Publications by authors named "Michael L Levy"

149 Publications

Prognostic factors in diffuse leptomeningeal glioneuronal tumor (DLGNT): a systematic review.

Childs Nerv Syst 2022 Jul 22. Epub 2022 Jul 22.

Department of Neurosurgery, University of California, San Diego-Rady Children's Hospital, San Diego, CA, 92123, USA.

Background: Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare tumor, first described by the WHO Classification of Central Nervous System Tumors in 2016. The clinical course is variable. Most tumors have low-grade histological findings although some may have more aggressive features. The goal of this systematic review was to identify prognostic factors for poor overall survival (OS).

Material And Methods: We performed a systematic review using three databases (PubMed, Google Scholar, and Embase) and the following search terms: diffuse leptomeningeal glioneuronal tumor, DLGNT, DLMGNT. Statistical analysis was performed using Statistica 13.3.

Results: We included 34 reports in our review comprising 63 patients, published from 2016 to 2022. The median OS was 19 months (range: 12-51 months). Using multivariable Cox survival analysis, we showed that Ki-67 ≥ 7%, age > 9 years, symptoms of elevated intracranial pressure (ICP) at admission, and the presence of contrast-enhancing intraparenchymal tumor are associated with poor OS. Receiver operating characteristic (ROC) analysis identified Ki-67 ≥ 7% as a significant predictor of poor OS.

Conclusions: Signs or symptoms of increased ICP with imaging findings of diffuse leptomeningeal enhancement should raise suspicion for DLGNT. In our systematic review, Ki-67 ≥ 7% was the most important prognostic factor for OS in DLGNT. The presence of intraparenchymal tumor with contrast enhancement was thought to represent disease progression and, together with patient age, was associated with poor OS.
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http://dx.doi.org/10.1007/s00381-022-05600-wDOI Listing
July 2022

Maturation of the sella turcica and parasellar region: Surgical relevance for anterior skull base approaches in pediatric patients.

Clin Neurol Neurosurg 2022 04 2;215:107168. Epub 2022 Mar 2.

Department of Neurosciences and Pediatrics, University of California San Diego, San Diego, CA, United States. Electronic address:

Objective: Traditional and extended transnasal transsphenoidal approaches provide direct access to a variety of anterior skull base pathologies. Despite increased utilization of transnasal approaches in children, anatomic studies on pediatric skull base maturation are limited. We herein perform a surgically relevant morphometric analysis of the sella and parasellar regions during pediatric maturation.

Methods: Measurements of sellar length (SL), sellar depth (SDp), sellar diameter (SDm), interclinoid distance (ID), intercavernous distance (ICD), and the presence of sphenoid sinus pneumatization (SSP), and sphenoid sinus type (SST) were made on thin-cut CT scans from 60 patients (evenly grouped by ages 0-3, 4-7, 8-11 12-15, 16-18, and >18 years) for analysis. Data were analyzed by sex and age groups using t-tests and linear regression.

Results: Sella and parasellar parameters did not differ by sex. SL steadily increased from 8.5 ± 1.2 mm to 11.5 ± 1.6 mm throughout development. SDp and SDm increased from 6.0 ± 0.9 mm to 9.3 ± 1.4 mm and 9.0 ± 1.6 mm to 14.4 ± 1.8 mm during maturation, with significant interval growth from ages 16-18 to adult (p < 0.01). ID displayed significant growth from ages 0-3 to 4-7 (18.0 ± 2.4 mm to 20.7 ± 1.9 mm; p = 0.002) and ICD from ages 0-3 to 8-11 (12.0 ± 1.8 mm to 13.5 ± 2.1 mm; p < 0.001), without further significant interval growth. SSP was not seen in patients < 3, but was 100% by ages 8-11. SSTs progressed from conchal/presellar (60% at ages 4-7) to sellar/postsellar (80% at adulthood).

Conclusion: The sella and parasellar regions have varied growth patterns with development. Knowledge of the expected maturation of key anterior skull base structures may augment surgical planning in younger patients.
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http://dx.doi.org/10.1016/j.clineuro.2022.107168DOI Listing
April 2022

Maturation of the internal auditory canal and posterior petrous bone with relevance to lateral and posterolateral skull base approaches.

Sci Rep 2022 03 3;12(1):3489. Epub 2022 Mar 3.

Department of Neurosciences and Pediatrics, University of California San Diego, San Diego, CA, USA.

Anatomic knowledge of the internal auditory canal (IAC) and surrounding structures is a prerequisite for performing skull base approaches to the IAC. We herein perform a morphometric analysis of the IAC and surgically relevant aspects of the posterior petrous bone during pediatric maturation, a region well-studied in adults but not children. Measurements of IAC length (IAC-L), porus (IAC-D) and midpoint (IAC-DM) diameter, and distance from the porus to the common crus (CC; P-CC) and posterior petrosal surface (PPS) to the posterior semicircular canal (PSC; PPS-PSC) were made on thin-cut axial CT scans from 60 patients (grouped by ages 0-3, 4-7, 8-11 12-15, 16-18, and > 18 years). IAC-L increased 27.5% from 8.7 ± 1.1 at age 0-3 to 11.1 ± 1.1 mm at adulthood (p = 0.001), with the majority of growth occurring by ages 8-11. IAC-D (p = 0.52) and IAC-DM (p = 0.167) did not significantly change from ages 0-3 to adult. P-CC increased 31.1% from 7.7 ± 1.5 at age 0-3 to 10.1 ± 1.5 mm at adulthood (p = 0.019). PPS-PSC increased 160% from 1.5 ± 0.7 at age 0-3 to 3.9 ± 1.2 mm at adulthood (p < 0.001). The majority of growth in P-CC and PPS-PSC occurred by ages 12-15. Knowledge of these patterns may facilitate safe exposure of the IAC in children.
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http://dx.doi.org/10.1038/s41598-022-07343-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8894491PMC
March 2022

Atypical Giant Suprasellar Prolactinoma Presenting With Visual Field Changes in the Absence of Symptoms of Hyperprolactinemia.

