Basichondrocranium anomalies in adult Chiari type I malformation: a morphometric study

Vega, Alfonso; Quintana, Fernando Utrera; Berciano, José

doi:10.1016/0022-510x(90)90150-l

Cited by 167 publications

(125 citation statements)

References 23 publications

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“…In recent times, however, several authors using x-ray, tomography and MRI examinations have also demonstrated that the posterior fossa volume, in the presence of BI and CM, is smaller than the observed in normal people [36][37][38][39] .…”

Section: Discussionmentioning

confidence: 97%

Basilar impression, Chiari malformation and syringomyelia: a retrospective study of 53 surgically treated patients

Silva¹,

Holanda

2003

Arq. Neuro-Psiquiatr.

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-The present study shows the results of 53 patients who have been treated surgically for basilar impression (BI), Chiari malformation (CM), and syringomyelia (SM). The patients were divided into two groups. Group I (24 patients) underwent osteodural decompression with large inferior occipital craniectomy, laminectomy from C 1 to C 3, dural opening in Y format, dissection of arachnoid adhesion between the cerebellar tonsils, medulla oblongata and spinal cord, large opening of the fourth ventricle and dural grafting with the use of bovine pericardium. Group II patients (29 patients) underwent osteodural-neural decompression with the same procedures described above plus dissection of the arachnoid adherences of the vessels of the region of the cerebellar tonsils, and tonsillectomy (amputation) in 10 cases, and as for the remainning 19 cases, intrapial aspiration of the cerebellar tonsils was performed. The residual pial sac was sutured to the dura in craniolateral position. After completion of the suture of the dural grafting, a thread was run through the graft at the level of the created cisterna magna and fixed to the cervical aponeurosis so as to move the dural graft on a posterior-caudal direction, avoiding, in this way, its adherence to the cerebellum. RESUMO -São analisados os resultados obtidos com o tratamento cirúrgico de 53 casos de impressão basilar (IB), malformação de Chiari (MC) e siringomielia (SM). Os pacientes foram divididos em dois grupos: no grupo I(24 casos) foi realizada a descompressão osteodural, caracterizada por craniectomia ampla occipital inferior, laminectomia variável de C 1 a C 3, abertura da dura-máter em forma de Y, dissecção das aderências aracnóideas das tonsilas cerebelares com o bulbo e medula cervical, abertura ampla do quarto ventrículo e enxerto dural; no grupo II (29 casos) foi utilizada a descompressão osteodural-neural, caracterizada pelos mesmos detalhes técnicos empregados no grupo I, acrescidos da tonsilectomia por amputação (10 casos) e por aspiração intrapial (19 casos). Nestes casos, o saco pial residual, resultante da aspiração intrapial das tonsilas cerebelares, foi suturado à dura-máter lateral e em posição cranial. Após o término do enxerto dural, foi passado um fio através do enxerto dural, à altura da cisterna criada, e fixado sobre a aponeurose cervical, com a finalidade de deslocar a plástica em direção caudal, evitando, desta forma, sua aderência ao cerebelo.PALAVRAS-CHAVE: impressão basilar, malformação de Chiari, siringomielia, tonsilectomia, enxerto da dura-máter.

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Section: Discussionmentioning

confidence: 97%

Basilar impression, Chiari malformation and syringomyelia: a retrospective study of 53 surgically treated patients

Silva¹,

Holanda

2003

Arq. Neuro-Psiquiatr.

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“…1,11,12,18,22,26,32,35,42 Children with CM-I have smaller posterior fossae than controls, 28,38,41,45 and in 1 study, patients with an associated syrinx had yet smaller posterior fossae than CM-I patients without a syrinx. 40 Syrinx has a strong association with scoliosis as well as motor and sensory symptoms.…”

Section: Discussionmentioning

confidence: 69%

Delayed resolution of syrinx after posterior fossa decompression without dural opening in children with Chiari malformation Type I

