Association between Brachycephaly, Chiari Malformation, and Basilar Invagination

Author(s):  
Ricardo Vieira Botelho ◽  
Pedro Bittencourt Botelho ◽  
Bruna Hernandez ◽  
Mauricio Bezerra Sales ◽  
José Marcus Rotta

Abstract Background There is evidence that Chiari malformation (CM) and basilar invagination (BI) are largely due to disproportion between the content and volume of the posterior fossa. A recent study identified an increased association between brachycephaly and BI. In several types of craniosynostosis, the posterior fossa volume is smaller than normal, and this is more pronounced in coronal synostosis. The aim of this study is to evaluate the association between CM and BI. Methods The cephalic index (CI) measured on magnetic resonance imaging (MRI) from a sample of patients with craniocervical malformation was compared with that of normal subjects. Results The average CI in the craniovertebral junction malformation (CVJM) group was significantly higher in BI patients than in normal subjects. The BI patients also had the highest CI among the whole sample of patients (p = 0.009). Conclusions In this study, BI patients had the highest CI among patients with CVJM and a significantly higher CI than those in the control group. Our data confirm the association between BI and brachycephaly.

1997 ◽  
Vol 86 (1) ◽  
pp. 40-47 ◽  
Author(s):  
Misao Nishikawa ◽  
Hiroaki Sakamoto ◽  
Akira Hakuba ◽  
Naruhiko Nakanishi ◽  
Yuichi Inoue

✓ To investigate overcrowding in the posterior cranial fossa as the pathogenesis of adult-type Chiari malformation, the authors studied the morphology of the brainstem and cerebellum within the posterior cranial fossa (neural structures consisting of the midbrain, pons, cerebellum, and medulla oblongata) as well as the base of the skull while taking into consideration their embryological development. Thirty patients with Chiari malformation and 50 normal control subjects were prospectively studied using neuroimaging. To estimate overcrowding, the authors used a “volume ratio” in which volume of the posterior fossa brain (consisting of the midbrain, pons, cerebellum, and medulla oblongata within the posterior cranial fossa) was placed in a ratio with the volume of the posterior fossa cranium encircled by bony and tentorial structures. Compared to the control group, in the Chiari group there was a significantly larger volume ratio, the two occipital enchondral parts (the exocciput and supraocciput) were significantly smaller, and the tentorium was pronouncedly steeper. There was no significant difference in the posterior fossa brain volume or in the axial lengths of the hindbrain (the brainstem and cerebellum). In six patients with basilar invagination the medulla oblongata was herniated, all three occipital enchondral parts (the basiocciput, exocciput, and supraocciput) were significantly smaller than in the control group, and the volume ratio was significantly larger than that in the Chiari group without basilar invagination. These results suggest that in adult-type Chiari malformation an underdeveloped occipital bone, possibly due to underdevelopment of the occipital somite originating from the paraxial mesoderm, induces overcrowding in the posterior cranial fossa, which contains the normally developed hindbrain. Basilar invagination is associated with a more severe downward herniation of the hindbrain due to the more severely underdeveloped occipital enchondrium, which further exacerbates overcrowding of the posterior cranial fossa.


1996 ◽  
Vol 1 (5) ◽  
pp. E1
Author(s):  
Misao Nishikawa ◽  
Hiroaki Sakamoto ◽  
Akira Hakuba ◽  
Naruhiko Nakanishi ◽  
Yuichi Inoue