Cureus 2021 Nov 16;13(11):e19632. Epub 2021 Nov 16.

Neurosciences and Pediatrics, University of California San Diego, San Diego, USA.

Prolactinomas are benign tumors that make up the majority of all pituitary adenoma cases and present most commonly in women. Prolactinomas presenting in adolescents and children, however, are extremely rare. We report a case of a 17-year-old male who presented with a six-month history of headaches and a previously unrecognized visual field deficit on examination. Neuroimaging revealed a large suprasellar tumor with imaging, more characteristic of a craniopharyngioma or suprasellar low-grade glioma impinging, on the left intracranial optic nerve causing right-sided hemianopsia. Due to the extensive mass effect and bitemporal hemianopsia on examination, the decision to proceed with initial surgical debulking was made following informed consent. A subtotal resection was performed where the pathology was consistent with a prolactinoma that correlated with markedly elevated prolactin (PRL) levels obtained pre and post-operatively that have not resulted until five days post procedure. The patient was subsequently treated with dopamine agonist (DA) cabergoline therapy and is now five-years disease-free with normal neurological examination and no residual tumor on neuroimaging. DA therapy has shown high clinical efficacy and should be considered prior to any surgical intervention; however, extensive mass effect may appropriate surgical debulking to increase therapy efficacy. Our case highlights an atypical appearance of a giant prolactinoma that may mimic other more common suprasellar tumors, a presentation associated with unrecognized visual field deficits, and the importance of rapid turnaround testing for serum PRL that may aid in the upfront diagnosis and management of prolactinomas.
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http://dx.doi.org/10.7759/cureus.19632DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8673687PMC
November 2021

Microsurgical Resection of a Giant Posterior Fossa Aneurysmal Malformation in a 21-Month-Old.

World Neurosurg 2022 Feb 27;158:165. Epub 2021 Nov 27.

Department of Neurosciences and Pediatrics, University of California San Diego, San Diego, California, USA. Electronic address:

Pediatric aneurysms commonly occur in the vertebrobasilar circulation with complex morphologies. "Aneurysmal malformations," or fistulous vessel dilations without a nidus, have also been described. Vessel friability and sensitivity to blood loss can complicate surgery. A 21-month-old male with motor and speech delay was found to have a giant posterior fossa aneurysmal malformation. He was lethargic, with minimal speech, and moved all extremities with mild hypotonia. Imaging demonstrated a 6.9 × 5.1 × 4.6 cm aneurysm arising from a fenestrated right V4 segment. This communicated via a single connection with the deep venous system, draining through the superior vermian cistern veins, posterior mesencephalic vein, basal vein of Galen, and inferior sagittal sinus, consistent with an arteriovenous fistula with secondary aneurysmal dilatation. Endovascular sacrifice was not feasible, in addition to concern for swelling after embolization. Three-dimensional modeling confirmed close proximity of the single inflow and outflow tracts. A suboccipital and left far lateral craniotomy for clip trapping and excision of the aneurysmal arteriovenous malformation was performed in a lateral position to completely decompress the brainstem (Video 1). Angiography before closure and postoperative vascular imaging demonstrated complete aneurysmal resection and fistula disconnection, with patency of normal vasculature. The postoperative course was notable for transient swallowing difficulties likely from lower cranial nerve irritation and refractory hydrocephalus requiring a shunt. The patient was meeting all developmental milestones at 2-year follow-up. This case highlights the complex vascular pathology often seen in pediatric patients, as well as the importance of presurgical planning and careful microsurgical technique in achieving a successful outcome.
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http://dx.doi.org/10.1016/j.wneu.2021.11.088DOI Listing
February 2022

Just Stick a Scope in: Laparoscopic Ventriculoperitoneal Shunt Placement in the Pediatric Reoperative Abdomen.

J Surg Res 2022 01 30;269:212-217. Epub 2021 Sep 30.

Division of Pediatric Surgery, Department of Surgery, University of California San Diego, San Diego, California. Electronic address:

Background: Ventriculoperitoneal shunt (VPS) placement into the reoperative abdomen can be challenging due to intraperitoneal adhesions. Laparoscopic guidance may provide safe abdominal access and identify an area for optimal cerebrospinal fluid drainage. The study aim was to compare laparoscopic-assisted VPS placement to an "open" approach in patients with prior abdominal surgery.

Materials And Methods: A retrospective review was performed of children undergoing VPS placement into a reoperative abdomen from 2009-2019. Clinical data were collected, and patients undergoing laparoscopy (LAP) were compared to those undergoing an open approach (OPEN).

Results: A total of 120 children underwent 169 VPS placements at a median age of 8 y (IQR 2-15 y), and a mean number of two prior abdominal operations (IQR 1-2). Laparoscopy was used in 24% of cases. Shunt-related complications within 30 d were lower in the LAP group (0% versus 19%, P = 0.001), as were VPS-related postoperative emergency department visits (0% versus 13%, P = 0.003) and readmissions (0% versus 13%, P = 0.013). Shunt malfunction rates were higher (42% OPEN versus 25% LAP, P = 0.03) and occurred sooner in the OPEN group (median 26 versus 78 wk, P = 0.01). The LAP group demonstrated shorter operative times (63 versus 100 min, P < 0.0001), and the only bowel injury. Time to feeds, length of stay, and mortality were similar between groups.