Kennedy¹,

Nelp²,

Kelly³

et al. 2015

PED

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OBJECT Chiari malformation Type I (CM-I) is associated with a syrinx in 25%–85% of patients. Although posterior fossa decompression (PFD) without dural opening is an accepted treatment option for children with symptomatic CM-I, many surgeons prefer to open the dura if a syrinx exists. The purpose of this study was to investigate the frequency and timing of syrinx resolution in children undergoing PFD without dural opening for CM-I. METHODS A retrospective review of 68 consecutive pediatric patients with CM-I and syringomyelia who underwent PFD without dural opening was conducted. Patient demographics, presenting symptoms and signs, radiographic findings, and intraoperative ultrasound and neuromonitoring findings were studied as well as the patients’ clinical and radiographic follow-up. RESULTS During the mean radiographic follow-up period of 32 months, 70% of the syringes improved. Syrinx improvement occurred at a mean of 31 months postoperatively. All patients experienced symptom improvement within the 1st year, despite only 26% of patients showing radiographic improvement during that period. Patients presenting with sensory symptoms or motor weakness had a higher likelihood of having radiographic syrinx improvement postoperatively. CONCLUSIONS In children with CM-I and a syrinx undergoing PFD without dural opening, syrinx resolution occurs in approximately 70% of patients. Radiographic improvement of the syrinx is delayed, but this does not correlate temporally with symptom improvement. Sensory symptoms or motor weakness on presentation are associated with syrinx resolution after surgery.

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“…Overcrowding of the posterior fossa or a normal amount of substance in an unusually small posterior fossa is likely the culprit of the downward displacement of the cerebellum (2,5,15,22,23,28,30,31,34,39,42,45,47). Many of the symptoms of Chiari may be the result of obstructed CSF flow (7) and the development of scar tissue of the cerebellar tonsils and the compression of the cerebellum and spinal cord (3,7,27).…”

Section: █ Discussionmentioning

confidence: 99%

Familial aggregation of chiari malformation: presentation, pedigree, and review of the literature

Nagy¹,

Mobley²,

Ray³

2014

Turkish Neurosurgery

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ABSTRACTprimary goals of the presentation of this family is to further illuminate the likely mode of genetic inheritance based on the large pedigree that is demonstrated below. █ CASE REpORTThis study is a case report of a patient's family of whom five individuals had a confirmed case of Chiari malformations and a total of eight were symptomatic (including the 5 confirmed). The incidence of Chiari malformations confirmed by CT scans occurred in two consecutive generations. The symptomatic individuals spanned three generations. The family of this child under our care also has a very large incidence of EhlersDanlos syndrome.Of the three daughters (III-7, III-8, and III-9) of the individual (II-3), all were radiographically confirmed cases of Chiari I Malformation (Figure 1). These patients lived in rural communities and thus were diagnosed with CT imaging instead of MRI, due to access and funding. However, in all of the patients the diagnosis was clear on the CT images, and was confirmed with MRI in some. Patient III-8 is the █ INTRODUCTION Chiari Malformation (CM) is typically considered to be the downward herniation of the cerebellar tonsils through the foramen magnum. The basic presentation of symptoms include occipital headaches, balance and coordination issues, and other associated cerebellar signs. This disease process affects both adult and pediatric patient populations, but symptoms vary by age. The presenting symptoms can be quite variable as well. Chiari malformations can be the result of a known etiology or can occur without any known causative factors. The Chiari that occurs from an unknown etiology is likely the result of a genetically determined small posterior fossa volume.There is a growing amount of data in the literature supporting a genetic basis for the Chiari malformation. However, familial aggregation in the literature is limited to twins, triplets, and a few cases of two consecutive generations being affected. While multiple projects are underway in the United States to identify a gene that can be specifically linked to the Chiari Malformation, none has yet elucidated the gene. One of the This article reports the largest familial aggregation of Chiari malformation in a single family to date as reported in the literature. This study is a retrospective case series of a family of whom five individuals have a confirmed case of Chiari malformation and three additional individuals have Chiari signs and symptoms. This contribution further supports the implication of genetics in the transmission of Chiari malformation. The family reported in this study also has a significant incidence of Ehlers-Danlos. Three sisters, including a set of twins, presented with confirmed cases of Chiari malformation and four of the five children of the twin sisters presented with confirmed or suspected Chiari malformation. Of note, the non-twin sister has three children who are unaffected. This report provides further evidence for a shared loci between the Chiari malformation and Ehlers-Danlos.

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Basichondrocranium anomalies in adult Chiari type I malformation: a morphometric study

Cited by 167 publications

References 23 publications

Basilar impression, Chiari malformation and syringomyelia: a retrospective study of 53 surgically treated patients

Basilar impression, Chiari malformation and syringomyelia: a retrospective study of 53 surgically treated patients

Delayed resolution of syrinx after posterior fossa decompression without dural opening in children with Chiari malformation Type I

Familial aggregation of chiari malformation: presentation, pedigree, and review of the literature

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