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The management of patients with Apert syndrome (AS) is complex and reflects the multisystem disease as a result of premature fusion of cranial vault, cranial base and midface sutures as well as extremity anomalies characterised by syndactyly. Early cranial sutural fusion results in craniocerebral disproportion which can lead to crisis surgical intervention due to raised intracranial pressure, ophthalmic and compromised airway concerns. Childhood inventions are often determined by psychosocial concerns and adult surgical interventions are often determined by cosmetic concerns. Treatments are provided by many different specialists within multidisciplinary teams (MDT). The treatment pathway extends from birth well into adulthood and is often associated with a heavy burden of care. Due to the extensive nature of the interaction with these patients MDT members have opportunities to provide enhanced patient-centred care and support.This case report provides an overview of the current knowledge of the aetiology of AS, illustrates the pathway of surgical and non-surgical management of AS and provides a long-term review of the dentofacial treatment outcomes.By having a better understanding of the impact of AS and treatment provided, MDT members can not only provide improved clinical treatment but also offer improved patient experiences for those with craniofacial anomalies, in particular, an increased awareness of the psychosocial challenges they endure.

Report the incidence of and treatment patterns for facial nerve palsy after skull base fracture.Retrospective cohort study.IBM MarketScan Commercial Database (2006-2019).Human subjects with skull base fracture, per International Classification of Diseases-9th and 10th Revisions-Clinical Modification diagnosis codes.The primary outcomes were the incidence and median time to facial nerve palsy diagnosis within 30 days of skull base fracture. Secondary outcomes were treatments (corticosteroids, antivirals, facial nerve decompression, botulinum toxin, and facial reanimation), demographics, and rates of hearing loss, vertigo, tympanic membrane rupture, cerebrospinal fluid leak, comorbidities, and loss of consciousness.The 30-day incidence of facial nerve palsy after skull base trauma was 1.0% (738 of 72,273 patients). The median (95% confidence interval [CI]) time to diagnosis was 6 (6-7) days, and only 22.9% were diagnosed within 1 day. There were significantly higher rates (risk difference, 95% CI) of hearing loss (26%, 22-29%), tympanic membrane rupture (6.3%, 4.5-8.1%), cerebrospinal fluid leak (6.4%, 4.5-8.3%), comorbidity (14%, 10.4-17.6%), and loss of consciousness (24.3%, 20.7-27.9%). Loss of consciousness was associated with longer median (95% CI) time to facial nerve palsy diagnosis: 10 (9-10) days. Corticosteroids were the most common treatment but only reported for less than one-third of patients. Only eight patients underwent facial nerve decompression.Facial nerve palsy after skull base fracture is associated with higher comorbidity, and the diagnosis is often delayed. Few patients were treated with surgery, and there are inconsistencies in the types and timing of treatments.

We describe the case of a 32-year-old man from Cape Verde having headache and increasing visual loss. Clinical and radiological investigations disclosed a massive destructive lesion involving the anterior and central skull base, orbit and nasoethmoid region initially interpreted as a malignant small round cell tumour. Surgical biopsies were negative for neoplasm, showing an intense inflammatory infiltrate together with fungus, later characterised as Aspergillus flavus spp. The patient was immunocompetent with no evidence of congenital or acquired immunodeficiencies. Invasive fungal infections in immunocompetent patients are rare and can be a diagnostic challenge. The best diagnostic clues include the patient’s origin from tropical climates, imaging features and the identification of fungal hyphae on pathology specimens. Although a devastating disease in immunocompromised patients, craniocerebral aspergillosis in immunocompetent patients carries a better prognosis. Available literature supports the combined used of ‘conservative’ surgical resection and antifungal therapy as the best treatment option.

Background The pseudoaneurysms of the internal carotid artery (ICA) at the skull base form a unique subset of craniofacial pseudoaneurysms with varied diagnostic and therapeutic challenges. Recurrence in a surgically treated pseudoaneurysm may become a nightmare due to very limited number of open and endovascular options. Report We report a rare case of recurrent pseudoaneurysm of petrous ICA, which presented with massive epistaxis following an initial successful occlusion by surgical trapping of the parent arterial segment with surgical clip. Cerebral angiography revealed filling of the pseudoaneurysm by small arterial channels from the external carotid, contralateral internal carotid and basilar arterial branches and emptying through the “slipped” distal clip. After a meticulous analysis of the cerebral angiogram, the recurrent pseudoaneurysm was eventually embolized retrogradely through the “slipped” clip after crossing the anterior communicating artery taking a contralateral internal carotid access. Conclusion Scrupulous planning and execution of ICA pseudoaneurysms is necessary to prevent recurrence. Naturally occuring collateral routes through the Circle of Willis aid in treatment of “unreachable” vascular lesions.

Mucormycosis is a very commonly encountered disease in the sinonasal region in patients with diabetes and immunocompromised status specially in the northern part of the Indian continent. Due to its fulminant nature and involvement in the rhino-orbital-cerebral region, prognosis is poor even after extensive debridement and amphotericin-B therapy. We present a case with diagnosis of sarcoidosis being treated with systemic steroids who developed sudden-onset ptosis and left lateral rectus palsy. On radiological evaluation with MRI, a heterogenously peripherally enhancing lesion was seen in the nasopharynx suggestive of nasopharyngeal abscess. The patient was taken up for emergency surgery; a necrotic lesion with destruction of sphenoid bone, pterygoids and clivus was seen originating in the nasopharynx. The lesion on fungal staining came out to be broad aseptate hyphae suggestive of mucormycosis.

Background: Cochlear implantation (CI) following endoscopic transcanal infrapromontorial vestibular schwannoma (VS) dissection is a feasible intervention in intracanalicular VS, with minimal extension into the cerebellopontine angle, but no audiologic results have ever been reported in the literature. Methods: From 2015 to 2021 in the Otorhynolaryngology Departments of Modena and Verona, three patients underwent this intervention. All were suffering from sporadic left-sided intracanalicular Koos I VS. Intraoperative electrically evoked auditory brainstem responses and electrophysiological measurements were performed before and after the placement of the electrode array, respectively. Since device activation one month after the surgery, each patient was followed up with audiometric tests, data logging, electrode impedance measurements and neural response telemetry performed at each scheduled fitting session at 15 days and 3, 6, 12 and 24 months. Results: Only in patient No. 3, an auditory benefit was observed and still evident even 36 months after activation. Impedances increased progressively in patient No. 1 and a benefit was never reported. Patient No. 2 left the follow-up for worsening comorbidities. Conclusions: CI following transcanal infrapromontorial VS resection is a beneficial intervention. The residual cochlear nerve after the tumour dissection and the course of electrophysiological measurements in the postoperative period were the main predictive factors for audiological outcomes.

Transcranial removal of the anterior clinoid process (ACP) provides access to the clinoidal segment of the internal carotid artery as well as superolateral decompression of the optic canal. Endoscopic endonasal approaches (EEAs) can access the entire medial and inferior portions of the optic canal, but no data exist to support what proportion of the anterior clinoid could be safely resected via an EEA.A cadaveric anatomical study was performed with removal of the ACP in 3 major steps in order of difficulty and risk. At each step, the removal was stopped when no more bone of the ACP could be seen without traction on neural structures. After each step, a CT scan was performed to allow volumetric measurement of the remaining ACP.Twenty ACPs in 10 cadaveric heads were removed to various degrees using the described stepwise technique. The mean portion of the ACP resected by each step was 21%, 46%, and 27%, respectively. Cumulated ACP removal at the end of step 3 was 94%, with complete removal achieved in 35% of the specimens.Using the safe route above the optic canal, removal of 21% of the ACP can be achieved via EEA. Although substantially more of the ACP can be drilled by accessing the optic strut, the benefits of pursuing additional removal must be weighed against the significant risks of drilling in this narrow corridor bordered by the internal carotid artery, the third cranial nerve, and the optic nerve.

