|Year : 2021 | Volume
| Issue : 2 | Page : 92-97
Optico-chiasmatic hypothalamic cavernomas - A report of three cases and review of literature
Roopesh V R Kumar, Arunkumar Karthikayan, Adhithyan Rajendran
Apollo Proton Centre, Chennai, Tamil Nadu, India
|Date of Submission||21-Jan-2022|
|Date of Decision||24-Jan-2022|
|Date of Acceptance||26-Jan-2022|
|Date of Web Publication||5-Apr-2022|
Dr. Roopesh V R Kumar
Apollo Proton Centre, Chennai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Optochiasmatic hypothalamic cavernous malformations (CMs) are exceedingly rare lesions and can manifest with visual deficits of varying magnitudes. They also can result in sudden-onset visual loss due to apoplexy. Magnetic resonance imaging can delineate the lesions and differentiate it from other common lesions like glioma or craniopharyngioma. Preoperative visual assessment including perimetry and optical coherence tomogram has to be performed whenever possible to assess the degree of deficits and also for prognostication. Microsurgical excision improves the visual deficits in majority of instances as documented by earlier reports and has to be undertaken as an emergency in apoplexy. Various corridors can be used to reach the lesion including anterolateral, midline transbasal anterior interhemispheric approach, and transnasal endoscopic approaches. The aim should be to achieve a gross total excision with minimal manipulation of surrounding white matter tracts to improve the visual outcome. There is a limited role for stereotactic radiosurgery. In the present study, we report three cases of CMs involving optic chiasm and hypothalamus including a case of apoplexy managed by gross total microsurgical resection with good outcome and also review the relevant literature on the natural history and management strategies
Keywords: Apoplexy, cavernous malformation, microsurgical excision, optochiasmatic hypothalamic, stereotactic radiosurgery
|How to cite this article:|
Kumar RV, Karthikayan A, Rajendran A. Optico-chiasmatic hypothalamic cavernomas - A report of three cases and review of literature. J Cerebrovasc Sci 2021;9:92-7
|How to cite this URL:|
Kumar RV, Karthikayan A, Rajendran A. Optico-chiasmatic hypothalamic cavernomas - A report of three cases and review of literature. J Cerebrovasc Sci [serial online] 2021 [cited 2022 May 22];9:92-7. Available from: http://www.jcvs.com/text.asp?2021/9/2/92/342565
| Introduction|| |
Cavernous malformations (CMs), also called cavernoma or cavernous angioma, are low flow, angiographically occult, histologically benign lesions occurring in the brain parenchyma, brain stem and the spinal cord.,, They constitute up to 20% of vascular malformations of the central nervous system. CMs involving optochiasmatic-hypothalamic (OPH) region are exceedingly rare and constitute <1% of all intra cranial CMs.,, Till date, only 87 cases had been reported in the literature. Most often, they manifest with varying degrees of visual deficits.,,, They can also cause sudden visual loss due to chiasmal apoplexy.,, Prompt recognition and early surgical intervention results in a good visual outcome in majority of the situations., Most often, a complete and curative excision is feasible as they are well circumscribed and do not have intervening white matter tracts., Biopsy alone has the risk of causing recurrent microbleeds resulting in progressive visual deficits.
| Case Reports|| |
A 32-year-old female presented with recurrent headache with visual blurring of 1-year duration. She was initially evaluated by an ophthalmologist and was managed with spectacles for refractory error. Her symptoms did not improve and hence was evaluated with visual perimetry. Her right eye acuity was 6/36 and left eye was 6/24. Visual field by perimetry showed bilateral inferior altitudinal defects [Figure 1]. A magnetic resonance imaging (MRI) of the brain with gadolinium-enhanced studies showed a heterogeneous lesion in the optic chiasm (OC) which was hyperintense on T1 W images and hypo on T2W images, not demonstrating enhancement on contrast [Figure 2]. The typical popcorn appearance on T1W images with hemosiderin rim was suggestive of CM of the OC. In view of the visual deficits, she was planned for microsurgical excision through a minimally invasive transciliary fronto-orbital craniotomy. After opening the right medial Sylvian fissure, the optic nerve (ON) and the internal carotid artery (ICA) were identified. On further medial dissection, the OC was seen and the lamina terminalis (LT) was bulging with bluish discoloration [Figure 3]. The LT was widely opened and the CM was seen. There were areas of microhemorrhages inside the lesion and the lesion as such was fibrous. However, there was a reasonably good plane and the capsule could thus be dissected from the surrounding gliotic plane. The lesion was gross totally excised except the hemosiderin rim over the right (ON) and chiasm junction [Figure 3]. Postoperatively, she had improvement in vision of both the eyes. Perimetry done after a month showed significant improvement in the inferior altitudinal field defect [Figure 1]. Visual acuity in the right improved to 6/12 and 6/6 in the left eye. MRI brain done after 6 weeks showed good excision of the tumor except for the hemosiderin rim along the right ON-OC junction. At 3 years of follow-up, she is doing well without visual decline and headache.
