scholarly journals Surgical treatment of Arnold-Chiari malformation type I in an adult patient

2008 ◽  
Vol 65 (8) ◽  
pp. 648-652
Author(s):  
Marko Markovic ◽  
Iva Berisavac ◽  
Vladimir Bojovic ◽  
Bojan Kostic ◽  
Vuk Djulejic
Keyword(s):  
Cranial Nerves ◽  
Clinical Signs ◽  
Foramen Magnum ◽  
Clinical Image ◽  
Posterior Arch ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Truncal Ataxia ◽  
Dural Graft ◽  
Cerebellar Tonsils

Background. Herniation of the cerebellar tonsils through the foramen magnum into the cervical spinal canal with obliteration of the cerebellomedullary cistern is the primary feature of Arnold-Chiari type I malformation (ACM I). It is considered to be congenital malformation, although there have been reported cases of an acquired form. Case report. We presented a female patient, 45-year old, with ACM I without syringomyelia as a rare and unusual clinical image, as well as the effect of decompressive surgery in the treatment of this malformation. The patient was admitted to the Department of Neurosurgery with clinical signs of truncal ataxia worsening during the last six years. Moderate quadriparesis with predominant lower extremity involvement and the signs of the cranial nerves damages occured during the last seven months before admission, with progressive clinical course up to the date of admission. Neurosurgical treatment that included suboccipital medial craniectomy with resection of posterior arch C1 vertebrae and C2 laminectomy resulted in a significant clinical improvement and a much better quality of life. Conclusion. Posterior craniovertebral decompression with microsurgical reduction of the cerebellar tonsils and placement of an artificial dural graft is a treatment of choice in severe forms of ACM I without syringomyelia. .

Trzyipółletni chłopiec z malformacją Chiariego typu I – opis przypadku

2017 ◽  
Vol 21 (3) ◽  
Author(s):  
Anna Worch ◽  
Małgorzata Wielopolska
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Common Type ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Chiari Type I Malformation ◽  
Large Head ◽  
Exacerbation Of Symptoms ◽  
Chiari Type I ◽  
Cerebellar Tonsils

Chiari malformation is the most common structural defect of the posterior fossa and cerebellum, consisting of displacements of hindbrain inclination through the foramen magnum, often associated with syringomyelia. In the most common type I of this disease, only the cerebellar tonsils are descended. Chiari type I malformation may remain asymptomatic in early childhood, however a number of unspecific symptoms, such as: neck pains and headaches, vertigos, balance and sight disorders, may strengthen with age. Because of unspecificity and various exacerbation of symptoms the disease is often misdiagnosed or undiagnosed. We describe a case of a 3.5-year old boy with whom his mother visited a pediatrician because of a chronic cough. During the examination mother of the patient informed that all infections start with vomiting and the patient is not able to stand up from horizontal position without turning first to the side and then to the abdomen. During the examination, a large head circumference was noticed (56 cm, > 97 percentile). MRI showed elongated cerebellar tonsils descended below the foramen magnum (left 14 mm, right 11 mm). Chiari malformation type I with associated syringomyelia was diagnosed. The patient was reffered to neurosurgery consultation.

scholarly journals Normalization of hindbrain morphology after decompression of Chiari malformation Type I

2012 ◽  
Vol 117 (5) ◽  
pp. 942-946 ◽  
Author(s):  
John D. Heiss ◽  
Giancarlo Suffredini ◽  
Kamran D. Bakhtian ◽  
Malisa Sarntinoranont ◽  
Edward H. Oldfield
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Control Group ◽  
Decompression Surgery ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Prospective Longitudinal Study ◽  
Tonsillar Ectopia ◽  
Prospective Longitudinal ◽  
Cerebellar Tonsils