The functional outcome, quality of life, and patient feedback related to a chosen treatment approach in skull base surgery have become a subject of interest and focused research in recent years. The current advances in endoscopic optical imaging technology and surgical precision have radically lowered the perioperative morbidity associated with skull base surgery. This has pushed toward a higher focus on patient-reported outcomes (PROs). It is now critical to ensure that the offered treatment plan and approach align with the patient's preferences and expectations, in addition to the surgeon's best clinical judgment and experience. PROs represent a view that reflects the patient's own thoughts and perspective on their condition and the management options, without input or interpretations from the surgeon. Having PRO data enables patients the opportunity to learn from the experiences and perspectives of other patients. This input empowers the patient to become an active participant in the decision-making process at different stages of their care. An in-depth PRO evaluation requires specific validated tools and scoring systems, namely the patient-reported outcomes measures (PROM) tools. In this review, we discuss the currently available skull-base-related PROs, the assessment tools used to capture them, and the future trends of this important topic that is in its infancy.

BACKGROUND: Multi Fractionated stereotactic radiosurgery (MF-SRS) of Linac has an essential role in the treatment of skull base meningiomas (sbMNG). However, Gamma Knife Icon (GK) allows MF-SRS using mask immobilization with onboard image guidance. OBJECTIVE: This dosimetric study aims to investigate whether equivalent plan quality can be achieved with Volumetric Modulated Arc Therapy (VMAT) in patients with large sbMNG (>10 cm3) previously treated with GK. METHODS: Twenty patients with the median target volume of 19.7cm3 are re-planned by using VMAT with 20 Gy in 5 fractions. Plan qualities are compared to tumor coverage, paddick conformity index (PCI), gradient index (GI), V4 Gy, V10 Gy, V12 Gy, optic chiasm V20 Gy, brainstem V23 Gy, optic nerve V25 Gy volumes, and maximum doses for all. Additionally, beam-on time and approximate planning time are also analyzed and compared. RESULTS: All plans provide adequate clinical requirements. First, the CI is comparable for the GK and VMAT (0.99±0.01 vs. 1.13±0.20; p = 0.18). Second, VMAT has a significantly higher GI than GK (3.81±0.35 vs. 2.63±0.09; p

A thorough observation of the root exit zone (REZ) and secure transposition of the offending arteries is crucial for a successful microvascular decompression (MVD) for hemifacial spasm (HFS). Decompression procedures are not always feasible in a narrow operative field through a retrosigmoid approach. In such instances, extending the craniectomy laterally is useful in accomplishing the procedure safely. This study aims to introduce the benefits of a skull base approach in MVD for HFS.The skull base approach was performed in twenty-eight patients among 335 consecutive MVDs for HFS. The site of the neurovascular compression (NVC), the size of the flocculus, and the location of the sigmoid sinus are measured factors in the imaging studies. The indication for a skull base approach is evaluated and verified retrospectively in comparison with the conventional retrosigmoid approach. Operative outcomes and long-term results were analyzed retrospectively.The extended retrosigmoid approach was used for 27 patients and the retrolabyrinthine presigmoid approach was used in one patient. The measurement value including the site of NVC, the size of the flocculus, and the location of the sigmoid sinus represents well the indication of the skull base approach, which is significantly different from the conventional retrosigmoid approach. The skull base approach is useful for patients with medially located NVC, a large flocculus, or repeat MVD cases. The long-term result demonstrated favorable outcomes in patients with the skull base approach applied.Preoperative evaluation for lateral expansion of the craniectomy contributes to a safe and secure MVD.

Cerebrospinal fluid (CSF) leakage is a major complication following endoscopic endonasal skull base surgery. Various skull base reconstruction methods are available, and the use of a vascularized nasoseptal flap (NSF) in skull base reconstruction has greatly contributed to a decrease in the CSF leak rate. A balloon catheter such as a sinus balloon or a Foley catheter is often used to support an NSF; however, in cases wherein nasal and/or paranasal structures supporting the balloon are lacking following the surgery, the NSF is not properly fixed and postoperative CSF leak may occur. Here we introduce a new technique of using multiple-balloon catheters to fix an NSF in such cases and provide the results of our analysis of the new technique's efficacy. Eight patients who underwent endonasal endoscopic surgery for the following cases were included: olfactory neuroblastoma (n = 6), recurrent craniofacial meningioma (n = 1), and recurrent chordoma (n = 1). After tumor resection, multilayered reconstruction with vascularized NSF was performed. Given that the Foley catheter was not stable to fix the flap in each case, we used an additional nasal catheter to support the Foley catheter. No complications such as postoperative CSF leak and necrosis of the vascularized flap were observed. These results suggest that the multiple-balloon catheter technique is a useful method for fixing the NSF to the skull base even when nasal cavity structures are missing due to surgical removal.

The management of base of skull (BS) chordomas is a neurosurgical conundrum owing to their close proximity to the critical neurovascular structures. Surgical resection is the gold standard treatment followed by adjuvant radiotherapy which includes photon therapy, proton beam therapy (PBT), gamma knife radiosurgery, etc. PBT has become an unparalleled therapeutic modality in the management of BS chordomas. The aim of this systematic review was to assess the outcomes in BS chordoma patients who received PBT as a primary or adjuvant therapy. PubMed and Cochrane databases were screened till May 2022. Following the PRISMA guidelines, studies were reviewed thoroughly, and the data of the included study was extracted. Statistical analysis was performed using the SAS 9.4 with P value .05 considered as significant. Sixteen studies with 752 patients were included. The majority of the patients were adults ( 18 years) with a male:female ratio of 1.2. The most common clinical features were cranial nerve (3rd, 6th, or 12th) palsy and hearing impairment. Ninety-five percent of the patients underwent surgical resection before PBT. The mean PBT dose received was 74.02 cGe (cobalt gray equivalent). Eighty percent of the patients showed a positive response to the therapy defined in terms of tumor regression. Five-year local control (LC), overall survival (OS), and progression-free survival (PFS) were calculated as 76.6%, 79.6%, and 89%, respectively. Statistical analysis revealed none of the factors had any significant association with 5-year LC. PBT is a growing therapeutic technique that has revolutionized the treatment of BS chordomas.

To evaluate the influence of facility case volume and type on skull base chordoma treatment and overall survival (OS).The 2004-2016 National Cancer Database was queried for skull base chordoma patients receiving definitive treatment. Facilities were categorized into 2 cohorts by calculating the mean number of patients treated per facility and using cutoff numbers that were 0.5 SD above and below the computed mean to separate the groups. As, by definition of the inclusion criteria, all included facilities treated at least 1 patient, low-volume facilities were defined as treating 1 patient, and high-volume facilities were defined as treating ≥7 patients; mid-volume facilities (facilities treating ≥2 but ≤6 patients) were excluded. Differences in treatment course, outcomes, and OS by facility type were assessed.The study included 658 patients (44.8% female, 79.5% White). The 187 unique facilities were categorized into 95 low-volume facilities (treating 1 patient during timeline) and 26 high-volume facilities (treating ≥7 patients during timeline). Kaplan-Meier log-rank analysis demonstrated a significant positive association between facility volume and OS (P0.001) and an improvement in OS in patients at academic facilities (P = 0.018). On Cox proportional hazards multivariate regression after adjusting for sex, age, Charlson-Deyo comorbidity index, and insurance type, high-volume facilities and academic facilities were associated with a lower mortality risk than low-volume facilities and nonacademic facilities (P0.001 and P = 0.03, respectively).Higher facility case volume and academic facility type appear to be associated with improved survival outcomes in treatment of skull base chordomas.

