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Osteogenesis Imperfecta (OI) is a genetic disease characterized by osteopenia and bone fragility in which the craniocervical junction is also affected. This is of special relevance due to the high prevalence in anomalies described in the literature as follows: basilar invagination, basilar impression, and platybasia. Methods. We analyzed 19 lateral skull radiographs and 14 magnetic resonance images (MRIs) of 28 patients with OI in which eight linear and five angular measurements were plotted to determine the existence of craniocervical junction anomalies and compare them with 38 lateral skull radiographs and 28 MRIs performed on age-matched healthy controls. Results. From the reference values obtained from the control sample for each age group, we established the limit value at which pathology could be suspected. Some of the variables studied showed a clear trend associated with growth. More than half of the patients (60.71%) presented an anomaly in the skull base. Conclusions. According to the diagnostic criteria used and taking +2.5 SD as the limit value, 10.71% of the patients had basilar invagination, 35.71% had basilar impression, and 39.29% had platybasia, the latter being the most common finding, Depto. de Especialidades Clínicas Odontológicas, Fac. de Odontología, TRUE, pub

Osteogenesis Imperfecta (OI) is a disease that causes bone fragility and deformities, affecting both the cranial base and the craniocervical junction, and may lead to other neurological disorders. A retrospective cross-sectional study was carried out based on cephalometric analysis of the cranial base in a sample of patients with OI, in lateral skull radiographs and magnetic resonance imaging (MRI), comparing them with a sample of age-matched controls. When the different variables of the craniocervical junction were analyzed, significance was found in comparisons with the different age groups. All measurements of the variables studied stabilized as growth progressed. For most of the variables, the severity of the disease influences the measurements of the skull base, with statistically significant differences. Both age and severity of the disease are factors that directly influence the anatomy of the craniocervical junction in these patients and may serve as indicators in the early detection and prevention of other derived alterations., Depto. de Especialidades Clínicas Odontológicas, Fac. de Odontología, TRUE, pub

[Objective] Malformations in the craniocervical junction (CCJ) are rare in the pediatric population but often need surgical treatment. We present a pediatric case series of patients treated with a 2-stage surgical approach with a halo vest and occipitocervical fusion and review complications and outcomes., [Methods] A retrospective analysis of a single-center case series was performed. Pediatric patients affected by congenital craniocervical junction anomalies and treated with a 2-stage approach were included. A halo vest was implanted in the first surgery, and ambulatory progressive reduction was performed. When a favorable anatomic situation was observed, arthrodesis was performed. Safety analysis was undertaken by analyzing the incidence of complications in both procedures. Effectivity analysis was carried out analyzing radiologic and clinical outcome (Goel grade and modified Japanese Orthopaedic Association score). Student t test was used for statistical analysis., [Results] Sixteen cases were included. Mean age of patients was 9.38 years. Safety analysis showed 2 halo loosenings, 1 pin infection, 2 wound infections, 1 cerebrospinal fluid leak, and 2 delayed broken rods. No major complications were observed. Radiologic analysis showed an improvement in the tip of the odontoid process to the McRae line distance (from −3.26 mm to −6.16 mm), atlantodental interval (from 3.05 mm to 1.88 mm), clival-canal angle (from 134.61° to 144.38°), and cervical kyphosis (from 6.39° to 1.54°). Clinical analysis also showed improvement in mean Goel grade (from 1.75 to 1.44) and modified Japanese Orthopaedic Association score (from 15.12 to 16.41)., [Conclusions] The 2-stage approach was a suitable and effective treatment for craniocervical junction anomalies in pediatric patients.

