Chiari I Deformity: Beyond 5 mm below the Foramen Magnum.

Although originally described as a consecutive series of pathologic changes, Chiari syndrome represents a spectrum of disease divided into two subsets: development deformities of the paraxial mesoderm manifesting after birth (types 0-1.5) and true congenital malformations due to failure of neural tube closure present in utero (types 2-5). Heterogeneity among patients with a Chiari deformity and incomplete understanding of its pathophysiologic characteristics have led to inconsistency in radiologic reporting and difficulty in defining appropriate management strategies tailored to an individual patient's condition. The radiologist is tasked with going beyond the criteria for cerebellar tonsillar herniation to define an individual patient's disease state, determine candidacy for surgery, and assist in selecting the proper surgical approach. In addition, the radiologist must be able to identify conditions that result in cerebellar tonsillar herniation that are not related to Chiari deformity to avoid inappropriate surgery. Last, the radiologist must be able to interpret postoperative imaging examinations to assess for adequacy of treatment and complications. The authors summarize recent literature regarding the pathophysiologic basis of Chiari 1 and related deformities and detail the ideal morphologic and physiologic imaging assessment, focusing on Chiari 1 and related deformities (Chiari 0, 0.5, and 1.5). Also discussed are surgical techniques and "pearls" of postsurgical imaging, including complications that must be recognized. This review provides clarity to a commonly encountered but less understood condition to optimize outcomes for patients with Chiari 1 and related deformities. ©RSNA, 2024 Supplemental material is available for this article. See the invited commentary by Huisman in this issue.

[Jugular Foramen, Foramen Magnum Meningiomas].

The jugular foramen, also known as the foramen magnum, is a highly intricate region of the skull base through which numerous critical blood vessels and nerves traverse. Meningiomas, the most common tumors in neurosurgical pathology, can arise at any location where the meninges are present, posing significant challenges. Meningiomas involving the jugular foramen and sublingual neural tube are particularly notable for their potential to extend from intracranial to extracranial sites, necessitating familiarity with extracranial anatomy, which is not typically encountered in clinical practice. A comprehensive understanding of anatomical characteristics, along with an ample field of view and working space, is crucial for handling the cerebellum, brainstem, and nerves meticulously. The use of surgical support tools such as neuromonitoring and navigation is essential for enhancing the safety of the procedure. Furthermore, preparedness for treatment options, rehabilitation, and adjunctive therapies is vital in the event of neurological symptoms such as those affecting the glossopharyngeal, vagal, or hypoglossal nerves.

Osseous bridging of the condylar fossa: report of a rare anatomical variation at the outer skull base.

PURPOSE: The topic of osseous variations of the craniocervical junction is a complex morphological and embryological chapter of human anatomy, with a possible impact on neurogical and vascular functionality in this morphological variable region. MATERIAL & METHODS: An until now undescribed anatomical variation of the exoccipital part of the occipital bone has been observed after maceration at the outer skull base of a West-European 68-year-old male body donor. RESULTS: On both sites of the foramen magnum accessory osseous processes were observed that arise from the jugular process and point towards the lateral margin of the foramen magnum. On the left site this process forms a full arc that bridges the condylar fossa completely. CONCLUSION: The observed osseous bridge over the condylar fossa has not been reported on before and can be explained by the partial persistence of a primordial vertebra between atlas and occipital bone: the Proatlas. The resulting accessory structure may affect due to its topographic conditions the V3-Segment of the vertebral artery and its accompanying nerves, and thus, play a role in diagnosis and therapy of vascular and/or neurological symptoms of head and neck.

Prevalence and Morphometry of Occipital Foramen in Dry Skulls and its Clinical Implications.

