Patient-specific mutation of Dync1h1 in mice causes brain and behavioral deficits.

AIMS: Cytoplasmic dynein heavy chain (DYNC1H1) is a multi-subunit protein complex that provides motor force for movement of cargo on microtubules and traffics them back to the soma. In humans, mutations along the DYNC1H1 gene result in intellectual disabilities, cognitive delays, and neurologic and motor deficits. The aim of the study was to generate a mouse model to a newly identified de novo heterozygous DYNC1H1 mutation, within a functional ATPase domain (c9052C > T(P3018S)), identified in a child with motor deficits, and intellectual disabilities. RESULTS: P3018S heterozygous (HET) knockin mice are viable; homozygotes are lethal. Metabolic and EchoMRI™ testing show that HET mice have a higher metabolic rate, are more active, and have less body fat compared to wildtype mice. Neurobehavioral studies show that HET mice perform worse when traversing elevated balance beams, and on the negative geotaxis test. Immunofluorescent staining shows neuronal migration abnormalities in the dorsal and lateral neocortex with heterotopia in layer I. Neuron-subtype specific transcription factors CUX1 and CTGF identified PI+ neurons from layers II/III and VI respectively in cortical layer I, and abnormal pyramidal neurons with MAP2+ dendrites projecting downward from the pial surface. CONCLUSION: The HET mice are a good model for the motor deficits seen in the child, and highlights the importance of cytoplasmic dynein in the maintenance of cortical function and dendritic orientation relative to the pial surface. Our results are discussed in the context of other dynein mutant mice and in relation to clinical presentation in humans with DYNC1H1 mutations.

Local control and survival after stereotactic radiosurgery for colorectal cancer brain metastases: an international multicenter analysis.

OBJECTIVE: The goal of this study was to characterize local tumor control (LC), overall survival (OS), and safety of stereotactic radiosurgery for colorectal brain metastasis (CRBM). METHODS: Ten international institutions participating in the International Radiosurgery Research Foundation provided data for this retrospective case series. This study included 187 patients with CRBM (281 tumors), with a median age of 62 years and 56.7% being male. Most patients (53.5%) had solitary tumors, although 10.7% had > 5 tumors. The median tumor volume was 2.7 cm3 (IQR 0.22-8.1 cm3), and the median margin dose was 20 Gy (IQR 18-22 Gy). RESULTS: The 3-year LC and OS rates were 72% and 20%, respectively. Symptomatic adverse radiation effects occurred in 1.6% of patients. In the multivariate analysis, age > 65 years and tumor volume > 4.0 cm3 were significant predictors of tumor progression (hazard ratio [HR] 2.6, 95% CI 1.4-4.9; p = 0.003 and HR 3.4, 95% CI 1.7-6.9; p < 0.001, respectively). Better performance status (Karnofsky Performance Scale score > 80) was associated with a reduced risk of tumor progression (HR 0.38, 95% CI 0.19-0.73; p = 0.004). Patient age > 62 years (HR 1.6, 95% CI 1.1-2.3; p = 0.03) and the presence of active extracranial disease (HR 1.7, 95% CI 1.1-2.4; p = 0.009) were significantly associated with worse OS. CONCLUSIONS: Stereotactic radiosurgery offers a high LC rate and a low rate of symptomatic adverse radiation effects for the majority of CRBMs. The OS and LC favored younger patients with high functional performance scores and inactive extracranial disease.

Complications of Endovascular and Open Aneurysm Surgery in the Era of Flow Diversion.

The techniques used for treatment of intracranial aneurysms have progressed dramatically over the decades. The introduction of modern endovascular techniques and the continued refinement of progressively less invasive neurosurgical approaches have contributed to steadily improving clinical outcomes. Moreover, innovations such as flow-diverting stents have achieved dramatic success and have gained rapid widespread adoption. Particularly in lesions for which the application of conventional treatment techniques is difficult, flow diversion technology has revolutionized aneurysm management. This review provides a discussion on the morbidity and mortality encountered in the treatment of intracranial aneurysms in the modern era. Common adverse events faced in the management of these lesions with open surgery and various endovascular techniques are highlighted.

