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Open access

A rare case of atypical intradural extramedullary glioblastoma diagnosed utilizing next-generation sequencing and methylation profiling: illustrative case

William J Shelton, Andrew P Mathews, Karrar Aljiboori, J. Stephen Nix, Murat Gokden, and Analiz Rodriguez

BACKGROUND

Primary spinal cord tumors, especially primary spinal cord glioblastoma multiforme (PSC-GBM), are exceptionally rare, accounting for less than 1.5% of all spinal tumors. Their infrequency and aggressive yet atypical presentation make diagnosis challenging. In uncertain cases, a surgical approach for tissue diagnosis is often optimal.

OBSERVATIONS

A 76-year-old male presented with a rapidly progressing clinical history marked by worsening extremity weakness, urinary retention, and periodic fecal incontinence alongside diffuse changes on neuraxis imaging. The patient, in whom subacute polyneuropathy was initially diagnosed, received multiple rounds of steroids and intravenous immunoglobulin without clinical improvement. Histopathological review of the biopsy tissue yielded an initial diagnosis of spindle cell neoplasm. Next-generation sequencing (NGS) is done routinely on all neuropathology specimens at the authors’ institution, and methylation profiling is pursued in difficult cases. Ultimately, NGS and methylation profiling results were essential to an integrated final diagnosis of GBM.

LESSONS

PSC-GBM is a rare but highly aggressive occurrence of this tumor. Prolonged back pain, rapid neurological decline, and imaging changes warrant the consideration of lesional biopsy for precise disease characterization. In inconclusive cases, NGS has proved invaluable for clinical clarification and diagnosis, underscoring its importance for integrated diagnoses in guiding appropriate treatment strategies.

Open access

Successful coil embolization of a ruptured pseudoaneurysm of the superior gluteal artery after a percutaneous awake robot-assisted sacroiliac joint fusion: illustrative case

Samah Morsi, Alyssa M Bartlett, Andrew A Hardigan, Mounica Paturu, Shawn W Adams, Malcolm R DeBaun, Waleska Pabon-Ramos, and Muhammad M Abd-El-Barr

BACKGROUND

Robot-assisted sacroiliac joint (SIJ) fusion has gained popularity, but it carries the risk of complications such as injury to the superior gluteal artery (SGA). The authors present the case of an awake percutaneous robot-assisted SIJ fusion leading to an SGA pseudoaneurysm.

OBSERVATIONS

An 80-year-old male, who had undergone an awake percutaneous robot-assisted SIJ fusion, experienced postoperative left hip pain and bruising. Subsequent arteriography demonstrated an SGA branch pseudoaneurysm requiring coil embolization.

LESSONS

An SGA injury, although uncommon (1.2% incidence), can arise from percutaneous screw placement, aberrant anatomy, or hardware contact. Thorough preoperative imaging, precise robot-assisted screw insertion, and soft tissue protection are crucial to mitigate risks. Immediate angiography aids in prompt diagnosis and effective intervention. Comprehensive knowledge of anatomical variants is essential for managing complications and optimizing preventative measures in robot-assisted SIJ fusion.

Open access

Immunocompetent isolated cerebral mucormycosis presenting with obstructive hydrocephalus: illustrative case

Khoa N Nguyen, Lindsey M Freeman, Timothy H Ung, Steven Ojemann, and Fabio Grassia

BACKGROUND

Isolated cerebral mucormycosis is rare in immunocompetent adults and is only sparsely reported to be associated with obstructive hydrocephalus.

OBSERVATIONS

Here, the authors report a case of obstructive hydrocephalus secondary to central nervous system mucormycosis without other systems or rhino-orbital involvement and its technical surgical management. A 23-year-old, incarcerated, immunocompetent patient with history of intravenous (IV) drug use presented with syncope. Although clinical and radiographic findings failed to elucidate an infectious pathology, endoscopy revealed an obstructive mass lesion at the level of the third ventricle, which, on microbiological testing, was confirmed to be Rhizopus fungal ventriculitis. Perioperative cerebrospinal fluid diversion, endoscopic third ventriculostomy, endoscopic biopsy technique, patient outcomes, and the literature are reviewed here. The patient received intrathecal and IV amphotericin B followed by a course of oral antifungal treatment and currently remains in remission.

LESSONS

The patient’s unique presentation and diagnosis of isolated cerebral mucormycosis reveal this pathogen as a cause of ventriculitis and obstructive hydrocephalus in immunocompetent adult patients, even in the absence of infectious sequelae on neuroimaging.