|Figure 1: Visual fields by perimetry showing bilateral inferior altitudinal defects in the left eye (a) and the right eye (b). Perimetry done after a month showed significant improvement in the inferior altitudinal field defect in both the left (c) and the right side (d)|
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|Figure 2: T1 hyperintense (a, T1 axial) and T2, T2 FLAIR (b- T2 coronal, c- T2 FLAIR axial) mixed intense well defined bi-lobed mass lesion seen at the optic chiasm region. Lesion showed no significant contrast enhancement (d- T1 postcontrast axial). Magnetic resonance imaging features are in favor of a lesion with varying stages of bleed, possibly cavernoma. Postoperative magnetic resonance imaging, T2 and T1 axial magnetic resonance imaging sequences (e and f) showed near-complete excision of the mass lesion with tiny residual|
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|Figure 3: The lamina terminalis is bulging with bluish discoloration (a). The lesion was gross totally excised (b) except the hemosiderin rim over the right optic nerve and chiasm junction|
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|Figure 4: Computerized tomogram scan plain coronal section (a) showed hyperdense bleed at bilateral foramen of Monro with a hyperdense lesion at the optic chiasm, hypothalamus, 3rd ventricle region. Magnetic resonance imaging scan showed T1 (b-T1 sagittal) and T2 FLAIR (c- T2 FLAIR axial) hyperintense an irregular lobulated lesion at the optic chiasm, hypothalamus, inferior third ventricle region, with peripheral rim of T2 hypointensity (d-T2 axial) suggestive of a mass lesion with subacute bleed. Postoperative magnetic resonance imaging (e- sagittal T2, f- coronal T2) showed complete excision of the mass lesion with no significant residual|
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|Figure 5: Magnetic resonance imaging showed T2 mixed intense lesion (a, b-T2 axial) with “popcorn” like appearance noted involving the optic chiasm, hypothalamus, third ventricle region. Lesion showed hyperintensities in T1 postcontrast (c,d) (no significant contrast enhancement seen in subtraction sequence). Lesion causes mass effect over the optic tracts, midbrain, bilateral thalamus. Magnetic resonance imaging features are classical for cavernoma. Post-surgery magnetic resonance imaging-T2 (e-T2 coronal), postcontrast sagittal T1 (f) showed complete excision of the mass lesion with no significant residual|
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A 15-year-old female developed a sudden-onset severe headache and altered sensorium. She was rushed to a nearby hospital where a plain computerized tomogram (CT) of the brain was done which showed acute hemorrhage in the suprasellar cistern with intraventricular extension. She was diagnosed as a ruptured anterior communicating artery and was then referred to our center. On receiving, she was drowsy, arousable, and following simple commands. An emergency CT angiogram of the brain did not reveal any vascular pathology. MRI of the brain [Figure 4]showed a lesion arising from the posterior aspect of OC with hemorrhage and surrounding hemosiderin rim. In addition, she had a left Sylvian large arachnoid cyst without major mass effect. She underwent an emergency left pterional craniotomy and through the left trans-Sylvian route (the arachnoid cyst aided in dissection), the left ON, OC was dissected. The LT was seen bulging with bluish discolouration and was opened. Fresh clots were evacuated and the typical mulberry like lesion was found and was excised en mass. The plane was good and the fresh hemorrhage aided in dissection. Ventricular cavities were irrigated with warm saline and wound closed in layers. There was transient diabetes insipidus in the early postoperative period and was managed by pediatric endocrinologist. Sensorium improved well and she was discharged after a week. Repeat MRI of the brain at 2-month follow-up showed no residual disease and her visual and endocrinological assessment was normal.