The occipital emissary foramen (OEF) located on the occipital bone transmits the occipital emissary vein, which connects the occipital vein to the confluence of cranial venous sinuses. The OEF varies in incidence, number, size, and location. Knowledge of this foramen is essential for carrying out suboccipital and transcondylar surgeries without clinical implications. Hence, the study was planned. The aim of the present study is to elaborate on incidence, location, and morphometry consisting of the number and size of this foramen in light of clinical bearing in the context of the Indian population. The study was carried out in the Department of Anatomy using 80 skulls of unknown age and sex. The occipital bone of the skull was observed for the incidence, number, size, and location of the occipital emissary vein and associated clinical implications were elaborated. The incidence of occipital foramen was 36.25% and detected in 29 skulls. All these occipital foramina were patent. The mean diameter of this foramen was 0.6 mm. The most common location of these foramina was the left side of the foramen magnum, followed by the left side of the external occipital crest. The information about the incidence, number, size, and location of OEF is important to prevent catastrophic bleeding during surgery in the region of the occipital bone. The awareness of differential morphometry and morphology of occipital foramina is of great importance for neurosurgeons during suboccipital craniotomy and skull base surgeries, including far lateral and transcondylar approaches to access posterior cranial fossa for management of pathologies in the cranial cavity.

Cervicomedullary Compression in Achondroplasia: A Feared Complication and an Unforeseen Risk Factor of Posterior Circulation Stroke.

Achondroplasia is an autosomal dominant disorder with defect in the ossification of the cartilage of long bones. Many bony abnormalities constitute its clinical features, with craniovertebral junction (CVJ) anomalies being one of most common issues which need to be addressed at the earliest. CVJ anomalies in individuals may cause neurovascular compression, which may warrant an early surgery to prevent catastrophic complications. Posterior circulation strokes secondary to CVJ anomalies are well known. We hereby present an unusual case of posterior circulation stroke in an achondroplastic dwarf who presented to our tertiary care centre. Prospective case study. The present case adds to the existing literature about one of the preventable causes of fatal posterior circulation strokes in the young. A high index of suspicion for neurovascular compression at the foramen magnum and early initiation of treatment in achondroplastic young individuals may have gratifying results.

A comparative analysis of neuroendoscopic foramen magnum decompression versus traditional open surgery for Chiari Malformation Type I.

PURPOSE: Chiari Malformation Type I (CM1) is characterized by the downward displacement of the cerebellar tonsils below the foramen magnum. The standard surgical treatment for CM1 is foramen magnum decompression and atlas laminectomy (FMD-AL). However, there is a growing interest in exploring minimally invasive techniques, such as neuroendoscopically assisted FMD-AL, to optimize surgical outcomes. The aim is to present the results of the less invasive neuroendoscopic-assisted system application as an alternative to decompression surgery in patients with CM-1 with/without syringomyelia. PATIENTS AND METHODS: A retrospective analysis was conducted on 76 patients with CMI who underwent either neuroendoscopic-assisted FMD-AL (n = 23) or open surgery (n = 53). Preoperative and postoperative assessments were performed, including pain levels, functional assessment, outcome and serum creatinine kinase (CK) levels. Surgical parameters and radiological imaging were also evaluated and compared. RESULTS: Both surgical groups showed improvements in pain levels and increase in postoperative CK levels. There were no statistically significant differences between the groups in terms of postoperative JOA scores, VAS scores, CCOS, or syrinx resolution. However, the neuroendoscopic group had significantly lower CK levels, shorter hospital stays, less blood loss, and shorter operation times compared to the open surgery group, indicating reduced muscle damage and potential benefits of the neuroendoscopic assisted approach. CONCLUSION: Both neuroendoscopy and open surgery groups can effectively alleviate symptoms and improve outcomes in patients with CM1. The neuroendoscopic assisted technique offers the advantage of reduced muscle damage and shorter hospital stays. The choice of surgical technique should be based on individual patient characteristics and preferences. LEVEL OF EVIDENCE: 3 (Retrospective case-control study) according to using the Oxford Centre for Evidence-Based Medicine (CEBM) Table.

Association of the Achondroplasia Foramen Magnum Score and intraoperative neuromonitoring.

