An isolated cavernous malformation of the sixth cranial nerve: A case report and review of literature
- Department of Neurosurgery, Shahid Beheshti University of Medical Sciences, Tehran, Iran,
- Skull Base Research Center, Loghman Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran,
- Faculty of Medicine, Tehran University of Medical Sciences, Valiasr, Tehran, Iran,
- Department of Neurosurgery, Day General Hospital, Valiasr, Tehran, Iran,
- Department of Neurosurgery, Neurological Institute, Cleveland Clinic-Taussig Cancer Center, Cleveland, Ohio, United States.
Mehrdad Hosseinzadeh Bakhtevari, Department of Neurosurgery, Day General Hospital, Valiasr, Tehran, Iran.
DOI:10.25259/SNI_811_2021Copyright: © 2021 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Mohammad Samadian1,2, Seyed Farzad Maroufi2,3, Mehrdad Hosseinzadeh Bakhtevari4, Hamid Borghei-Razavi5. An isolated cavernous malformation of the sixth cranial nerve: A case report and review of literature. 16-Nov-2021;12:563
How to cite this URL: Mohammad Samadian1,2, Seyed Farzad Maroufi2,3, Mehrdad Hosseinzadeh Bakhtevari4, Hamid Borghei-Razavi5. An isolated cavernous malformation of the sixth cranial nerve: A case report and review of literature. 16-Nov-2021;12:563. Available from: https://surgicalneurologyint.com/surgicalint-articles/11226/
Background: Isolated cavernous malformation (CM) of the abducens nerve has not been reported in the literature. Herein, the authors address the clinical importance of these lesions and review the reported cases of CM from 2014 to 2020.
Case Description: A 21-year-old man presented with binocular diplopia and headache from 2 months before his admission. The neurological examination revealed right-sided abducens nerve palsy. The brain MRI revealed an extra-axial pontomedullary lesion suggestive of a CM. The lesion was surgically removed. During the operation, the abducens nerve was resected considering the lesion could not be separated from the nerve and an anastomosis was performed using an interposition nerve graft and fibrin glue. Pathological examination of the resected lesion revealed that it was originated from within the nerve. The patient’s condition improved in postoperative follow-ups.
Conclusion: Surgical resection of the cranial nerves CMs is appropriate when progressive neurological deficits are present. If the lesion is originated from within the nerve, we suggest resection of the involved nerve and performing anastomosis. Novel MRI sequences might help surgeons to be prepared for such cases and fibrin glue can serve as an appropriate tool to perform anastomosis when end-to-end sutures are impossible to perform.
Keywords: Abducens nerve, Cavernous hemangioma, Cranial nerves, Surgical resection
Cavernous malformations (CMs) are occult vascular lesions, defined as hyalinized capillary clusters, without an intervening muscle or neural tissue. These lesions account for 5–15% of all central nervous system vascular lesions[
The supratentorial region is the most common location for CMs followed by the posterior fossa in the pons and the cerebellum.[
Although uncommon, CMs with the involvement of different cranial nerves have been reported in the literature. In general, lesions on the abducens nerve are extremely rare. There is only one case of CM associated with the abducens nerve that originated from the Dorello’s canal and encircled the nerve. Herein, we describe the first case of a pathologically proven isolated intraneural and intrafascicular CM of the cranial nerve.
