman R, A. Awad I, et al. Synopsis of Guidelines for the Clinical

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Management of Cerebral Cavernous Malformations: Consensus Recommendations Based on

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Systematic Literature Review by the Angioma Alliance Scientific Advisory Board Clinical

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Experts Panel. Neurosurgery. 2017;80(5):665-680.

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stereotactic radiosurgery for cavernous malformations. J Neurosurg. 1995;83(5):825-831.

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Horne MA, Flemming KD, Su IC, et al. Clinical course of untreated cerebral cavernous malformations: a meta-analysis of individual patient data. Lancet Neurol. 2016;15(2):166-173.

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Wang CC, Liu A, Zhang JT, Sun B, Zhao YL. Surgical management of brain-stem cavernous malformations: report of 137 cases. Surg Neurol. 2003;59(6):444-454; discussion 454.

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Kondziolka D, Lunsford LD, Flickinger JC, Kestle JR. Reduction of hemorrhage risk after

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Arauz A, Patiño-Rodriguez HM, Chavarria-Medina M, Becerril M, Longo GM, Nathal E.

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Rebleeding and Outcome in Patients with Symptomatic Brain Stem Cavernomas.

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Cerebrovascular Diseases. 2017;43(5-6):283-289.

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Taslimi S, Modabbernia A, Amin-Hanjani S, Barker FG, Macdonald RL. Natural history of

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cavernous malformation. Systematic review and meta-analysis of 25 studies. 2016;86(21):1984-

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of 12 patients and review of the literature. J Neurosurg. 2010;112(1):140-149.

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Moro M, Longatti PL, Cisotto P, Baratto V, Carteri A. Growing patterns of cavernous angioma in the fourth ventricle. Case report. J Neurosurg Sci. 1998;42(4):221-225.

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Koyama T, Kyoshima K, Okudera H, Kobayashi S. Surgery of a cavernous angioma making a tunnel between the fourth ventricle and tubercle. Neurosurg Rev. 2001;24(1):38-40.

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Kivelev J, Niemela M, Kivisaari R, Hernesniemi J. Intraventricular cerebral cavernomas: a series

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Finkelnburg R. Zur Differentialdiagnose zwischen Kleinhirntumoren und chronischem

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Hydrocephalus. (Zugleich ein Beitrag zur Kenntnis der Angiome des Zentralnervensystems).

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Deutsche Zeitschrift f Nervenheilkunde. 1905;29(1-2):135-151.

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and review of the literature. Acta Neurochir (Wien). 1978;40(1-2):61-82.

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Yoshimoto T, Suzuki J. Radical surgery on cavernous angioma of the brainstem. Surg Neurol. 1986;26(1):72-78.

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Yamasaki T, Handa H, Yamashita J, et al. Intracranial and orbital cavernous angiomas. 1986;64(2):197.

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Terao H, Hori T, Matsutani M, Okeda R. Detection of cryptic vascular malformations by computerized tomography. Report of two cases. J Neurosurg. 1979;51(4):546-551.

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Pozzati E, Gaist G, Poppi M, Morrone B, Padovani R. Microsurgical removal of paraventricular cavernous angiomas. Report of two cases. J Neurosurg. 1981;55(2):308-311.

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Kuroiwa T, Fujimoto T, Aoyagi M, Inaba Y. [Cavernous hemangioma on the fourth ventricle floor. Report of a successfully treated case]. Neurol Med Chir (Tokyo). 1983;23(4):305-310.

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Kendall B, Reider-Grosswasser I, Valentine A. Diagnosis of masses presenting within the ventricles on computed tomography. Neuroradiology. 1983;25(1):11-22.

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Itoh J, Usui K. Cavernous angioma in the fourth ventricular floor--case report. Neurol Med Chir (Tokyo). 1991;31(2):100-103.

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Giombini S, Morello G. Cavernous angiomas of the brain. Account of fourteen personal cases

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Ohue S, Fukushima T, Kumon Y, Ohnishi T, Friedman AH. Surgical management of brainstem

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cavernomas: selection of approaches and microsurgical techniques. Neurosurg Rev.

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2010;33(3):315-322; discussion 323-314.

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Georgieva VB, Krastev ED. Surgical Treatment of Brainstem Cavernous Malformation with

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Concomitant Developmental Venous Anomaly. In: Asian J Neurosurg. Vol 14. India2019:557-

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Hauck EF, Barnett SL, White JA, Samson D. Symptomatic brainstem cavernomas. Neurosurgery. 2009;64(1):61-70; discussion 70-61.