Conclusions: Laparoscopic guidance during VPS placement into the reoperative abdomen is associated with a decrease in shunt-related complications, longer shunt patency, and shorter operative times. Prospective study may clarify the potential benefits of laparoscopy in this setting.
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http://dx.doi.org/10.1016/j.jss.2021.07.045DOI Listing
January 2022

Maturation of the anterior petrous apex: surgical relevance for performance of the middle fossa transpetrosal approach in pediatric patients.

J Neurosurg 2021 Sep 17:1-7. Epub 2021 Sep 17.

2Department of Neurosciences and Pediatrics, University of California, San Diego, San Diego.

Objective: The middle fossa transpetrosal approach to the petroclival and posterior cavernous sinus regions includes removal of the anterior petrous apex (APA), an area well studied in adults but not in children. To this end, the authors performed a morphometric analysis of the APA region during pediatric maturation.

Methods: Measurements of the distance from the clivus to the internal auditory canal (IAC; C-IAC), the distance of the petrous segment of the internal carotid artery (petrous carotid; PC) to the mesial petrous bone (MPB; PC-MPB), the distance of the PC to the mesial petrous apex (MPA; PC-MPA), and the IAC depth from the middle fossa floor (IAC-D) were made on thin-cut CT scans from 60 patients (distributed across ages 0-3, 4-7, 8-11, 12-15, 16-18, and > 18 years). The APA volume was calculated as a cylinder using C-IAC (length) and PC-MPB (diameter). APA pneumatization was noted. Data were analyzed by laterality, sex, and age.

Results: APA parameters did not differ by laterality or sex. APA pneumatization was seen on 20 of 60 scans (33.3%) in patients ≥ 4 years. The majority of the APA region growth occurred by ages 8-11 years, with PC-MPA and PC-MPB increasing 15.9% (from 9.4 to 10.9 mm, p = 0.08) and 23.5% (from 8.9 to 11.0 mm, p < 0.01) between ages 0-3 and 8-11 years, and C-IAC increasing 20.7% (from 13.0 to 15.7 mm, p < 0.01) between ages 0-3 and 4-7 years. APA volume increased 79.6% from ages 0-3 to 8-11 years (from 834.3 to 1499.2 mm3, p < 0.01). None of these parameters displayed further significant growth. Finally, IAC-D increased 51.1% (from 4.3 to 6.5 mm, p < 0.01) between ages 0-3 and adult, without significant differences between successive age groups.

Conclusions: APA development is largely complete by the ages of 8-11 years. Knowledge of APA growth patterns may aid approach selection and APA removal in pediatric patients.
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http://dx.doi.org/10.3171/2021.3.JNS202648DOI Listing
September 2021

Optimizing international neurosurgical outreach missions: 15-year appraisal of operative skill transfer in Lima, Peru.

Surg Neurol Int 2021 24;12:425. Epub 2021 Aug 24.

Department of Pediatric Neurosurgery, University of California San Diego, San Diego, California, United States.

Background: While several medical outreach models have been designed and executed to alleviate the unmet need for international neurosurgical care, disparate strategies have evolved. There is a need to determine the optimal pediatric neurosurgical outreach model through which resources are efficiently utilized while imparting the largest possible impact on global health. This study evaluates the efficacy of an international pediatric neurosurgery outreach model at transferring operative skill in a sustainable and scalable manner in Lima, Peru over a 15-year duration.

Methods: Three 1-week neurosurgical missions were carried out (2004-2006) in Lima, Peru to teach neuroendoscopic techniques and to provide equipment to host neurosurgeons, equipping the hosts to provide care to indigent citizens beyond the duration of the missions. Follow-up data were obtained over a 15 year span, with collaboration maintained over email, two in-person visits, and video-conferencing services.

Results: Since the outreach missions in 2004-2006, the host neurosurgeons demonstrated sustainability of the neuroendoscopic instruction by independently performing neuroendoscopic operations on a growing caseload: at baseline, 0 cases were performed in 2003, but since 2012 and onwards, 40-45 cases have been performed annually. Scalability is illustrated by the fact that the institution established a rigorous neuroendoscopy training program to independently pass on the techniques to resident physicians.

Conclusion: The described international pediatric neurosurgical outreach model, centered around teaching operative technique as opposed to solely providing care to citizens, allowed operative skill to be sustainably transferred to surgeons in Lima, Peru. Having served the neuroendoscopic needs of hundreds of citizens, the strategic design is replicable and should be mirrored by future medical endeavors seeking to substantially impact the deficit in global surgical care.
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http://dx.doi.org/10.25259/SNI_241_2021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8422466PMC
August 2021

Palliative endoscopic third ventriculostomy for pediatric primary brain tumors: a single-institution case series.

J Neurosurg Pediatr 2021 Aug 6;28(4):387-394. Epub 2021 Aug 6.

Departments of1Neurological Surgery and.

Objective: Children with nonoperative brain tumors, such as diffuse intrinsic pontine gliomas (DIPGs), often have life-threatening hydrocephalus. Palliative shunting is common in such cases but can be complicated by hardware infection and mechanical failure. Endoscopic third ventriculostomy (ETV) is a minimally invasive alternative to treat hydrocephalus without implanted hardware. Herein, the authors report their institutional experience with palliative ETV for primary pediatric brain tumors.