TMEM106B Puncta Is Increased in Multiple Sclerosis Plaques, and Reduced Protein in Mice Results in Delayed Lipid Clearance Following CNS Injury.

During inflammatory, demyelinating diseases such as multiple sclerosis (MS), inflammation and axonal damage are prevalent early in the course. Axonal damage includes swelling, defects in transport, and failure to clear damaged intracellular proteins, all of which affect recovery and compromise neuronal integrity. The clearance of damaged cell components is important to maintain normal turnover and restore homeostasis. In this study, we used mass spectrometry to identify insoluble proteins within high-speed/mercaptoethanol/sarcosyl-insoluble pellets from purified white matter plaques isolated from the brains of individuals with relapsing-remitting MS (RRMS). We determined that the transmembrane protein 106B (TMEM106B), normally lysosome-associated, is insoluble in RRMS plaques relative to normal-appearing white matter from individuals with Alzheimer's disease and non-neurologic controls. Relative to wild-type mice, hypomorphic mice with a reduction in TMEM106B have increased axonal damage and lipid droplet accumulation in the spinal cord following myelin-oligodendrocyte-glycoprotein-induced experimental autoimmune encephalomyelitis. Additionally, the corpora callosa from cuprizone-challenged hypomorphic mice fail to clear lipid droplets efficiently during remyelination, suggesting that when TMEM106B is compromised, protein and lipid clearance by the lysosome is delayed. As TMEM106B contains putative lipid- and LC3-binding sites, further exploration of these sites is warranted.

Postischemic Trigeminal Neuropathy Treated With Radiofrequency Ablation.

Trigeminal neuralgia is a pain syndrome that is defined by sharp electrical shock-like pain that radiates in the sensory distribution of the trigeminal nerve. The classical cause of this syndrome is vascular compression, but other causes, such as stroke, have also been described. Instances of post-ischemic trigeminal pain have been described as meeting the classic description, and are termed trigeminal neuropathy. The treatment paradigms for trigeminal neuralgia versus neuropathy differ significantly, especially with the consideration of surgical management.We present a case of a 78-year-old man with post-ischemic trigeminal neuropathy that was successfully treated with radiofrequency ablation after failure of conservative management.We also summarize three previous cases of post-ischemic trigeminal neuropathy that were also successfully treated with percutaneous surgical treatment, showing that percutaneous surgical management should be considered in patients with post-ischemic trigeminal neuropathy that fail conservative management.

Local Control and Survival Outcomes After Stereotactic Radiosurgery for Brain Metastases From Gastrointestinal Primaries: An International Multicenter Analysis.

BACKGROUND: There are limited data regarding outcomes for patients with gastrointestinal (GI) primaries and brain metastases treated with stereotactic radiosurgery (SRS). OBJECTIVE: To examine clinical outcomes after SRS for patients with brain metastases from GI primaries and evaluate potential prognostic factors. METHODS: The International Radiosurgery Research Foundation centers were queried for patients with brain metastases from GI primaries managed with SRS. Primary outcomes were local control (LC) and overall survival (OS). Kaplan-Meier analysis was used for univariate analysis (UVA) of prognostic factors. Factors significant on UVA were evaluated with a Cox multivariate analysis proportional hazards model. Logistic regressions were used to examine correlations with RN. RESULTS: We identified 263 eligible patients with 543 brain metastases. Common primary sites were rectal (31.2%), colon (31.2%), and esophagus (25.5%) with a median age of 61.6 years (range: 37-91.4 years) and a median Karnofsky performance status (KPS) of 90% (range: 40%-100%). One-year and 2-year LC rates were 83.5% (95% CI: 78.9%-87.1%) and 73.0% (95% CI: 66.4%-78.5%), respectively. On UVA, age >65 years ( P = .001), dose <20 Gy ( P = .006) for single-fraction plans, KPS <90% ( P < .001), and planning target volume ≥2cc ( P = .007) were associated with inferior LC. All factors other than dose were significant on multivariate analysis ( P ≤ .002). One-year and 2-year OS rates were 68.0% (95% CI: 61.5%-73.6%) and 31.2% (95% CI: 24.6%-37.9%), respectively. Age > 65 years ( P = .006), KPS <90% ( P = .005), and extracranial metastases ( P = .05) were associated with inferior OS. CONCLUSION: SRS resulted in comparable LC with common primaries. Age and KPS were associated with both LC and OS with planning target volume and extracranial metastases correlating with LC and OS, respectively. These factors should be considered in GI cancer patient selection for SRS.

