scholarly journals Craniofacial and Craniocervical Features in Cartilage-Hair Hypoplasia: A Radiological Study of 17 Patients and 34 Controls

2021 ◽  
Vol 12 ◽  
Author(s):  
Heidi Arponen ◽  
Marjut Evälahti ◽  
Outi Mäkitie
Keyword(s):  
Skull Base ◽  
Cranial Base ◽  
Basilar Invagination ◽  
Craniocervical Junction ◽  
Skeletal Class ◽  
Base Angle ◽  
Cartilage Hair Hypoplasia ◽  
Skeletal Class Ii ◽  
Anterior Cranial Base ◽  
Age Range

BackgroundBiallelic mutations in the non-coding RNA gene RMRP cause Cartilage-hair hypoplasia (CHH), a rare skeletal dysplasia in which the main phenotypic characteristic is severe progressive growth retardation.ObjectiveThis study compared the cranial dimensions of individuals with CHH to healthy subjects.MethodsLateral skull radiographs of 17 patients with CHH (age range 10 to 59 years) and 34 healthy individuals (age range 10 to 54 years) were analyzed for relative position of the jaws to skull base, craniofacial height and depth, as well as vertical growth pattern of the lower jaw, anterior cranial base angle, and the relationship between the cervical spine and skull base.ResultsWe found that the length of the upper and lower jaws, and clivus were significantly decreased in patients with CHH as compared to the controls. Anterior cranial base angle was large in patients with CHH. Basilar invagination was not found.ConclusionThis study found no severe craniofacial involvement of patients with CHH, except for the short jaws. Unexpectedly, mandibular deficiency did not lead to skeletal class II malocclusion.Clinical ImpactAlthough the jaws were shorter in patients with CHH, they were proportional to each other. A short posterior cranial base was not associated with craniocervical junction pathology.

scholarly journals Cranial base pathology in pediatric osteogenesis imperfecta patients treated with bisphosphonates

2015 ◽  
Vol 15 (3) ◽  
pp. 313-320 ◽  
Author(s):  
Heidi Arponen ◽  
Ilkka Vuorimies ◽  
Jari Haukka ◽  
Helena Valta ◽  
Janna Waltimo-Sirén ◽  
...  
Keyword(s):  
Osteogenesis Imperfecta ◽  
Cranial Base ◽  
Basilar Invagination ◽  
Craniocervical Junction ◽  
Normative Values ◽  
Bisphosphonate Treatment ◽  
Basilar Impression ◽  
Duration Of Treatment ◽  
Cross Sectional ◽  
Kaplan Meier

OBJECT Cranial base pathology is a serious complication of osteogenesis imperfecta (OI). Our aim was to analyze whether bisphosphonate treatment, used to improve bone strength, could also prevent the development of craniocervical junction pathology (basilar impression, basilar invagination, or platybasia) in children with OI. METHODS In this single-center retrospective study the authors analyzed the skull base morphology from lateral skull radiographs and midsagittal MR images (total of 94 images), obtained between the ages of 0 and 25 years in 39 bisphosphonate-treated OI patients. The results were compared with age-matched normative values and with findings in 70 OI patients who were not treated with bisphosphonates. In addition to cross-sectional data, longitudinal data were available from 22 patients with an average follow-up period of 7.6 years. The patients, who had OI types I, III, IV, VI, and VII, had been treated with zoledronic acid, pamidronate, or risedronate for 3.2 years on average. RESULTS Altogether 33% of the 39 bisphosphonate-treated patients had at least 1 cranial base anomaly, platybasia being the most prevalent diagnosis (28%). Logistic regression analysis suggested a higher risk of basilar impression or invagination in patients with severe OI (OR 22.04) and/or older age at initiation of bisphosphonate treatment (OR 1.45), whereas a decreased risk was associated with longer duration of treatment (OR 0.28). No significant associations between age, height, or cumulative bisphosphonate dose and the risk for cranial base anomaly were detected. In longitudinal evaluation, Kaplan-Meier curves suggested delayed development of cranial base pathology in patients treated with bisphosphonates but the differences from the untreated group were not statistically significant. CONCLUSIONS These findings indicate that cranial base pathology may develop despite bisphosphonate treatment. Early initiation of bisphosphonate treatment may delay development of craniocervical junction pathology. Careful followup of cranial base morphology is warranted, particularly in patients with severe OI.

scholarly journals Endoscopic endonasal resection of midline cranial base tumors

2018 ◽  
Vol 19 (2) ◽  
pp. 7-17
Author(s):  
Enrico De Divitiis ◽  
Felice Esposito ◽  
Paolo Cappabianca ◽  
Luigi M. Cavallo ◽  
Oreste De Divitiis ◽  
...  
Keyword(s):  
Skull Base ◽  
Cranial Base ◽  
Transsphenoidal Approach ◽  
Csf Leak ◽  
Endoscopic Endonasal ◽  
Visual Deficits ◽  
Extended Transsphenoidal Approach ◽  
Anterior Cranial Base ◽  
Subtotal Removal