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Sandalcioglu IE, Wiedemayer H, Secer S, Asgari S, Stolke D. Surgical removal of brain stem

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cavernous malformations: surgical indications, technical considerations, and results. J Neurol

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Neurosurg. 2001;95(5):825-832.

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Samii M, Eghbal R, Carvalho GA, Matthies C. Surgical management of brainstem cavernomas. J

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Kyoshima K, Kobayashi S, Gibo H, Kuroyanagi T. A study of safe entry zones via the floor of

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the fourth ventricle for brain-stem lesions. Report of three cases. J Neurosurg. 1993;78(6):987-

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Mesina BVQ, Sosuan GMN, Reyes KB. Eight-and-a-half syndrome: a rare potentially lifethreatening disease. In: GMS Ophthalmol Cases. Vol 8. Germany2018:Doc04.

272 273

Figure Legends

274

Figure 1—Preoperative imaging of 4th ventricle cavernous malformation. MRI (A, Axial T2;

275

B, Axial T1; C, Sagittal T1) shows mixed intensity lesion at the left side of the 4th ventricular

276

floor consistent with acute on chronic hemorrhage of a cavernous malformation. Mixed density

277

round lesion on CT head with no contrast (D) is suggestive of acute on chronic hemorrhage.

278

Figure 2—Intraoperative images showing A) pial surface of the 4th ventricular floor with

279

discoloration indicating the target lesion underneath (green arrow). This coincided with the safe

280

entry region of the 4th ventricular floor. B) After corticectomy, the lesion was readily seen, and a

281

dissection plane was identified (green dashed line). C) Post-resection cavity after careful

282

hemostasis and irrigation (green arrow).

283

Figure 3—Postoperative imaging of 4th ventricle cavernous malformation. MRI (A, Axial

284

T2; B, Axial T1; C, Sagittal T1) shows a cavity in the floor of the 4th ventricle, previously

285

occupied by the lesion, no longer demonstrating blood products.

286

Figure 4—Anatomy of the Suprafacial and Infrafacial Triangles in the Rhomboid Fossa.

287

The suprafacial triangle (A) is bordered by the medial longitudinal fasciculus, the facial nerve,

288

and the cerebellar peduncle. The infrafacial triangle (B) is bordered by the medial longitudinal

289

fasciculus, the striae medullaris, and the facial nerve. FC = facial colliclus, VI = abducens

290

nucleus, VII = facial nucleus, VII nerve = facial nerve, MLF = medial longitudinal fasciculus.

291

Table 1—Characteristics, presentation, treatment, and outcome among 18 cases of 4th ventricular

292

cavernous malformations.

Table 1 Author, Year Finkelnburg, 1905

Age*, Sex 14, M

Dandy, 1928

31, M

Giombini & Morello, 1978

27, M

Terao et al., 1979

29, F

Pozzati et al., 1981

36, M

Kendall et al., 1983 Kuroiwa et al., 1983

60, F

Yamasaki et al., 1986

47, M

Yoshimoto & Suzuki, 1986

16, M

Itoh & Usui, 1991

44, F

Moro et al., 1998 Koyama et al., 2001

66, F

44, F

53, F

Kivelev et al., 43, F 2010

Presentation Headache, vomiting, left CN VI palsy, dizziness, multiple bleeds Headache, nystagmus, left extremity motor loss and hypesthesia Seizures, left CN VII palsy, cerebellar symptoms, nystagmus, left limb hypotonia, right hypesthesia Headache, nausea, vomiting, dizziness, multiple bleeds

Treatment Partial resection

Outcome Died (7 hours after operation)

Total resection

Improved

Partial resection, radiation

Died (2 months after operation)

Total resection

Vomiting, diplopia, dizziness, cerebellar symptoms, gait disturbance Mass effect

Total resection

Cerebellar symptoms persisted for 2 weeks; resolved by discharge Cerebellar symptoms persisted for 4 weeks; resolved by 1 year Symptom recurrence

Diplopia, ophthalmoplegia, right CN V hypesthesia, left CN VII palsy Headache, vomiting, cerebellar symptoms, SAH, IPH Nausea, vomiting, left CN V, VII-X, XII palsies, cerebellar symptoms Headache, vomiting, bilateral papilledema, diplopia, left CN V-VI palsies, dysarthria, cerebellar symptoms, IVH Left CN VI and VII palsy, nystagmus, gait disturbance CN deficit, cerebellar symptoms, right extremity motor loss and hypesthesia Headache, nausea, vomiting