Pearls & Oy-sters: Pivoting Treatment Regimens of Pediatric Atypical Teratoid Rhabdoid Tumors to Optimize Care in Adult ATRT: A Case Report.

Atypical teratoid rhabdoid tumor (ATRT) is a highly malignant embryonal tumor of the CNS, largely affecting pediatric patients, with exceedingly rare cases in adults at an estimated annual incidence of 1/1,000,000. We report a unique case of ATRT in a 43-year-old female patient who first presented with progressive focal headaches. Imaging revealed a sellar mass with suprasellar extensions, which was partially removed via a transsphenoidal resection. The tumor aggressively recurred just 1 month postoperatively. Her care team pursued a novel treatment plan by using a slightly modified COG ACNS 0332 regimen, which involved radiation, followed by 4 cycles of monthly chemotherapy including vincristine, cyclophosphamide, and cisplatin. Hematopoietic stem cells were collected between radiation and chemotherapy in the event that the patient required stem cell salvage therapy postadjuvant chemotherapy. The MRIs taken at 2 and 4 months postrecurrence indicated a substantial decrease in tumor volume, with corresponding clinical improvements to cranial nerve deficits. Given the scarcity of literature on adult cases of ATRT and the lack of a standard of care for these cases, discussing the efficacy of our patient's treatment plan may aid clinical decision making for adult ATRT cases.

A Case Study Using Papaya Leaf Extract to Reverse Chemotherapy-Induced Thrombocytopenia in a GBM Patient.

Chemotherapy-induced thrombocytopenia (CIT) is a critical condition in which platelet counts are abnormally reduced following the administration of chemotherapeutic compounds. CIT poses a treatment conundrum to clinicians given the increased risk of spontaneous bleeding, obstacles to surgical management of tumors, and exclusion from clinical trials. Treatment of CIT involves the removal of the offending agent combined with platelet infusion or thrombopoietin agonist treatment. However, due to the autoimmune and infection risks associated with infusions, this treatment is only reserved for patients with critically low platelet counts. One potential solution for patients in the mid to low platelet count range is Carica papaya leaf extract (CPLE). In this case, we report the novel use of CPLE as a method of bolstering platelet counts in a patient presenting with CIT. The patient was initiated on CPLE therapy consisting of 1 tablespoon twice daily with meals. Following CPLE treatment, the patient's platelet counts rebounded from less than 10,000/µL to 113,000/µL. This clinical vignette supports the use of CPLE in the clinical context of CIT when thrombopoietin agonists are not a viable option. The potential benefits of CPLE as a method for increasing platelet count deserve further exploration, especially as a treatment option for refractory patients or those ill-suited for other traditional thrombocytopenia therapies.

The Saga of Pulse Oximetry Screening for Critical Congenital Heart Disease in Israel: A Historical Perspective.

BACKGROUND: Many countries have adopted a mandatory routine pulse oximetry screening of newborn infants to identify babies with otherwise asymptomatic critical congenital heart disease (CCHD). OBJECTIVES: To describe the current status of pulse oximetry CCHD screening in Israel, with a special emphasis on the experience of the Shaare Zedek Medical Center. METHODS: We review the difficulties of the Israeli Medical system with adopting the SaO2 screening, and the preliminary results of the screening at the Shaare Zedek Medical Center, both in terms of protocol compliance and CCHD detection. RESULTS: Large scale protocol cannot be implemented in one day, and regular quality assessment programs must take place in order to improve protocol compliance and identify the reasons for protocol failures. CONCLUSIONS: Quality control reviews should be conducted soon after implementation of the screening to allow for prompt diagnosis and quick resolution.