Objective: The advent of the endoscope in transsphenoidalsurgery has permitted to expand the indications of such approach also for the treatment of on tumors located in supra, para, retro and infrasellar regions, enabling the neurosurgeon to work under direct visual control in a minimally invasive way. Since 2004 we have started to use the extended endonasal transsphenoidal approach for a variety of lesions involving the midline skull base and, in particular, the suprasellar area, the cavernous sinus and the retroclival prepontine region. Methods: Over a 36-month period, sixty-four procedures have been performed. The series consisted of 29 males and 35 females, aged from 24 to 80 years (median 49.8 years). The mean follow-up was of 18 months (ranging from 3 to 36 months). Among the patients with midline lesions, who were 90.6 % of the total, seven patients had a pituitary adenoma, sixteen patients were affected by a craniopharyngioma, six patients had a suprasellar Rathke’s cleft cyst, seven subjects had a tuberculum sellae meningioma, four had an olfactorygroove meningioma, and six a clival tumor. Other lesions ofthe midline skull base were, 1 chiasmatic astrocytoma, 1 neuroendocrine tumor, 4 post-traumatic cerebro-spinal fluid rhinorrhea, and one optic nerve glioma. Three other patients had anterior cranial base meningoencephaloceles. Results: Overall, gross total removal of the lesion was achieved in 30/49 tumoral lesions (61.2%); subtotal removal was achieved in 12/49 cases (24.5%). The three cases of meningoencephaloceles were all successfully treated. Among the patients with preoperative visual deficits, most of them fully recovered or improved and only two worsened in one eye. Major complications consisted in 2 deaths (one not directly related with the surgical procedure), 6 postoperative CSF leak (one complicated with bacterial meningitis), one ICA injury, and 6 cases of permanent diabetes insipidus.Conclusion: The extended transsphenoidal approach tothe supra and parasellar lesions seems Endoscopy; Transsphenoidal surgery; Extended approach; Parasellar; Tumors; Anterior skull base. A promising minimally invasivetechnique for the removal of lesions affecting these areas,once thought to be suitable only of the transcranial routes.Concerning the lesion removal and the recurrence rate compared with the transcranial routes, it is too early to pose a definitive word, since the follow-up is still too short.

Vascularized Pericranial Flap for Endonasal Anterior Skull Base Reconstruction

2021 ◽  
Author(s):  
Jonathan Giurintano ◽  
Michael W. McDermott ◽  
Ivan H. El-Sayed
Keyword(s):  
Minimally Invasive ◽  
Skull Base ◽  
San Francisco ◽  
Middle Turbinate ◽  
Cranial Base ◽  
Anterior Skull Base ◽  
Craniofacial Resection ◽  
Skull Base Reconstruction ◽  
Pericranial Flap ◽  
Anterior Cranial Base

Abstract Importance As the limits of advanced skull base malignancies that can be managed through an endoscopic endonasal approach continue to be expanded, the resultant anterior skull base defects are of increasing size and complexity. In the absence of nasoseptal or turbinate flaps, the vascularized pericranial flap has been employed at our institution with excellent results. Objective The study aimed to review the outcomes of patients who underwent endonasal anterior craniofacial resection with anterior skull base reconstruction using a vascularized pericranial flap. Design Retrospective chart review of patients treated by the University of California – San Francisco minimally invasive skull base service from the years 2011 to 2017. Average duration of follow-up was 16.4 months. Setting This study was conducted at Academic tertiary referral center. Participants A total of nine patients with advanced anterior cranial base malignancies were identified who were treated with a minimally invasive, endoscopic anterior craniofacial resection from the years 2011 to 2017. Due to the nature of the resection in these patients, nasoseptal flaps and inferior/middle turbinate flaps were unavailable or insufficient for anterior skull base defect repair. Each patient underwent reconstruction of the anterior cranial base defect using an anteriorly based pericranial flap harvested by bicoronal incision, and tunneled anteriorly to the nasal cavity through a frontoethmoidal incision.

