BACKGROUND Spinal extradural arachnoid cysts (SEACs) communicate with the subarachnoid space through small communicating dural holes. The precise preoperative detection of all communicating holes, ...followed by minimally invasive dural closure, is the ideal treatment to prevent postoperative spinal deformities, especially in cases of multiple SEACs. However, standard imaging methods often fail to detect communicating hole locations. Although a few cases of successful single-hole detection via cinematic magnetic resonance imaging (MRI) have been reported, this modality’s ability to detect multiple holes has not been demonstrated. OBSERVATIONS The authors describe the case of a 14-year-old male with myelopathy due to multiple SEACs at T5–8 and T8–12. Myelography revealed a complete block at the T8 level; no cephalic cyst or communicating holes were identified. Time-spatial labeling inversion pulse (T-SLIP) MRI revealed cerebrospinal fluid flow into the cyst at T10 and T7. A limited laminectomy or hemilaminectomy was performed at T7 and T10, and two dural holes were closed without a total cystectomy. The patient’s gait disturbance and rectal disorder disappeared. The cysts were confirmed to have completely disappeared on conventional MRI at 1 year postoperatively. LESSONS T-SLIP MRI, a cinematic MRI, is useful for detecting multiple communicating holes in SEACs.
BACKGROUND Revascularization for extracranial vertebral artery dissection or vertebral artery atherosclerotic occlusive lesions caused by vertebrobasilar insufficiency or posterior circulation ...infarction is relatively rare. When bypassing the cervical external carotid artery (ECA) or common carotid artery (CCA) using a radial artery (RA) or saphenous vein (SV) graft, it is difficult to determine whether the recipient site should be the V2 or V3 portion. OBSERVATIONS In case 1, cervical ECA-RA-V3 bypass was performed for bilateral extracranial vertebral artery dissection with the onset of ischemia, and cervical CCA-SV-V3 bypass was added 12 days later. Nine years after surgery, the bilateral vertebral artery dissection had improved, and the patient still had a patent bypass. In case 2, cervical ECA-RA-V2 bypass was performed for arteriosclerotic bilateral extracranial vertebral artery occlusion. The bypass was patent 5 years after surgery. The postoperative course was uneventful in both patients. LESSONS The authors present cases of posterior fossa revascularization using the vertebral artery V3 and V2 portions via skull base surgery and note that it is important to consider each patient’s individual characteristics when selecting the V3 or V2 portion.
BACKGROUND Essential tremor (ET) is one of the most common movement disorders worldwide. In medically refractory ET, deep brain stimulation (DBS) of the ventral intermediate nucleus of the thalamus ...is the current standard of care. However, DBS carries an inherent 2% to 3% risk of hemorrhage, a risk that can be much higher in patients with concomitant coagulopathy. Magnetic resonance imaging–guided focused ultrasound (MRgFUS) thalamotomy is a surgical alternative that is highly effective in treating ET, with no reports of intracranial hemorrhage to date. OBSERVATIONS This is the first documented case of successful MRgFUS thalamotomy in a patient with von Willebrand disease (VWD). A 60-year-old left-handed male had medically refractory ET, VWD type 2B, and a family history of clinically significant hemorrhage after DBS. He underwent right-sided MRgFUS thalamotomy and received a perioperative course of VONVENDI (recombinant von Willebrand factor) to ensure appropriate hemostasis. Postprocedure imaging confirmed a focal lesion in the right thalamus without evidence of hemorrhage. The patient reported 90% improvement of his left-hand tremor and significant improvement in his quality of life without obvious side effects. LESSONS MRgFUS thalamotomy with peri- and postoperative hematological management is a promising alternative to DBS for patients with underlying coagulopathies.