Spontaneous Cerebrospinal Fluid Rhinorrhea from a Prolactin-Secreting Pituitary Macroadenoma.

Cerebrospinal fluid (CSF) rhinorrhea is a rare complication of macroprolactinomas that, in the vast majority of cases, is subsequent to either medical or surgical intervention. Here, we present the successful management of a rare case of spontaneous, noniatrogenic CSF rhinorrhea in a patient with an untreated macroprolactinoma. A 27-year-old man with no significant medical history presented with six months of persistent CSF rhinorrhea, which was confirmed by testing for beta-2-transferrin. He had had decreased libido since adolescence and impaired growth of secondary sexual characteristics. Workup revealed an elevated prolactin level, and imaging demonstrated erosion of the anterior sellar floor and soft tissue within the sphenoid sinus, concerning for tumor. The patient underwent surgical repair of the CSF leak via a transnasal transsphenoidal approach, with resection and biopsy of tumor material within the sinus. No tumor was noted within the sella itself. The patient tolerated the procedure well and had subsequent normalization of his prolactin level with no further CSF egress. Spontaneous noniatrogenic CSF rhinorrhea, although rare, should be considered in the differential diagnosis of invasive pituitary macroadenomas, especially prolactinomas. The mechanism of CSF leak from a prolactinoma is not completely understood, but the CSF leak should be urgently repaired through a transnasal transsphenoidal approach. Concurrently, tumor resection should be performed and a postoperative lumbar puncture or lumbar drain should be considered to reinforce the skull base reconstruction.

Surgical Management of Multifocal Trigeminal Schwannomas.

BACKGROUND: Isolated trigeminal schwannomas occur in 0.07% to 0.3% of intracranial tumors and account for 0.8% to 8% of intracranial schwannomas and 1/3 of Meckel cave tumors. The presence of multisegmental schwannoma is rare, resulting in a limited understanding of its optimal management. OBJECTIVE: To describe potential surgical options to manage this rare entity. METHODS: A 2-institution retrospective review of all patients with pathologically confirmed trigeminal schwannoma managed with resection from January 2009 through January 2019 was conducted. A manual chart review was performed to verify patients' inclusion and collect data about age, sex, tumor size, tumor site, treatment modality, surgical approach, complications, and follow-up duration and status. RESULTS: A total of 4 patients (age range 12-50 yr) who underwent a variety of cranial and orbitocranial approaches for tumor resection were identified. Patients achieved good outcomes with improvement of visual outcomes. One case of infection and 1 case of partial tumor recurrence requiring reresection were identified. CONCLUSION: Multisegmental trigeminal schwannoma is a rare and unique entity, often associated with trigeminal schwannomatosis. Interdisciplinary management has been shown to be the most effective method for improving patient outcomes with these complex and poorly understood diseases.

Internal Auditory Canal Variability: Anatomic Variation Affects Cisternal Facial Nerve Visualization.

BACKGROUND: The internal auditory canal (IAC) is an important landmark during surgery for lesions of the cerebellopontine angle. There is significant variability in the position and orientation of the IAC radiographically, and the authors have noted differences in surgical exposure depending on the individual anatomy of the IAC. OBJECTIVE: To test the hypothesis that IAC position and orientation affects the surgical exposure of the IAC and facial nerve, especially when performing the translabyrinthine approach. METHODS: The authors retrospectively reviewed magnetic resonance imaging studies of 50 randomly selected patients with pathologically confirmed vestibular schwannomas. Measurements, including the anterior (APD) and posterior (PPD) petrous distances, the anterior (APA) and posterior (PPA) petro-auditory angles, and the internal auditory angle (IAA), were obtained to quantify the position and orientation of the IAC within the petrous temporal bone. RESULTS: The results quantitatively demonstrate tremendous variability of the position and orientation of the IAC in the petrous temporal bone. The measurement ranges were APD 10.2 to 26.1 mm, PPD 15.1 to 37.2 mm, APA 104 to 157°, PPA 30 to 96°, and IAA -5 to 40°. CONCLUSION: IAC variability can have a substantial effect on the surgical exposure of the IAC and facial and vestibulocochlear nerves. Specifically, a horizontally oriented IAC with a small IAA may have significant impact on visualization of the facial nerve within its cisternal segment with the translabyrinthine approach. The retrosigmoid approach is less affected with IAC variability in position and angle.