Osteomalacia Caused by Skull Base Tumors: Report of 2 Cases

Neurosurgery ◽  
2011 ◽  
Vol 69 (1) ◽  
pp. E239-E244 ◽  
Author(s):  
Takeshi Uno ◽  
Kensuke Kawai ◽  
Naoto Kunii ◽  
Seiji Fukumoto ◽  
Junji Shibahara ◽  
...  
Keyword(s):  
Skull Base ◽  
Muscle Power ◽  
Cranial Base ◽  
Whole Body ◽  
Entire Body ◽  
Whole Body Imaging ◽  
Skull Base Tumors ◽  
En Bloc ◽  
True Incidence ◽  
Anterior Cranial Base

Abstract BACKGROUND AND IMPORTANCE: Tumor-induced osteomalacia (TIO) is an uncommon paraneoplastic syndrome rarely encountered in neurosurgical practice. We report on 2 cases of TIO caused by skull base tumors. Although the diagnosis of TIO is difficult to make and often is delayed because of the insidious nature of the symptoms, mostly systemic pain and weakness, it is curable once it is diagnosed and properly treated. CLINICAL PRESENTATION: Both patients presented with severe pain developing in the lower extremities and moving out to the entire body, as well as difficulty moving. They were diagnosed with TIO several years after onset. A high level of serum FGF23 was confirmed, and whole-body imaging studies demonstrated tumors in the middle and anterior cranial base, respectively. The patient with the anterior cranial base tumor had a history of hemorrhage into the frontal lobe and partial resection. En bloc resection of tumor with surrounding skull bone was performed. The histological diagnosis for both cases was phosphaturic mesenchymal tumor, mixed connective tissue variant. CONCLUSION: The level of FGF23 normalized immediately after surgery. Both patients experienced a dramatic relief of pain and recovery of muscle power. Although reports of osteomalacia caused by tumors in the neurosurgical field are extremely rare in the literature, its true incidence is unknown. We emphasize the importance of recognition of this syndrome and recommend total resection of tumors when possible.

scholarly journals Cephalometric Evaluation Of The Size And Morphology Of Sella Turcica In Different Types Of Malocclusion Among Romanian Subjects

2016 ◽  
Vol 12 (21) ◽  
pp. 1
Author(s):  
Irinel Panainte ◽  
Reka Gyergyay ◽  
Krisztina Martha
Keyword(s):  
Sella Turcica ◽  
Cranial Base ◽  
Class I ◽  
Base Length ◽  
Class Iii ◽  
Linear Measurements ◽  
Mean Values ◽  
Skeletal Class ◽  
Skeletal Class Ii ◽  
Different Types

Introduction: The vertical and sagital position of the maxilla and mandible is influenced by the size and the angulation of the cranial base. Sellae turcica is part of the cranial base. It is located in the middle cranial fossa. Thus, the growth and the development of this bony structure are influenced by neural and general skeletal pattern as well. Cephalometric analysis is an important part of orthodontic diagnosis and treatment planning. From numerous cephalometric landmarks, the S- sellae point is commonly used to describe the cranial base. Also, it is used to evaluate other bony structures’ position towards it. Objective: The purpose of this study is to evaluate the shape and the dimension of the sellae turcica in different types of malocclusions. Materials and Methods: 136 randomly selected lateral cephalometric radiographs were analyzed. Also, skeletal and facial pattern was identified and the shape and sagital dimension of the sellae was measured. Results: Statistical analysis presented no significancy regarding sellae’s shape in different types of malocclusion. However, the skeletal class II cases presented the most anarchic sellae shapes. Comparing linear measurements of skeletal length and sellae diameter, we found that the smallest diameter of the sellae appears in class III malocclusions. Thus, other skeletal length presents the lowest mean values also. Statistically significant differences among maxillary, mandibular, and cranial base length and sellae diameter were found in class I malocclusion (p=0.013). Conclusions: Sella morphology appears to have certain correlation with cranial and jaw base length and jaw base relationship in skeletal Class I Romanian population

scholarly journals Evaluation of Cranial Base Morphology of Pakistani Population in Skeletal Class I, II and III Malocclusions

2019 ◽  
Vol 9 (2) ◽  
pp. 43-46
Author(s):  
Faizan Alia ◽  
Rabia Aziz ◽  
Amna Malik ◽  
Hadiqa Afzal
Keyword(s):  
Pearson Correlation ◽  
Class Ii ◽  
Cranial Base ◽  
Class I ◽  
Base Length ◽  
Class Iii ◽  
Skeletal Class ◽  
Anterior Cranial Base ◽  
Angular Measurements ◽  
Jaw Position

 Introduction: Cranial Base integrate different patterns of growth in various regions of the skull such as the nasal cavity, the oral cavity, and the pharynx. Anteroposterior jaw position is thought to be affected by cranial base growth. Although scientific literature shows conflicting results regarding both positive and negative correlation between the cranial base and skeletal malocclusions. Materials & Method: 138 patients selected according to the Inclusion criteria were divided into three major categories depending upon ANB angle. Class I, class II group and class III group. Pretreatment lateral cephalometric radiographs were taken. Linear and Angular measurements were measured. All the data was entered in SPSS version 25 and then analyzed by using descriptive statistics including mean, standard deviation and by using inferential statistics including Pearson correlation and ANOVA test. Result: No significant differences were recorded between cranial base deflection angles (N-S-Ar and N-S-Ba) and malocclusions groups while Anterior cranial base length S-N was found to be maximum in class III and posterior cranial base length S-Ba was greater in class I. Conclusion: Cranial base angles N-S-Ar and N-S-Ba did not demonstrate any statistically significant correlation with three malocclusion groups i.e. Class I, Class II and Class III and their correlation was negative.