Object Chiari malformation Type I (CM-I) is characterized by hindbrain deformity. We investigated the effects of craniocervical decompression surgery on the anatomical features of hindbrain deformity with a prospective MRI study of patients with CM-I. Methods A prospective longitudinal study was conducted in 48 patients with CM-I (39 with syringomyelia) treated with craniocervical decompression. Clinical examinations and cervical MRI were performed before surgery and 1 week, 3–6 months, and annually after surgery. Hindbrain deformity was defined by tonsillar ectopia, pointed cerebellar tonsils, and/or cervicomedullary protuberance. The length of the clivus, basiocciput (sphenooccipital synchondrosis to basion), supraocciput (internal occipital protuberance to opisthion), and anteroposterior (AP) width of CSF pathways at the foramen magnum were measured and compared with those from 18 healthy volunteers (control group). Results Before surgery, the patients' posterior fossa bones were short and their CSF pathways were narrow. All patients had tonsillar ectopia (mean [± SD] 12.3 ± 5.1 mm; normal 0.3 ± 1.0). The majority of patients had pointed tonsils and more than two-thirds exhibited a cervicomedullary protuberance. Clivus and basiocciput lengths were significantly shorter than the values obtained in the control group. However, the supraocciput length did not differ significantly from control measurements. The mean bulbopontine sulcus distance superior to the basion was 9.5 ± 2.6 mm (vs 13.6 ± 2.8 mm in controls; p < 0.0001). The AP widths of the CSF pathways at the level of the foramen magnum were significantly narrowed. After surgery, CSF pathways significantly expanded both ventrally and dorsally. By 3–6 months after surgery, pointed tonsils became round, cervicomedullary protuberance disappeared, and tonsillar ectopia diminished by 51% (to 6.0 ± 3.3 mm; p < 0.0001). Conclusions The cerebellar tonsils and brainstem assumed a normal appearance within 6 months after craniocervical decompression. These findings support the concept that the CM-I is not a congenital malformation of the neural elements but rather an acquired malformation that arises from pulsatile impaction of the cerebellar tonsils into the foramen magnum. Clinical trial registration no.: NCT00001327.

Soft tissue density within the foramen magnum, a predictor for surgical intervention in pediatric patients with Chiari malformation type I

2020 ◽  
Vol 26 (6) ◽  
pp. 671-675
Author(s):  
William Fuell ◽  
Reem Elwy ◽  
Thomas Harkey ◽  
Matthew Carey ◽  
Gregory W. Albert
Keyword(s):  
Soft Tissue ◽  
Pediatric Patients ◽  
Surgical Intervention ◽  
Foramen Magnum ◽  
Type I ◽  
Soft Tissue Density ◽  
Tonsillar Herniation ◽  
Chiari Malformation Type I ◽  
Tissue Density ◽  
Cerebellar Tonsils

OBJECTIVEChiari malformation type I (CMI) is diagnosed as herniation of the cerebellar tonsils by at least 5 mm below the foramen magnum. However, the degree of tonsillar herniation is a poor predictor of the need for decompression surgery. Exploration for an alternative morphological predictor for surgical intervention could provide greater insight into the development of an appropriate treatment plan for these patients. To investigate this issue, the authors calculated the soft tissue density within the foramen magnum as a measure of impaction of the cerebellar tonsils. Soft tissue density within the foramen magnum and degree of tonsillar herniation were then assessed for their correlation with the need for surgical intervention.METHODSThe authors conducted a retrospective, longitudinal chart review of pediatric patients with CMI. Those who had undergone surgical intervention were considered symptomatic and those who had been treated conservatively, as asymptomatic. Soft tissue density was found by dividing the soft tissue occupancy of the foramen magnum (brainstem and cerebellar tonsils) by the total area of the bony foramen magnum. The predictive value of these two measurements for the need of surgery was determined.RESULTSOf the 465 patients seen for CMI at the authors’ institution between July 1, 2011, and May 31, 2017, 80 underwent surgical intervention and 385 were asymptomatic. The average tissue density was significantly greater in the surgical group than in the asymptomatic group (83.3% and 78.6%, respectively, p < 0.0001). The average tonsillar descent for surgical patients was 10.8 mm compared to 9.8 mm for asymptomatic patients (p = 0.140). The point-biserial correlation coefficient was assessed, and soft tissue density was found to positively correlate with the need for surgical intervention (rpb = 0.199, p = 0.0001), whereas tonsillar herniation did not correlate with the need for surgery (rpb = 0.083, p = 0.115). Additionally, the degree of tonsillar herniation did not correlate with soft tissue density (r = 0.09), indicating that soft tissue density is an independent morphological parameter.CONCLUSIONSThe study findings suggest that the need for surgical intervention in CMI patients is positively correlated with increasing soft tissue density within the foramen magnum, whereas the degree of tonsillar herniation did not show a correlation with the need for surgical intervention. Additionally, soft tissue density is a factor independent of the degree of tonsillar herniation. Further investigation of tissue density within the foramen magnum is needed in the hope of discovering a clinically applicable parameter that would indicate a need for surgical intervention in patients with CMI.

scholarly journals The surgical procedure of syringomyelie with Chiari I in adults regarding the intrapial aspiration of cerebellar tonsils: does this procedure improve symptoms with less complication?

