Crouzon Syndrome: Cephalometric Analysis and Evaluation of Pathogenesis

1994 ◽  
Vol 31 (3) ◽  
pp. 201-209 ◽  
Author(s):  
Francesco Carinci ◽  
Anna Avantaggiato ◽  
Curioni Camillo

Crouzon syndrome is a craniofaciostenosis characterized by brachycephaly, ocular proptosis, and maxillary retrusion. The hypothesis has been forwarded that an alteration in anterior cranial base synchondrosis activity is responsible for the skeleton abnormalities which are associated with this disorder. The present work was aimed at assessing this pathogenetic hypothesis. Cephalometry was used as the analysis method and care was taken in determining the three-dimensional measurements of some functional spaces (e.g., orbit, rhinopharynx, and nasal cavity). The results indicate that in Crouzon syndrome the craniofacial alterations depend not only on reduced synchondrosis activity of the anterior cranial base, but also of the posterior cranial base.

2017 ◽  
Vol 87 (6) ◽  
pp. 847-854 ◽  
Author(s):  
Juliana Macêdo de Mattos ◽  
Juan Martin Palomo ◽  
Antonio Carlos de Oliveira Ruellas ◽  
Paula Loureiro Cheib ◽  
Manhal Eliliwi ◽  
...  

ABSTRACT Objectives: To test the null hypotheses that the positions of the glenoid fossae and mandibular condyles are identical on the Class I and Class II sides of patients with Class II subdivision malocclusion. Materials and Methods: Retrospective three-dimensional (3D) assessments of the positions of the glenoid fossae and mandibular condyles were made in patients with Class II malocclusion. Relative to a fiducial reference at the anterior cranial base, distances from the glenoid fossae and condyles were calculated in pretreatment cone beam computed tomographic scans of 82 patients: 41 with Class II and 41 with Class II subdivision malocclusions. The 3D distances from glenoid fossae to sella turcica in the X (right-left), Y (anterior-posterior), Z (inferior-superior) projections were calculated. Results: Patients with Class II malocclusion displayed a symmetric position of the glenoid fossae and condyles with no statistically significant differences between sides (P > .05), whereas patients with Class II subdivision showed asymmetry in the distance between the glenoid fossae and anterior cranial base or sella turcica (P < .05), with distally and laterally positioned glenoid fossae on the Class II side. (P < .05). Male patients had greater distances between glenoid fossae and anterior cranial fossae (P < .05). The condylar position relative to the glenoid fossae did not differ between the two malocclusion groups nor between males and females (P > .05). Conclusions: The null hypotheses were rejected. Patients with Class II subdivision malocclusion displayed asymmetrically positioned right- and left-side glenoid fossae, with a distally and laterally positioned Class II side, although the condyles were symmetrically positioned within the glenoid fossae.


1997 ◽  
Vol 87 (4) ◽  
pp. 625-628 ◽  
Author(s):  
Keisuke Imai ◽  
Kounosuke Tsujiguchi ◽  
Chiaya Toda ◽  
Ki-Chul Sung ◽  
Sadao Tajima ◽  
...  

✓ The benign osteoblastoma is rarely seen as a tumor of the facial bone in infancy or early childhood. Only five cases with nasal involvement have been reported in the literature. The authors present a case of osteoblastoma of the nasal cavity, the nasal bone, the ethmoid sinus, and the anterior cranial base. This 3-year-old girl presented with a tumor surrounding the left medial canthus. Imaging studies, including x-ray films, computerized tomography scans, magnetic resonance images, a 99mTc-scintigram, and angiograms, confirmed the location of the tumor. A biopsy specimen of tumor was obtained intranasally and the pathological diagnosis was an osteoblastic tumor suggestive of osteoblastoma. Although the tumor margin was well defined on the radiological images, it was difficult to determine the exact margin during the operation. Therefore, it is important to show how to excise the tumor completely under direct view. With the use of a “dismasking flap,” it was possible to resect the benign osteoblastoma completely from the nasal cavity, even though it extended into the orbit, the maxilla, and the anterior cranial base.