Primary dural closure and anterior cranial base reconstruction using pericranial and nasoseptal multi-layered flaps in endoscopic-assisted skull base surgery

2014 ◽  
Vol 156 (10) ◽  
pp. 1911-1915 ◽  
Author(s):  
Ryosuke Tomio ◽  
Masahiro Toda ◽  
Toshiki Tomita ◽  
Masaki Yazawa ◽  
Maya Kono ◽  
...  
Keyword(s):  
Skull Base ◽  
Skull Base Surgery ◽  
Cranial Base ◽  
Dural Closure ◽  
Anterior Cranial Base

scholarly journals Cranial Base Features in Skeletal Class III Patients

2008 ◽  
Vol 78 (3) ◽  
pp. 433-439 ◽  
Author(s):  
Peter Proff ◽  
Florian Will ◽  
Ivan Bokan ◽  
Jochen Fanghänel ◽  
Tomas Gedrange
Keyword(s):  
Cranial Base ◽  
Class I ◽  
Base Length ◽  
Class Iii ◽  
Skeletal Class ◽  
Anterior Cranial Base ◽  
Condylar Displacement ◽  
Mandibular Protrusion

Abstract Objective: To investigate the cranial base configuration in skeletal Class III patients to clarify the conflicting findings from literature. Materials and Methods: Initial lateral radiographs of 54 skeletal Class III patients and 54 matched controls (Class I, II/1, II/2) aged 14 to 24 years were analyzed retrospectively for 21 cephalometric basicranial variables and jaw lengths relative to anterior cranial base length. Results: In contrast to overall cranial base length, the anterior (N-S) and posterior (S-Ba, S-Ar) sections failed to show a significant reduction in Class III patients. The significantly more acute angles Ca-S-Ba and Se-S-Ba reflected increased cranial base flexure. Resulting anterior condylar displacement was shown by significant reduction of Se-S-Cd and Ar-Ca. Relative mandibular length was significantly increased. Conclusions: Decreased basicranial angulation associated with Class III mandibular protrusion was clearly confirmed for skeletal Class III patients. Overall shortening of the cranial base apparently resulted from various minor alterations. The results are compatible with the deficient orthocephalization hypothesis of Class III morphogenesis. The basicranial-maxillary relationship in skeletal Class III remains unclear.

scholarly journals Mandibular shape prediction using cephalometric analysis: applications in craniofacial analysis, forensic anthropology and archaeological reconstruction

2020 ◽  
Vol 42 (1) ◽  
Author(s):  
Ahmed Omran ◽  
David Wertheim ◽  
Kathryn Smith ◽  
Ching Yiu Jessica Liu ◽  
Farhad B. Naini
Keyword(s):  
Cranial Base ◽  
Mean Difference ◽  
Dental Occlusion ◽  
Cephalometric Analysis ◽  
Skeletal Class ◽  
Shape Prediction ◽  
Straight Line ◽  
Anterior Cranial Base ◽  
Mandibular Body ◽  
Archaeological Reconstruction

Abstract Background The human mandible is variable in shape, size and position and any deviation from normal can affect the facial appearance and dental occlusion. Objectives The objectives of this study were to determine whether the Sassouni cephalometric analysis could help predict two-dimensional mandibular shape in humans using cephalometric planes and landmarks. Materials and methods A retrospective computerised analysis of 100 lateral cephalometric radiographs taken at Kingston Hospital Orthodontic Department was carried out. Results Results showed that the Euclidean straight-line mean difference between the estimated position of gonion and traced position of gonion was 7.89 mm and the Euclidean straight-line mean difference between the estimated position of pogonion and the traced position of pogonion was 11.15 mm. The length of the anterior cranial base as measured by sella-nasion was positively correlated with the length of the mandibular body gonion-menton, r = 0.381 and regression analysis showed the length of the anterior cranial base sella-nasion could be predictive of the length of the mandibular body gonion-menton by the equation 22.65 + 0.5426x, where x = length of the anterior cranial base (SN). There was a significant association with convex shaped palates and oblique shaped mandibles, p = 0.0004. Conclusions The method described in this study can be used to help estimate the position of cephalometric points gonion and pogonion and thereby sagittal mandibular length. This method is more accurate in skeletal class I cases and therefore has potential applications in craniofacial anthropology and the ‘missing mandible’ problem in forensic and archaeological reconstruction.

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