To investigate overcrowding in the posterior cranial fossa as the pathogenesis of adult-type Chiari malformation, the authors studied the morphology of the brainstem and cerebellum within the posterior cranial fossa (neural structures consisting of the midbrain, pons, cerebellum, and medulla oblongata) as well as the base of the skull while taking into consideration their embryological development. Thirty patients with Chiari malformation and 50 normal control subjects were prospectively studied using neuroimaging. To estimate overcrowding, the authors used a "volume ratio" in which volume of the posterior fossa brain (consisting of the midbrain, pons, cerebellum, and medulla oblongata within the posterior cranial fossa) was placed in a ratio with the volume of the posterior fossa cranium encircled by bony and tentorial structures. Compared to the control group, in the Chiari group there was a significantly larger volume ratio, the two occipital enchondral parts (the exocciput and supraocciput) were significantly smaller, and the tentorium was pronouncedly steeper. There was no significant difference in the posterior fossa brain volume or in the axial lengths of the hindbrain (the brainstem and cerebellum). In six patients with basilar invagination the medulla oblongata was herniated, all three occipital enchondral parts (the basiocciput, exocciput, and supraocciput) were significantly smaller than in the control group, and the volume ratio was significantly larger than that in the Chiari group without basilar invagination. These results suggest that in adult-type Chiari malformation an underdeveloped occipital bone, possibly due to underdevelopment of the occipital somite originating from the paraxial mesoderm, induces overcrowding in the posterior cranial fossa, which contains the normally developed hindbrain. Basilar invagination is associated with a more severe downward herniation of the hindbrain due to the more severely underdeveloped occipital enchondrium, which further exacerbates overcrowding of the posterior cranial fossa.


Author(s):  
Paweł Szaro ◽  
Khaldun Ghali Gataa ◽  
Mateusz Polaczek

Abstract Purpose The aim of the study was to examine the ligaments of the os trigonum. Methods The ankle joint magnetic resonance imaging (MRI) of 104 patients with the os trigonum (experimental group) and 104 patients without the os trigonum (control group) were re-reviewed. The connections of the os trigonum and posterior talofibular ligament (PTFL), the fibulotalocalcaneal ligament (FTCL), the paratenon of the Achilles tendon, the posterior talocalcaneal ligament (PTCL), the osteofibrous tunnel of the flexor hallucis longus (OF-FHL) and the flexor retinaculum (FR) were studied. Results The os trigonum is connected to structures. The posterior part of the PTFL inserted on the os trigonum in 85.6% of patients, whereas in all patients in the control group, the posterior part of the PTFL inserted on the posterior talar process (p < 0.05). The connection of the PTCL was seen in 94.2% of patients in the experimental group, while it was seen in 90.4% of patients in the control group (p > 0.05). The connection to the FTCL in the experimental group was 89.4%, while in the control group, it was 91.3% (p > 0.05). The communication with the paratenon was seen more often in the control group compared to that in the experimental group (31.7% vs. 63.8%, p < 0.001). The FTCL was prolonged medially into the FR in 85.6% of patients in the experimental group and in 87.5% of patients in the control group (p > 0.05). The flexor hallucis longus (FHL) run at the level of articulation between the os trigonum 63.5% and the posterior process of the talus 25% and less often on the os trigonum 11.5%. Conclusion The os trigonum is connected with all posterior ankle structures and more connections than previously reported.


1998 ◽  
Vol 112 (9) ◽  
pp. 860-864 ◽  
Author(s):  
P. J. D. Dawes ◽  
J.-P. Jeannon

AbstractAn audit of 334 patients who underwent magnetic resonance imaging (MRI) as investigation for a possible diagnosis of vestibular schwannoma was carried out to assess adherence to previously agreed screening guidelines. This represents one year's activity. A posterior fossa tumour was identified in 12 patients. Scan requests were judged to be inappropriate for 28 cases. The issues surrounding the screening for acoustic neuroma are discussed.


2005 ◽  
Vol 25 (1) ◽  
pp. 87-97 ◽  
Author(s):  
Guangliang Ding ◽  
Quan Jiang ◽  
Li Zhang ◽  
Zheng Gang Zhang ◽  
Lian Li ◽  
...  

Suppression of platelet activation improves the efficacy of thrombolytic therapy for stroke. Thus, combination treatment with recombinant tissue plasminogen activator (r-tPA) and 7E3 F(ab′)2, a GPIIb/IIIa inhibitor that binds the platelet to fibrin, may improve the efficacy of thrombolytic therapy in embolic stroke. Magnetic resonance imaging (MRI) was used to monitor treatment response in rats subjected to embolic middle cerebral artery (MCA) occlusion (MCAo). Animals were randomized into treated ( n = 12) and control ( n = 10) groups and received intravenous combination therapy or saline, respectively, 4 hours after MCAo. Magnetic resonance imaging (MRI) measurements performed 1 hour after MCAo showed no difference between groups. However, an increased incidence (50%) of MCA recanalization was found in the treated group at 24 hours compared with 20% in the control group. The area of low cerebral blood flow at 24 and 48 hours was significantly smaller in the combination treatment group, and the lesion size, as indicated from the T2 and T1 maps, differed significantly between groups. Fluorescence microscopy measurements of cerebral microvessels perfused with fluorescein isothiocyanate-dextran and measurements of infarct volume revealed that the combination treatment significantly increased microvascular patency and reduced infarct volume, respectively, compared with the control rats. The efficacy of combination treatment 4 hours after ischemia is reflected by MRI indices of tissue perfusion, MCA recanalization, and reduction of lesion volume. The treatment also reduced secondary microvascular perfusion deficits.