A 26-year-old male presented with progressive headache, change in behavior, and visual blurring of 6 months' duration. Visual assessment showed visual acuity of 6/24 in both the eyes with mild temporal pallor. As his attention span was diminished, a formal perimetry could not be performed. Endocrinological evaluation revealed hypothyroidism and prolactin levels were marginally elevated. His serum osmolality and sodium levels were high suggesting chronic diabetes insipidus. MRI of the brain with contrast [Figure 5] revealed a large heterogeneous lesion filling the entire third ventricle and lifting the foramen of Monro bilaterally. On gadolinium administration, there was a subtle enhancement. The interior core of the lesion showed a mosaic pattern-like appearance with areas of hemosiderin rim around. A diagnosis of hypothalamic glioma versus craniopharyngioma was considered, although the possibility of CM was not completely ruled out. Through a right fronto-orbito zygomatic craniotomy and trans-LT approach, the lesion was approached. The internal core of the lesion showed multiple areas of hemorrhages in different stages and the typical mulberry nature of the lesion was suggestive of cavernous angioma as confirmed by squash cytology. Through a meticulous microsurgical dissection, the lesion was removed in piecemeal with great care while dissecting off the walls of the third ventricle. Gross total tumor excision was achieved as confirmed by postoperative imaging. He had a stormy postoperative period with fluid, electrolyte disturbances, intermittent seizure episodes, and waxing and waning of sensorium. After 10 days of intensive medical management in critical care unit involving neuro-intensivist and endocrinologist, he was shifted to step down care unit and underwent rigorous neurorehabilitation. He was finally discharged after a month of stay in hospital and continued on best medical management. He showed slow and steady neurological recovery and at 3 months of follow-up, he was independent and could take care of his daily activities. A repeat MRI of the brain showed no residual disease and visual acuity was 6/6 in both the eyes.
| Discussion|| |
Incidence and presentation
CMs of the brain have an incidence of 0.3%–0.7%.,,,, CMs involving OPH region are extremely rare and constitute <1%.,, A detailed systematic literature searches of the reported cases suggested only 87 cases of CMs appearing in the OPH region. If we add the three cases in this study, the total cases would be 90. OC was more commonly involved (87%), followed by ON and least in hypothalamus (5/87). Most patients present with symptoms in their 2nd–4th decade of life. There is generally no sex predilection, but hemorrhage reportedly occurs more frequently in females than in males.,, Chiasmal apoplexy is one of the most common clinical presentations as in case 2, characterized by sudden visual disturbance, headache, and altered sensorium.,,,, Symptoms can also occur in a chronic or progressive manner with intermittent episodes of headache and visual loss, as in case 1. Hypothalamic disturbances can be the predominant symptom if the epicenter is in hypothalamus, as in case 3.
The natural history of OPH CMs is not clear because most of the reported cases have undergone surgical resection., The annual rate of hemorrhage associated with cerebral CM is around 0.7%–3.1%. The risk of re-bleed ranges from 3.8%–22% which is high in the first 3 years after bleed and reduces thereafter.,,
MRI of the brain is the most sensitive test to diagnose CM. It is characterized by the typical popcorn appearance attributed to the different stages of blood products and a surrounding hemosiderin rim. Unlike in the cerebral hemispheres, the hemosiderin rim may not be obvious in the ventricular side of the OPH region as it can be washed away by the flow of cerebrospinal fluid (CSF). Conventionally, they do not enhance after administering gadolinium and are almost always angiographically occult.,
A detailed visual assessment is mandatory prior to intervention. Perimetry can quantify the field defect attributable to the CM and can be used to demonstrate the improvement post surgery. However, in the acute presentation, as in chiasmal apoplexy (like in case 2), perimetry may not be possible always. Optical coherence tomogram is emerging as a valuable tool nowadays to assess the retinal nerve fiber layer thickness (RNFL) and thickness could be used to prognosticate the visual recovery post surgery. A markedly reduced RNFL thickness can be a poor prognostic factor for visual recovery.,
Indications for surgery
Considering the natural history of the disease and the progressive visual impairment owing to the mass effect on the chiasm, most cases reported in the literature have undergone surgical excision.,,,,,,,,,, Most cases reported had documented dramatic improvement of the visual deficits following surgical extirpation.,,,,,,,,,,,, Hence, just observation or wait and watch policy may not be the ideal approach in CMs of the optic chasm unless they are incidentally detected without visual deficits. Chiasmal apoplexy is a surgical emergency and an ultra-early intervention can reverse the visual deficits, as shown in many reports.,,,,, In the review study by Liu et al., most patients (97%) were treated surgically which included gross total resection in 60%, subtotal resection in 6%, biopsy in 6%, biopsy with decompression of the haemorrhage in 23%, and biopsy followed by radiation in 2% of cases.,,,,,,,,,
In the present study, case 1 had progressive visual deficits and had promptly reversed following surgery. In case 2, it was apoplexy requiring emergency intervention and had an excellent outcome. Although preoperative visual assessment including perimetry could o't be done in view of altered sensorium, we strongly believe that visual deficits would have been reversed following emergency intervention and postoperative visual assessment was normal. In case 3, it was more of hypothalamic syndrome and the aim was to stabilize the disease progression by preventing rebleed and relieving the mass effect on hypothalamus.