1995 ◽  
Vol 83 (4) ◽  
pp. 733-736 ◽  
Author(s):  
Paul C. Francel ◽  
T. S. Park ◽  
Jeffrey L. Marsh ◽  
Bruce A. Kaufman

✓ Frontal plagiocephaly may arise from either synostotic or deformational forces. Deformational causes of frontal plagiocephaly can be distinguished from synostotic causes by differences seen on physical examination, which can then be confirmed by skull x-ray films and if necessary three-dimensional computerized tomography (CT). Unilateral coronal synostosis is the main synostotic cause of frontal plagiocephaly, although it has also been seen with fusion of the frontozygomatic suture. In several syndromes presenting with bilateral coronal synostosis, fusion of the frontosphenoidal and frontoethmoidal sutures is also present. The authors report, for perhaps the first time, a case showing synostotic frontal plagiocephaly secondary to fusion of the frontosphenoidal suture alone. Although the phenotypic appearance is superficially similar to that seen in unilateral coronal synostosis, analysis of the cranial base shows markedly different effects: angulation of the anterior cranial base with respect to the posterior cranial base away from the synostotic side and angulation of the posterior cranial base with respect to the midpalatal suture also away from the synostotic side. In unilateral coronal synostosis, both angulations are toward the synostotic side. These effects on the cranial base alter its relationship to the cranial vault and the facial skeleton. Most important, frontal plagiocephaly secondary to fusion of the frontosphenoidal suture should not be overlooked as being deformational. Because this fusion is difficult or impossible to visualize by skull x-ray films, three dimensional CT must be obtained in cases that are not clearly identified as deformational plagiocephaly by physical examination.


2020 ◽  
Author(s):  
Yufei Liu ◽  
Jihu Yang ◽  
Xiejun Zhang ◽  
Yanhua Sun ◽  
Fanfan Chen ◽  
...  

Abstract BACKGROUND: Collision occurrences of sinonasal carcinosarcoma and pituitary adenoma are rarely reported. Sinonasal carcinosarcomas represent rare neoplasms with invasive characteristics and unfavorable prognoses.CASE DESCRIPTION: We present a rare case of a collision occurrence of sinonasal carcinosarcoma and pituitary adenoma in a 45‐year‐old male patient. MRI demonstrated a large mass involving the sellar region, nasal cavity, paranasal sinuses and anterior cranial base. Total surgical resection with a pure endoscopic expanded endonasal approach was performed successfully with neuronavigational assistance. Histopathologic results were a carcinosarcoma in the nasal cavity, paranasal sinuses, and anterior cranial base and a pituitary adenoma in the intrasellar zone. The Ki-67 index of the carcinosarcoma was high (more than 95%). Although the patient received chemotherapy, he died 6 months after surgery because of in situ recurrence and extensive metastatic growth.CONCLUSIONS: Collision occurrences of sinonasal carcinosarcoma and pituitary adenoma are rare events. Such tumor could be removed successfully by neuronavigational guidance with a pure endoscopic expanded endonasal approach. A contralateral nasal septum mucosa flap without tumor invasion can be used as a kind of skull base repair material. A high Ki-67 index may be a biomarker of rapid tumor progression and poor prognosis in such patients.


Neurosurgery ◽  
2003 ◽  
Vol 53 (5) ◽  
pp. 1126-1137 ◽  
Author(s):  
James K. Liu ◽  
David Decker ◽  
Steven D. Schaefer ◽  
Augustine L. Moscatello ◽  
Richard R. Orlandi ◽  
...  

Abstract OBJECTIVE Anterior cranial base tumors are surgically resected with combined craniofacial approaches that frequently involve disfiguring facial incisions and facial osteotomies. The authors outline three operative zones of the anterior cranial base and paranasal sinuses in which tumors can be resected with three standard surgical approaches that minimize transfacial incisions and extensive facial osteotomies. METHODS The zones were defined by performing dissections on 10 cadaveric heads and by evaluating radiographic images of patients with anterior cranial base tumors. The three approaches performed on each cadaver were transbasal, transmaxillary, and extended transsphenoidal. RESULTS Three zones of approach were defined for accessing tumors of the anterior cranial base, nasal cavity, and paranasal sinuses. Zone 1 is exposed by the transbasal approach, which is limited anteriorly by the supraorbital rim, posteriorly by the optic chiasm and clivus, inferiorly by the palate, and laterally by the medial orbital walls. This approach allows access to the entire anterior cranial base, nasal cavity, and the majority of maxillary sinuses. The limitation imposed by the orbits results in a blind spot in the superolateral extent of the maxillary sinus. Zone 2 is exposed by a sublabial maxillotomy approach and accesses the entire maxillary sinus, including the superolateral blind spot and the ipsilateral anterior cavernous sinus. However, access to the anterior cranial base is limited. Zone 3 is exposed by the transsphenoidal approach. This approach accesses the midline structures but is limited by the lateral nasal walls and intracavernous carotid arteries. An extended transsphenoidal approach allows further exposure to the anterior cranial base, clivus, or cavernous sinuses. The use of the endoscope facilitates tumor resection in the nasal cavity and paranasal sinuses. CONCLUSION The operative zones outlined offer minimally invasive craniofacial approaches to accessing lesions of the anterior cranial base and paranasal sinuses, obviating facial incisions and facial osteotomies. Case illustrations demonstrating the approach selection paradigm are presented.