INTRODUCTION: Foramen magnum stenosis in achondroplasia carries a risk of sudden death. A proportion of these patients benefit from foramen magnum decompression (FMD). The Achondroplasia Foramen Magnum Score (AFMS) was developed to stratify those most at risk. We hypothesise that this score may be reflected in neurophysiological findings. METHODS: Patients with achondroplasia who had undergone FMD (n=20) were retrospectively grouped into AFMS 2, 3 and 4. Amplitude from tibialis anterior (TA) and the percentage change in somatosensory evoked potential (SSEP) latency after FMD were reported. RESULTS: Baseline motor evoked potential amplitudes for patients with AFMS=4 were significantly lower left (p=0.0017 and p=0.02 for right and left TA, respectively) compared with AFMS grades 2 and 3. Median reduction (% change) in SSEP latency (ms) after surgery was not significantly different in any of the patients. CONCLUSIONS: This short report cross-references AFMS to intraoperative neuromonitoring. Baseline amplitudes were noticeably lower in the most severe AFMS group. This observation supports the notion that AFMS can help risk stratify patients and aid in surgical selection.

Novel classification of foramen magnum meningiomas predicted by topographic position relative to neurovascular bundle.

PURPOSE: Proximity to critical neurovascular structures can create significant obstacles during surgical resection of foramen magnum meningiomas (FMMs) to the detriment of treatment outcomes. We propose a new classification that defines the tumor's relationship to neurovascular structures and assess correlation with postoperative outcomes. METHODS: In this retrospective review, 41 consecutive patients underwent primary resection of FMMs through a far lateral approach. Groups defined based on tumor-neurovascular bundle configuration included Type 1, bundle ventral to tumor; Type 2a-c, bundle superior, inferior, or splayed, respectively; Type 3, bundle dorsal; and Type 4, nerves and/or vertebral artery encased by tumor. RESULTS: The 41 patients (range 29-81 years old) had maximal tumor diameter averaging 30.1 mm (range 12.7-56 mm). Preoperatively, 17 (41%) patients had cranial nerve (CN) dysfunction, 12 (29%) had motor weakness and/or myelopathy, and 9 (22%) had sensory deficits. Tumor type was relevant to surgical outcomes: specifically, Type 4 demonstrated lower rates of gross total resection (65%) and worse immediate postoperative CN outcomes. Long-term findings showed Types 2, 3, and 4 demonstrated higher rates of permanent cranial neuropathy. Although patients with Type 4 tumors had overall higher ICU and hospital length of stay, there was no difference in tumor configuration and rates of postoperative complications or 30-day readmission. CONCLUSION: The four main types of FMMs in this proposed classification reflected a gradual increase in surgical difficulty and worse outcomes. Further studies are warranted in larger cohorts to confirm its reliability in predicting postoperative outcomes and possibly directing management decisions.

Topographic Anatomy and Pneumatization of the Posterior Clinoid Process in Chiari Type I Malformation.

OBJECTIVE: This study aimed to examine pneumatization and topographic location of the posterior clinoid process (PCP) in Chiari type I malformation (CIM) for skull base approaches. METHODS: Computed tomography images of 52 (23 males/29 females) CIM subjects aged 23.87 ± 16.09 years and 71 (26 males/45 females) healthy subjects aged 42.48 ± 21.48 years constituted the study universe. RESULTS: The distances of PCP to the foramen magnum (P = 0.037), superior orbital fissure (P < 0.001), foramen rotundum (P < 0.001), and foramen ovale (P < 0.001) were smaller, but the distance of PCP to the crista galli (P = 0.038) was greater in CIM patients, compared with normal subjects. In CIM, the fusion between PCP and the anterior clinoid process was observed in 9 sides (8.70%), while in controls it was observed in 12 sides (8.50%). PCP pneumatization was observed in 40 sides (38.50%) in CIM patients, while it was observed in 28 sides (19.70%) in normal subjects. These data displayed that PCP pneumatization was affected by CIM (P < 0.001). CONCLUSIONS: The distances of PCP to the crista galli and foramen magnum indicate the anterior fossa length and the posterior fossa depth, respectively; thus CIM patients have a longer anterior fossa and a shallow posterior fossa. In addition, the distances of PCP to the superior orbital fissure, foramen rotundum, and foramen ovale indicate the middle fossa width; hence CIM patients have less middle fossa width than normal individuals. CIM patients have an approximately 50% higher PCP pneumatization rate, and this may increase the risk of complications such as cerebrospinal fluid fistula during the application of posterior clinoidectomy.