Repeat Resection of a Cavernous Malformation of the Optic Nerve/Chiasm via a Frontotemporal Approach: 2-Dimensional Operative Video.

Cavernous malformations are benign vascular lesions that can arise throughout the central nervous system. The occurrence of a cavernous malformation within the optic nerve or chiasm, however, is extremely uncommon. The case described in this video involved a 36-yr-old woman who presented 3 mo after undergoing a left frontotemporal craniotomy for resection of an optic nerve cavernous malformation. She had initially presented to an outside hospital with vision loss, and the left optic nerve lesion was identified and resected. Although her vision had reportedly improved slightly postoperatively, she awoke 3 mo later with bilateral subjective blurriness and new visual field deficits. Magnetic resonance imaging revealed enlargement of the left optic apparatus hemorrhagic lesion, corresponding to residual cavernous malformation. Given the recurrence of hemorrhage and the associated visual symptoms, the patient underwent a redo left frontotemporal craniotomy for resection of the optic nerve and chiasmal lesion. Histopathologic evaluation revealed thick-walled vessels with focal intervening glial tissue, an absence of neoplastic cells, and hemorrhage, consistent with a cavernous malformation. The patient tolerated the procedure well. Postoperatively, she experienced immediate amelioration in her visual symptoms. She was discharged home on postoperative day 3, and her bitemporal visual field deficit continued to progressively improve through her last ophthalmologic appointment 14 mo after surgery. Postoperative and subsequent surveillance neuroimaging demonstrated complete resection of the cavernous malformation without evidence of recurrence. This case demonstrates the techniques utilized to ensure complete resection of the malformation in this very eloquent region. The patient provided consent for publication.

Outcomes of decompressive surgery for cavernous sinus meningiomas: long-term follow-up in 50 patients.

OBJECTIVE: Cavernous sinus meningiomas are complex tumors that offer a perpetual challenge to skull base surgeons. The senior author has employed a management strategy for these lesions aimed at maximizing tumor control while minimizing neurological morbidity. This approach emphasizes combining "safe" tumor resection and direct decompression of the roof and lateral wall of the cavernous sinus as well as the optic nerve. Here, the authors review their experience with the application of this technique for the management of cavernous sinus meningiomas over the past 15 years. METHODS: A retrospective analysis was performed for patients with cavernous sinus meningiomas treated over a 15-year period (2002-2017) with this approach. Patient outcomes, including cranial nerve function, tumor control, and surgical complications were recorded. RESULTS: The authors identified 50 patients who underwent subtotal resection via frontotemporal craniotomy concurrently with decompression of the cavernous sinus and ipsilateral optic nerve. Of these, 25 (50%) underwent adjuvant radiation to the remaining tumor within the cavernous sinus. Patients most commonly presented with a cranial nerve (CN) palsy involving CN III-VI (70%), a visual deficit (62%), headaches (52%), or proptosis (44%). Thirty-five patients had cranial nerve deficits preoperatively. In 52% of these cases, the neuropathy improved postoperatively; it remained stable in 46%; and it worsened in only 2%. Similarly, 97% of preoperative visual deficits either improved or were stable postoperatively. Notably, 12 new cranial nerve deficits occurred postoperatively in 10 patients. Of these, half were transient and ultimately resolved. Finally, radiographic recurrence was noted in 5 patients (10%), with a median time to recurrence of 4.6 years. CONCLUSIONS: The treatment of cavernous sinus meningiomas using surgical decompression with or without adjuvant radiation is an effective oncological strategy, achieving excellent tumor control rates with low risk of neurological morbidity.

Resection of a Neuroenteric Cyst Using a Far Lateral Approach.