Radical resection of complex lesions occupying multiple compartments at the central skull base remains a significant challenge, since surgical outcomes may be compromised by insufficient exposure and inappropriate techniques. However, the efficiency of the maxillary swing approach for these lesions has not been sufficiently evaluated. Careful assessment of lesion characteristics must be performed when selecting the appropriate procedure.Between May 2006 and February 2017, 17 patients underwent resection of extensive lesions in the central skull base using the maxillary swing approach. As shown in the representative cases, data regarding clinical findings and technical considerations were reviewed.Complete resection was achieved in all patients. The pathological findings were diverse, and the majority were schwannomas (9 cases, 52.94%), followed by meningiomas (World Health Organization II) (3 cases, 17.65%). Complications were managed as described in the case illustrations, and symptoms improved with time. The follow-up duration ranged from 62 to 192 months (median, 114 months), while 2 patients were lost to the follow-up. No mortality was observed. Two patients who experienced malignancy relapse were still under observation due to their asymptomatic status.Our preliminary results suggest that the maxillary swing approach can be an alternative option for managing extreme cases, such as large, extensive, hypervascularized masses with fibrous or calcified consistency, or for recurrent lesions in the central skull base. En bloc resection can be successfully obtained, resulting in long-term local control.

This section reviews the selection criteria and best practices for endoscopic cerebrovascular surgery, focusing on purely endoscopic endonasal approaches. Although these approaches still play a limited role in open vascular neurosurgery, they offer a robust and potentially safer technique for establishing visualization and vascular control of particular, well-selected pathologies, such as aneurysms of the proximal circulation; this requires strong, multidisciplinary experience with endoscopic anatomy and surgical technique, advanced reconstruction techniques, and instruments designed to be maneuvered within this relatively novel corridor and application.

Mastoidectomy involves drilling the temporal bone while avoiding the facial nerve, semicircular canals, sigmoid sinus, and tegmen. Optical topographic imaging (OTI) is a novel registration technique that allows rapid registration with minimal navigational error. To date, no studies have examined the use of OTI in skull-base procedures.In this cadaveric study, 8 mastoidectomies were performed in 2 groups-4 free-hand (FH) and 4 OTI-assisted mastoidectomies. Registration accuracy for OTI navigation was quantified with root mean square (RMS) and target registration error (TRE). Procedural time, percent of mastoid resected, and the proximity of the mastoidectomy cavity to critical structures were determined.The average RMS and TRE associated with OTI-based registration were 1.44 mm (±0.83 mm) and 2.17 mm (±0.89 mm), respectively. The volume removed, expressed as a percentage of the total mastoid volume, was 37.5% (±10.2%) versus 31.2% (±2.3%), P = 0.31, for FH and OTI-assisted mastoidectomy. There were no statistically significant differences between FH and OTI-assisted mastoidectomies with respect to proximity to critical structures or procedural time.This work is the first examining the application of OTI neuronavigation in lateral skull-base procedures. This pilot study revealed the RMS and TRE for OTI-based navigation in the lateral skull base are 1.44 mm (±0.83 mm) and 2.17 mm (±0.89 mm), respectively. This pilot study demonstrates that an OTI-based system is sufficiently accurate and may address barriers to widespread adoption of navigation for lateral skull-base procedures.

To identify factors associated with skull base involvement (SBI) of maxillary ameloblastomas (MA).This retrospective cohort study was composed of MA patients treated during a 7-year period. Demographic, radiographic, and nine immunohistopathologic predictor variables were included. The outcome variable was presence of SBI (yes/no). Descriptive, bi- and multivariate statistics were computed, and P ≤ .05 in multivariate analyses was considered statistically significant.The sample comprised 23 subjects (34.8% females; 21.7% with SBI) with a mean age of 50.3 ± 18.2 years. Candidate predictors of an SBI in MAs were 1) male gender, 2) a low Karnofsky Performance Status score (KPS), 3) multilocular radiolucency, 4) ill-defined margins, 5) cortical perforation, 6) inclusion of an unerupted tooth, 7) moderate to strong reactivity to p53, Ki-67, CD10, astrocyte elevated gene-1 (AEG-1) protein, carbonic anhydrase IX (CA IX), calretinin (calbindin2; CALB2), and BRAF-V600E, and 8) negative to low immunopositivity to α-smooth muscle actin (α-SMA) and syndecan-1 (CD138). However, multivariate analyses confirmed the significant associations of SBI with negative/low syndecan-1 reactivity (P = .003; adjusted odds ratio [ORsubadj./sub], 4.04; 95% confidence interval [95% CI], -.89 to -.48; Pearson's Correlation Coefficient [r] = -.74) and with KPS (P = .003; ORsubadj./sub, 4.04; 95% CI, -.78 to -.17; r = -.54) only.Our findings suggest an aggressive approach to MAs with negative to low syndecan-1 immunopositivity and/or in multi-morbid patients (who may have difficulty in access to health care). Otherwise, health care inequalities due to low KPS scores should be minimized or eliminated.

A defect in the skull base can result in leakage of cerebrospinal fluid (CSF) out of the subarachnoid space into the sinonasal cavities, termed CSF rhinorrhea. Patients presenting with CSF rhinorrhea often require surgical repair, typically performed endoscopically. Successful surgical intervention is highly reliant on accurate identification of the leak site. Identification can be enhanced by the administration of intrathecal fluorescein (IF) via a lumbar drain before surgery. The objective of this systematic review is to better characterize the benefits and limitations of IF administration.A systematic search was conducted for literature documenting the use of IF for CSF leak localization. The results of this search were subjected to initial review, followed by full-text evaluation of selected texts, and final inclusion based on predetermined selection criteria. Primary outcomes were specificity and sensitivity of IF administration in identifying CSF leak sites. Secondary outcomes included administration technique and safety data.A total of 25 studies representing 3801 instances of IF administration met inclusion criteria. When injected slowly for up to 30 minutes at doses of50 mg, IF administration results in minimal complications. A total of 25 mg doses (92.36%) of IF appear more sensitive than 10 mg (71.88%) doses of IF while maintaining a similar safety profile. Mixing fluorescein with double distilled water instead of CSF or saline may decrease waiting times before fluorescein can be identified and increase its safety profile.Intrathecal fluorescein may play an important role in the identification of CSF leak site during endoscopic repair.

Objective/hypothesisTo investigate the clinical features, management strategies and outcomes for patients with metastatic primary skull base chordomas.Study designSystematic review.MethodsA systematic search through Pubmed/Medline, Web of Science, and EBSCOhost (CINAHL) was conducted without restriction on dates. After study screening and full-text assessment, two authors independently extracted all data using a pre-established abstraction form.ResultsForty cases were included from 38 studies. The average age (standard deviation [SD]) of the sample at presentation was 28.5 (23.3) and was equally distributed across genders. The average time (SD) between initial diagnosis to local recurrence was 40.1 (60.3) months. The average time (SD) from primary tumor detection to the diagnosis of metastatic disease was 55.2 (49.0) months. The most common subsite for metastatic spread were the lungs (32.5%). Of the 33 patients with data on outcomes, 48.5% were found to have expired by the time of publication. The median overall survival was estimated to be 84 months (95% confidence interval [CI] 62.3-105.7).ConclusionsThe most common subsites for metastatic spread of skull base chordoma were the lungs and bone. Overall survival for patients in the current cohort was a median of 84 months, with no significant differences noted when stratifying by the extent of surgery or the site of metastases.Level of evidence3a.

(1) Background: The normal A1 segment of the anterior cerebral artery (ACA) has a supraoptic course. The proximal infraoptic course of an A1 segment leaving the internal carotid artery (ICA) near the origin of the ophthalmic artery is a rare possibility. This study aimed to determine the prevalence and detailed anatomy of infraoptic A1 segments. (2) Methods: We retrospectively studied 145 computed tomography angiograms from 92 male and 53 female cases, with ages varying from 61 to 78 y.o. (3) Results: In 21/145 cases, infraoptic or infrachiasmatic horizontal-medial courses of A1 segments that emerged distally from the ICA were found. Distal infraoptic A1 segments were bilateral in 16/145 cases and unilateral in 3/145 cases. Infrachiasmatic A1 segments were found bilaterally in 2/145 male cases. All the infraoptic/infrachiasmatic A1 segments left long ICAs with low bifurcations. In 7/34 sides with distal infraoptic or infrachiasmatic A1 segments, supracarotid courses were present. In one female, the right A1 segment had an anterior supraclinoid, supracarotid and infraoptic course. In two female cases with a bilateral distal infraoptic A1, the segment was almost contacting the respective posterior cerebral artery. (4) Conclusions: In cases with dolicho(ectatic) ICAs, the A1 segments could have infraoptic and supracarotid courses the neurosurgeons should be aware of.

Chondrosarcomas of the sinonasal cavity and skull base are uncommon malignancies. We sought to provide long-term outcomes at two tertiary care centers.Patients with chondrosarcoma treated between 2000 and 2021 were included. The primary outcomes were overall survival (OS) and disease-specific survival (DSS).Thirty-eight patients met inclusion criteria. Fourteen patients had sinonasal (36.8%), 7 petroclival (18.4%), and 17 other primary skull base lesions (44.7%). Twenty-eight patients (73.7%) underwent radiation with an average dose of 67.3 ± 15.1 Gy. Eighteen patients (47.4%) required revision surgery for recurrence. 1, 5, and 10-year OS were 97.3%, 93.1%, and 74.7%. DSS at 5- and 10-year survival was 95.7%. Adjuvant radiation was associated with improved OS (HR: 0.12; 95% CI: 0.02-0.75, p = 0.023).We present our experience over the last 20 years treating chondrosarcomas. Favorable survival outcomes can be achieved but recurrence requiring repeat resection is common.

The prevalence of increased intra-cranial pressure (ICP) in patients with scaphocephaly is controversial. Here, based on anthropological material, we aimed to determine whether adults with non-operated sagittal synostosis show indirect signs of increased ICP.Thirty-eight dry skulls (21 skulls with sagittal craniosynostosis and 17 controls) were selected from the collections of the National Museum of Natural History (Paris, France). All skulls registered as 'fused sagittal suture' or 'scaphocephaly' in the registry of the Museum were included. All had total fusion of the sagittal suture. Controls were selected within skulls of similar origin (France), without visible craniofacial anomalies. The 38 skulls were CT-scanned using a standard medical CT-scan with a protocol dedicated to dry bone imaging. Eight radiological signs associated with raised ICP were assessed: (1) calvaria and (2) skull base thinning, (3) dorsum sellae erosion, (4) sella turcica lengthening, (5) copper beaten skull, (6) suture diastasis, (7) persistent metopic suture, and (8) small frontal sinus. Scaphocephaly was assessed based on head circumference, cranial index, intra-cranial volume, fronto-nasal angle, and inter-zygomatic distance. Linear and non-linear logistic models were used to compare groups.19/21 skulls with sagittal synostosis were significantly scaphocephalic. None of the criteria for ICP were significantly different in skulls with scaphocephaly relative to controls. Nevertheless, 5 individual skulls with scaphocephaly had ≥ 3 signs in favor of a history of raised ICP. We do not report the significant prevalence of indirect signs of raised ICP in adults with scaphocephaly. These results do not allow ruling out a history of early raised ICP or of minor prolonged raised ICP. Even though our findings support the fact that scaphocephaly is not significantly associated with prolonged raised ICP, individual cases (5/21) with clear signs in favor of a history of brain compression indicate that scaphocephaly correction should be considered as a functional procedure until the production of clear evidence. Cognitive assessments of non-operated adult patients with scaphocephaly could contribute to tackle this recurring question in craniofacial surgery.

Background Endoscopic endonasal skull base surgery (EESBS) is performed through a non-sterile corridor. Intracranial infection rates in a pediatric population have not been extensively studied and the exact effect of antibiotic prophylaxis on rates of infection after EESBS in pediatric patients remains unclear. Objective The purpose of our study is to investigate risk factors for postoperative intracranial infection during EESBS in a pediatric population and help elucidate the role of antibiotic prophylaxis. Methods We conducted a retrospective chart review of 99 consecutive patients under the age of 18 who underwent EESBS at our institution from 2013 to 2021. Centers for Disease Control and Prevention/National Healthcare Safety Network criteria for diagnosis of meningitis were used to identify postoperative intracranial infections. Results The average age was 12.3 years (range 1.6-18) with 66 male patients and 33 female patients. 49 patients had an intraoperative cerebrospinal fluid (CSF) leak, of which 4 had a postoperative CSF leak. We identified 3 postoperative intracranial infections (3%), which were all meningitis cases. The infection rate was 6% (3/49) among those with intraoperative CSF leaks. All patients with meningitis had a postoperative CSF leak. All infections were transclival approaches (2 chordoma and 1 neurenteric cyst). Conclusion This investigation represents one of the largest pediatric endoscopic skull base surgery cohorts. EESBS is safe to perform in pediatric populations, but transclival approaches and postoperative CSF leaks are risk factors for postoperative meningitis.

Anterior skull base defects present a unique reconstructive challenge. Risk factors such as previous operation, radiation and infection require a multidisciplinary approach to create a stable wound. Reconstructive microsurgery serves an important role when attempts with pericranial flap placement and nonvascularized graft obliteration fail. Our series analyzes the outcomes of 5 patients who underwent vascularized reconstruction of craniofacial defects at Northwestern Memorial Hospital from 2014-2021. Each patient presented with a complex, hostile scalp and bony wound. Common risk factors included previous craniotomy, failed obliteration, chemoradiation, osteomyelitis and epidural abscess. Patients underwent multi-stage procedures to obtain wound source control, reconstruct the frontal sinus and skull base, and frontal bone cranioplasty. Each patient underwent successful free flap reconstruction without flap loss or donor site complications. No patients suffered ongoing symptomatic CSF leak or pneumocephalus. However, only two patients have had sustained success with their alloplastic cranioplasty. A perfect, watertight seal is required to prevent seeding the implant and avoiding infection. Overall, free flap reconstruction of the skull base and frontal sinus is a necessary solution when patients fail more conservative locoregional options. Close collaboration with neurosurgery can help with diagnosis and establish the optimal timing for surgical care.

Complete resection combined with postoperative radiotherapy is ideal for skull base chordomas. The recent literature suggests that the degree of surgical resection is the most important prognostic factor.We retrospectively analyzed the clinical data of 16 patients with initial chordoma treated at our center between August 2015 and December 2021 and conducted a retrospective study on the prognosis of surgical treatment of skull base chordoma between 2013 and 2022.According to the Kaplan-Meier method, there was a significant difference in PFS between patients aged 50 years and 50 years, and no significant difference was observed in PFS for tumor involvement of the internal carotid artery, dura, or superior or inferior clivus. However, there was still a correlation with prognosis. As observed in the included literature, the 5-year overall survival rate was significantly higher in patients undergoing total skull base chordoma resection than in those undergoing subtotal resection (STR), which in turn was significantly higher than in those undergoing partial resection (PR). Patients undergoing subtotal resection had significantly better 5-year PFS rates than those undergoing PR.Our study shows that gross total resection and STR have better survival in patients with skull base chordomas compared to PR.

Differentiation of skull base tumors, including chondrosarcomas, chordomas, and metastases, on conventional imaging remains a challenge. We aimed to test the utility of DWI and dynamic contrast-enhanced MR imaging for skull base tumors.Fifty-nine patients with chondrosarcomas, chordomas, or metastases between January 2015 and October 2021 were included in this retrospective study. Pretreatment normalized mean ADC and dynamic contrast-enhanced MR imaging parameters were calculated. The Kruskal-Wallis H test for all tumor types and the Mann-WhitneyFifteen chondrosarcomas (9 men; median age, 62 years), 14 chordomas (6 men; median age, 47 years), and 30 metastases (11 men; median age, 61 years) were included in this study. Fractional plasma volume helped distinguish all 3 tumor types (DWI and dynamic contrast-enhanced MR imaging sequences can be beneficial for differentiating the 3 common skull base tumors.

Objective: \ud \ud IgG4-related disease (IgG4-RD) involving the temporal bone is an uncommon and underrecognized pathology often mistaken for malignancy. This systematic review is the first that aims to thoroughly analyze IgG4-RD of the temporal bone.\ud \ud Databases Reviewed: \ud \ud Ovid MEDLINE, EMBASE, Cochrane Library, and Google Scholar.\ud \ud Methods: \ud \ud We used the following search keywords: “lgG4-RD,” “skull,” “skull base,” “cranial,” “temporal bone,” “inner ear.” We additionally manually searched the bibliographies of relevant articles. The JBI Critical Appraisal Checklist for Case Reports and Case Series was used to assess the risk of bias; because of the scarcity of the reports, data were available through limited case series and reports; thus, data synthesis was not possible.\ud \ud Results: \ud \ud We identified 17 studies with 22 cases with temporal bone involvement. The most common presenting symptoms were hearing loss, otalgia, and headache. The mastoid and petrous bone were the most affected anatomical areas. Both computed tomography and magnetic resonance imaging were used. Biopsies showed the characteristic lymphoplasmacytic infiltrate in all cases, with histopathology being the diagnostic modality that set the diagnosis. Most patients were treated with corticosteroids ± surgery or a combination of corticosteroids and immunosuppressants with 95.5% symptomatic response and disease control.\ud \ud Conclusion: \ud \ud IgG4-RD of the temporal bone radiologically manifests as space-occupying, lytic lesions; clinically, it presents with vague otological symptoms. Diagnosis involves a thorough workup, with histopathology being crucial in setting a definite diagnosis. IgG4-RD tends to respond well to systemic corticosteroids, whereas surgery is mostly required for diagnostic purposes.

Procedures performed along the skull base require technical prowess and a thorough knowledge of cranial anatomy to navigate the operative field. Anatomical triangles created by unique anatomical structures serve as landmarks to guide the surgeon during meticulous skull base procedures. The corridors rapidly orient the surgeon to the operative field and permit greater confidence regarding skull base position during dissection. A literature review was performed with use of the PubMed database and reference list searches from full-text reviewed articles, which resulted in the identification of 31 distinct anatomical triangles of the skull base. The 31 anatomical triangles are categorized into a corresponding cranial fossa or the extracranial subsection. The triangles described in the manuscript include junctional, interoptic, precommunicating, opticocarotid, supracarotid, parasellar, clinoidal, oculomotor, carotid-oculomotor, supratrochlear, infratrochlear, anteromedial, quadrangular, anterolateral, posteromedial, posterolateral, lateral, superior petrosal, oculomotor-tentorial, inferomedial, inferolateral, glossopharyngo-cochlear, vagoaccessory, suprahypoglossal, hypoglossal-hypoglossal, infrahypoglossal, parapetrosal, suprameatal, retromeatal, suboccipital, and the inferior suboccipital. The goal of this review is to create a comprehensive resource for existing skull base triangles that includes borders, contents, surgical applications, and illustrations to enhance awareness and inform microsurgical dissection.

We aim to distill the current body of evidence to consider when selecting an endoscopic versus open approach to address cranial base disease. We evaluate the evolution of modern surgical techniques and their respective limitations.Greater understanding of the role of tumor biology in relation to patient outcomes has led to increasingly sophisticated treatment algorithms. This combined with further development of advanced instrumentation and technique has led to the adoption of new surgical corridors previously unapproachable via traditional surgery.Endoscopic and open craniofacial approaches both remain important surgical techniques in the management of cranial base tumors. The modern skull base surgeon may employ a combination of approaches to optimize tumor extirpation while preserving functional outcomes. Patient history, anatomic tumor extent, histology, and functional goals should be considered thoroughly when planning any surgical intervention.

Cardiac paragangliomas are exceedingly rare. Herein, we describe a patient with a large dopaminesecreting cardiac paraganglioma who had a history of pheochromocytoma after right adrenalectomy. The cardiac surgery was uneventful and without blood pressure fluctuations.The measurement of plasma-free metanephrines or urinary fractionated metanephrines is used as an initial screening test for pheochromocytoma or paraganglioma detection. However, these results must be combined with those of a plasma 3-methoxytyramine test to accurately establish the rare dopaminergic phenotype of pheochromocytomas or paragangliomas, if suspected. F-FDOPA (6-[18F]-L-fluoro-L-3, 4-dihydroxyphenylalanine)-based positron emission tomography (PET) and PET-computed tomography are relatively sensitive and specific; therefore, these techniques are recommended for patients with pheochromocytomas or paragangliomas before operation or during postoperative follow-up.

Objectives There are several instruments to assess health-related quality of life (HRQoL) in chronic rhinosinusitis (CRS). Unfortunately, none of them evaluates all three health domains (physical, social and psychological) important to assess the overall well-being of the patient. The Endoscopic Endonasal Sinus and Skull Base Surgery Questionnaire (EES-Q) does assess all these elements. Initially, the EES-Q is validated to evaluate the impact of endoscopic endonasal surgery (EES) on HRQoL. The aim of this study is to assess whether EES-Q outcomes differ in patients with CRS compared with healthy individuals. Therefore, extending the use of the EES-Q for all CRS patients. Design Cross-sectional study. Setting Tertiary referral hospital. Participants One hundred patients with uncontrolled CRS (50% with nasal polyps) scheduled to receive EES. The questionnaire was completed preoperatively. Healthy control subjects (n = 100) without any history of sinusitis or a known current medical treatment at a hospital were included. Main outcome measures Mann-Whitney U test was performed to identify differences in EES-Q scores (domain scores and EES-Q score). Results The median EES-Q score in CRS patients (33.8) was significantly higher (p < 0.001) than in the control group (10.4). As well as the physical (52.5 vs. 16.4, p < 0.001), psychological (13.8 vs. 5.0, p < 0.001) and social (37.5 vs. 2.5, p < 0.001) domain scores. Conclusions With this study, we are extending the use of the EES-Q. It indicates that the EES-Q can be a valuable clinical tool to assess multidimensional HRQoL in all patients with CRS.

Pre-surgical diagnosis of skull base chondrosarcoma (SBC) is often challenging due to the resemblance to chordoma. The goal of this study was to develop an optimal method for predicting SBC diagnosis.This retrospective study included patients with histologically diagnosed SBC and skull base chordoma. Their clinical and radiologic features were compared, and the predictive factors of SBC were examined.Forty-one patients with SBC and 41 with chordoma were included. Most SBCs exhibited hypointensity (25, 64.1%) or isointensity (12, 30.8%) on T1-weighted images, and hyperintensity (34, 87.1%) or mixed intensity (5, 12.8%) on T2-weighted images. MRI contrast enhancement was usually avid or fair (89.7%) with "arabesque"-like pattern (41.0%). The lateral/paramidline location was more common in SBC than in chordoma (85.4% vs. 9.8%; P 0.01), while midline SBCs (14.6%) were also possible. Multivariate analysis demonstrated that higher apparent diffusion coefficient (ADC) value (unit odds ratio 1.01; 95% confidence interval 1.00-1.02; P 0.01) was associated with an SBC diagnosis. An ADC value of ≥ 1750 × 10The ADC-based method is helpful in distinguishing SBC from chordoma and readily applicable in clinical practice. The prediction accuracy increases when other characteristics of SBC, such as non-midline location and arabesque-like enhancement, are considered together.