Preliminary study on cranial measurements and sexual dimorphism in skull bones of gaur (Bos gaurus gaurus, Smith 1827).

Cranial measurements have been widely used in various studies in wildlife sciences, ranging from understanding predator ecology to wildlife forensics. However, detailed description of morphometry and sexual dimorphism of the skull of gaur Bos gaurus gaurus is lacking. The present study was undertaken to determine the sexual dimorphism based on the cranial measurements of gaur. A total of 12 individual gaur skulls of male (n = 6) and female (n = 6) were studied in the field from the naturally deceased animals between January 2018 and December 2021 in different ranges of Bandhavgarh tiger reserve (BTR), Madhya Pradesh, India. The skull measurements were analysed using univariate and multivariate statistics to determine whether cranial dimensions could be used to differentiate male and female skulls reliably. A total of 43 morphometrical parameters grouped into nine indices were calculated. Select morphometrical parameters viz PL, GFL, AKI, LBB, LFB, GBEE, GBAN, BPOP and GTCH were significantly different (p < 0.05) between sexes, whereas GBAN were significantly higher in female skulls. The measurements demonstrated that the skull of the gaur was dolichocephalic as the profile length and the otion to otion breath in both male and female were <75% of the length. Overall, 28 linear measurements of both the sexes were statistically significant (p < 0.05; <0.01). The calculated indices revealed that the foramen magnum index in the female gaur were significantly higher. In calculated cranial indices the facial index (a) was higher in female and facial index (b) were higher in males. The two important parameters, facial breadth in facial index (a) and the greatest breadth in facial index (b) were positively correlated, though facial index (a) was statistically not significant between the sexes. The greater inner length of the foramen magnum in female skull resulted in foramen being oval whereas it was circular in males. These parameters were decisive for sexual dimorphism, skull comparison and craniological studies. This study ascertained that the frontal index and skull index had no significant influence and were not good indices for discriminating skulls between male and female. Based on the Principal Component Analysis, it was found that skull of male and female gaurs exhibits differences in cranial morphology viz. cranial profile length or total length (PL) and the least inner height of the temporal groove (LIHT). The findings of the present study provide baseline information on various craniometrical measurements of skull of gaur, indices and parameters for sex identification that can be effectively used in understanding sex biased predation ecology, provide base line information to describe variation across its geographic range, and in identifying skulls recovered in wildlife offence cases.

Evaluation of Craniometric Parameters and Efficacy of Posterior Surgical Intervention Types in Basilar Invagination Patients.

OBJECTIVE: Basilar invagination is one of the most frequently observed abnormalities at the craniovertebral junction, in which the odontoid process of C2 prolapses into the foramen magnum. METHODS: The current study included 27 patients who underwent surgery for basilar invagination between October 2013 and January 2023. The study group was divided into 2 groups according to basilar invagination types; type I (the presence of type A atlantoaxial instability and instability is the main pathology) and type II (the presence of type B and C atlantoaxial instability and skull base dysgenesis is the main pathology). Craniometric parameters included in the study were atlantodental interval, posterior atlantodental interval, Chamberlain's line violation, clivus-canal angle, Welcher's basal angle, and Boogaard angle. RESULTS: The mean age of the patients was 24.30 ± 14.36 years (5-57 years). Fourteen patients (51.9%) were female, and 13 patients (48.1%) were male. Ten patients (37%) had type I basilar invagination, and 17 patients (63%) had type II basilar invagination. Preoperative and postoperative atlantodental interval and Boogaard angle were significantly higher in type I basilar invagination, as preoperative and postoperative posterior atlantodental interval and clivus-canal angle were significantly higher in type II basilar invagination. There was a positive strong correlation between Chamberlain's line violation and Boogaard angle. Postoperative Chamberlain's line violation was significantly higher in occipitocervical fixation (P = 0.035). C1 lateral mass screw fixation was found more successful in Chamberlain's line violation correction than occipital plates. Occipitocervical fixation was found to be associated with higher postoperative Nurick scores (P = 0.015) and complication rates (P = 0.020). Cages applied to the C1-C2 joint space were found to be associated with higher fusion rates (P = 0.023) and lower complication rates (P = 0.024). CONCLUSIONS: In the present study, it was found that C1-C2 fixation was more successful in correcting craniometric parameters and had lower complication rates than occipitocervical fixation. In appropriate patients, it was determined that cage application increased the success rates of the operations.