Open access

Supraorbital keyhole approaches in the first 3 years of practice: outcomes and lessons learned. Patient series

Adnan Hussain Shahid, Danner Butler, Garrett Dyess, Maxon Bassett, Luke Harris, Ursula Hummel, Danielle Chason, and Jai Deep Thakur

BACKGROUND

Supraorbital keyhole approaches (SKAs) have garnered criticism for a limited surgical exposure, restrictive surgical freedom, blind spots, and the learning curve. This retrospective study of patients who underwent SKA aims to explore the outcomes, technical nuances, and the learning curve reflected in a single surgeon’s experience in the initial 3 years of practice.

OBSERVATIONS

A total of 20 SKA operations were performed in 19 patients. Gross- or near-total resection was achieved in 14 of 17 tumor cases. The mean blood loss was 80.5 mL, the mean duration of surgery was 5 hours, and the median stay was 3 days. Endoscopic augmentation was used in 11 cases in which additional tumor removal occurred in 8 of the 11 cases. There were no cases of cerebrospinal fluid leakage or wound infection. A 30-day readmission and typical narcotics after discharge were seen in one patient each. When comparing two halves of a neurosurgery practice over 3 years, the duration of surgery was significantly longer in the later year, which is likely due to operating on a larger tumor size as the years progressed. No cases required static retractors or conversion to larger craniotomies.

LESSONS

Careful case selection and respecting the learning curve allows the safe incorporation of SKA in the early stages of neurosurgical practice.

Open access

Ruptured pial-pial collateral aneurysm associated with left internal carotid artery occlusion: nuances of surgical management. Illustrative case

Juan Silvestre G Pascual, Eddie Guo, Runze Yang, Kristopher D Langdon, Sanju Lama, and Garnette R Sutherland

BACKGROUND

Carotid occlusion often leads to the formation of a collateral network. On rare occasions, due to hemodynamic influence, aneurysms can occur. Here, the authors describe a 69-year-old male presenting with intracerebral hemorrhage secondary to a ruptured aneurysm within such a network.

OBSERVATIONS

The patient presented to the emergency department with an altered level of consciousness. Imaging showed a left temporal lobe hemorrhage extending into the ventricle, subdural hematoma, and evidence of contrast extravasation. Digital subtraction angiography revealed an occluded left internal carotid artery with the left middle cerebral artery territory reconstituted by flow through an external carotid artery–internal carotid artery anastomosis. The latter was formed by the superficial temporal artery–superior orbital artery, as well as pial-pial collaterals from the posterior temporal artery. Notably, a 4-mm aneurysm arising from the pial-pial collateral network was identified. Surgical intervention involved a left temporal craniectomy and aneurysm excision, with special attention paid to preserving the anastomotic flow through the superficial temporal artery.

LESSONS

This case underscores the importance of recognizing and preserving collateral vascular pathways in cases of carotid occlusion with an associated aneurysm. It emphasizes the necessary balance between managing aneurysm risk and maintaining cerebral perfusion, highlighting the need for careful preoperative planning and intraoperative caution.

Open access

Enlarged tumefactive perivascular, or Virchow-Robin, spaces and hydrocephalus: do we need to treat? Illustrative cases

Belal Neyazi, Vanessa Magdalena Swiatek, Klaus-Peter Stein, Karl Hartmann, Ali Rashidi, Seraphine Zubel, Amir Amini, and I. Erol Sandalcioglu

BACKGROUND

Perivascular spaces (PVSs) are spaces in brain parenchyma filled with interstitial fluid surrounding small cerebral vessels. Massive enlargements of PVSs are referred to as “giant tumefactive perivascular spaces” (GTPVSs), which can be classified into three types depending on their localization. These lesions are rare, predominantly asymptomatic, and often initially misinterpreted as cystic tumor formations. However, there are several reported cases in which GTPVSs have induced neurological symptoms because of their size, mass effect, and location, ultimately leading to obstructive hydrocephalus necessitating neurosurgical intervention. Presented here are three diverse clinical presentations of GTPVS.

OBSERVATIONS

Here, the authors observed an asymptomatic case of type 1 GTPVS and two symptomatic cases of type 3 GTPVS, one causing local mass effect and the other hydrocephalus.

LESSONS

GTPVSs are mostly asymptomatic lesions. Patients without symptoms should be closely monitored, and biopsy is discouraged. Hydrocephalus resulting from GTPVS necessitates surgical intervention. In these cases, third ventriculostomy, shunt implantation, or direct cyst fenestration are surgical options. For patients presenting with symptoms from localized mass effect, a thorough evaluation for potential neurosurgical intervention is imperative. Follow-up in type 3 GTPVS is recommended, particularly in untreated cases. Given the infrequency of GTPVS, definitive guidelines for neurosurgical treatment and subsequent follow-up remain elusive.

