The Role of Stereotactic Radiosurgery in the Management of Foramen Magnum Meningiomas—A Multicenter Analysis and Review of the Literature

Background: Foramen magnum meningiomas (FMMs) represent a considerable neurosurgical challenge given their location and potential morbidity. Stereotactic radiosurgery (SRS) is an established non-invasive treatment modality for various benign and malignant brain tumors. However, reports on single-session or multisession SRS for the management and treatment of FMMs are exceedingly rare. We report the largest FMM SRS series to date and describe our multicenter treatment experience utilizing robotic radiosurgery. Methods: Patients who underwent SRS between 2005 and 2020 as a treatment for a FMM at six different centers were eligible for analysis. Results: Sixty-two patients met the inclusion criteria. The median follow-up was 28.9 months. The median prescription dose and isodose line were 14 Gy and 70%, respectively. Single-session SRS accounted for 81% of treatments. The remaining patients received three to five fractions, with doses ranging from 19.5 to 25 Gy. Ten (16%) patients were treated for a tumor recurrence after surgery, and thirteen (21%) underwent adjuvant treatment. The remaining 39 FMMs (63%) received SRS as their primary treatment. For patients with an upfront surgical resection, histopathological examination revealed 22 World Health Organization grade I tumors and one grade II FMM. The median tumor volume was 2.6 cubic centimeters. No local failures were observed throughout the available follow-up, including patients with a follow-up ≥ five years (16 patients), leading to an overall local control of 100%. Tumor volume significantly decreased after treatment, with a median volume reduction of 21% at the last available follow-up (p < 0.01). The one-, three-, and five-year progression-free survival were 100%, 96.6%, and 93.0%, respectively. Most patients showed stable (47%) or improved (21%) neurological deficits at the last follow-up. No high-grade adverse events were observed. Conclusions: SRS is an effective and safe treatment modality for FMMs. Despite the paucity of available data and previous reports, SRS should be considered for selected patients, especially those with subtotal tumor resections, recurrences, and patients not suitable for surgery.

Occipital cranioplasty using customized titanium prosthesis yields successful outcome in association with foramen magnum decompression in dogs suffering by Chiari-like malformation

OBJECTIVE To describe the use of a customized 3–D-printed titanium prosthesis as adjunctive treatment for foramen magnum decompression (FMD) in dogs with Chiari-like malformation (CM) and syringomyelia (SM). ANIMALS 8 dogs with clinical signs and MRI findings of CM-SM. PROCEDURES 3-D reconstruction of CT images of the head was used to simulate an occipital craniectomy and design the prosthesis. FMD was performed, and the prosthesis was implanted. Follow-up was performed 1, 6, and 12 months later, and clinical status was scored. Repeated MRI images were compared to identify changes involving the neural structures, particularly the syrinx. RESULTS All prostheses were easily positioned based on the preoperative 3-D models, with no complications. At 12 months after surgery, 3 dogs were free of previous medications, 4 were still receiving steroid medications but at lower doses, and 1 was occasionally receiving acupuncture. MRI of 5 dogs 6 to 20 months after surgery revealed resolution of SM (n = 1), reduced size of SM (3), or worse SM (1). All dogs showed an increase in size of the caudal cranial fossa. Dogs with a longer presurgical duration of the clinical signs and wider syrinx generally had worse outcomes than other dogs. CLINICAL RELEVANCE Findings suggested that use of customized 3–D-printed titanium prosthesis and associated FMD can represent an adjunctive option to medically treated dogs with CM-SM. Although the small number of cases precludes definitive conclusions, early surgical treatment, particularly in dogs with a small syrinx, could ensure better long-term outcomes, as previously suggested.

Sonographic Measurement of Brainstem Through the Foramen Magnum in Premature Neonates Can Predict Neurodevelopment Outcome?

Background: To investigate whether serial morphometric measurements of the brainstem using high resolution trans-foramen-magnum ultrasound (US) in premature neonates correlate with neurological outcomes.Methods: Serial brain ultrasound scans were performed in 36 consecutive preterm infants born at &lt;34 weeks of gestation from birth until term-equivalent age. Two-dimensional brainstem measurements of the pons and medulla oblongata were compared with those in a cohort of 67 healthy full-term newborns. Neurologic assessment of the premature infants was assessed at 5 years of age.Results: Of the 36 preterm infants born between 25 and 34 weeks of gestation, eight had significantly delayed growth profiles in both the pons and medulla and developed neurological sequelae by 5 years of age.Conclusions: Morphometric measurements of the developing brainstem using high resolution trans-foramen-magnum ultrasound (US) may help predict neurological outcome in high-risk neonates, particularly in those who are born extremely premature.

IgG4-related pachymeningitis masquerading as foramen magnum meningioma: illustrative case

BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition with potential multiorgan involvement. Common manifestations include autoimmune pancreatitis and retroperitoneal fibrosis. Pathological analysis reveals lymphoplasmacytic infiltrate rich in IgG4-positive cells and characteristic storiform fibrosis. Early treatment with glucocorticoids may prevent progression to poorly responsive fibrotic disease. OBSERVATIONS A 63-year-old female patient presented with reports of left-sided headaches, nausea, and photophobia in addition to recently diagnosed chronic rhinosinusitis (CRS). Neurological examination revealed dysarthria secondary to left hypoglossal nerve palsy. Computed tomography (CT) revealed a contrast-enhancing extraaxial mass at the left craniocervical junction, CRS with secondary hyperostotic reaction, and multiple hypodense lesions involving the occipital bone. Magnetic resonance imaging revealed a dural-based lesion involving the foramen magnum and invading the left hypoglossal canal. The patient underwent a far-lateral craniotomy. Histopathological analysis revealed severe lymphoplasmacytic inflammation, storiform fibrosis and rich plasma-cell population positive for IgG4. Serum IgG4 was markedly elevated. Total-body CT showed no systemic involvement. The patient was diagnosed with IgG4-RD and was prescribed prednisone, with normalization of her IgG4 levels after 1 month. LESSONS IgG4-RD may mimic a variety of diseases, including skull-base meningiomas and CRS. Accurate diagnosis and expedited administration of steroids may prevent unnecessary interventions and progression to treatment-resistant fibrosis.

Complete posterior cranial vault distraction osteogenesis to correct Chiari malformation type I associated with craniosynostosis

OBJECTIVE Posterior vault distraction osteogenesis (PVDO) is an effective tool to increase intracranial volume and expand the posterior cranial fossa. During PVDO, the authors extended osteotomy posterior to the foramen magnum to fully expand the posterior cranial fossa. The aim of this study was to investigate the efficacy of complete PVDO in posterior fossa expansion and treatment of Chiari malformation type I (CM-I) in patients with craniosynostosis. METHODS Patients with craniosynostosis who had undergone complete PVDO between January 2012 and May 2020 were reviewed retrospectively. A coronal osteotomy extending to the foramen magnum was performed and the foramen magnum was decompressed by removing its posterior rim with a 1-mm Kerrison rongeur. Four distractor devices were placed and the vector of distraction was controlled from the posterior to the inferior-posterior direction, depending on the deformity. Changes in the intracranial volume, posterior cranial fossa area, and cerebellar tonsillar descent were measured after complete PVDO by using CT and MRI. RESULTS A total of 11 patients with craniosynostosis and concurrent CM-I were included in the study. The mean age was 34.6 ± 24.0 months (continuous variables are expressed as the mean ± SD throughout). One patient had sleep apnea, which was consistent with CM-I, and another patient had a headache, which was nonspecific. The intracranial volume increased from 1179.6 ± 180.2 cm3 to 1440.6 ± 251.5 cm3 (p = 0.003; 24.5% increase compared to the preoperative volume). The posterior skull base area increased from 44.9 ± 19.3 cm2 to 72.7 ± 18.1 cm2 (p = 0.004). Cerebellar tonsillar descent decreased in all 11 patients after complete PVDO (preoperative: 10.8 ± 3.7 mm, postoperative: 2.7 ± 3.0 mm; p = 0.003). Among the 11 patients, 5 showed complete resolution of cerebellar tonsillar herniation. CONCLUSIONS Complete PVDO can more efficiently expand the posterior cranial fossa, unlike conventional methods. Moreover, it helps to relieve cerebellar tonsillar herniation. Complete PVDO is a powerful tool to increase the intracranial and posterior fossa volumes in patients with craniosynostosis and concurrent CM-I.

Bilateral typeIIpersistent proatlantal intersegmental artery: a rare variant of persistent carotid-vertebrobasilar anastomoses

The persistent proatlantal intersegmental artery is a rare variant of persistent carotid-vertebrobasilar anastomoses, especially their bilateral presence is rarer. We report a case of bilateral typeII persistent proatlantal intersegmental artery. The absence of bilateral vertebral arteries was incidentally noted on neck ultrasound examination. Subsequent time-of-flight MR angiography confirmed this. The bilateral typeIIpersistent proatlantal intersegmental artery arose from the cervical external carotid artery, penetrated the C1 transverse foramen, entered the skull via the foramen magnum, and joined the lower portion of the basilar artery.