Methods: The authors conducted a retrospective review of consecutive patients who had undergone palliative ETV for hydrocephalus secondary to nonresectable primary brain tumors over a 10-year period at Rady Children's Hospital. Collected variables included age, sex, tumor type, tumor location, presence of leptomeningeal spread, use of a robot for ETV, complications, ETV Success Score (ETVSS), functional status, length of survival, and follow-up time. A successful outcome was defined as an ETV performed without clinically significant perioperative complications or secondary requirement for a new shunt.

Results: Fifteen patients met the study inclusion criteria (11 males, 4 females; average age 7.9 years, range 0.8-21 years). Thirteen patients underwent manual ETV, and 2 patients underwent robotic ETV. Preoperative symptoms included gaze palsy, nausea/vomiting, headache, lethargy, hemiparesis, and seizures. Tumor types included DIPG (3), intraventricular/thalamic glioblastoma (2), and leptomeningeal spread of medulloblastoma (2), anaplastic oligo-/astrocytoma (2), rhabdoid tumor (2), primitive neuroectodermal tumor (1), ganglioglioma (1), pineoblastoma (1), and embryonal carcinoma (1). The mean preoperative ETVSS was 79 ± 8.8. There was 1 perioperative complication, a wound breakdown consistent with refractory hydrocephalus. The mean follow-up was 4.9 ± 5.5 months overall, and mean survival for the patients who died was 3.2 ± 3.6 months. Two patients remained alive at a mean follow-up of 15.7 months. Palliative ETV was successful in 7 patients (47%) and unsuccessful in 8 (53%). While patients with successful ETV were significantly older (11.9 ± 5.6 vs 4.4 ± 4.1 years, p = 0.010), there were no significant differences in preoperative ETVSS (p = 0.796) or postoperative survival (p = 0.476) between the successful and unsuccessful groups. Overall, functional outcomes were similar between the two groups; there was no significant difference in posttreatment Karnofsky Performance Status scores (68.6 ± 19.5 vs 61.3 ± 16.3, p = 0.454), suggesting that including ETV in the treatment algorithm did not worsen outcomes.

Conclusions: Palliative ETV is a safe and potentially efficacious treatment option in selected pediatric patients with hydrocephalus from nonoperative brain tumors. Close follow-up, especially in younger children, is required to ensure that patients with refractory symptoms receive appropriate secondary CSF diversion.
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http://dx.doi.org/10.3171/2021.3.PEDS20952DOI Listing
August 2021

Influence of Pediatric Endoscopic Endonasal Skull Base Resections on Midface and Skull Base Development.

Pediatr Neurosurg 2021 19;56(4):345-356. Epub 2021 May 19.

University of California San Diego School of Medicine, La Jolla, California, USA.

Objective: The purpose of this study is to use imaging data to determine if endoscopic endonasal surgery (EES) for skull base tumor resection interrupts skull base growth and development, resulting in an atrophic midface skeletal structure, compared to matched normal controls.

Methods: Data were collected by a retrospective chart review done on children aged 16 years and below who underwent endoscopic tumor resection and had pre- and postoperative magnetic resonance imaging with relevant midface anatomy. 121 normal controls were matched to 20 EES patients by age and gender. Three measurements related to midface anatomy were taken from 1 sagittal T1 slice and 1 axial T2 slice of each scan. Statistical analysis was used to compare growth measures between cases and controls.

Results: Twenty patients who underwent EES between November 2015 and April 2018 met our inclusion criteria. The mean age of the patients, 11 males and 9 females, was 10 years, and 8 patients (38%) were aged 7 years or younger. Six patients who had a high-flow CSF leak obtained a nasoseptal flap. A student T test and multivariate regression analysis found that EES did not affect midface and skull base growth. Among the variables assessed, age appears to be the only driver of growth.

Conclusion: There were no identified differences in craniofacial growth in pediatric patients undergoing EES for skull base tumor resection as compared to the control group. EES does not appear to significantly interfere with midface/skull base development and is a good surgical option for pediatric patients.
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http://dx.doi.org/10.1159/000516236DOI Listing
October 2021

Clinical and Neuroimaging Features of Magnetic Resonance-Guided Stereotactic Laser Ablation for Newly Diagnosed and Recurrent Pediatric Brain Tumors: A Single Institutional Series.

World Neurosurg 2021 06 17;150:e378-e387. Epub 2021 Mar 17.

Department of Neurosurgery, University of California San Diego, La Jolla, California, USA; Rady Children's Hospital, San Diego, California, USA.

Objective: We describe our single-institutional experience with magnetic resonance-guided stereotactic laser ablation (SLA) for the treatment of newly diagnosed and recurrent pediatric brain tumors.

Methods: Eighteen consecutive ablation procedures were performed in 17 patients from March 2016-April 2020. Patient demographics, indications, procedures, neuroimaging features, and outcomes were reviewed retrospectively.

Results: Seventeen patients (mean age of 11.4 years, 11 boys, 6 girls) underwent SLA with a mean follow-up of 24 months (range: 3-45 months). Tumor histologies included pilocytic astrocytoma (n = 5), ganglioglioma (n = 3), low-grade glioma not otherwise specified (n = 4), glioblastoma (n = 2), meningioma (n = 1), medulloblastoma (n = 1), and metastatic malignant peripheral nerve sheath tumor (n = 1). SLA was first-line therapy in 10 patients. Mean procedure duration including anesthesia time was 328 minutes (range: 244-529 minutes), and mean postoperative length of stay was 1.5 days (range 1-5 days). The complication rate was 29%, which included 3 patients who experienced postoperative motor changes, which resolved within several weeks of surgery, 1 patient with self-limited intraoperative bradycardia and hypotension, and 1 patient who died postoperatively due to intracranial hemorrhage from a distant lesion. Twelve of 17 patients had a neuroimaging response after SLA (4 complete responses, 8 partial responses, 1 stable disease). Percentage of tumor shrinkage from baseline ranged from 33%-100% (mean 75%). Patients with low-grade glioma exhibited the best responses to SLA (range 3%-100% decrease; mean 90%; 36% complete response rate).