2019 ◽  
pp. 37-48
Author(s):  
Ait Bachir Mustapha ◽  
T. Benbouzid
Keyword(s):  
Clinical Signs ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Spinal Fluid ◽  
Motor Deficits ◽  
Common Denominator ◽  
Consecutive Series ◽  
Dural Graft ◽  
Chiari I ◽  
Cerebellar Tonsils

Introduction. Cranio-vertebral decompression remains the common denominator for the treatment of syringomyelia associated with Chiari I. On the other hand, the details of the procedure, remains controversial. The success of the surgery is to restore the circulation of cerebro-spinal fluid at the level of the foramen magnum. How is this circulation restored to the level of foramen is the question? We offer our attitude towards the treatment of syringomyelia with Chiari I. Material and method. Consecutive series of 32/121 patients benefiting from cranio-vertebral decompression associated with intrapial aspiration of cerebellar tonsils treated for syringomyelia with a Chiari I malformation in adults. Result. Motor deficits were present in 20 / 22 patients, representing 90% of the entire patient group. these motor deficits are improved in 16 out of 22 cases, and remained unchanged in 06cas. no motor aggravation occurred. in our study, bone decompression and intra pial aspiration of cerebellar tonsils (sub arachnoids manipulation) were found to be associated with favourable results on clinical signs and symptoms. However, sub arachnoids manipulation and intra pial aspiration of cerebellar tonsils showed a little more complication compared with bone decompression with dural plasty. Conclusion. The bone decompression with dural graft and intradural dissection of adhesions and reduction by intra pial aspiration or resection of the tonsils is indicated on the MRI aspect of cerebellar tonsils of considerable size totally obstructing the foramen Magnum, the intraoperative finding, through the arachnoid, of the absence of passage of the cerebro-spinal fluid because of the bulging of the cerebellar tonsils.

Prognostic significance of C1–C2 facet malalignment after surgical decompression in adult Chiari malformation type I: a pilot study based on the Chicago Chiari Outcome Scale

2020 ◽  
pp. 1-7
Author(s):  
Michael Lumintang Loe ◽  
Tito Vivas-Buitrago ◽  
Ricardo A. Domingo ◽  
Johan Heemskerk ◽  
Shashwat Tripathi ◽  
...  
Keyword(s):  
Pilot Study ◽  
Chiari Malformation ◽  
Prognostic Significance ◽  
Foramen Magnum ◽  
Bivariate Analysis ◽  
Type I ◽  
Foramen Magnum Decompression ◽  
Swallowing Function ◽  
Chiari Malformation Type I

OBJECTIVEThe authors assessed the prognostic significance of various clinical and radiographic characteristics, including C1–C2 facet malalignment, in terms of surgical outcomes after foramen magnum decompression of adult Chiari malformation type I.METHODSThe electronic medical records of 273 symptomatic patients with Chiari malformation type I who were treated with foramen magnum decompression, C1 laminectomy, and duraplasty at Mayo Clinic were retrospectively reviewed. Preoperative and postoperative Neurological Scoring System scores were compared using the Friedman test. Bivariate analysis was conducted to identify the preoperative variables that correlated with the patient Chicago Chiari Outcome Scale (CCOS) scores. Multiple linear regression analysis was subsequently performed using the variables with p < 0.05 on the bivariate analysis to check for independent associations with the outcome measures. Statistical software SPSS version 25.0 was used for the data analysis. Significance was defined as p < 0.05 for all analyses.RESULTSFifty-two adult patients with preoperative clinical and radiological data and a minimum follow-up of 12 months were included. Motor deficits, syrinx, and C1–C2 facet malalignment were found to have significant negative associations with the CCOS score at the 1- to 3-month follow-up (p < 0.05), while at the 9- to 12-month follow-up only swallowing function and C1–C2 facet malalignment were significantly associated with the CCOS score (p < 0.05). Multivariate analysis showed that syrinx presence and C1–C2 facet malalignment were independently associated with the CCOS score at the 1- to 3-month follow-up. Swallowing function and C1–C2 facet malalignment were found to be independently associated with the CCOS score at the 9- to 12-month follow-up.CONCLUSIONSThe observed results in this pilot study suggest a significant negative correlation between C1–C2 facet malalignment and clinical outcomes evaluated by the CCOS score at 1–3 months and 9–12 months postoperatively. Prospective studies are needed to further validate the prognostic value of C1–C2 facet malalignment and the potential role of atlantoaxial fixation as part of the treatment.

scholarly journals Standard and cardiac-gated phase-contrast magnetic resonance imaging in the clinical course of patients with Chiari malformation Type I