The anatomy and variations of the anterior ethmoidal artery (AEA) are clinically relevant. The anterior ethmoidal foramen (AEF) can be used to locate the initial site of the AEA, and the anterior nasal spine (ANS) is a constant bony marker in the anterior nasal atrium. However, there is no relevant research on AEF and ANS targeting the AEA. Hence, this study aimed to accurately locate the AEA through AEF and ANS using computed tomography.A total of 120 (240 sides) sinus computed tomography scans were retrospectively selected and studied. The AEA was classified into grades I, Ⅱ, and Ⅲ group based on the relationship between the AEA and the skull base. The distance between AEF and ANS and the angle between AEF-ANS and the axial plane were measured.The average distance from AEF to ANS was 58.26±3.64 mm, and the corresponding angle was 60.05±5.93 degrees. The AEF-ANS distances and angles were negatively correlated with age. Moreover, the distances from AEF to ANS were significantly increased in the grade Ⅲ group compared with the grade Ⅱ group.The measurements obtained in this study add anatomic knowledge that can serve as a better intraoperative localization method of the AEA, which can help surgeons avoid relative complications during endoscopic sinus surgery.

McCune-Albright syndrome (MAS) and fibrous dysplasia (FD) have been reported to cause Chiari type I malformation (CM1) and skull base invagination (BI).A 6-year-old girl was diagnosed with MAS and FD. She was diagnosed with CM1 at age 8 years, and the syringomyelia had gradually increased by age 20 years. We performed foramen magnum decompression and C1 laminectomy, and the syringomyelia stopped spreading after surgery.This patient underwent long-term radiological observation and morphological evaluations, which revealed that the skull thickening was progressing, while the posterior cranial fossa volume (PCFV) remained unchanged for 14 years. Therefore, although PCFV did not decrease, it was considered to be relatively inadequate due to the increase in brain volume with growth, resulting in posterior fossa overcrowding, causing CM1.In patients with FD/MAS, long-term evaluation of bone thickening, odontoid position, and PCFV is necessary.

Expanded endonasal approaches (EEA) have become the main approach to the anterior skull base. A specific questionnaire, the Sino-Nasal Outcome Test for Neurosurgery (SNOT-NC), was developed in German to assess quality of life after EEA. The aim of this study was the cross-cultural adaptation and validation of the Italian version of SNOT-NC.Three hundred patients who underwent EEA for anterior skull base diseases were included in the study. An Italian version of SNOT-NC was cross-culturally adapted. Internal consistency, test-retest reliability, construct, and clinical and group validity were analyzed. The Short-Form 36 questionnaire was used for construct validity analysis.The Cronbach α coefficient was 0.862. Only 1 subscale (olfactory disturbance) showed an insufficient internal consistency. The test-retest reliability was excellent (intraclass correlation coefficient between 0.934 and 0.997). The good correlation between SNOT-NC and Short-Form 36 scores (P0.05) showed the construct validity of the questionnaire. SNOT-NC was able to distinguish between patients with more or fewer nasal symptoms (P0.05). Patients who underwent a transtuberculum/transplanum approach had greater olfactory disturbances compared with other approaches (P0.05).The Italian version of SNOT-NC showed good internal consistency, test-retest reliability, construct, and clinical and group validity, as well as original version. It can be considered a good instrument to evaluate the impact of endoscopic EEA to the anterior skull base.

The greater wings of the sphenoid bone (GWS) comprise the components of the sphenoid bone that make up most of the posterior orbital wall and form the anterior and medial parts of the floor of the middle cranial fossa. Many important skull base foramina, which transmit vital neurovascular structures, are present in these paired wings on either side of the central body of the sphenoid bone. A wide variety of diseases can affect the GWS, ranging from benign osseus lesions to malignant primary and secondary bone abnormalities. The complex three-dimensional curved (winged) shape of the GWS and the wide array of pathologic entities that affect this bone can make it challenging for the radiologist to report the imaging findings accurately, especially in relation to the important skull base foramina. The authors describe a systematic approach to understanding the three-dimensional anatomy of the GWS and review important diseases, with the aid of imaging examples. Useful imaging "pearls" that can help in making specific diagnoses are provided throughout the article.

Introduction Endoscopic management of frontal sinus cerebrospinal fluid leaks has become the gold standard of treatment, with high success rates and low morbidity. The aim of this study is to review our experience in managing this challenging condition. Objective To review our experience in treating frontal sinus cerebrospinal fluid leaks through an endonasal endoscopic approach. Methods A retrospective evaluation of patients undergoing endoscopic surgery for frontal sinus cerebrospinal fluid leaks was performed. Demographics, defect location and etiology, surgical and reconstructive technique, complications, and postoperative followup were examined. Results Twenty-two patients with a mean age of 40.4 years were treated surgically by the senior author between 2015 and 2019. Cerebrospinal fluid leak was either traumatic (17) or spontaneous (5). Successful first-attempt endoscopic repair was accomplished in all cases. A combined endoscopic-trephination approach was necessary in 5 patients (22.8%). No serious complications were reported, and frontal sinus drainage pathway was patent in all our cases. Revision surgery was necessary in only 2 patients for synechia formation. The mean patient followup was 22.7 months (range: 7 − 41 months). Conclusion Progress in the field of endoscopic surgery has shifted the paradigm, establishing endoscopic repair of frontal sinus leaks as the standard of care. A few remaining limits of this approach could be addressed by combining endoscopy with frontal trephination.

This study aimed to identify the characteristics of cranial-base morphology in platybasic and nonplatybasic patients with palatal anomalies and velopharyngeal dysfunction (VPD) compared with control subjects to investigate structural factors related to craniofacial morphology that affect the nasopharyngeal space and may influence velopharyngeal function, and to develop precise treatments for specific patients with VPD.Three hundred eighty-six patients with VPD and various palatal anomalies were studied retrospectively. The control group included 126 healthy patients with normal speech. Lateral cephalometric images assessed craniofacial morphology.Nonplatybasic patients and control subjects had larger SNA, S-Ba-Ptm, and N-Ba-PP angles (in the craniomaxillary complex), and platybasic patients had larger nasopharyngeal ANS-Ptm-Ve and Ba-S-Ptm angles and longer Ve-T and Ve-Ba distances than the nonplatybasic patients. All study patients had larger ANB, Gn-Go-Ar, and PP-MP angles (in the craniomandibular complex). Nonplatybasic patients had smaller Ba-SN angles than platybasic patients and controls because of more acute N-S-Ptm angle. Among the nonplatybasic patients, Ve-T length tended to be shorter (with no significant difference between groups) and located more inferiorly (because of the smallest ANS-Ptm-Ve angle) in relation to the maxilla. Thus, the nasopharynx was narrower horizontally but longer vertically than in patients with platybasia.Cranial-base flexure influences the shape of the skull base and facial-skeletal structure and may alter the pharyngeal space between them. This finding should help improve preoperative planning regarding the effect of the pharyngeal flap height relative to the nasopharynx and oropharynx ratio that affects surgical outcomes, such as resonance and residual VPD. In patients with Class III malocclusion and maxillary constriction, careful planning of presurgical orthodontic treatment is needed in maxillary advancement procedures. Orthodontic and surgical collaboration can help prevent postoperative VPD, especially in platybasic patients.