This operative video highlights a rare case of a neuroenteric cyst at the ventral craniocervical junction. The case involved a 30-year-old man who initially presented 13 years earlier with acute onset of headache and visual changes. At that time, he was found to have a small, enhancing ventral intradural extramedullary mass at the rostral aspect of C1 thought to be a meningioma. The lesion was managed conservatively, and surveillance imaging tracked its slow progressive enlargement to a size of 1.4 cm ( Fig. 1A, B ). Although he remained asymptomatic, nonurgent elective resection was recommended because of his age and mass progression. The patient underwent a left far lateral approach to the craniocervical junction for resection of the mass. This involved dissection of the suboccipital musculature to expose the C1 transverse process in the suboccipital triangle and ultimately the vertebral artery. After a small craniectomy and C1 hemilaminectomy, the dura was opened and a cystic lesion encountered ( Fig. 2 ). The cystic contents were debulked and the capsule resected. Histopathologic examination revealed abundant goblet cells consistent with a neuroenteric cyst. Dural closure was bolstered with fascia lata and autologous fat graft. Postoperative magnetic resonance imaging (MRI) was consistent with gross total resection ( Fig. 1C, D ). The patient tolerated the procedure well with no new postoperative neurological deficits and was discharged home on postoperative day 2. On completing a 3-day decadron taper, he developed steroid-responsive symptoms consistent with aseptic meningitis, possibly related to cerebrospinal fluid contamination with the cyst contents during resection. The link to the video can be found at: https://youtu.be/SskETPe5PXQ .

Spinal cord stimulation failure: evaluation of factors underlying hardware explantation.

OBJECTIVE: Spinal cord stimulation has been shown to improve pain relief and reduce narcotic analgesic use in cases of complex refractory pain syndromes. However, a subset of patients ultimately undergoes removal of the spinal cord stimulator (SCS) system, presumably because of surgical complications or poor efficacy. This retrospective study addresses the paucity of evidence regarding risk factors and underlying causes of spinal cord stimulation failures that necessitate this explantation. METHODS: In this retrospective single-center review, 129 patients underwent explantation of SCS hardware during a 9-year period (2005-2013) following initial placement at the authors' institution or elsewhere. Medical history, including indication of implantation, device characteristics, revision history, and reported reasons for removal of hardware, were reviewed. RESULTS: The 74 (57%) women and 55 (43%) men were a median of 49 years old (IQR 41-61 years) at explantation; the median time to explantation was 20 months (IQR 7.5-45.5 months). Thoracic or upper lumbar leads were placed in 89.9% of patients primarily for the diagnosis of postsurgical failed-back surgery syndrome (70.5%), chronic regional pain syndrome (14.7%), and neuropathic pain (8.5%). More than half of patients were legally disabled. Initial postoperative reduction in pain was reported in 81% of patients, and 37.8% returned to work. Among 15 patients with acute postsurgical complications (12 infections, 2 hemorrhages, 1 immediate paraplegia), the median time to removal was 2 months. Primary reasons for hardware removal were lack of stimulation efficacy (81%), electrode failure due to migration (14%), and allergic reactions to implanted hardware in 2 patients. The 72 patients who underwent formal psychiatric evaluation before implantation were affected by high rates of major depression (64%), anxiety (34%), posttraumatic stress disorder (PTSD) (12%), drug or alcohol abuse (12%), and physical or sexual abuse (22%). CONCLUSIONS: The authors' findings provide insight regarding the mechanisms of spinal cord stimulation failure that resulted in total removal of the implanted system. The relationship between spinal cord stimulation failure and certain psychiatric disorders, such as PTSD, depression, and anxiety, is highlighted. Ultimately, this work may shed light on potential avenues to reduce morbidity and improve patient outcomes.

Surgical Trapping With Revascularization of Concomitant Cervical and Petrous Internal Carotid Artery Aneurysms: 2-Dimensional Operative Video.