Open access

Microvascular decompression of a vertebral artery loop causing cervical radiculopathy: illustrative case

Alexa Semonche, Lorenzo Rinaldo, Young Lee, Todd Dubnicoff, Harlan Matles, Dean Chou, Adib Abla, and Edward F Chang

BACKGROUND

Vertebral artery loops are a rare cause of cervical radiculopathy. Surgical options for nerve root decompression include an anterior or posterior approach, with or without additional microvascular decompression.

OBSERVATIONS

The authors describe a case of a 49-year-old man with a long-standing history of left-sided neck pain and migraines, who was found to have a vertebral artery loop in the left C3–4 neural foramen compressing the left C4 nerve root. The patient underwent a posterior cervical decompression with instrumented fusion and macrovascular decompression of the left C4 nerve root via Teflon felt insertion. In a literature review, we identified 20 similar cases that had also been managed surgically.

LESSONS

Although the anterior approach is more frequently described in the literature, a posterior approach for nerve compression by a vertebral artery loop is also a safe and effective treatment. The authors report the third case of this surgical approach with a good outcome.

Open access

Brainstem anesthesia during awake craniotomy: illustrative case

Yun Chen, Mei Sun, Hongmin Bai, Ruixin Yang, and Huan He

BACKGROUND

Awake craniotomy (AC) is performed to remove the lesions near or in eloquent areas, during which the patients are alert and without any airway instrument. Apnea is a severe complication in AC. Here, the authors describe a case of sudden apnea induced by unexpected local anesthesia of the brainstem during AC.

OBSERVATIONS

A 42-year-old male underwent AC for a large, recurrent, bilateral frontal lobe mass and experienced transient apnea and loss of brainstem reflexes during the surgery. The patient recovered spontaneous breath rhythm just a few minutes after the removal of a lidocaine cotton pledget, which was found near the patient’s midbrain. Then the patient awoke and cooperated to finish the surgery.

LESSONS

The administration of a local anesthetic subdurally in AC is common but risky. The scouring action of cerebral spinal fluid can spread those agents and cause unexpected brainstem anesthesia. A lower concentration of the anesthetic and keeping away from the cistern can make it safer.

Open access

Arteriovenous malformation presenting as complex regional pain syndrome: illustrative case

Dayna C Sloane, Diego D Luy, Atul K Mallik, Joseph C Serrone, and Douglas E Anderson

BACKGROUND

Complex regional pain syndrome (CRPS) is typically described as a peripheral nerve disorder in which exaggerated allodynia and hyperalgesia follow a minor injury. Some researchers propose a central mechanism, although current evidence is lacking.

OBSERVATIONS

A 14-year-old female presented with classic CRPS symptoms of left upper-extremity weakness and hyperalgesia after a bout of sharp pain in her thumb while shoveling snow. A possible seizure prompted magnetic resonance imaging, revealing a right frontal Spetzler-Martin grade II arteriovenous malformation (AVM) adjacent to the primary motor cortex. Brodmann areas 1, 3a, and 3b, which are responsible for localizing and processing burning and painful sensations, were also involved. The patient underwent transarterial Onyx embolization in two sessions and microsurgical resection, after which her CRPS symptoms completely resolved.

LESSONS

To our knowledge, this is the first reported case of a cerebral AVM presenting as CRPS, which supports a central mechanism. The authors propose that rapid growth of the AVM led to a vascular steal phenomenon of surrounding parenchyma, which disrupted the patient’s normal motor function and nociceptive processing. Further validation in other series is needed.

Open access

Misplaced intraspinal venous stent causing cauda equina syndrome: illustrative case

Vaibhavi Shah, Thomas Johnstone, Ghani Haider, Neelan J Marianayagam, Martin N Stienen, Venita Chandra, and Anand Veeravagu

BACKGROUND

Endovenous stents for deep venous thrombosis treatment can be unintentionally placed in the spinal canal, resulting in neurological deficit.

OBSERVATIONS

The authors report the case of a patient presenting to our institution with intraspinal misplacement of an endovenous stent, resulting in cauda equina syndrome. The authors also performed a systematic literature review, evaluating the few previously reported cases. This review was performed according to the updated Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. In four of five cases describing stent misplacement into the spinal canal, the authors report that only anteroposterior monoplanar imaging modalities were utilized for venous localization and stent deployment. The anteroposterior plane cannot assess the relative depth of structures, nor can it distinguish between superimposed structures well. Therefore, the use of biplanar imaging should at least be considered before stent deployment, as intraspinal stent placement can lead to disastrous consequences.

LESSONS

This report should serve as an impetus for the use of biplanar or three-dimensional imaging modalities for iliac venous stent placement. Additionally, this work should increase spine surgeons’ awareness about management and operative techniques when faced with this complication.