Conclusions: SLA is a minimally invasive modality for the treatment of newly diagnosed and recurrent low-grade pediatric brain tumors. Low-grade glioma exhibited the best responses. Identification of ideal candidates for SLA, mitigation of perioperative complications, and demonstration of long-term outcomes need to be better defined in a clinical trial setting.
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http://dx.doi.org/10.1016/j.wneu.2021.03.027DOI Listing
June 2021

Rare case of mutant anaplastic pleomorphic xanthroastrocytoma in a 5-year survivor of acute lymphoblastic leukaemia.

BMJ Case Rep 2021 Feb 26;14(2). Epub 2021 Feb 26.

Neurosciences and Pediatrics, University of California San Diego, La Jolla, California, USA

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http://dx.doi.org/10.1136/bcr-2021-241815DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7919580PMC
February 2021

Pediatric Nasal Chondromesenchymal Tumors: Case Report and Review of the Literature.

Pediatr Neurosurg 2021 11;56(1):61-66. Epub 2021 Feb 11.

Department of Neurosurgery, University California San Diego, San Diego, California, USA,

Introduction: Nasal chondromesenchymal tumors (NCMT) are rare benign neoplasms that usually present in children <1 year of age. They can display rapid growth and significant local bony remodeling that can mimic a malignant process. Of the ∼50 published cases to date, few have documented the need for neurosurgical intervention. We herein report a NCMT in an infant treated with a staged cranial and transnasal approach, as well as summarize the available literature on this pathology.

Case Report: A newborn male with a compromised airway was noted to have a large sinonasal lesion. After stabilization, MRI demonstrated a 4-cm enhancing mass with diffuse sinus involvement and significant extension into the anterior cranial fossa, with displacement of the optic apparatus and hypothalamic pituitary axis. After an initial biopsy, the patient underwent a bifrontal craniofacial approach at 2 months of age, followed by a second-stage transnasal endoscopic approach at 15 months which resulted in a complete resection. There were no neurosurgical complications. Pathology was consistent with a NCMT.

Discussion: Although rare, neurosurgical involvement is critical for the treatment of NCMTs with intracranial extension. Staged cranial and endonasal endoscopic approaches may be needed for complete resection of such lesions.
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http://dx.doi.org/10.1159/000512717DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7969402PMC
October 2021

Unusual case of occipital lobe dysembryoplastic neuroepithelial tumour with fusion.

BMJ Case Rep 2021 Jan 27;14(1). Epub 2021 Jan 27.

Neurosciences and Pediatrics, University of California San Diego, La Jolla, California, USA

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http://dx.doi.org/10.1136/bcr-2020-241440DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7843307PMC
January 2021

Arteriovenous malformation surgery in children: the Rady Children's Hospital experience (2002-2019).

Childs Nerv Syst 2021 04 6;37(4):1267-1277. Epub 2021 Jan 6.

Department of Neurosurgery, University of California-San Diego, San Diego, CA, USA.

Purpose: Compared to adult AVMs, there is a paucity of data on the microsurgical treatment of pediatric AVMs. We report our institutional experience with pediatric AVMs treated by microsurgical resection with or without endovascular embolization and radiation therapy.

Methods: We retrospectively reviewed all patients ≤ 18 years of age with cerebral AVMs that underwent microsurgical resection at Rady Children's Hospital 2002-2019.

Results: Eighty-nine patients met inclusion criteria. The mean age was 10.3 ± 5.0 years, and 56% of patients were male. In total, 72 (81%) patients presented with rupture. Patients with unruptured AVMs presented with headache (n = 5, 29.4%), seizure (n = 9, 52.9%), or incidental finding (n = 3, 17.7%). The mean presenting mRS was 2.8 ± 1.8. AVM location was lobar in 78%, cerebellar/brainstem in 15%, and deep supratentorial in 8%. Spetzler-Martin grade was I in 28%, II in 45%, III in 20%, IV in 6%, and V in 1%. Preoperative embolization was utilized in 38% of patients and more frequently in unruptured than ruptured AVMs (62% vs. 32%, p = 0.022). Radiographic obliteration was achieved in 76/89 (85.4%) patients. Complications occurred in 7 (8%) patients. Annualized rates of delayed rebleeding and recurrence were 1.2% and 0.9%, respectively. The mean follow-up was 2.8 ± 3.1 years. A good neurological outcome (mRS score ≤ 2) was obtained in 80.9% of patients at last follow-up and was improved relative to presentation for 75% of patients.

Conclusions: Our case series demonstrates high rates of radiographic obliteration and relatively low incidence of neurologic complications of treatment or AVM recurrence.
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http://dx.doi.org/10.1007/s00381-020-04994-9DOI Listing
April 2021

Surgical Relevance of Pediatric Anterior Clinoid Process Maturation for Anterior Skull Base Approaches.

Oper Neurosurg (Hagerstown) 2021 02;20(3):E200-E207

Department of Neurosciences and Pediatrics, University of California, San Diego, San Diego, California.

Background: Removal of the anterior clinoid process (ACP) can expand anterior skull base surgical corridors. ACP development and anatomical variations are poorly defined in children.

Objective: To perform a morphometric analysis of the ACP during pediatric maturation.