Approaches to the jugular foramen can be challenging. Adding to this difficulty is the potential for a bony septation to occur in the foramen thus subdividing its contents. Although such bony septations in the jugular foramen are known, the anatomic details of these structures have not been studied well. Therefore, the present anatomic study was performed.One hundred adult human skulls (200 sides) underwent evaluation for the presence or absence of a bony septation within the jugular foramen. The source, morphology, and size were all documented. A classification scheme was developed to better describe the varied types of morphology of the jugular foramen bony septa. Select bony septations were submitted to histologic analysis using hematoxylin and eosin and Masson's trichrome.Bony bridges were found on 8.5% of sides. These were bilateral in 3% of skulls. Eight (47%) were incomplete (type I). Nine sides (53%) were found to have completely articulated bony bridges (type II) and these had articulation between the bony processes as either touching (type IIa), joint-like (type IIb), or completely fused (type IIc) morphologies. Multiple septa were observed on 2.5% of sides and these were classified as type III septa. Histologically, both incomplete and complete septa were found to be composed of normal bone tissue.Our study found significant variations regarding the bony septa of the jugular foramen. Knowledge of this anatomy can be useful for preoperative planning and intraoperative navigation with approaches to the skull base that target pathology of the jugular foramen.

Background The jugular foramen (JF) can be the site of several tumours. Paragangliomas, schwannomas and meningiomas are the most commonly reported. We describe a case of melanocytoma originating from the JF and presenting with an accessory nerve palsy. Illustrative case A 48-year-old woman presented with a 6-month history of cervical and left shoulder pain with wasting and weakness of the left trapezius. A Magnetic Resonance Imaging (MRI) showed a T1-hyperintense, T2-isointense, heterogeneously enhancing lesion involving the left JF and extending into the cerebello-medullary and cerebello-pontine cisterns. A retrosigmoid craniotomy was performed and a near-total removal achieved. The accessory nerve was involved by tumour and could not be preserved. Given the diagnostic uncertainty between melanotic schwannoma, metastatic melanoma and meningeal melanocytoma, next generation sequencing and genome-wide DNA methylation arrays were performed, documenting a mutation in GNA11 (c.6226A>T, p. Gln209Leu) and a methylation profile consistent with melanocytoma. The patient underwent adjuvant fractionated radiotherapy of the tumour remnant. A follow-up MRI 4 years after surgery did not show any tumour recurrence. Conclusions The differential diagnosis of skull base pigmented tumours can be challenging, particularly when they occur in unusual locations such as the JF. They can be misdiagnosed given their similar clinical, neuroradiological and pathological features if anatomy of the site of origin is not carefully considered and molecular tests are not performed, leading to erroneous treatment and follow-up planning.

BACKROUND: The feasibility of a novel skull base approach, the navigated minimal ly invasive presigmoidal suprabulbar infralabyrinthine approach (NaMIPSI-A) without rerouting of the fallopian canal for selected jugular foramen tumors (JFTs), has been demonstrated in a neuroanatomical laboratory study. OBJECTIVE: Here, we present our clinical experience with this approach for selected JFTs, with a particular focus on its efficacy and safety. METHODS: All patients with JFTs who were treated via the NaMIPSI-A were included in this study. The JFTs were classified according to a modified Fisch classification. The neurological and neuroradiological outcome, the extent of tumor resection, and the approach-related morbidity were examined.RESULTS: Five patients (two women, three men; mean age 57y, range 48-65) were available. According to the modified Fisch classification, two JFTs were graded as C1, one as De1 and two as De2. Gross total resection (GTR) was achieved in three patients and near-total resection (NTR) in two. Postsurgically, no new neurological deficits and no approach-related morbidity and mortality occurred. One case with a postoperative cerebrospinal fluid leak was managed successfully with lumbar drainage. During the follow-up period (mean 67,6 months, range 12-119 months) tumor recurrence was noted in the NTR group but not in the GTR group. CONCLUSIONS: The NaMIPSI-A to the jugular foramen without rerouting of the fallopian canal is highly valuable for selected tumors of the jugular foramen. It is less invasive than other skull approaches, and it allows safe and complete tumor removal in appropriate patients.

Postoperative management strategies for endoscopic skull base surgery (ESBS) vary widely because of limited evidence-based guidance.The PubMed, EMBASE, and Cochrane databases were systematically reviewed from January 1990 through February 2022 to examine 18 postoperative considerations for ESBS. Nonhuman studies, articles written in a language other than English, and case reports were excluded. Studies were assessed for levels of evidence, and each topic's aggregate grade of evidence was evaluated.A total of 74 studies reporting on 18 postoperative practices were reviewed. Postoperative pain management, prophylactic antibiotics, and lumbar drain use had the highest grades of evidence (B). The literature currently lacks high quality evidence for a majority of the reviewed ESBS precautions. There were no relevant studies to address postoperative urinary catheter use and medical intracranial pressure reduction.The evidence for postoperative ESBS precautions is heterogeneous, scarce, and generally of low quality. Although this review identified the best evidence available in the literature, it suggests the urgent need for more robust evidence. Therefore, additional high-quality studies are needed in order to devise optimal postoperative ESBS protocols.

The sphenoid bone is an unpaired bone that contributes to the formation of the skull base. Despite the enormous progress in transnasal endoscopic visualisation, imaging techniques remain the cornerstones to characterise any pathological condition arising in this area. In the present review, we offer a bird’s-eye view of the developmental, inflammatory, and neoplastic alterations affecting the sphenoid body and clivus, with the aim to propose a practical diagnostic aid for radiologists based on clinico-epidemiological, computed tomography, and magnetic resonance imaging features.

Summary The integration of the growth of the brain and the cranial base suggests that each system may influence the other, notably during the first three years of life, although this influence has never been proven to be exclusive. The aim of our work was to analyse the dynamics of normal growth on the one hand, and the development and ontogenetic allometry of the cranial base in the infant on the other hand. Material and method A total of 32 infants (17 males/15 femeles) having been included in the unexpected infant death french protocol were analyzed. Three-dimensional reconstructions of the cranial base were performed from CT scans. The technique combined manual segmentation of regions of interest, contour extraction and surface reconstruction. Nineteen landmarks were positioned on each of the bone surfaces. Results No correlation was observed between sex assigned at birth and shape, weight, crown-heel length, or head circumference. Principal component analysis showed that 85.5% of the variance observed on the first component was secondary to growth. After Procrustes superimposition, 25% of the shape variance observed was explained by the first principal component. It showed anteroposterior lengthening of the cranial base. In addition, the height, width and length of the posterior fossa increased and the relative position of the basion was displaced inferiorly and anteriorly with flexion of the sphenoid angle. Negative allometry was also observed. Conclusion Our study, carried out in a rigorously selected population of infants, presents a fundamental approach to ontogeny through study of shape, growth and ontogenetic allometry.

PURPOSE/OBJECTIVES To develop a normal tissue complication probability (NTCP) model including clinical and dosimetric parameters for high-grade temporal lobe radionecroses (TRN) after pencil beam scanning (PBS) proton therapy (PT). MATERIALS/METHODS Data of 299 patients with skull base and Head and Neck tumors treated with PBS PT with a total dose of ���60 GyRBE from 05/2004-11/2018 were included. Patients with a ��� grade (G) 2 TRN (CTCAE v5.0 criteria) were considered as having a high-grade TRN. Nine clinical and 27 dosimetric parameters were considered for structure-wise modelling. After elimination of strongly cross-correlated variables, logistic regression models were generated using penalized LASSO regression. Bootstrapping was performed to assess parameter selection robustness. Model performance was evaluated via cross-correlation by assessing the area under the curve of receiver operating characteristic curves (AUC-ROC) and calibration with a Hosmer-Lemeshow test statistic. RESULTS After a median radiological follow-up of 51.5 months (range, 4-190), 27 (9%) patients developed a ��� G2 TRN. Eleven patients had bitemporal necrosis, resulting in 38 events in 598 temporal lobes for structure-wise analysis. During Bootstrapping analysis, the highest selection frequency was found for prescription dose (PD), followed by Age, V40Gy[%], Hypertension (HBP) and D1cc[Gy]. During cross validation Age*PD* D1cc[Gy]*HBP was superior in all described test statistics. Full cohort structure wise and patient wise models were built with a maximum AUC-ROC of 0.79 (structure-wise) and 0.76 (patient-wise). CONCLUSION While developing a logistic regression NTCP model to predict ��� G2 TRN, the best fit was found for the model containing Age, PD, D1cc[Gy] and HBP as risk factors. External validation will be the next step to improve generalizability and potential introduction into clinical routine.