The Crocodylian proatlas functions to redistribute venous blood and cerebrospinal fluid.

The proatlas, a bone located between the skull and the neural spines of the cervical vertebrae, is best known from reptiles. Most previous studies of the proatlas have centered on its developmental, debating the relationship between the proatlas and the cervical neural arches. The present study was intended as a description of the proatlas in the American alligator (Alligator mississippiensis) and an experimental test of its hypothesized role in venous blood and cerebrospinal fluid (CSF) distribution. In Alligator, the proatlas is chevron-shaped; ventrally it has a loose connection to the dorsal surface of the first cervical vertebrae, dorsally it has a robust elastic tissue tether on the otoccipital and supraoccipital bones. The ventral surface of the proatlas parallels the dorsal margin of the foramen magnum and rests on the dorsal surface of the spinal venous sinus. Experimental manipulation of the proatlas demonstrated that displacement of the proatlas causes pressure changes in both the spinal venous sinus and the enclosed spinal CSF. The results of this study represent the first demonstration of an explicit functional role for the proatlas, the circulation of fluids between the cranial and spinal compartments of the central nervous system.

Dysphagia as a primary manifestation of basilar impression: A case report.

Secondary basilar invagination or basilar impression is an anomaly at the craniovertebral junction where the odontoid process prolapses into the foramen magnum with the risk of compressing adjacent structures and obstructing the proper flow of cerebrospinal fluid (CSF). The incidence is less than 1% in the general population and occurs mainly in the first three decades of life when it is associated with malformations of the neuroaxis. In older age, the main aetiologies are diseases that alter bone mineral density. The clinical course is usually progressive and the most common symptoms are asthenia, cervical pain and restricted movement, but also dysphonia, dyspnoea and dysphagia. It is a progressive disease which, if left untreated, can cause severe neurological damage and death. We report the case of a 79-year-old woman with osteoporosis and progressive dysphagia leading to severe malnutrition, which conditioned the decision not to intervene due to the high perioperative risk.

Comparison of craniometric measurements of New Zealand rabbit (Oryctolagus cuniculus L.) using three-dimensional scanner with digital calliper measurements: A methodological study.

As technology has developed in recent years, the use of three-dimensional (3D) scanners and printers has become widespread in the medical field. However, since this field is new, all kinds of methodological and experimental related studies gain importance. This study aimed to identify the differences between the calliper measurements by determining the craniometric data on the models constructed by scanning the crania of New Zealand Rabbits (Oryctolagus cuniculus L.), preferred as experimental animals, with a three-dimensional scanner. Therefore, a total of 12 New Zealand rabbits including 6 females and 6 males were used. After the crania that comprised the study material were macerated, they were subjected to 3D scanning. After the scanning process was completed, they were craniometrically measured both on the scanned models and by using a digital calliper. Analysis of the craniometric data of the 3D scanner showed that there was a difference between sexes at the level of p < 0.05 in widest length between the external acoustic meatus (WLBEAM), skull width and Foramen magnum height (FMH) parameters and cranial index data, and at the level of p < 0.001 in the largest nasal width (LNW) parameter. A statistical difference was found between sexes in frontal length, WLBEAM, LNW and FMH parameters and cranial index values in craniometric data collected with the digital calliper (p < 0.05). Consequently, the data collected in this study were found to be close to each other in both methods, suggesting that the 3D scanner may be used in morphometric studies.

Retroflexed dens in paediatric Chiari 1 patients and implications: Single centre retrospective study.