Methods: Measurements of ACP base thickness (ACP-BT), midpoint thickness (ACP-MT), length (ACP-L), length from optic strut to ACP tip (ACP-OS), pneumatization (ACP-pneumo), and the presence of an ossified carotico-clinoid ligament (OCCL) or interclinoid ligament (OIL) were made from high-resolution computed-tomography scans from 60 patients (ages 0-3, 4-7, 8-11 12-15, 16-18, and >18 yr). Data were analyzed by laterality, sex, and age groups using t-tests and linear regression.

Results: There were no significant differences in ACP parameters by laterality or sex, and no significant growth in ACP-BT or ACP-MT during development. From ages 0-3 yr to adult, mean ACP-L increased 49%, from 7.7 to 11.5 mm. The majority of ACP-L growth occurred in 2 phases between ages 0-3 to 8-11 and ages 16-18 to adult. Conversely, ACP-OS was stable from ages 0-3 to 8-11 but increased by 63% between ages 8-11 to adult. Variations in ACP morphology (OCCL/OIL/ACP-pneumo) were found in 15% (9/60) of scans. OCCL and OIL occurred in patients as young as 3 yrs, whereas ACP-pneumo was not seen in patients younger than 11 yrs.

Conclusion: The ACP demonstrates stable thickness and a complex triphasic elongation and remodeling pattern with development, the understanding of which may facilitate removal in patients <12. Clinically relevant ACP anatomic variations can occur at any age.
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http://dx.doi.org/10.1093/ons/opaa374DOI Listing
February 2021

Robot-assisted stereotactic biopsy of pediatric brainstem and thalamic lesions.

J Neurosurg Pediatr 2020 Dec 25;27(3):317-324. Epub 2020 Dec 25.

1Department of Neurosurgery, University of California, San Diego, La Jolla, California.

Objective: Biopsies of tumors located in deep midline structures require highly accurate stereotaxy to safely obtain lesional tissue suitable for molecular and histological analysis. Versatile platforms are needed to meet a broad range of technical requirements and surgeon preferences. The authors present their institutional experience with the robotic stereotactic assistance (ROSA) system in a series of robot-assisted biopsies of pediatric brainstem and thalamic tumors.

Methods: A retrospective analysis was performed of 22 consecutive patients who underwent 23 stereotactic biopsies of brainstem or thalamic lesions using the ROSA platform at Rady Children's Hospital in San Diego between December 2015 and January 2020.

Results: The ROSA platform enabled rapid acquisition of lesional tissue across various combinations of approaches, registration techniques, and positioning. No permanent deficits, major adverse outcomes, or deaths were encountered. One patient experienced temporary cranial neuropathy, and 3 developed small asymptomatic hematomas. The diagnostic success rate of the ROSA system was 91.3%.

Conclusions: Robot-assisted stereotactic biopsy of these lesions may be safely performed using the ROSA platform. This experience comprises the largest clinical series to date dedicated to robot-assisted biopsies of brainstem and diencephalic tumors.
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http://dx.doi.org/10.3171/2020.7.PEDS20373DOI Listing
December 2020

Improving Pediatric Neuro-Oncology Survival Disparities in the United States-Mexico Border Region: A Cross-Border Initiative Between San Diego, California, and Tijuana, Mexico.

JCO Glob Oncol 2020 11;6:1791-1802

Peckham Center for Cancer and Blood Disorders, Rady Children's Hospital San Diego, San Diego, CA.

Purpose: Treatment of children with CNS tumors (CNSTs) demands a complex, interdisciplinary approach that is rarely available in low- and middle-income countries. We established the Cross-Border Neuro-Oncology Program (CBNP) between Rady Children's Hospital, San Diego (RCHSD), and Hospital General, Tijuana (HGT), Mexico, to provide access to neuro-oncology care, including neurosurgic services, for children with CNSTs diagnosed at HGT. Our purpose was to assess the feasibility of the CBNP across the United States-Mexico border and improve survival for children with CNSTs at HGT by implementing the CBNP.

Patients And Methods: We prospectively assessed clinicopathologic profiles, the extent of resection, progression-free survival, and overall survival (OS) in children with CNSTs at HGT from 2010 to 2017.

Results: Sixty patients with CNSTs participated in the CBNP during the study period. The most common diagnoses were low-grade glioma (24.5%) and medulloblastoma (22.4%). Of patients who were eligible for surgery, 49 underwent resection at RCHSD and returned to HGT for collaborative management. Gross total resection was achieved in 78% of cases at RCHSD compared with 0% at HGT ( < .001) and was a predictor of 5-year OS (hazard ratio, 0.250; 95% CI, 0.067 to 0.934; = .024). Five-year OS improved from 0% before 2010 to 52% in 2017.

Conclusion: The CBNP facilitated access to complex neuro-oncology care for underserved children in Mexico through binational exchanges of resources and expertise. Survival for patients in the CBNP dramatically improved. Gross total resection at RCHSD was associated with higher OS, highlighting the critical role of experienced neurosurgeons in the treatment of CNSTs. The CBNP model offers an attractive alternative for children with CNSTs in low- and middle-income countries who require complex neuro-oncology care, particularly those in close proximity to institutions in high-income countries with extensive neuro-oncology expertise.
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http://dx.doi.org/10.1200/GO.20.00377DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7713516PMC
November 2020

Functional Precision Medicine Identifies New Therapeutic Candidates for Medulloblastoma.

Cancer Res 2020 12 12;80(23):5393-5407. Epub 2020 Oct 12.

Rady Children's Institute for Genomic Medicine, San Diego, California.