To study the morphological types and relative location of the pterion and its precise relationship with the middle meningeal artery (MMA) in the skulls of adults from southeastern China.Dry skulls (n = 250) of adults were obtained from a university specimen bank and analyzed. The morphological types of the pterions were observed. The distances from the center of the external pterion (Pec) to the relevant intracranial and extracranial marker points were measured using a digital vernier caliper. The anterior, middle, and posterior end points of the external pterion were drilled perpendicular to the bone surface. The precise relationships of the external pterion with the internal pterion and the groove of the frontal branch of the MMA were observed and measured after sawing the skull.The morphological types of the pterion in the skulls of adults from southeastern China were sphenoparietal suture (SP) (85%), epipteric (12.4%), frontotemporal suture (1.4%), and stellate (1.2%) types. The mean widths of the external and internal pterions were R, 10.68 ± 4.22 mm; L, 11.13 ± 4.40 mm and R, 14.66 ± 4.04 mm; L, 14.14 ± 4.29 mm, respectively, and the width of the internal pterion was slightly longer than that of the external (P 0.05). No significant difference in pterion width was found between the genders or sides of the skull (both P 0.05). The distances from the Pec to the posterolateral aspect of the frontozygomatic suture, zygomatic process of the frontal bone, midpoint of the zygomatic arch, and external acoustic meatus were 29.95 ± 3.75 mm, 34.88 ± 4.08 mm, 40.86 ± 3.59 mm, and 53.79 ± 3.82 mm, respectively. These distances were slightly longer on the right side of the skull than on the left side (P 0.01) and longer in men than in women (P 0.01). The distances from the Pec to the frontal crest, optic canal, and anterior clinoid process were 62.79 ± 1.15 mm, 45.39 ± 2.48 mm, and 45.47 ± 2.05 mm, respectively. The external and internal pterions were not on the same level, and all the internal pterions were located below the external ones. In the vast majority of the skulls, the groove of the frontal branch of the MMA passed through the posterior end of the external pterion (Pep) or the area between the Pec and Pep.The morphology of the pterion in the skulls of adults from southeastern China is predominantly of the SP type, mostly symmetrically distributed. The distance from the pterion to the extracranially relevant marker points differs among the ethnic groups, between the genders, and between the sides of the skull. All the internal pterions are located below the external ones. Most of the frontal branch of the MMA is located below the mid-posterior segment of the lateral pterion. The characterization of the morphology, the relative position of the pterion and the precise relationship of this structure with the MMA in the skulls of adults from southeastern China may provide an anatomical basis for teaching and clinical practices.

Preoperative embolization for intracranial meningiomas can cause tumor necrosis, reduce intraoperative blood loss, and facilitate surgery. This study aimed to evaluate the efficacy of tumor embolization using Embosphere microspheres for skull base meningiomas and analyze postembolization plain computed tomography (CT) and magnetic resonance imaging (MRI) scans to identify findings that could potentially predict treatment response.Between April 2014 and April 2020, 80 patients with skull base meningiomas presenting at our medical center underwent embolization with Embosphere microspheres. The effects of tumor embolization were evaluated through a comparison of postembolization plain CT and contrast-enhanced MRI.A total of 143 vessels (102 of 108 external carotid artery branches and 41 of 65 internal carotid artery branches) from 80 skull base meningiomas were embolized with Embosphere microspheres. Microspheres 100-300 μm in size were used in 2 cases, microspheres 300-500 μm in size were used in 12 cases, and microspheres 500-700 μm in size were used in 66 cases. Postembolization contrast-enhanced MRI showed reductions in enhancing lesions within the tumor in 55 of 80 cases. Postembolization plain CT scans showed high-density lesions within the tumor in 41 of 55 cases. Thus, reductions in enhancing lesions on postembolization contrast-enhanced MRI were statistically significantly associated with the presence of high-density lesions on postembolization plain CT (P0.001). Embolization-related neurological complications occurred in 3 cases.Embosphere microspheres are user friendly and effective embolic materials for the embolization of skull base meningiomas. Postembolization contrast-enhanced MRI and plain CT findings may be useful for evaluating the effects of tumor embolization.

Chondrosarcoma and chordoma are often grouped together because of their similar anatomic locations, clinical presentations, histopathological and radiological findings, and growth patterns. In the present study, we investigated the clinical and prognostic differences of chondrosarcomas and chordomas of the skull base and spine.We accessed the Surveillance, Epidemiology, and End Results database to search for patients from 2000 to 2018 with chondrosarcomas and chordomas of the skull base and spine for inclusion in the present study.We included 1346 and 1536 cases of chondrosarcoma and chordoma for analysis, respectively. Chondrosarcomas of the cranial base and spine were seen in younger patients and were associated with a larger tumor size compared with chordomas. Among the tumors of the skull base, chondrosarcomas were more common in women, with a male predominance found for chordomas. We also observed a male predilection for both spinal chondrosarcomas and chordomas. Distinct metastatic patterns were found for chondrosarcomas versus chordomas, and spinal chondrosarcomas showed a greater risk of distant metastases at presentation compared with spinal chordomas. Cranial base chondrosarcomas were associated with superior outcomes compared with chordomas. However, we demonstrated an opposite survival pattern for spinal chondrosarcomas and chordomas.Chondrosarcomas and chordomas have divergent clinical manifestations and prognoses depending on the anatomic location.

Objectives Prognoses for nasopharyngeal carcinoma (NPC) between categories T2 and T3 in the Eighth American Joint Committee on Cancer (AJCC) staging system were overlapped. We explored the value of skull base invasion (SBI) subclassification in prognostic stratification and use of induction chemotherapy (IC) to optimize T2/T3 categorization for NPC patients. Methods We retrospectively reviewed 1752 NPC patients from two hospitals. Eight skull base bone structures were evaluated. Survival differences were compared between slight SBI (T3 patients with pterygoid process and/or base of the sphenoid bone invasion only) and severe SBI (T3 patients with other SBIs) with or without IC using random matched-pair analysis. We calculated the prognosis and Harrel concordance index (C-index) for the revised T category and compared IC outcomes for the revised tumor stages. Results Compared to severe SBI, slight SBI showed better 5-year overall survival (OS) (81.5% vs. 92.3%, p = 0.001) and progression-free survival (PFS) (71.5% vs. 83.0%, p = 0.002). Additional IC therapy did not significantly improve OS and PFS in slight SBI. The proposed T category separated OS, PFS, and locoregional recurrence-free survival in T2 and T3 categories with statistical significance. An improved C-index for OS prediction was observed in the proposed T category with combined confounding factors, compared to the AJCC T staging system (0.725 vs. 0.713, p = 0.046). The survival benefits of IC were more obvious in the advanced stage. Conclusions NPC patients with slight SBI were recommended to downstage to T2 category. The adjustment for T category enabled better prognostic stratification and guidance for IC use. Key Points • For nasopharyngeal carcinoma (NPC) patients in T3 category, slight skull base invasion was a significant positive predictor for OS and PFS. • NPC patients with slight SBI might not gain significant survival benefits from induction chemotherapy. • Downstaging slight SBI NPC patients to T2 category would make a more accurate risk stratification, improve the predicting performance in OS, and have a better guidance in the use of IC for patients in advanced stage.