INTRODUCTION: Chiari 1 malformation and hind brain hernia can be associated with skull base and craniocervical anomalies. One of the more recently associated anomalies is a retroverted or retroflexed odontoid process or dens. We conducted a retrospective study of our operated symptomatic and conservatively managed asymptomatic Chiari cohort to assess the impact of dens retroflexion on rate of revision or cerebrospinal fluid diversion following primary foramen magnum decompression (FMD). METHODS: We undertook a retrospective study of all foramen magnum decompression (FMD) cases for Chiari type 1 malformation performed over a 15-year period in a single tertiary paediatric neurosurgical unit. For comparison, non-operated asymptomatic Chiari cases were considered as reference cohort. Information gathered included: demographics, age, sex, length of cerebellar tonsils below McRae's line, pB-C2 distance (a line drawn perpendicular to one drawn between the basion and the posterior aspect of the C2 body), angle of retroflexion (angle formed between a line drawn through the odontoid synchondrosis and its intersection with a line drawn from the tip of the odontoid process) and angle of retroversion (angle formed between the line drawn from the base of C2 and its intersection with a line drawn from the tip of the odontoid process). Grade of retroflexion was measured using pre-operative mid-sagittal MR images and classified as grade 0 (> 90°), grade 1 (85°-89°); grade 2 (80°-84°) and grade 3 (< 80°). The rates for redo surgery or need for cerebrospinal fluid (CSF) diversion were obtained from clinical records and compared in the operated and non-operated groups. RESULTS: One hundred twenty-six Chiari 1 patients were included in this study with adequate imaging. Sixty-five patients were in the non-operated asymptomatic cohort with 61 patients in the operated symptomatic cohort. Mean age of non-operated cohort was 10.2 years with M:F ratio (30:35). Mean cerebellar tonsillar length below McRae's line was 10.3 mm. 7.7% of this cohort had associated syrinx. Mean angles of retroversion and retroflexion were 76 and 78°, respectively. Retroflexion grades included (9.2% grade 1, 35% grade 2 and 52.3% grade 3). pB-C2 distance was 6.8 mm. Mean age of operated cohort was 11.3 years, with M:F ratio (21:40). Mean cerebellar tonsillar length below McRae's line was 15 mm. 45.9% of this cohort had associated syrinx. Mean angles of retroversion and retroflexion were 73 and 74.5°, respectively. Retroflexion grades included (4.9% grade 1, 16.5% grade 2 and 78.6% grade 3). pB-C2 distance was 6.9 mm. No association was identified between retroflexion grade and rate of revision or CSF diversion following primary foramen magnum decompression. CONCLUSION: The operated Chiari 1 cohort had more retroflexed dens, longer tonsils and associated syrinx compared to the non-operated asymptomatic cohort.

Genetic characterization and mutational profiling of foramen magnum meningiomas: a multi-institutional study.

OBJECTIVE: Foramen magnum (FM) meningiomas pose significant surgical challenges and have high morbidity and mortality rates. This study aimed to investigate the distribution of clinically actionable mutations in FM meningiomas and identify clinical characteristics associated with specific mutational profiles. METHODS: The authors conducted targeted next-generation sequencing of 62 FM meningiomas from three international institutions, covering all relevant meningioma genes (AKT1, KLF4, NF2, POLR2A, PIK3CA, SMO, TERT promoter, and TRAF7). Patients with a radiation-induced meningioma or neurofibromatosis type 2 (NF2) were excluded from the study. Additionally, patient and tumor characteristics, including age, sex, radiological features, and tumor location, were retrospectively collected and evaluated. RESULTS: The study cohort consisted of 46 female and 16 male patients. Clinically significant driver mutations were detected in 58 patients (93.5%). The most commonly observed alteration was TRAF7 mutations (26, 41.9%), followed by AKT1E17K mutations (19, 30.6%). Both mutations were significantly associated with an anterolateral tumor location relative to the brainstem (p = 0.0078). NF2 mutations were present in 11 cases (17.7%) and were associated with posterior tumor location, in contrast to tumors with TRAF7 and AKT1E17K mutations. Other common mutations in FM meningiomas included POLR2A mutations (8, 12.9%; 6 POLR2AQ403K and 2 POLR2AH439_L440del), KLF4K409Q mutations (7, 11.3%), and PIK3CA mutations (4, 6.5%; 2 PIK3CAH1047R and 2 PIK3CAE545K). POLR2A and KLF4 mutations exclusively occurred in female patients and showed no significant association with specific tumor locations. All tumors harboring AKT1E17K and POLR2A mutations displayed meningothelial histology. Ten tumors exhibited intratumoral calcification, which was significantly more frequent in NF2-mutant compared with AKT1-mutant FM meningiomas (p = 0.047). CONCLUSIONS: These findings provide important insights into the molecular genetics and clinicopathological characteristics of FM meningiomas. The identification of specific genetic alterations associated with tumor location, volume, calcification, histology, and sex at diagnosis may have implications for personalized treatment strategies in the future.