Medulloblastoma is among the most common malignant brain tumors in children. Recent studies have identified at least four subgroups of the disease that differ in terms of molecular characteristics and patient outcomes. Despite this heterogeneity, most patients with medulloblastoma receive similar therapies, including surgery, radiation, and intensive chemotherapy. Although these treatments prolong survival, many patients still die from the disease and survivors suffer severe long-term side effects from therapy. We hypothesize that each patient with medulloblastoma is sensitive to different therapies and that tailoring therapy based on the molecular and cellular characteristics of patients' tumors will improve outcomes. To test this, we assembled a panel of orthotopic patient-derived xenografts (PDX) and subjected them to DNA sequencing, gene expression profiling, and high-throughput drug screening. Analysis of DNA sequencing revealed that most medulloblastomas do not have actionable mutations that point to effective therapies. In contrast, gene expression and drug response data provided valuable information about potential therapies for every tumor. For example, drug screening demonstrated that actinomycin D, which is used for treatment of sarcoma but rarely for medulloblastoma, was active against PDXs representing Group 3 medulloblastoma, the most aggressive form of the disease. Functional analysis of tumor cells was successfully used in a clinical setting to identify more treatment options than sequencing alone. These studies suggest that it should be possible to move away from a one-size-fits-all approach and begin to treat each patient with therapies that are effective against their specific tumor. SIGNIFICANCE: These findings show that high-throughput drug screening identifies therapies for medulloblastoma that cannot be predicted by genomic or transcriptomic analysis.
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http://dx.doi.org/10.1158/0008-5472.CAN-20-1655DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7718387PMC
December 2020

Low-grade glioneuronal tumour with novel molecular features associated with unusual partial epilepsy in a child.

BMJ Case Rep 2020 Sep 7;13(9). Epub 2020 Sep 7.

Neurosciences and Pediatrics, University of California San Diego, San Diego, California, USA

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http://dx.doi.org/10.1136/bcr-2020-237373DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7478008PMC
September 2020

Three-Dimensional Modeling of Complex Pediatric Intracranial Aneurysmal Malformations With a Virtual Reality System.

Simul Healthc 2021 Aug;16(4):295-300

From the Department of Neurological Surgery, University of California, San Diego, La Jolla, CA.

Introduction: Surgical simulation is valuable in neurovascular surgery given the progressive rarity of these cases and their technical complexity, but its use has not been well described for pediatric vascular pathologies. We herein review the use of surgical simulation at our institution for complex pediatric aneurysmal malformations.

Methods: A retrospective review of patients treated for middle cerebral artery aneurysmal malformations with surgical simulation assistance (SuRgical Planner [SRP]; Surgical Theater, Mayfield Village, OH) during a 2-year period at Rady Children's Hospital of San Diego was performed.

Results: In 5 pediatric patients with complex MCA aneurysmal malformations (mean age = 33.2 ± 49.9 months), preoperative 3-dimensional (3D) interactive modeling informed treatment planning and enhanced surgeon understanding of the vascular pathology. Availability of intraoperative simulation also aided real-time anatomical understanding during surgery. Specific benefits of simulation for these cases included characterization of involved perforating vessels, as well as an enhanced understanding of flow patterns within associated complex arteriovenous fistulas and feeding vessel/daughter branch anatomy. Despite the complexity of the lesions treated, use of simulation qualitatively enhanced surgeon confidence. There were no perioperative complications for patients treated with open surgery.

Conclusions: Surgical simulation may aid in the treatment of complex pediatric aneurysmal malformations.
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http://dx.doi.org/10.1097/SIH.0000000000000498DOI Listing
August 2021

Middle fossa approach for a pediatric facial nerve meningioma.

J Neurosurg Pediatr 2020 Aug 28;26(5):578-582. Epub 2020 Aug 28.

3Neurosciences and Pediatrics, University of California, San Diego, California.

Pediatric cerebellopontine angle (CPA) meningiomas are extremely rare and are usually treated with a retrosigmoid surgical approach or radiation. The authors present the use of a middle fossa approach for the treatment of a symptomatic CPA meningioma in a 22-month-old female. The patient initially presented at 17 months with isolated progressive, long-standing right-sided facial weakness. MRI demonstrated a 5.0 × 5.0-mm right CPA lesion just superior to the cisternal segment of cranial nerve (CN) VII, which demonstrated growth on interval imaging. At 22 months of age she underwent a successful middle fossa craniotomy, including wide exposure of the porus acusticus, allowing for a gross-total resection with preservation of CNs VII and VIII. Pathological analysis revealed a WHO grade I meningioma. The patient remained neurologically stable on follow-up. The middle fossa approach can be used to safely access the CPA in properly selected pediatric patients.
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http://dx.doi.org/10.3171/2020.5.PEDS2034DOI Listing
August 2020

A Paramedian Supracerebellar, Infratentorial Approach for Resection of Midbrain Tumor.

World Neurosurg 2020 11 9;143:83. Epub 2020 Jul 9.

Pediatric Division of Neurosurgery, University of California at San Diego, San Diego, California, USA. Electronic address:

Brainstem tumors represent formidable lesions for neurosurgical intervention. They should be approached with a thorough understanding of the anatomy and clear sense of surgical goals. A 14-year-old previously healthy girl presented with 2 weeks of nausea, headaches, diplopia, and gait instability. Workup revealed a 3.5 x 2.5 x 2.5 cm contrast-enhancing mass within the right midbrain. The patient consented to the surgical procedure. Preoperative imaging demonstrated superior displacement of the deep venous system because of the tumor, as well as inferior displacement of the fourth cranial nerve exit zone and posterior bowing of the entire tectal region. This allowed a safe corridor from a supracerebellar infratentorial approach. The three quarters lateral position was chosen to optimize surgeon ergonomics and allow for gravity to drain blood from the operative field. Near total resection was obtained without any new neurologic deficit. Final pathology was consistent with pilocytic astrocytoma, World Health Organization grade I. The video demonstrates the surgical approach in addition to techniques for brainstem tumor resection (Video 1).
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http://dx.doi.org/10.1016/j.wneu.2020.06.243DOI Listing
November 2020

Atypical central neurocytoma with aggressive features in a child.