Petrous internal carotid artery (ICA) aneurysms are rare, complicated lesions to treat. The management paradigms include observation, endovascular exclusion, or surgical trapping with or without revascularization. The case described in this video involved a 67-yr-old woman with a known history of chronic lymphocytic leukemia, who presented after a mechanical ground-level fall. Clinically, she had a nasal deformity and resolving epistaxis consistent with mild facial trauma. Computed tomography (CT) revealed a comminuted nasal bone fracture and an incidental 3-cm right petrous ICA aneurysm. Subsequent vascular imaging demonstrated a concurrent 1.5-cm right cervical ICA dissecting pseudoaneurysm. Flow diversion with a Pipeline stent (Medtronic, Dublin, Ireland) was unsuccessful because the aneurysm's size precluded microcatheter selection of the ICA distal to the lesion. When the patient did not tolerate balloon test occlusion of the ICA, we proceeded with surgical trapping of both aneurysms and high-flow extracranial-to-intracranial bypass. The patient underwent a right frontotemporal craniotomy and an external carotid artery-to-frontal M2 middle cerebral artery bypass with a radial artery graft. Following a clinoidectomy, an aneurysm clip was applied to the paraclinoid ICA, and the cervical ICA was ligated just distal to the bifurcation, effectively trapping both aneurysms. The patient tolerated the procedure well. Postoperatively, she experienced symptomatic hypotension requiring vasopressor therapy and a transient partial oculomotor palsy that resolved during her hospital course. She was discharged home without neurological sequelae. Postoperative CT angiography demonstrated complete exclusion of the ICA aneurysms and a patent radial bypass graft after surgery and at 6-month follow-up. The patient provided consent for publication.

Multidisciplinary Approach to Malignant Peripheral Nerve Sheath Tumor of the Trigeminal Nerve: 2-Dimensional Operative Video.

Malignant peripheral nerve sheath tumors (MPNSTs) of the trigeminal nerve are uncommon lesions that pose a surgical challenge to neurosurgeons. The case described in this video involved a 67-yr-old man who presented with a 2-yr history of left-sided facial numbness spreading from his chin along the left mandible to the preauricular area. He also reported left-sided tongue numbness and decreased taste on the left side of the tongue. On examination, he had left-sided facial numbness along the V3 distribution to pinprick and light touch. Magnetic resonance imaging (MRI) revealed an enhancing left V3 lesion extending from Meckel's cave to the angle of the mandible. The patient underwent a left temporal craniotomy for biopsy of the lesion, formalizing the diagnosis of a MPNST of the left trigeminal nerve. A multidisciplinary resection of his lesion was performed. Left infratemporal fossa approach with neck dissection, mandibulectomy, and frontotemporal craniotomy were performed. Additionally, a frontal external ventricular drain was placed for 3 d to aid in CSF diversion to avoid CSF leak, and free-flap reconstruction was undertaken. The patient tolerated the procedure well. Postoperatively, he retained his facial numbness, dysphagia, and dysarthria. The patient was discharged to inpatient rehabilitation on postoperative day 12. Postoperative computed tomography and MRI depicted complete resection of the left trigeminal nerve MPNST. At his last follow-up appointment, 3 mo after surgery, the patient reported significant improvement in his symptoms. Neuroimaging demonstrated no residual tumor and adjuvant radiotherapy was recommended. The patient provided consent for publication.

Upper Lip Tie and Breastfeeding: A Systematic Review.

BACKGROUND: Upper lip tie (ULT) articles have been recorded in Medline since 1998, while "labial frenum" articles have been recorded since 1946. OBJECTIVE: to study the existing medical literature on ULT (or labial frenum or fraenum) as they relate to breastfeeding. MATERIALS AND METHODS: Medline search engine was used to determine and subsequently retrieve all articles published on ULT from 1946 to 2018. Key-words of upper lip tie OR labial frenum were used for the search. We also used Google Scholar and Embase to widen our search, and used the PRISMA criteria for systematic reviews (SRs). Articles were classified as case reports (or series), reviews, editorials (or opinions), cohort studies, clinical trials (nonrandomized), randomized controlled trials (RCT), and SRs according to Medline's own classification. We systematically summarized all articles published to date. RESULTS AND CONCLUSION: No RCT were found, and the evidence for routine ULT release in infants with breastfeeding difficulties is poor. The classification system proposed by Kotlow has not been found reliable both in terms of inter and intraobserver agreement and in terms of predicting the severity of the breastfeeding difficulties.