Preoperative estimation of intracranial compliance in symptomatic children with Chiari malformation type 1: impact on outcome and risk of complications.

BACKGROUND: The role of reduced intracranial compliance (ICC) in the outcome after foramen magnum decompression (FMD) was demonstrated in adults with Chiari malformation Type 1 (CMI). However, similar observations from children treated for CMI are missing. METHODS: We reviewed pediatric cases of CMI referred to FMD between 2006 and 2022. Children with clinical and/or radiological signs suggesting reduced ICC (Group A) underwent overnight measurements of the pulsatile intracranial pressure (ICP): mean ICP wave amplitude (MWA) served as a surrogate marker of ICC. Children with more typical symptoms of CMI (Group B) underwent FMD without preoperative ICC estimation. This study presents the clinical, radiological, and outcome differences between these groups. RESULTS: Sixty-four children (mean age 11.1 ± 4.3 years) underwent FMD: In Group A (n = 30), the finding of reduced ICC as estimated from preoperative ICP measurement resulted in CSF diversion (ventriculoperitoneal shunt) before FMD in 11 children. Two patients required shunt due to complications after FMD (total shunt rate 43%). In Group B (n = 34) treated with FMD without preoperative ICC estimation, five children (15%) required shunting due to complications. In Group A, we found a significantly higher frequency of headache, nausea, fatigue, and dizziness. The outcome assessed by the modified Chicago Chiari Outcome Scale (mean follow-up 83 ± 57 months) was comparable between the groups, but the complication rate after FMD was significantly lower in Group A (7% vs. 32%; p = 0.011). The number of procedures (ICP measurement, FMD, shunt, re-do FMD, shunt revisions) was significantly higher in Group A (2.6 ± 0.9 vs. 1.5 ± 1.1 per patient; p < 0.001). CONCLUSION: In symptomatic children with CMI, the preoperative estimation of ICC from the overnight measurement of pulsatile ICP was more reliable for identifying those with reduced ICC than clinical and radiological assessment alone. When children with abnormally reduced ICC were identified and treated with CSF diversion before FMD, the complication rate was significantly reduced.

[Clinical evaluation methods for craniovertebral junction abnormalities].

Craniovertebral junction malformation is a congenital malformation located in the foramen magnum and upper cervical spine, including bone and nerve malformation, resulting in motor and sensory disorders, cerebellar and lower cranial nerves, etc. The evaluation methods of clinical symptoms and efficacy of craniovertebral junction malformation are important for the surgical indications and effects, mainly including the evaluation of clinical symptoms and the quality of life. At present, the commonly used methods in clinical work and literature are the Japanese orthopaedic association scores, visual analogue scales, 36-item short-form health survey, etc. Most of these clinical evaluations are not aimed at craniovertebral junction diseases but focus on the description of a certain type of clinical symptoms. Chicago Chiari outcome scale and syringomyelia outcome scale of Xuanwu hospital are dedicated to Craniovertebral junction malformation, but more clinical studies are needed to prove their effectiveness. Based on the literature reports, this article reviewed the previous clinical evaluation methods of craniovertebral junction malformation and discusses their applications and limitations.