BMJ Case Rep 2020 Jun 21;13(6). Epub 2020 Jun 21.

Neurosciences and Pediatrics, University of California San Diego, San Diego, California, USA

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http://dx.doi.org/10.1136/bcr-2020-236262DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7307550PMC
June 2020

Tumor necrosis factor overcomes immune evasion in p53-mutant medulloblastoma.

Nat Neurosci 2020 07 18;23(7):842-853. Epub 2020 May 18.

Department of Pediatrics, Oregon Health & Science University, Portland, OR, USA.

Many immunotherapies act by enhancing the ability of cytotoxic T cells to kill tumor cells. Killing depends on T cell recognition of antigens presented by class I major histocompatibility complex (MHC-I) proteins on tumor cells. In this study, we showed that medulloblastomas lacking the p53 tumor suppressor do not express surface MHC-I and are therefore resistant to immune rejection. Mechanistically, this is because p53 regulates expression of the peptide transporter Tap1 and the aminopeptidase Erap1, which are required for MHC-I trafficking to the cell surface. In vitro, tumor necrosis factor (TNF) or lymphotoxin-β receptor agonist can rescue expression of Erap1, Tap1 and MHC-I on p53-mutant tumor cells. In vivo, low doses of TNF prolong survival and synergize with immune checkpoint inhibitors to promote tumor rejection. These studies identified p53 as a key regulator of immune evasion and suggest that TNF could be used to enhance sensitivity of tumors to immunotherapy.
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http://dx.doi.org/10.1038/s41593-020-0628-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7456619PMC
July 2020

Commentary: What Variables Correlate With Different Clinical Outcomes of Abusive Head Injury?

Neurosurgery 2020 09;87(4):E497

Department of Neurological Surgery, University of California, San Diego, La Jolla, California.

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http://dx.doi.org/10.1093/neuros/nyaa153DOI Listing
September 2020

Unusual extradural chordoma in an adolescent presenting with lumbar radiculopathy.

BMJ Case Rep 2020 Apr 6;13(4). Epub 2020 Apr 6.

Neurosciences and Pediatrics, University of California San Diego, San Diego, California, USA

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http://dx.doi.org/10.1136/bcr-2020-234942DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7244276PMC
April 2020

Magnetic resonance-guided stereotactic laser ablation therapy for the treatment of pediatric brain tumors: a multiinstitutional retrospective study.

J Neurosurg Pediatr 2020 Mar 27;26(1):13-21. Epub 2020 Mar 27.

Departments of7Neurosurgery and.

Objective: This study aimed to assess the safety and efficacy of MR-guided stereotactic laser ablation (SLA) therapy in the treatment of pediatric brain tumors.

Methods: Data from 17 North American centers were retrospectively reviewed. Clinical, technical, and radiographic data for pediatric patients treated with SLA for a diagnosis of brain tumor from 2008 to 2016 were collected and analyzed.

Results: A total of 86 patients (mean age 12.2 ± 4.5 years) with 76 low-grade (I or II) and 10 high-grade (III or IV) tumors were included. Tumor location included lobar (38.4%), deep (45.3%), and cerebellar (16.3%) compartments. The mean follow-up time was 24 months (median 18 months, range 3-72 months). At the last follow-up, the volume of SLA-treated tumors had decreased in 80.6% of patients with follow-up data. Patients with high-grade tumors were more likely to have an unchanged or larger tumor size after SLA treatment than those with low-grade tumors (OR 7.49, p = 0.0364). Subsequent surgery and adjuvant treatment were not required after SLA treatment in 90.4% and 86.7% of patients, respectively. Patients with high-grade tumors were more likely to receive subsequent surgery (OR 2.25, p = 0.4957) and adjuvant treatment (OR 3.77, p = 0.1711) after SLA therapy, without reaching significance. A total of 29 acute complications in 23 patients were reported and included malpositioned catheters (n = 3), intracranial hemorrhages (n = 2), transient neurological deficits (n = 11), permanent neurological deficits (n = 5), symptomatic perilesional edema (n = 2), hydrocephalus (n = 4), and death (n = 2). On long-term follow-up, 3 patients were reported to have worsened neuropsychological test results. Pre-SLA tumor volume, tumor location, number of laser trajectories, and number of lesions created did not result in a significantly increased risk of complications; however, the odds of complications increased by 14% (OR 1.14, p = 0.0159) with every 1-cm3 increase in the volume of the lesion created.

Conclusions: SLA is an effective, minimally invasive treatment option for pediatric brain tumors, although it is not without risks. Limiting the volume of the generated thermal lesion may help decrease the incidence of complications.
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http://dx.doi.org/10.3171/2020.1.PEDS19496DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7885863PMC
March 2020

Novel cavernous sinus mutant hemangioendothelioma in a teenager.

BMJ Case Rep 2020 Mar 18;13(3). Epub 2020 Mar 18.

Neurosciences and Pediatrics, University of California San Diego, San Diego, California, USA

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http://dx.doi.org/10.1136/bcr-2020-234677DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7101000PMC
March 2020
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