Most of the OC CMs project through the LT and the surgical corridors should lead to OC and LT in the shortest route possible. Various surgical approaches had been undertaken for OPH CMs in the earlier case studies and the most commonly used corridor was anterolateral approach through frontotemporal, pterional, or its larger variant, fronto-temporo-orbito-zygomatic (FTOZ) craniotomy (76%).,,,,,,,,, Few studies have used transbasal corridor through anterior interhemispheric approach (17%). Minimally invasive procedures like transciliary mini supra brow craniotomy (two cases) and transnasal endoscopic excision (three cases) were also rarely performed.,,, The limitations of the anterolateral approach are the limited visibility of the ipsilateral ON-OC junction which remains a blind corner.,, Midline approaches through transbasal corridor can give bilateral symmetric exposure but are more extensive and requires significant brain retraction. Transnasal endoscopic approach involves opening ventricles through a potentially contaminated corridor with risk of infection and CSF leak.,, In the present study, case 1 was approached through minimally invasive transciliary fronto-orbito craniotomy as the lesion was relatively small. In case 2, a pterional craniotomy was used in view of an emergency situation. In case 3, the lesion was large reaching up to FOM, and hence, FTOZ craniotomy was used.
In view of the eloquent location, it is very important to remain strictly in the gliotic plane surrounding the cavernoma to prevent damage to the visual fibres by surgical manipulation.,,, Internal decompression of the micro hemorrhages and soft components will allow the tumor to shrink aiding in peripheral dissection. Gentle warm saline irrigation in the interface between the tumor and the surrounding gliotic plane can aid in meticullous microsurgical dissection (as done in excision of brainstem cavernoma). Damage to the visual fibres can happen either mechanically due to surgical manipulation or thermally due to bipolar coagulation. Both can be prevented by avoiding cautery in the tumor - visual fibre interface and by leaving behind the hemosiderin rim.
Role of radiosurgery
The role of stereotactic radiosurgery for CM is very controversial and had been used in eloquent unresectable locations like brain stem with equivocal results. However, there is a very limited role for radiosurgery in OC CMs in view of the high risk of radiation-induced toxicity to the optic apparatus., Furthermore, preoperative diagnosis of CM is still difficult and doing radiosurgery without histopathological proof may not be the ideal way of treatment.
In the review article by Liu et al., 75% of patients experienced an improvement in vision following surgical decompression and the remaining 20% had a stable vision. There was visual deterioration only in 5%. Maximum improvement was seen in those who underwent gross total excision.,, In chiasmal apoplexy, even complete visual loss had been reversed by emergency decompression of the optic apparatus.
| Conclusion|| |
Optochiasmatic hypothalamic CMs are rare lesions and can cause varying degrees of visual deficits including sudden visual loss due to apoplexy. Prompt and early intervention by meticulous microsurgical excision of the lesion could result in a good visual outcome. Surgeons should use the most familiar corridor to extirpate the lesion in the least traumatic manner. The role of radiosurgery is limited.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest/b>
There are no conflicts of interest.
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