2011 ◽  
Vol 31 (3) ◽  
pp. E5 ◽  
Author(s):  
Uwe Max Mauer ◽  
Andreas Gottschalk ◽  
Carolin Mueller ◽  
Linda Weselek ◽  
Ulrich Kunz ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Chiari Malformation ◽  
Phase Contrast ◽  
Foramen Magnum ◽  
Central Canal ◽  
Type I ◽  
Csf Flow ◽  
Chiari Malformation Type I ◽  
Cerebrospinal Fluid Flow ◽  
Contrast Magnetic Resonance

Object The causal treatment of Chiari malformation Type I (CM-I) consists of removing the obstruction of CSF flow at the level of the foramen magnum. Cerebrospinal fluid flow can be visualized using dynamic phase-contrast MR imaging. Because there is only a paucity of studies evaluating CSF dynamics in the region of the spinal canal on the basis of preoperative and postoperative measurements, the authors investigated the clinical usefulness of cardiacgated phase-contrast MR imaging in patients with CM-I. Methods Ninety patients with CM-I underwent preoperative MR imaging of CSF pulsation. Syringomyelia was present in 59 patients and absent in 31 patients. Phase-contrast MR imaging of the entire CNS was used to investigate 22 patients with CM-I before surgery and after a mean postoperative period of 12 months (median 12 months, range 3–33 months). In addition to the dynamic studies, absolute flow velocities, the extension of the syrinx, and tonsillar descent were also measured. Results The changes in pulsation were highly significant in the region of the (enlarged) cistern (p = 0.0005). Maximum and minimum velocities (the pulsation amplitude) increased considerably in the region where the syrinx was largest in diameter. The changes of pulsation in these patients were significant in the subarachnoid space in all spinal segments but not in the syrinx itself and in the central canal. Conclusions The demonstration of CSF flow pulsation can contribute to assessments of surgical outcomes. The results presented here, however, raise doubts about current theories on the pathogenesis of syringomyelia.

Complete posterior cranial vault distraction osteogenesis to correct Chiari malformation type I associated with craniosynostosis

2021 ◽  
pp. 1-7
Keyword(s):  
Foramen Magnum ◽  
Posterior Cranial Fossa ◽  
Type I ◽  
Intracranial Volume ◽  
Tonsillar Herniation ◽  
Chiari Malformation Type I ◽  
Cerebellar Tonsillar Herniation ◽  
Cranial Fossa ◽  
The Mean ◽  
Tonsillar Descent

OBJECTIVE Posterior vault distraction osteogenesis (PVDO) is an effective tool to increase intracranial volume and expand the posterior cranial fossa. During PVDO, the authors extended osteotomy posterior to the foramen magnum to fully expand the posterior cranial fossa. The aim of this study was to investigate the efficacy of complete PVDO in posterior fossa expansion and treatment of Chiari malformation type I (CM-I) in patients with craniosynostosis. METHODS Patients with craniosynostosis who had undergone complete PVDO between January 2012 and May 2020 were reviewed retrospectively. A coronal osteotomy extending to the foramen magnum was performed and the foramen magnum was decompressed by removing its posterior rim with a 1-mm Kerrison rongeur. Four distractor devices were placed and the vector of distraction was controlled from the posterior to the inferior-posterior direction, depending on the deformity. Changes in the intracranial volume, posterior cranial fossa area, and cerebellar tonsillar descent were measured after complete PVDO by using CT and MRI. RESULTS A total of 11 patients with craniosynostosis and concurrent CM-I were included in the study. The mean age was 34.6 ± 24.0 months (continuous variables are expressed as the mean ± SD throughout). One patient had sleep apnea, which was consistent with CM-I, and another patient had a headache, which was nonspecific. The intracranial volume increased from 1179.6 ± 180.2 cm3 to 1440.6 ± 251.5 cm3 (p = 0.003; 24.5% increase compared to the preoperative volume). The posterior skull base area increased from 44.9 ± 19.3 cm2 to 72.7 ± 18.1 cm2 (p = 0.004). Cerebellar tonsillar descent decreased in all 11 patients after complete PVDO (preoperative: 10.8 ± 3.7 mm, postoperative: 2.7 ± 3.0 mm; p = 0.003). Among the 11 patients, 5 showed complete resolution of cerebellar tonsillar herniation. CONCLUSIONS Complete PVDO can more efficiently expand the posterior cranial fossa, unlike conventional methods. Moreover, it helps to relieve cerebellar tonsillar herniation. Complete PVDO is a powerful tool to increase the intracranial and posterior fossa volumes in patients with craniosynostosis and concurrent CM-I.

scholarly journals A Case of Chiari Malformation Type I : Improvement of Syringomyelia and Scoliosis by Foramen Magnum Decompression Alone

1999 ◽  
Vol 8 (11) ◽  
pp. 727-731
Author(s):  
Tetsuya Kubota ◽  
Kenichi Nishiyama ◽  
Akira Tamura ◽  
Kouichi Kawasaki ◽  
Hiroshi Masuda ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Type I ◽  
Foramen Magnum Decompression ◽  
Chiari Malformation Type I

*Siringomielia asociada a Malformación de Chiari I. Reporte de caso. [Syringomyelia associated with Chiari I Malformation. Case report.]