BACKGROUND Thalamic lesions located in the floor of the lateral ventricle pose significant surgical challenges, given their proximity to critical neurovascular structures. Transcortical approaches ...are often limited by risks of injuring the eloquent cortex and nearby vessels. Furthermore, lesions extending into the third ventricle further impede accessibility. The corticospinal tract (CST), situated close to the thalamus, presents a major obstacle. Diffusion tensor imaging plays a crucial role in overcoming these challenges by accurately delineating the CST’s location relative to the lesion, enabling surgeons to plan minimally invasive and safe access. OBSERVATIONS A 32-year-old female presented with progressive right-sided hemiparesis over several days. Magnetic resonance imaging revealed a cystic ring-enhancing thalamic lesion extending into the third ventricle. While supine, the patient underwent surgery via a right pericoronal parasagittal craniotomy followed by a contralateral interhemispheric transcallosal transchoroidal approach. LESSONS This case exemplifies the utility of the contralateral interhemispheric transcallosal approach for treating thalamic lesions, particularly those extending into the third ventricle. This minimally invasive approach minimizes retraction of the eloquent cortex and reduces the risk of neurovascular injury, potentially leading to improved surgical outcomes and faster recovery.
Anaplastic lymphoma kinase (ALK)-positive histiocytosis (ALK-H) is an emerging entity in the category of histiocytic neoplasms that was first reported as a multisystemic disease in three infants in ...2008. The clinicopathological spectrum of ALK-H has been expanded to include localized disorders in specific organs, but the features of this subtype are not well known. The authors report a case of ALK-H localized in the central nervous system that was difficult to treat and review the relevant literature.
The authors reviewed archival histiocytic tumors at their institute and found a pediatric case of ALK-H localized in a cerebellar hemisphere that had previously been reported as histiocytic sarcoma. Chemotherapy (approximately 1 year), additional surgery, and conventional chemotherapy (approximately 2.5 years) led to clinical remission, and maintenance chemotherapy was continued (approximately 1.5 years). Three years after completing treatment, a high-grade glioma was found in a cerebral hemisphere, and the patient died of the glioma 2 years later.
Although the prognosis of ALK-H is generally good according to prior cases, the authors' case required long-term conventional chemotherapy, suggesting the tumor displayed aggressive characteristics. Early administration of ALK inhibitors may be necessary.
Resection remains the optimal treatment for hemangioblastomas, highly vascular tumors commonly located in the cerebellum or spinal cord. Preoperative embolization can be used with caution to reduce ...intraoperative blood loss and promote gross-total resection while reducing neurological morbidity.
The authors report a case of a 44-year-old male who presented with worsening dizziness, gait imbalance, and diplopia. Imaging revealed a large vascular cerebellar mass with brainstem compression and hydrocephalus, concerning for hemangioblastoma. The patient underwent preoperative embolization of the main arterial supply from the right superior cerebellar artery, which devascularized the tumor and reduced the early venous shunting, followed by gross-total resection of the tumor. Preoperative embolization helped to facilitate safe microsurgical resection because this main arterial supply was deep to the planned surgical approach, and the Onyx cast served as a landmark of the dorsal midbrain and remaining arterial supply. This resulted in minimal intraoperative blood loss. At 2-year follow-up, the patient's diplopia and vertigo had resolved, and his gait continues to improve.
Despite controversy regarding the use of preoperative embolization for hemangioblastoma resection, the authors emphasize its safety and utility during resection of a large cerebellar hemangioblastoma. Onyx embolization provided benefit in both reducing the tumor blood supply and serving as intraoperative visual guidance.
Visual impairment due to delayed optic neuropathy is a rare complication of the endovascular treatment of paraclinoid aneurysms. An inflammatory response induced in the treated aneurysm wall is a ...known mechanism underlying this pathophysiology. The authors describe a case with severe and progressive optic neuropathy leading to neuronal degeneration and severe visual dysfunction.
A 42-year-old female with a history of surgical clipping for a paraclinoid aneurysm presented with a recurrence. Although the patient was unaware of any visual dysfunction, a preoperative ophthalmological examination revealed mild inferior quadrantanopia in the left eye. The coil embolization procedure was uneventful; however, the following day, the patient experienced progressive visual impairment, which worsened despite the initiation of steroid therapy. Ophthalmological examination revealed a severe decrease in visual acuity and further deterioration of the visual field. Magnetic resonance imaging showed remarkable swelling and edema of the left optic nerve adjacent to the treated aneurysm. Despite continued steroid therapy, the patient's visual function did not recover well due to subsequent optic nerve degeneration.
Optic neuropathy after endovascular procedures can lead to severe visual dysfunction. Careful management is essential, particularly when treating a symptomatic paraclinoid aneurysm, even if symptoms are only apparent on detailed examination.
Skull lesions are a common finding in children, with dermoid cysts and eosinophilic granulomas observed most frequently. However, primary intraosseous xanthomas of the calvaria, which are lytic, ...expansile lesions that develop without underlying hyperlipidemic disease, are rare in children, with only one prior case reported.
The authors describe the case of a healthy 6-year-old male who presented with a 2-month history of an enlarging midline skull mass that developed after a recent minor trauma. Imaging showed a full-thickness, lytic frontal bone lesion with an aggressive appearance and heterogeneous contrast enhancement. The patient underwent gross-total resection of the lesion with placement of a mesh cranioplasty. Histopathology revealed a primary intraosseous xanthoma. The patient was discharged on postoperative day 2 and required no further treatment at the 1-month follow-up.
This is the first reported case of a primary intraosseous xanthoma in the frontal bone of a pediatric patient. It emphasizes the need to include primary xanthomas in the differential diagnosis for pediatric skull lesions, particularly when the lesion has an aggressive radiographic appearance or the patient has a history of focal trauma. Furthermore, our findings indicate that resection, together with subsequent monitoring for lesion reccurrence, is an adequate first-line treatment.
Unilateral cranial nerve (CN) VI, or abducens nerve, palsy is rare in children and has not been reported in association with Chiari malformation type 1 (CM1) in the absence of other classic CM1 ...symptoms.
A 3-year-old male presented with acute incomitant esotropia consistent with a unilateral, left CN VI palsy and no additional neurological symptoms. Imaging demonstrated CM1 without hydrocephalus or papilledema, as well as an anterior inferior cerebellar artery (AICA) vessel loop in the immediate vicinity of the left abducens nerve. Given the high risk of a skull base approach for direct microvascular decompression of the abducens nerve and the absence of other classic Chiari symptoms, the patient was initially observed. However, as his palsy progressed, he underwent posterior fossa decompression with duraplasty (PFDD), with the aim of restoring global cerebrospinal fluid dynamics and decreasing possible AICA compression of the left abducens nerve. Postoperatively, his symptoms completely resolved.
In this first reported case of CM1 presenting as a unilateral abducens palsy in a young child, possibly caused by neurovascular compression, the patient's symptoms resolved after indirect surgical decompression via PFDD.
Plasmacytoma, a rare plasma cell disorder, often presents as a solitary or multiple tumors within the bone marrow or soft tissues, typically associated with multiple myeloma. Extramedullary ...plasmacytomas (EMPs), particularly those located in the external auditory canal (EAC), are exceedingly rare and pose significant treatment challenges given their location, anatomical complexity, and high risk of recurrence.
The authors report the case of a 72-year-old male with a history of multiple myeloma, presenting with recurrent left EAC plasmacytoma. After initial conventional radiotherapy for the lesion, a recurrence was documented in 7 years. The patient subsequently underwent stereotactic radiosurgery, which proved successful, leading to complete resolution of the lesion without any long-term adverse effects or radiation-related complications over a 45-month period.
This case is a unique instance of utilizing stereotactic radiosurgery for recurrent EMP in the EAC, highlighting its potential as an effective approach in managing complex plasmacytomas.