Background: The extreme lateral approach is a direct lateral approach which allows a good control of the entire length of the vertebral artery (VA), the jugular foramen, the lowest cranial nerves, and the jugular–sigmoid complex. Herein we try to exploit the variants of the approach and we identify indications, advantages, and disadvantages. Methods: All phases of the study were conducted at the Institute of Public Health Section of Legal Medicine and Insurance of the University. We performed the extreme lateral approach in four subjects, who died between 24 and 48 h before in non-traumatic circumstances (three men and one woman). Results: The great auricular nerve, the spinal accessory, the branches of the first ventral spinal nerves, the jugular vein, and the vertebral artery were identified in all the cadavers. In all cases the right VA exited from the transverse foramen of C1. The site of SCM piercing the accessory nerve was at a distance from the tip of the mastoid between 3 and 4 cm (3.3 in one case, 3.4 in 2 cases, and 3.7 in one case). No vessels and nerves have been damaged after being identified and isolated. Conclusions: Extradural lesions at the ventro-lateral aspect of the CVJ may require an extreme lateral approach, a procedure more aggressive comparing with far lateral approach, which represents a reasonable option for large anterior and anterolateral lesions when greater exposure is required.

Purpose: We propose an magnetic resonance imaging (MRI)-based quantitative morphovolumetric approach to the posterior cranial fossa (PCF) and craniocervical junction (CCJ) changes in achondroplastic patients investigating possible associations with ventriculomegaly and medullary compression. Methods: We analyzed MRI of 13 achondroplastic children not treated by surgery. 3D FSPGR T1-weighted images were used to analyze (1) PCF synchondroses; (2) PCF volume (PCFV), PCF brain volume (PCFBV), PCFV/PCFBV ratio, cerebellar volume, cerebrospinal fluid (CSF) spaces volume, and IV ventricle volume; (3) PCF (clivus, supraocciput, exocciput lengths, tentorial angle) and CCJ (AP and LL diameters of foramen magnum (FM)) morphometry; (4) measurements of FM and jugular foramina (JF) areas; and (5) supratentorial ventricular volume. Results: All patients showed synostosis of spheno-occipital synchondroses, eight showed synostosis of intra-occipital synchondroses, nine showed CCJ impingement on the cervical cord but only three had cervical myelopathy. Compared to controls, clivus and exocciput lengths, LL and AP diameters of FM, FM area and JF area were significantly reduced, supraocciput length, tentorial angle, PCFV, PCFBV, cerebellar volume, supratentorial ventricular system volume were significantly increased. A correlation was found between clivus length and supratentorial ventricular volume, premature closure of intra-occipital synchondroses and FM area while a trend was found between FM area and supraocciput length. Conclusion: Our analysis demonstrates a relationship between the shortening of the clivus and the ventriculomegaly. On the other hand the premature closure of PCF synchondroses, the shape, and the growth direction of supraocciput bone contribute to reduce the FM area, causing in some patients medullary compression.