Author(s):  
Ahmed Omran ◽  
David Wertheim ◽  
Kathryn Smith ◽  
Ching Yiu Jessica Liu ◽  
Farhad B. Naini

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.


2010 ◽  
Vol 6 (4) ◽  
pp. 368-371
Author(s):  
Emily B. Ridgway ◽  
Alexander E. Ropper ◽  
John B. Mulliken ◽  
Bonnie L. Padwa ◽  
Liliana C. Goumnerova

Complications of Le Fort III midfacial advancement include CSF rhinorrhea, meningitis, and ocular and cerebral injury. This report reviews the anatomy of the Le Fort III osteotomies and their relevance to the unusual complication of meningoencephalocele. In this report, a young male patient with Crouzon syndrome underwent subcranial midfacial advancement at the age of 10 years for obstructive sleep apnea and ocular exposure. He presented 4 years later complaining of nasal obstruction. On physical examination, a mucous-covered mass was noted in the left upper nasal vault medial to the turbinates. Computed tomography scanning and MR imaging confirmed the diagnosis of frontoethmoidal meningoencephalocele. Repair of the meningoencephalocele was accomplished using a combined neurosurgery and plastic surgery approach. Meningoencephalocele is a rare complication of subcranial midfacial advancement. The abnormal anatomy of the anterior cranial base in patients with syndromic craniosynostosis places them at greater risk for fracture of the cribriform plate and dural tears during this procedure. Unrecognized dural injury is the etiology of this complication in this young patient; however, elevated intracranial pressure may have been a confounding factor. Attention to the anatomy of the anterior cranial base, as seen on sagittal CT images, will aid in preventing this complication.


2017 ◽  
Vol 88 (2) ◽  
pp. 233-245 ◽  
Author(s):  
Cecilia Ponce-Garcia ◽  
Manuel Lagravere-Vich ◽  
Lucia Helena Soares Cevidanes ◽  
Antonio Carlos de Olivera Ruellas ◽  
Jason Carey ◽  
...  

ABSTRACT Objective: The purpose of this systematic review was to synthesize the available literature concerning the reliability of three-dimensional superimposition methods when assessing changes in craniofacial hard tissues. Materials and Methods: Four electronic databases were searched. Two authors independently reviewed potentially relevant articles for eligibility. Clinical trials, cohort, case-control, and cross-sectional studies that evaluated the reliability of three-dimensional superimposition methods on the anterior cranial base were included. Results: Six studies fulfilled the inclusion criteria. Four studies used the voxel-based registration method, one used the landmark-based method and one used the surface-based method. Regarding reliability, the voxel-based studies showed on average a difference of 0.5 mm or less between images. The optimized analysis using a six-point correction algorithm in the landmark-based method showed 1.24 mm magnitude of error between images. Conclusions: Although reliability appears to be adequate, the small sample size and high risk of bias among studies make available evidence still insufficient to draw strong conclusions.


2018 ◽  
Vol 55 (10) ◽  
pp. 1367-1374 ◽  
Author(s):  
Xiyang Liu ◽  
Zhenqi Chen

Objective: To identify the effects of palate repair on cranial base and maxillary morphology in patients with unilateral cleft lip and palate (UCLP) and to discover the relevance between cranial base and maxilla through cephalometric analysis. Design: Retrospective. Patients: Thirty-seven UCLP patients with operated lip (OL) and unoperated palate constituted OL group and were classified into 5 cervical vertebral maturation (CVM) stages. Thirty-seven UCLP patients with operated lip and palate (OLP) and 37 noncleft people with skeletal class I malocclusion were CVM- and sex-matched with the OL group as OLP group and control group, respectively. CVM stage I and II were combined into group 1, CVM stage III to V were combined into group 2. Interventions: Lateral cephalograms of all participants were obtained. Main Outcome Measures: Cephalometric analysis was employed, and data were compared among groups. Results: Length of posterior cranial base (Ba-S) of the OL group was shorter than controls in group 1; Ba-S and the ratio between length of posterior and anterior cranial base (Ba-S/S-N) of the OL and OLP groups were smaller than controls in group 2. No significant differences in cranial base were found between the OL and OLP groups. In group 1, patients of the OLP group showed smaller SNA, ANS-Ptm, and ANS-Ptm/S-N, and patients of the OL group showed smaller ANS-Ptm. In group 2, both OL and OLP groups had smaller sella-nasion-A point angle (SNA), projection distance between ANS and Ptm points on FH plane (ANS-Ptm), and the ratio between ANS-Ptm and anterior cranial base length (ANS-Ptm/S-N). Conclusions: Palate repair seems to have no obvious effects on cranial base morphology in patients with UCLP. Those patients with lip operated, whether cleft palate operated or not, tend to have a smaller length of maxilla sagittally and this deformity progresses with age.


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