2021 ◽  
Vol 40 (03) ◽  
Author(s):  
Flor Wilson Giraldo ◽  
Hector Lezcano ◽  
Leonardo Barrios
Keyword(s):  
Cranial Nerves ◽  
Chiari I Malformation ◽  
Clinical Suspicion ◽  
Unknown Etiology ◽  
Type I ◽  
Imaging Studies ◽  
Structural Alterations ◽  
Truncal Ataxia ◽  
Chiari I ◽  
The Right

<p>Introducción: Las Malformaciones de Chiari (M.C.) son alteraciones estructurales a nivel del cerebelo de etiología en estudio. Muy raras, afectan al 0,5% de la población. Más de la mitad de los pacientes cursan con siringomielia. Caso clínico: Femenina de 47 años con antecedente de M.C. Tipo I, acude con cuadro de dos semanas de evolución de cefalea holocraneana de intensidad 8/10, asociado a mareo e inestabilidad a la marcha. Hallazgos positivos de alteración en los pares craneales III, VIII, IX; hipertonía, signo de Babinski y Hoffman positivos bilaterales; ataxia truncal; nistagmus a la derecha; fuerza muscular disminuida en miembros superiores e hiperreflexia. Discusión: Ante la sospecha clínica, y las múltiples alteraciones a nivel del cerebelo y bulbo, se procede a realizar estudios de imagen y se confirma el diagnóstico de siringomielia.</p><p><strong>Abstract: </strong></p><p>Introduction: Chiari malformations (C.M.) are structural alterations in cerebellum. They are of unknown etiology, at present in study. They are very rare and affect 0,5 % of the population. More than half of the patients have syringomyelia. Clinical case: A 47-year-old female with medical record of C.M. Type I, presents with a two-week evolution holocranial headache, 8/10 in intensity, associated with dizziness and gait instability. Positive findings of alteration in cranial nerves III, VIII, IX; hypertonia, Babinski and Hoffman bilateral sign; truncal ataxia; nystagmus on the right; decreased muscle strength in the upper limbs and hyperreflexia. Discussion: Due to clinical suspicion and multiple alterations in cerebellum and bulb, imaging studies are performed and the diagnosis of syringomyelia is confirmed.</p>

Complications following decompression of Chiari malformation Type I in children: dural graft or sealant?

2011 ◽  
Vol 8 (2) ◽  
pp. 177-183 ◽  
Author(s):  
Stephen R. Parker ◽  
Peggy Harris ◽  
Thomas J. Cummings ◽  
Timothy George ◽  
Herbert Fuchs ◽  
...  
Keyword(s):  
Aseptic Meningitis ◽  
Complication Rate ◽  
Chiari Malformation ◽  
Csf Leak ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Complication Rates ◽  
Chiari Malformation Type I ◽  
Dural Graft ◽  
Tissue Sealant

Object Posterior fossa decompression with duraplasty for Chiari malformation Type I (CM-I) is a common pediatric neurosurgery procedure. Published series report a complication rate ranging from 3% to 40% for this procedure. Historically, many dural substitutes have been used, including bovine grafts, human cadaveric pericardium, synthetic dura, and autologous pericranium. The authors hypothesized that a recently observed increase in complications was dependent on the graft used. Methods Between January 2004 and January 2008, 114 consecutive patients ≤ 18 years old underwent primary CM-I decompression using duraplasty. Records were retrospectively reviewed for short- and intermediate-term complications and operative technique, focusing on the choice of duraplasty graft with or without application of a tissue sealant. Results The average age of the patients was 8.6 years. The dural graft used was variable: 15 were treated with cadaveric pericardium, 12 with Durepair, and 87 with EnDura. Tisseel was used in 75 patients, DuraSeal in 12, and no tissue sealant was used in 27 patients. The overall complication rate was 21.1%. The most common complications included aseptic meningitis, symptomatic pseudomeningocele, or a CSF leak requiring reoperation. The overall complication rates were as follows: cadaveric pericardium 26.7%, