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Open access

Kota Hiraga, Shigemasa Hayashi, Ryosuke Oshima, Tatsuma Kondo, Fumiaki Kanamori, and Ryuta Saito

BACKGROUND

Developmental venous anomalies (DVAs) are congenital anatomical variants of the normal deep parenchymal veins. DVAs are occasionally found incidentally on brain imaging, and most cases are asymptomatic. However, they rarely cause central nervous disorders. Herein, a case of mesencephalic DVA that caused aqueduct stenosis and hydrocephalus and discuss its diagnosis and treatment is reported.

OBSERVATIONS

The patient was a 48-year-old female who presented with depression. Computed tomography and magnetic resonance imaging (MRI) of the head revealed obstructive hydrocephalus. Contrast-enhanced MRI revealed an abnormally distended linear region with enhancement on the top of the cerebral aqueduct, which was confirmed as a DVA by digital subtraction angiography. An endoscopic third ventriculostomy (ETV) was performed to improve the patient’s symptoms. Intraoperative endoscopic imaging showed obstruction of the cerebral aqueduct by the DVA.

LESSONS

This report describes a rare case of obstructive hydrocephalus caused by DVA. It highlights the usefulness of contrast-enhanced MRI for diagnosing cerebral aqueduct obstructions due to DVAs and the effectiveness of ETV as a treatment option.

Open access

Hiroki Seto, Ryosuke Ogura, Tetsuya Hiraishi, Yoshihiro Tsukamoto, Taiki Saito, Satoshi Shibuma, Kohei Shibuya, Kouichirou Okamoto, Makoto Oishi, and Yukihiko Fujii

BACKGROUND

Cerebellopontine angle (CPA) lipoma–associated hemifacial spasm (HFS) is rare. As the removal of CPA lipomas has a high risk of worsening the neurological symptoms, surgical exploration is warranted only in selected patients. Preoperative identification of the lipoma affected site of the facial nerve, and offending artery are crucial for patient selection and successful microvascular decompression (MVD).

OBSERVATIONS

Presurgical simulation using three-dimensional (3D) multifusion imaging showed a tiny CPA lipoma wedged between the facial and auditory nerves, as well as an affected facial nerve by the anterior inferior cerebellar artery (AICA) at the cisternal segment. Although a recurrent perforating artery from the AICA anchored the AICA to the lipoma, successful MVD was achieved without lipoma removal.

LESSONS

The presurgical simulation using 3D multifusion imaging could identify the CPA lipoma, affected site of the facial nerve, and offending artery. It was helpful for patient selection and successful MVD.

Open access

Youngkyung Jung, Jennifer L. Quon, and James M. Drake

BACKGROUND

Sinus pericranii (SP) is a rare vascular anomaly, with an uncertain etiology. Often discovered as superficial lesions, they can be primary or secondary in nature. Herein, we report a rare case of SP in the setting of a large posterior fossa pilocytic astrocytoma associated with a significant venous network.

OBSERVATIONS

A 12-year-old male presented with acute clinical deterioration in extremis with a 2-month history of lethargy and headaches. Outside plain computed tomography imaging revealed a large posterior fossa cystic lesion, probably a tumor, with severe hydrocephalus. There was also a midline small skull defect at the opisthocranion, without visible vascular anomalies. An external ventricular drain was placed with rapid recovery. Contrast imaging revealed a large midline SP emanating from occipital bone with a large intraosseous, and subcutaneous venous plexus in the midline draining inferiorly into venous plexus around the craniocervical junction. A posterior fossa craniotomy without contrast imaging could have resulted in catastrophic hemorrhage. A small modified off-center craniotomy provided access to the tumor with a gross total excision.

LESSONS

SP is a rare but significant phenomenon. Its presence does not necessarily preclude resection of underlying tumors, provided that a careful preoperative assessment of the venous anomaly is undertaken.

Open access

Atsuhiko Ninomiya, Hidenori Endo, Ryosuke Tashiro, Atsushi Kanoke, and Teiji Tominaga

BACKGROUND

Moyamoya disease (MMD) is linked to the formation of intracranial aneurysms. The authors recently observed an effective use of magnetic resonance vessel wall imaging (MR-VWI) to detect de novo unruptured MMD-associated microaneurysms.

OBSERVATIONS

The authors describe a 57-year-old female who was diagnosed with MMD 6 years ago after suffering a left putaminal hemorrhage. MR-VWI revealed point-like enhancement in the right posterior paraventricular region during the annual follow-up. On the T2-weighted image, this lesion was surrounded by high intensity. Angiography revealed a microaneurysm in the periventricular anastomosis. Right combined revascularization surgery was performed to prevent future hemorrhagic events. Another de novo circumferential enhanced lesion on MR-VWI appeared in the left posterior periventricular region 3 months after surgery. Angiography revealed that the enhanced lesion was a de novo microaneurysm on the periventricular anastomosis. The left combined revascularization surgery went well. The bilateral microaneurysms vanished on follow-up angiography.

