Combined deficit of the four lower cranial nerves (CN IX, X, XI, and XII) was originally described by French physicians Collet (1915) and Sicard (1917) during World War I. To date though, this rare neurological clinical picture lacks systematic evidence regarding its epidemiology, clinical presentation, treatment strategies, and outcome. We conducted a systematic review and meta-analysis concerning Collet-Sicard syndrome (CSS) on Medline database in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The research yielded 84 articles among which 73 individual case reports were eventually retained. Mean age was 53.7 (± 16) years old and the male-to-female ratio was 1.8/1. CSS was firstly caused by tumors (38.4%), following by vascular etiologies (28.8%), trauma (16.4%), and infection (6.8%), among others. Temporary enteral nutrition was required for 17 patients (23.3%). The four CN presented significant chances of complete or partial recovery: 52.1% for CN IX ( p  < 0.001), 46.6% for CN X and CN XII ( p  < 0.001), and 39.7% for CN XI ( p  = 0.002). Tumoral causes presented significantly lower chances of favorable CN recovery (7.1%) compared to infection (60%), vascular (52.4%), and trauma (41.7%) ( p  < 0.001). Older age (> 53 years old) was not associated with a dismal CN prognostic ( p  = 0.763). Most patients (71.2%) presented a favorable outcome (Glasgow Outcome Scale score ≥ 4). All the patients who died (6.8%) suffered from skull base tumors. CSS is a rare condition requiring prompt clinical and radiologic diagnostic and multidisciplinary management. Vascular or infectious-related CSS seem to present a rather good prognostic, closely followed by trauma, whereas tumoral-related CSS seem to suffer from a more dismal prognostic.

UNASSIGNED: The reconstruction of frontobasal defects following oncologic resections of paranasal and anterior skull base (ASB) malignancies remains challenging. Ineffective reconstruction could lead to cerebrospinal fluid leak, meningitis, and tension pneumocephalus.
UNASSIGNED: Aim of this investigation was to analyse postoperative complication rates with or without bone graft for anterior skull base reconstruction.
UNASSIGNED: In this retrospective study, we included patients following resection of paranasal and/or anterior skull base malignancies between October 2013 and December 2022. Complications were analysed with regards to the type of skull base reconstruction.
UNASSIGNED: Eleven patients were identified (2 female, 9 male, age (median, SD) 64 ± 14.1 years (range 38-81). There were nine cases of paranasal sinus and nasal cavity carcinomas and two cases of olfactory neuroblastomas. Overall survival was 22.5 ± 28 months (range: 5-78), progression free survival was 17.0 ± 20.3 months (range: 11-78). Bone skull base reconstruction using a split graft was performed in three cases. Postoperative complications requiring surgical intervention were seen in 33% (one tension pneumocephalus) of cases in the bone reconstruction group and 50% (three patients with cerebrospinal fluid leak, one infection) in the non-bone reconstruction group.
UNASSIGNED: The structural reinforcement of structural bone chip grafting might provide additional support of the ASB and prevent CSF leakage or encephalocele. Especially in large (>10 cm2) bone defects of advanced sinonasal malignancies extending into the middle cranial fossa, the full armamentarium of reconstruction possibilities should be considered.