2016 ◽  
Vol 8 (2) ◽  
pp. 87-92
Author(s):  
Lucas M. Gutierrez ◽  
Matias Cullari ◽  
Maximiliano Ranaletta ◽  
Ignacio Tanoira ◽  
César Gomez ◽  
...  

Introducción: El síndrome del espacio cuadrilátero (QSS) es un cuadro clínico causado por la compresión nerviosa y/o vascular a nivel del espacio axilar lateral. La prevalencia de este síndrome se desconoce y se cree que es baja, sin embargo, formas de presentación incompleta podrían llevar a que el mismo sea subdiagnosticado. Objetivo: Describir un protocolo diagnóstico utilizado en una serie de casos consecutivos de pacientes con omalgia inespecífica y sospecha de QSS. Materiales y Métodos: De manera prospectiva se evaluaron 10 pacientes con omalgia agravada con la rotación externa forzada, uno de ellos con síntomas bilaterales. Se realizó electromiografía (EMG) de deltoides y redondo menor bilaterales, con estudios de conducción nerviosa. Los valores se compararon con el hombro sano, y con un grupo control. El diagnóstico se confirmó mediante resonancia magnética nuclear (RMN). Resultados: El EMG mostró en todos los casos atrapamiento con compromiso axonal de la rama para el redondo menor, con preservación de la inervación del deltoides. La RMN mostró en los 11 hombros afectados atrofia selectiva del redondo menor. Conclusión: El QSS en sus formas incompletas suele ser subdiagnosticado, siendo necesario mantener un alto índice de sospecha clínica frente a un cuadro de omalgia inespecífica. Palabras Claves: espacio cuadrilátero, omalgia, electromiografía, redondo menor, nervio axilar. Introduction: The quadrilateral space syndrome (QSS) has clinical features caused by a nervous and /or vascular compression at the lateral axillary space. The prevalence of this syndrome is unknown but it is believed to be low. Nevertheless, some cases with an incomplete clinical presentation might be misdiagnosed. Purpose: To describe a diagnostic protocol used in a series of consecutive cases of patients with non-specific omalgia and suspected QSS. Materials and Methods: Ten patients of both genders suffering shoulder pain aggravated by forced external rotation were prospectively evaluated.  One of them had bilateral symptoms. Evaluation included clinical examination, electromyography (EMG) and nerve conduction studies of the deltoid and teres minor bilaterally. The results were compared with an age-matched control group. Diagnosis was confirmed by magnetic resonance imaging (MRI). Results: The EMG showed in all the cases a selective entrapment of the branch for the teres minor, with preservation of the innervation of the deltoid. The RMN showed in the 11 affected shoulders selective atrophy of the teres minor. Conclusion: Incomplete presentations of the QSS are usually underdiagnosed, making it necessary to maintain a high index of clinical suspicion in front of any non-specific omalgia case. Key words: quadrilateral space, omalgia, electromyography, teres minor, axillary nerve.


2013 ◽  
Vol 32 (01) ◽  
pp. 40-47
Author(s):  
José Alberto Gonçalves da Silva ◽  
Adailton Arcanjo dos Santos Júnior ◽  
José Demir Rodrigues

Abstract Objective: The objective of this paper is to analyze the surgical treatment of impacted cisterna magna without syringomyelia (SM) associated or not with basilar impression (BI) and/or Chiari malformation (CM). Method: The authors present, in this work, the results of five cases with impacted cisterna magna without SM which were associated with BI in four cases, tonsillar herniation in three patients when they were in the sitting position and in the other two cases there was not herniation in the sitting position. Results: The surgical treatment was characterized by a large craniectomy with the patient in the sitting position, tonsillectomy, large opening of the fourth ventricle and duraplasty with creation of a large artificial cisterna magna. An upward migration of the posterior fossa structures was detected by postoperative magnetic resonance imaging (MRI). Conclusion: The surgical treatment of impacted cisterna magna without SM remains unclear, however, a large craniectomy associated with tonsillectomy and creation of a large cisterna magna showed good results and a tendency of upward migration of the posterior fossa structures.


2017 ◽  
Vol 63 (11) ◽  
pp. 946-949 ◽  
Author(s):  
Marcelo Ferreira Sabba ◽  
Beatriz Souza Renor ◽  
Enrico Ghizoni ◽  
Helder Tedeschi ◽  
Andrei Fernandes Joaquim

Summary Chiari malformation (CM) is the most common and prevalent symptomatic congenital craniocervical malformation. Radiological diagnosis is established when the cerebellar tonsils are located 5 mm or more below the level of the foramen magnum on magnetic resonance imaging (MRI). Surgical treatment is indicated whenever there is symptomatic tonsillar herniation or syringomyelia/hydrocephalus. The main surgical treatment for CM without craniocervical instability (such as atlantoaxial luxation) is posterior fossa decompression, with or without duraplasty. The authors describe in details and in a stepwise fashion the surgical approach of patients with CM as performed at the State University of Campinas, emphasizing technical nuances for minimizing the risks of the procedure and potentially improving patient outcome.


2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Tae-Ha Lim ◽  
Soo Il Choi ◽  
Hyung Rae Cho ◽  
Keum Nae Kang ◽  
Chang Joon Rhyu ◽  
...  

Background. We devised a new morphological parameter called the superior articular process area (SAPA) to evaluate the connection between lumbar foraminal stenosis (LFS) and the superior articular process. Objective. We hypothesized that the SAPA is an important morphologic parameter in the diagnosis of LFS. Methods. All patients over 60 years of age were included. Data regarding the SAPA were collected from 137 patients with LFS. A total of 167 control subjects underwent lumbar magnetic resonance imaging (MRI) as part of a routine medical examination. We analyzed the cross-sectional area of the bone margin of the superior articular process at the level of L4-L5 facet joint in the axial plane. Results. The average SAPA was 96.3±13.6 mm2 in the control group and 128.1±17.2 mm2 in the LFS group. The LFS group was found to have significantly higher levels of SAPA (p<0.001) in comparison to the control group. In the LFS group, the optimal cut-off value was 112.1 mm2, with 84.4% sensitivity, 83.9% specificity, and AUC of 0.94 (95% CI: 0.91–0.96). Conclusions. Higher SAPA values were associated with a higher possibility of LFS. These results are important in the evaluation of patients with LFS.


2021 ◽  
Vol 23 (1) ◽  
pp. 83-90
Author(s):  
D. S. Epifanov ◽  
V. B. Lebedev ◽  
A. A. Zuev

Introduction. Each of the pathologies is rare and can lead to compression of the brainstem and spinal cord, with the development of a “mixed” clinical picture. The tactics of treating such patients differs from one author to another.The objective is to present the author’s experience in treating patients with basilar invagination associated with type I Chiari malformation and to analyze the literature on the methods of surgical treatment of this pathology.Materials and methods. From 2014 to 2019, 3 patients with a basilar invagination and type I Chiari malformation were surgically treated at the N. I. Pirogov National Medical and Surgical Center. Indications for surgical intervention were long-term progressive neurological disorders, lack of effect from conservative therapy. Patients underwent anterior transnasal and posterior decompression of nerve structures followed by stabilization.Results. All patients were evaluated 12 months after surgery. All patients in neurological status showed positive dynamics. According to magnetic resonance imaging of the craniovertebral junction, 2 patients showed positive dynamics: regression of the myelopathy, a decrease in the diameter of the syringomyelic cyst.Conclusion. The criteria for anterior transnasal decompression are the predominance of bulbar disorders and / or paresis in the extremities in the clinical picture; posterior decompression of nerve structures, indicated in the presence of specific symptoms of type I Chiari malformation and / or syringomyelia. Taking into account the results of the latest published studies it can be assumed that stabilization is a necessary option in the treatment of patients with this pathology.


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