LESSONS

Unruptured MMD-associated microaneurysms on the periventricular anastomosis can be detected using MR-VWI. Revascularization surgery can eliminate microaneurysms by reducing hemodynamic stress on the periventricular anastomosis.

Restricted access

Blessing N. R. Jaja, Christopher D. Witiw, Erin M. Harrington, Yingshi He, Ali Moghaddamjou, Michael G. Fehlings, and Jefferson R. Wilson

OBJECTIVE

There is a need to better understand and predict postsurgical outcomes for degenerative cervical myelopathy (DCM) patients, particularly to support treatment decisions for patients with mild DCM. The goal of this study was to identify and predict outcome trajectories for DCM patients up to 2 years postsurgery.

METHODS

The authors analyzed two North American multicenter prospective DCM studies (n = 757). Functional recovery and physical health component quality of life were assessed in DCM patients at baseline, 6 months, and 1 and 2 years postoperatively using the modified Japanese Orthopaedic Association (mJOA) score and Physical Component Summary (PCS) of the SF-36, respectively. Group-based trajectory modeling was used to identify recovery trajectories for mild, moderate, and severe DCM. Prediction models for recovery trajectories were developed and validated in bootstrap resamples.

RESULTS

Two recovery trajectories were identified for the functional and physical components of quality of life: good recovery and marginal recovery. Depending on outcome and myelopathy severity, one-half to three-fourths of the study patients followed the good recovery trajectory characterized by improvement in mJOA and PCS scores over time. The remaining one-half to one-fourth of patients followed the marginal recovery trajectory, experiencing little improvement and, in certain cases, worsening postoperatively. The prediction model for mild DCM had an area under the curve of 0.72 (95% CI 0.65–0.80), with preoperative neck pain, smoking, and posterior surgical approach noted as dominant predictors of marginal recovery.

CONCLUSIONS

Surgically treated DCM patients follow distinct recovery trajectories in the first 2 years postoperatively. While most patients experience substantial improvement, a significant minority experience little improvement or worsening. The ability to predict DCM patient recovery trajectories in the preoperative setting facilitates the formulation of individualized treatment recommendations for patients with mild symptoms.

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Rodrigo Saad Berreta, Helen Zhang, Daniel Alsoof, Christopher L. McDonald, Bassel G. Diebo, Eren Kuris, and Alan H. Daniels

OBJECTIVE

Spinal osteomyelitis and spondylodiscitis (SD) are infections of the vertebral body and disc, respectively, with more than 50% associated with Staphylococcus aureus. Methicillin-resistant S. aureus (MRSA) has become a pathogen of interest in cases of SD due to increasing prevalence. The purpose of this investigation was to characterize the current epidemiological and microbiological landscape in SD cases, in addition to medical and surgical challenges in treating these infections.

METHODS

The PearlDiver Mariner database was queried for ICD-10 codes to identify cases of SD from 2015 to 2021. The initial cohort was stratified by offending pathogens, including methicillin-sensitive S. aureus (MSSA) and MRSA. Primary outcome measures included epidemiological trends, demographics, and rates of surgical management. Secondary outcomes included length of hospital stay, rate of reoperation, and complications associated with surgical cases. Multivariable logistic regression was used to control for age, gender, region, and Charlson Comorbidity Index (CCI).

RESULTS

Nine thousand nine hundred eighty-three patients met the inclusion criteria and were retained for this study. Approximately half (45.5%) of SD cases resulting from S. aureus infections each year were resistant to beta-lactam antibiotics. The proportion of cases that were managed surgically was 31.02%. Among the cases that involved surgical intervention, 21.83% underwent revision operations within 30 days of the original procedure and 37.29% of cases returned to the operating room within 1 year. Substance abuse, specifically alcohol, tobacco, and drug use, were strong predictors for surgical intervention in cases of SD (all p < 0.001), in addition to obesity (p = 0.002), liver disease (p < 0.001), and valvular disease (p = 0.025). After adjusting for age, gender, region, and CCI, cases of MRSA were more likely to undergo surgical management (OR 1.19, p = 0.003). MRSA SD also exhibited higher rates of reoperation within 6 months (OR 1.29, p = 0.001) and 1 year (OR 1.36, p < 0.001). Surgical cases resulting from MRSA infections also exhibited higher morbidity and significant rates of transfusion (OR 1.47, p = 0.030), acute kidney injury (OR 1.35, p = 0.001), pulmonary embolism (OR 1.44, p = 0.030), pneumonia (OR 1.49, p = 0.002), and urinary tract infection (OR 1.45, p = 0.002) compared with MSSA SD infections.

CONCLUSIONS

More than 45% of cases of S. aureus SD in the US are resistant to beta-lactam antibiotics, presenting obstacles in treatment. Cases of MRSA SD are more likely to be managed surgically and have higher rates of complications and reoperations. Early detection and prompt operative management are imperative to reduce the risk of complications.

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Travis S. CreveCoeur, Nikita G. Alexiades, Christopher M. Bonfield, Douglas L. Brockmeyer, Samuel R. Browd, Jason Chu, Anthony A. Figaji, Mari L. Groves, Todd C. Hankinson, David H. Harter, Steven W. Hwang, Andrew Jea, Steven G. Kernie, Jeffrey R. Leonard, Jonathan E. Martin, Matthew E. Oetgen, Alexander K. Powers, Curtis J. Rozzelle, David L. Skaggs, Jennifer M. Strahle, John C. Wellons III, Michael G. Vitale, and Richard C. E. Anderson

OBJECTIVE

The focus of this modified Delphi study was to investigate and build consensus regarding the medical management of children with moderate and severe acute spinal cord injury (SCI) during their initial inpatient hospitalization. This impetus for the study was based on the AANS/CNS guidelines for pediatric SCI published in 2013, which indicated that there was no consensus provided in the literature describing the medical management of pediatric patients with SCIs.

METHODS

An international, multidisciplinary group of 19 physicians, including pediatric neurosurgeons, orthopedic surgeons, and intensivists, were asked to participate. The authors chose to include both complete and incomplete injuries with traumatic as well as iatrogenic etiologies (e.g., spinal deformity surgery, spinal traction, intradural spinal surgery, etc.) due to the overall low incidence of pediatric SCI, potentially similar pathophysiology, and scarce literature exploring whether different etiologies of SCI should be managed differently. An initial survey of current practices was administered, and based on the responses, a follow-up survey of potential consensus statements was distributed. Consensus was defined as ≥ 80% of participants reaching agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). A final meeting was held virtually to generate final consensus statements.

RESULTS

Following the final Delphi round, 35 statements reached consensus after modification and consolidation of previous statements. Statements were categorized into the following eight sections: inpatient care unit, spinal immobilization, pharmacological management, cardiopulmonary management, venous thromboembolism prophylaxis, genitourinary management, gastrointestinal/nutritional management, and pressure ulcer prophylaxis. All participants stated that they would be willing or somewhat willing to change their practices based on consensus guidelines.

CONCLUSIONS

General management strategies were similar for both iatrogenic (e.g., spinal deformity, traction, etc.) and traumatic SCIs. Steroids were recommended only for injury after intradural surgery, not after acute traumatic or iatrogenic extradural surgery. Consensus was reached that mean arterial pressure ranges are preferred for blood pressure targets following SCI, with goals between 80 and 90 mm Hg for children at least 6 years of age. Further multicenter study of steroid use following acute neuromonitoring changes was recommended.

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Mohammed O. Alalfi, Giuseppe Lanzino, and Kelly D. Flemming

OBJECTIVE

Newly diagnosed patients with a familial cavernous malformation (FCM) and their families are concerned about their future outlook, which is scarcely discussed in the literature. The authors studied a prospective contemporary cohort of patients with FCMs to assess demographics, mode of presentation, prospective risk of hemorrhage and seizures, need for surgery, and functional outcome over an extended interval.

METHODS

A prospectively maintained database beginning January 1, 2015, of patients diagnosed with a cavernous malformation (CM) was queried. Data on demographics, radiological imaging, and symptoms at first diagnosis were collected in adult patients who gave their consent to prospective contact. Follow-up was done using questionnaires, in-person visits, and medical record review to assess for prospective symptomatic hemorrhage (i.e., the first hemorrhage after enrollment in the database), seizure, functional outcome measured by the modified Rankin Scale (mRS), and treatment. The prospective hemorrhage rate was calculated by the number of prospective hemorrhages divided by patient-years of follow-up censored at last follow-up, first prospective hemorrhage, or death. A Kaplan-Meier curve of survival free of hemorrhage was obtained comparing patients with versus without hemorrhage at the time of presentation and compared with a log-rank test for p < 0.05.