Uvod: Interesovanje za kraniocervikalni prelaz (engl. „the craniocervical junction“ – CCJ) započelo je disekcijama i izvanrednim crtežima Leonarda da Vinčija, a nastavljeno je ispitivanjima Andreasa Vezalijusa u 16. veku, kao i italijanskih anatoma i umetnika u 17. i 18. stoleću (Saunders, 1973; Persaud, 1984; Düring and Poggesi, 2006; Nathan, 2007). Veliki doprinos dali su Buržer u 19. veku i Pernkopf u 20. stoleću (Pernkopf, 1963; Bourgery i Jacob, 2005). Ipak, najdetaljnija istraživanja ovog područja obavljena su u poslednjih nekoliko decenija (Martin i sar., 2010; Steinmetz i sar., 2010; Lopez i sar., 2015). Kraniocervikalni prelaz obuhvata, u najužem smislu, donji deo okcipitalne kosti, atlas i aksis. Spomenuti autori proučili su ove osteološke komponente i izvršili odgovarajuća merenja, ali ne u potpunosti. Zato smo odlučili da CCJ ispitamo mnogo detaljnije u anatomskom i radiološkom domenu, kako bismo dali doprinos neurohirurškim intervencijama u ovom području. Ciljevi: 1) precizno utvrđivanje anatomskih odnosa osteoloških komponenti CCJa, uključujući i morfometrijska ispitivanja, kao i proučavanja obližnjih neuralnih struktura, naročito odnosa kičmene moždine, moždanog stabla i tonzila cerebeluma; 2) utvrđivanje odnosa spomenutih elemenata nakon maksimalne fleksije i ekstenzije, u poređenju sa neutralnim položaj glave i vrata; 3) utvrđivanje patoloških poremećaja i urođenih malformacija komponenti CCJa; 4) poboljšanje neurohirurških intervencija u predelu CCJ u svetlosti dobijenih podataka tokom naših istraživanja. Materijal i metode: Korišćene su, najpre, standardne anatomske metode na 22 uzorka okcipitalne kosti, 7 atlasa i 7 aksisa, uključujući precizna morfometrijska proučavanja. Druga grupa uzoraka obuhvatala je tri glave s mozgom prethodno fiksiranih u 10% formalinu. Nakon zamrzavanja na minus 250 C, svaka glava je serijski sečena u predelu CCJ u jednoj od tri glavne ravni: aksijalnoj, koronalnoj i sagitalnoj. Na ovim presecima ispitani su odnosi pojed, Objective: The interest in the craniocervical junction (the CCJ) started with dissections and brilliant drawings of Leonardo da Vinci, and it was continued by the examinations of Andreas Vesalius in the 16th century, and the Italian anatomists and artists in the 17th and 18th century (Saunders, 1973; Persaud, 1984; Düring and Poggesi, 2006; Nathan, 2007). A great contribution was given by Bourgery in the 19th century and Pernkopf in the 20th century (Pernkopf, 1963; Bourgery and Jacob, 2005). However, the most detailed study of this region was performed in the recent few decades (Martin et al., 2010; Steinmetz et al., 2010; Lopez et al., 2015). The craniocervical junction, in the narrower sense, comprises the lower part of the occipital bone, the atlas and the axis. The mentioned authors examined these osteological components and made certain measurements, although not fully. Therefore we decided to examine the CCJ in much more details and thus to give a contribution to the neurosurgical interventions in this region. Aims: 1) a precise determination of the anatomic relationships of the CCJ osteological components, including the morphometric examination, as well as a study of the adjacent neural structures, especially the relationships of the spinal cord, the brain stem and the cerebellar tonsils; 2) a determination of the relationships of the mentioned elements following a maximum flextion and extension, compared to the neutral position of the head and neck; 3) a determination of the pathological disorders and the congenital malformations of the CCJ components; 4) an improvement of the neurosurgical interventions in the CCJ region in the light of the data obtained in our study. Material and methods: First of all, some standard anatomic methods were applied in 22 occipital bones, 7 atlas and 7 axis specimens, including a precise morphometric examination. The second group of the specimens comprised three heads with a brain previously fixed in a 10% formaldehyde soluti

PURPOSE: Occipitocervical fusion is required when the occipitoatlantal joint is unstable. The purpose of this paper is to discuss the role of imaging in the pre- and postoperative evaluation of posterior occipitocervical fusion (POCF), focusing on contoured loop fixation by Hartshill and Songer instrumentation. MATERIALS AND METHODS: We studied 21 patients (eight males, 12 females; age range 6-70 years; mean age 32.6 years) with craniocervical instability who underwent POCF with Hartshill U-shaped rod and Songer sublaminar wires. Pre- and postoperative radiographic, computed tomography (CT) and magnetic resonance (MR) imaging examinations were performed in all patients. A 3-to 6-month period of external orthosis with halo vest, sterno-occipitalmandibular immobiliser (SOMI) brace or Philadelphia collar followed surgery. Follow-up was 12-96 (mean 53.1) months. RESULTS: Clinical assessment using the Frankel scale revealed improvement or deterioration arrest in all but two patients: one with C3 failure and halo destabilisation; the other, who had exhibited myelopathy signs on preoperative MR imaging and persistent basilar impression, showed increasing and progressive neurological deficits despite successful POCF. CONCLUSIONS: Pre- and postoperative imaging is extremely useful in patients scheduled to undergo POCF. Preoperative MR screening of basilar impression associated with possible spinal cord lesions appears mandatory to predict possible deterioration and prevent undesired failure of the operation and it may suggest the need for an alternative surgical approach, such as the transoral approach.