BACKGROUND: Pediatric skull base lesions occur rarely and are of various etiologies. Traditionally, open craniotomy has been the treatment of choice; however, nowadays, endoscopic approaches are increasingly applied. In this retrospective case series, we describe our experience in treating pediatric skull base lesions and provide a systematic overview of the literature on the treatment and outcome of pediatric skull base lesions.
METHODS: We conducted a retrospective data collection of all pediatric patients (<18 years) treated for a skull base lesion at the Division of Pediatric Neurosurgery, University Children\'s Hospital Basel, Switzerland, between 2015 and 2021. Descriptive statistics and a systematic review of the available literature were additionally conducted.
RESULTS: We included 17 patients with a mean age of 8.92 (±5.76) years and nine males (52.9%). The most common entity was sellar pathologies (n = 8 47.1%), with craniopharyngioma being the most common pathology (n = 4, 23.5%). Endoscopic approaches, either endonasal transsphenoidal or transventricular, were used in nine (52.9%) cases. Six patients (35.3%) suffered from transient postoperative complications, while in none of the patients these were permanent. Of the nine (52.9%) patients with preoperative deficits, two (11.8%) showed complete recovery and one (5.9%) partial recovery after surgery. After screening 363 articles, we included 16 studies with a total of 807 patients for the systematic review. The most common pathology reported in the literature confirmed our finding of craniopharyngioma (n = 142, 18.0%). The mean PFS amongst all the studies included was 37.73 (95% CI [36.2, 39.2]) months, and the overall weighted complication rate was 40% (95% CI [0.28 to 0.53] with a permanent complication rate of 15% (95% CI [0.08 to 0.27]. Only one study reported an overall survival of their cohort of 68% at five years.
CONCLUSIONS: This study highlights the rarity and heterogeneity of skull base lesions in the pediatric population. While these pathologies are often benign, achieving GTR is challenging due to the deep localization of the lesions and eloquent adjacent structures, leading to high complication rates. Therefore, skull base lesions in children require an experienced multidisciplinary team to provide optimal care.

UNASSIGNED: Intracranial neurenteric cysts (NCs) are extremely rare tumors that more commonly involve the posterior fossa than any other cranial part. While transcranial skull base surgery has been the mainstay of treatment, the utility of endoscopic transnasal surgery (ETS) remains to be established.
UNASSIGNED: We report a case of a large posterior fossa NC extensively involving the suprasellar region, cerebellopontine angle, and prepontine cistern, which we successfully resected with ETS through a combination of transtubercular and transclival routes. Before surgery, the patient presented with abducens nerve and pseudobulbar palsies, which resolved within 2 weeks postoperatively. The patient remained free from recurrence for 3 years postoperatively.
UNASSIGNED: Extended ETS may offer a minimally invasive option for the posterior fossa NC, extensively occupying the ventral space of the brainstem.

Osteoblastoma of the skull is a rare entity, and they account only for 2-4% of all the cases of osteoblastoma. We perform a comprehensive review of the pertinent literature on the subject and we report a case of a 3-year-old girl presenting with a 6-month history of a supraorbital mass and exophthalmos due to an osteoblastoma of the frontal and ethmoid bones involving the orbit and anterior skull base. A 3D printed model of the patient\'s skull was used for the preoperative planning and reconstruction strategy. Total en-bloc resection of the tumor followed by immediate reconstruction was achieved. No recurrence was detected 3 years after the surgery. Gross total resection is strongly advised with skull osteoblastoma, especially in young age, because of the risk of the recurrence and malignant transformation. 3D printing is proven to be a valuable tool to enhance surgical performance by avoiding complications while achieving total resection with accurate reconstruction. Long-term follow-up is important to detect recurrences and improve the management of these young patients.

BACKGROUND: Trigeminal schwannomas are rare tumors of the trigeminal nerve. Depending on the location, from which they arise along the trigeminal nerve, these tumors can present with a variety of symptoms that include, but are not limited to, changes in facial sensation, weakness of the masticatory muscles, and facial pain.
METHODS: We present a case of a 16-year-old boy with an atypical presentation of a large trigeminal schwannoma: painless malocclusion and unilateral masticatory weakness. This case is the first documented instance; to the best of our knowledge, in which a trigeminal schwannoma has led to underbite malocclusion; it is the 19th documented case of unilateral trigeminal motor neuropathy of any etiology. We discuss this case as a unique presentation of this pathology, and the relevant anatomy implicated in clinical examination aid in further understanding trigeminal nerve pathology.
CONCLUSIONS: We believe our patient\'s underbite malocclusion occurred secondary to his trigeminal schwannoma, resulting in associated atrophy and weakness of the muscles innervated by the mandibular branch of the trigeminal nerve. Furthermore, understanding the trigeminal nerve anatomy is crucial in localizing lesions of the trigeminal nerve.

Introduction: Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a small round blue cell tumor of nasal neuroepithelium first described in 1924. Though this tumor is especially rare in the pediatric population with an incidence of <0.1 per 100,000, it is the most common pediatric nasal cavity neoplasm. The purpose of this systematic review is to examine the treatment modalities utilized for pediatric esthesioneuroblastoma and overall survival. Methods: A systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Pubmed, EMBASE, and Ovid MEDLINE databases were queried for studies pertinent to treatment modalities for pediatric esthesioneuroblatoma and survival outcomes. Results: Two hundred and seventy-sixth articles were identified, with seven meeting inclusion criteria. Ninety-four patients with an age range of 0.9-21 years old with esthesioneuroblastoma were included. Nearly 90% of patients were of stage Kadish B or C at time of presentation, while 20% presented with cervical lymphadenopathy. Only about 10% of patients underwent single modality therapy. Overall, 5-year survival ranged from 44 to 91% with a median follow-up of 3-13 years. Conclusion: Children with esthesioneuroblastoma usually present at an advanced stage and undergo multi-modality therapy at a higher rate than adult patients. There is a wide range of documented overall survival though this lack of precision could be due to a paucity of patients.

BACKGROUND: Perivascular epithelioid cell tumors (PEComas) of the skull base are extremely rare. Here we report the first description of a malignant PEComa mimicking jugular foramen schwannoma and presenting as Collet-Sicard syndrome, and we review the previous literature on PEComas of the head, neck and skull base.
METHODS: A 29-year-old woman presented with hoarseness, dysphagia, vomiting, and headache. She was first diagnosed with Collet-Sicard syndrome caused by thrombosis of the sigmoid and transverse sinuses. She was treated with anticoagulant therapy, and the hoarseness and paralysis of the accessory nerve improved. Later, at age 31, the hoarseness again worsened. At another hospital, enhanced computed tomography revealed a tumor in the jugular foramen extending to the neck and medially displacing the internal carotid artery. She was referred to our hospital for further examination and was diagnosed with jugular foramen schwannoma causing thrombosis of the sinuses. At the one-year follow-up, the tumor had grown rapidly and had started to surround the internal carotid artery. We therefore performed a tissue biopsy of the tumor in the jugular foramen and neck. Based on pathological analysis, we made a definitive diagnosis of malignant PEComa.
CONCLUSIONS: It may be extremely challenging to reach an accurate diagnosis of PEComa in the skull-base region, which can cause a delay in treatment initiation. When atypical clinical features for a skull-base tumor are found, we recommend preliminary biopsy to obtain a definitive diagnosis and initiate an appropriate treatment strategy as early as possible.

OBJECTIVE: To review the impact of a weekly multidisciplinary neuroradiology imaging review on the management of patients undergoing radiotherapy.
METHODS: A prospective study of the management of 118 patients (30=head and neck, 40=skull base, central nervous system=48) was conducted over a 12-month period from January 2018 through January 2019. After review of each patient\'s history and relevant imaging, a radiation oncologist completed a form detailing the changes that were made in diagnosis and management. Imaging source (external and internal examinations), availability of outside reports, report timeliness, the value of reports, changes in interpretation, changes in clinical management, and changes in prognosis were documented. Changes in interpretation and management were designated as major or minor depending on the significance of the change. The managing radiation oncologist indicated whether the imaging review conference substituted for a peer-to-peer consultation with a neuroradiologist.
RESULTS: Nearly half (47%) of all patients had a change in interpretation. Of those, 32% of patients had a major change in interpretation, while 14% had a minor change in interpretation. The existence of the multidisciplinary imaging review conference prevented a peer-to-peer consultation (interruption) by the radiation oncologists to the neuroradiologists in 90% of the cases presented. Further analysis was performed.
CONCLUSIONS: The involvement of neuroradiologists in a joint radiation oncology imaging review conference resulted in changes in diagnostic imaging interpretation that led to significant changes in management, expected prognosis, and workflow.

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