A 21-year-old man was admitted to our clinic with a sudden and progressive headache and binocular diplopia. His symptoms had appeared 2 months before his first visit to the clinic. He had also experienced a similar attack 6 months earlier, but he was in a good general condition. The neurological examination revealed right-sided abducens nerve palsy. A brain computed tomography (CT) scan was acquired, which showed a suspected lesion in the cerebellopontine angle. Brain CT angiography was also performed, which did not reveal any prominent findings. The brain MRI showed an extra-axial pontomedullary lesion with the classic appearance of a CM, that is, a reticulated salt-and-pepper core, surrounded by a halo rim [
The patient underwent surgery using the suboccipital retrosigmoid approach with intraoperative neuromonitoring. The procedure was performed through general anesthesia as the patient laid in a lateral position, and his head was fixed in a three-pin Mayfield headrest. After opening the dura, a CM was detected, arising from the sixth cranial nerve, with no invasion to the brainstem or the surrounding tissues. Since the lesion could not be separated from the nerve and its integrity was unclear, we resected the CM by cutting the proximal and distal portions (before the Dorello’s canal) of the sixth cranial nerve [
Intraoperative images. (a) A reddish pulsating lesion in the cerebellopontine angle; (b) view of the CM (white arrow) and the distal course of the abducens nerve entering the Dorello’s canal (black arrow); (c) severed abducens nerve distal to the lesion (black arrow); (d) the resected CM with part of the abducens nerve (black arrow), passing through the lesion; (e) the prepared nerve graph (3 cm of the greater auricular nerve); (f) resection cavity after removal of the lesion, with the interposition nerve graft placed meticulously between the proximal and distal ends of the abducens nerve.
The greater auricular nerve (>3 cm) was harvested as an interposition nerve graft. An anastomosis was performed using a fibrin glue sealant, as end-to-end suture was impossible. One day after surgery, the patient showed complete sixth nerve palsy. He was discharged on the 4th postoperative day, without any neurological deficit, except for the right abducens nerve palsy. In the 5-month follow-up visit, he showed complete recovery of the cranial deficit and diplopia. The histopathological examination of the lesion demonstrated typical characteristics of a CM [
Tumors associated with the peripheral nerve are rare entities. Depending on their origin, peripheral nerve tumors are classified into nerve sheath tumors and nonneural sheath tumors.[
The cranial nerves, as a part of the peripheral nervous system, may be affected by CMs. In 2014, Rotondo et al.[
To the best of our knowledge, this is the first case of a pathologically proven isolated intraneural and intrafascicular CM of the cranial nerve. Only one case of the abducens nerve involvement has been described in the literature.[
Our patient presented with recurrent attacks of progressive diplopia, which showed a similar pattern to other cases of cranial nerve CMs, with acute or progressive neurological deficits, associated with the involved nerve. The recurrence of attacks is probably due to hemorrhage, as reported in the latter case. Although some reported cases of cranial nerve CMs associated with nerve deficits resolved without any intervention,[
MRI imaging is crucial for the evaluation of CMs, especially if hemorrhage has occurred previously. In T2-weighted gradient-echo images, lesions with mixed intensity and reticulated cores, surrounded by hypointense rims (corresponding to hemosiderin deposition), suggest CMs. If intact CMs occur, there may not be any prominent feature characteristic of CMs, except for a vague enhancement on gadolinium-based contrast-enhanced MRI. In our case, MRI showed a CM with a previous hemorrhage located at the pontomedullary angle, but we could not determine the origin of the lesion. An important issue that has not been resolved yet is whether CMs develop within the nerve or originate from outside, encompassing the nerve. Although T1- and T2-weighted images are frequently used for the diagnosis of CMs, they cannot provide adequate information about these lesions. The constructive interference in steady-state (CISS) and fast imaging employing steady-state acquisition (FIESTA) sequences is proven to be useful in revealing the lesion boundaries and their relations to the surrounding tissues.[
Full functional recovery after the surgical repair of the transected abducens nerve has been previously reported.[
CMs of the cranial nerve, although rare, pose a significant threat to the nerve functions. Except for our patient, only one other case of CM affecting the abducens nerve was reported earlier. Due to the limited number of cases and scarce evidence, no definite recommendation is available on the indications of surgery and treatment. However, it seems essential to treat patients with progressive neurological deficits, and physicians should consider novel MRI sequences (e.g., CISS/FIESTA), as the traditional sequences do not provide enough information regarding the nature and location of the lesions.
The authors certify that they have obtained all appropriate patient consent.
There are no conflicts of interest.
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