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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.

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.

(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.