Partial resection Total resection

Left CN VII palsy

Total resection

Mild left CN VI palsy

Total resection

Mild left CN V, VII-IX palsies

Total resection

Cerebellar symptoms persisted for 3 weeks; resolved at follow-up

Partial resection Total resection

Symptom persistence and regrowth of lesion Symptom-free

Total resection

Cerebellar symptoms at discharge; resolved at follow-up Worsened CN VI-VII palsies at discharge; returned to same as pre-op by follow-up New diplopia, CN VII palsy, and cerebellar symptoms at discharge; CN VII palsy persisted at

58, F

Headache, nausea, vomiting, CN VI-VII palsies, IVH

Total resection

15, M

CN deficit, IVH

Total resection

follow-up

Current study

*Age in years

49, F

CN deficit

Total resection

49, F

Headache, hydrocephalus, IVH

Total resection

27, M

Headache, nausea, vomiting, diplopia, left CN V1/V2 paresthesia, gait disturbance, multiple bleeds

Total resection

New CN VI-VII palsies, worsening diplopia and cerebellar symptoms at discharge; CN VI-VII palsies persisted at followup Nausea and vomiting at discharge; resolved at follow-up Improved CN V paresthesia, left CN VI palsy, left eightand-a-half syndrome

Table 1 Author, Year Finkelnburg, 1905

Age*, Sex 14, M

Dandy, 1928

31, M

Giombini & Morello, 1978

27, M

Terao et al., 1979

29, F

Pozzati et al., 1981

36, M

Kendall et al., 1983 Kuroiwa et al., 1983

60, F

Yamasaki et al., 1986

47, M

Yoshimoto & Suzuki, 1986

16, M

Itoh & Usui, 1991

44, F

Moro et al., 1998 Koyama et al., 2001

66, F

Kivelev et al., 2010

43, F

44, F

53, F

Presentation Headache, vomiting, left CN VI palsy, dizziness, multiple bleeds Headache, nystagmus, left extremity motor loss and hypesthesia Seizures, left CN VII palsy, cerebellar symptoms, nystagmus, left limb hypotonia, right hypesthesia Headache, nausea, vomiting, dizziness, multiple bleeds

Treatment Partial resection

Outcome Died (7 hours after operation)

Total resection

Improved

Partial resection, radiation

Died (2 months after operation)

Total resection

Vomiting, diplopia, dizziness, cerebellar symptoms, gait disturbance Mass effect

Total resection

Cerebellar symptoms persisted for 2 weeks; resolved by discharge Cerebellar symptoms persisted for 4 weeks; resolved by 1 year Symptom recurrence

Diplopia, ophthalmoplegia, right CN V hypesthesia, left CN VII palsy Headache, vomiting, cerebellar symptoms, SAH, IPH Nausea, vomiting, left CN V, VII-X, XII palsies, cerebellar symptoms Headache, vomiting, bilateral papilledema, diplopia, left CN V-VI palsies, dysarthria, cerebellar symptoms, IVH Left CN VI and VII palsy, nystagmus, gait disturbance CN deficit, cerebellar symptoms, right extremity motor loss and hypesthesia Headache, nausea, vomiting

Partial resection Total resection

Left CN VII palsy

Total resection

Mild left CN VI palsy

Total resection

Mild left CN V, VII-IX palsies

Total resection

Cerebellar symptoms persisted for 3 weeks; resolved at follow-up

Partial resection Total resection

Symptom persistence and regrowth of lesion Symptom-free

Total resection

Cerebellar symptoms at discharge; resolved at follow-up Worsened CN VI-VII palsies at discharge; returned to same as pre-op by follow-up New diplopia, CN VII palsy, and cerebellar symptoms at discharge; CN

58, F

Headache, nausea, vomiting, CN VI-VII palsies, IVH

Total resection

15, M

CN deficit, IVH

Total resection

Current study

*Age in years

49, F

CN deficit

Total resection

49, F

Headache, hydrocephalus, IVH

Total resection

27, M

Headache, nausea, vomiting, diplopia, left CN V1/V2 paresthesia, gait disturbance, multiple bleeds

Total resection

VII palsy persisted at follow-up New CN VI-VII palsies, worsening diplopia and cerebellar symptoms at discharge; CN VI-VII palsies persisted at followup Nausea and vomiting at discharge; resolved at follow-up Improved CN V paresthesia, left CN VI palsy, left eightand-a-half syndrome

Abbreviations

- CM: Cavernous malformation - MFL: medial longitudinal fasiculus - MRI: magnetic resonance imaging - HB: House-Brackmann - PPRF: paramedian pontine reticular formation - INO: internuclear ophthalmoplegia