RESULTS

A total of 75 patients with FCM were included, of whom 60% were female. The mean age at diagnosis was 41 ± 16 years. Most symptomatic or large lesions were located supratentorially. At first diagnosis, 27 patients had no symptoms, and the remaining were symptomatic. Over an average of 9.9 years, the rate of prospective hemorrhage was 4.0% per patient-year, and the rate of new seizure was 1.2% per patient-year, with 64% and 32% of patients experiencing at least one symptomatic hemorrhage and at least one seizure, respectively. Thirty-eight percent of the patients underwent at least 1 surgery and 5.3% underwent stereotactic radiosurgery. At the last follow-up, 83.0% of patients remained independent with an mRS score ≤ 2.

CONCLUSIONS

The authors’ findings provide clinically useful information on hemorrhage rate, seizure rate, the likelihood of surgery, and functional outcome. These findings can be helpful to practicing physicians when counseling patients with FCM and their families, who are often apprehensive about their future and well-being.

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Edward J. Ebani, Sara Strauss, Charlene Thomas, Arindam RoyChoudhury, Michael G. Kaplitt, and J. Levi Chazen

OBJECTIVE

The ability to predict final lesion characteristics during magnetic resonance–guided focused ultrasound (MRgFUS) thalamotomy for the treatment of essential tremor remains technically challenging, yet it is essential in order to avoid off-target ablation and to ensure adequate treatment. The authors sought to evaluate the technical feasibility and utility of intraprocedural diffusion-weighted imaging (DWI) in the prediction of final lesion size and location.

METHODS

Lesion diameter and distance from the midline were measured on both intraprocedural and immediate postprocedural diffusion and T2-weighted sequences. Bland-Altman analysis was utilized to determine differences in measurement between intraprocedural and immediate postprocedural images with both sequences.

RESULTS

Lesion size increased on both the postprocedural diffusion and T2-weighted sequences, although the difference was smaller on the T2-weighted sequence. There was only a small difference in intraprocedural and postprocedural lesion distance from the midline on both the diffusion and T2-weighted sequences.

CONCLUSIONS

Intraprocedural DWI is both feasible and useful with regard to predicting final lesion size and providing an early indication of lesion location. Further research should determine the value of intraprocedural DWI in predicting delayed clinical outcomes.

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Umberto Tosi, Alexandra Giantini-Larsen, Dimitrios Mathios, Ashutosh Kacker, Vijay K. Anand, Kiarash Ferdowssian, Ali Baaj, Roger Härtl, Benjamin I. Rapoport, Jeffrey P. Greenfield, and Theodore H. Schwartz

OBJECTIVE

Endonasal endoscopic odontoidectomy (EEO) is an alternative to transoral surgery for symptomatic ventral compression of the anterior cervicomedullary junction (CMJ), allowing for earlier extubation and feeding. Because the procedure destabilizes the C1–2 ligamentous complex, posterior cervical fusion is often performed concomitantly. The authors’ institutional experience was reviewed to describe the indications, outcomes, and complications in a large series of EEO surgical procedures in which EEO was combined with posterior decompression and fusion.

METHODS

A consecutive, prospective series of patients who underwent EEO between 2011 and 2021 was studied. Demographic and outcome metrics, radiographic parameters, extent of ventral compression, extent of dens removal, and increase in CSF space ventral to the brainstem were measured on the preoperative and postoperative scans (first and most recent scans).

RESULTS

Forty-two patients (26.2% pediatric) underwent EEO: 78.6% had basilar invagination, and 76.2% had Chiari type I malformation. The mean ± SD age was 33.6 ± 3.0 years, with a mean follow-up of 32.3 ± 4.0 months. The majority of patients (95.2%) underwent posterior decompression and fusion immediately before EEO. Two patients underwent prior fusion. There were 7 intraoperative CSF leaks but no postoperative CSF leaks. The inferior limit of decompression fell between the nasoaxial and rhinopalatine lines. The mean ± SD vertical height of dens resection was 11.98 ± 0.45 mm, equivalent to a mean ± SD resection of 74.18% ± 2.56%. The mean increase in ventral CSF space immediately postoperatively was 1.68 ± 0.17 mm (p < 0.0001), which increased to 2.75 ± 0.23 mm (p < 0.0001) at the most recent follow-up (p < 0.0001). The median (range) length of stay was 5 (2–33) days. The median time to extubation was 0 (0–3) days. The median time to oral feeding (defined as, at minimum, toleration of a clear liquid diet) was 1 (0–3) day. Symptoms improved in 97.6% of patients. Complications were rare and mostly associated with the cervical fusion portion of the combined surgical procedures.

CONCLUSIONS

EEO is safe and effective for achieving anterior CMJ decompression and is often accompanied by posterior cervical stabilization. Ventral decompression improves over time. EEO should be considered for patients with appropriate indications.