A 40-year-old female with past medical history of migraine and no family history of CM, presented with unilateral scotomas with a gradual onset over 7 months and progressive visual deterioration, referred as blurred vision of the right eye (RE), with a significant worsening few days before seeking medical care.\nShe was initially admitted at Ophthalmology outpatient clinic for observation. The ophthalmologic evaluation revealed a decreased visual acuity (7/10) and a visual field defect on the RE. Her left eye (LE) was completely normal (10/10, without field defect). The computerized campimetry (CC) disclosed a supero-inferior arciform defect in the RE. LE CC was normal [].\nVisual evoked potential responses showed results compatible with a right second cranial nerve dysfunction due to compressive optic neuropathy on the RE. A noncontrast enhanced computed tomography (CT) scan was performed and revealed a spontaneous hyperdense focal nodular lesion, heterogeneous, with irregular contours and a punctate area of increased attenuation coefficient related to calcification, on the right optic-carotid cistern, adjacent to the ipsilateral ON. No surrounding edema or mass effect was documented. No evidence of subarachnoid or intraventricular hemorrhage [].\nBased on the CT scan result, the patient was referred to the outpatient clinic of the Neurosurgery Department. She completed the diagnostic neuro-imaging evaluation with a CT angiography (CTA) [], a magnetic resonance imaging (MRI), and a MR angiography (MRA) of the brain.\nThe MRI showed a focal solitary well-defined rounded lesion involving the right ON in its intracranial prechiasmatic segment, sparing the chiasm. The T1-weighted noncontrasted sequence showed a spontaneously hyperintense lesion [Figures -]. In the T2-weighted MRI, the lesion exhibited a heterogeneous signal intensity and a peripheral hypointense rim [. No large vessels adjacent to the lesion were identified.\nThe MRA demonstrated a hyperintense mass due to hemosiderin. No enlarged vessels were identified [].\nThese features were considered to be consistent with an occult vascular malformation, presenting an inner hematoma (blood and blood by-products in various states of evolution) surrounded by a peripheral hemosiderin rim, suggesting the diagnosis of a CM of the intracranial segment of the right ON.\nThe patient complaints remained unchanged for one week. After this period of time, she was electively operated on, through a right pterional craniotomy. An intradural optic canal unroofing, through a drilling technique, was performed to completely expose and microsurgically remove the CM []. The CM was found over the superior surface of the ON at the entrance of the optic canal with an anterior canalicular extension.
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 []. With the diagnosis of brain stem CM with a history of multiple hemorrhages, progressive neurological deficit, and pial presentation of the CM, the patient was enlisted for the surgical resection of the lesion.\nThe 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 monitoring was used to detect changes in the function of the nervous structure. Motor and somatosensory evoked potentials were normal during the surgery. Direct nerve stimulation of the sixth nerve was also performed during the surgery. The response to stimulation was normal when stimulating distal to the lesion and weak proximal to the lesion.\nThe 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 []. To determine the origin of the lesion, immunohistochemistry staining for the S100 marker was carried out, and the results showed the intraneural origin of the lesion []. The follow-up imaging was normal after 2 years, and the patient was symptom free during this period.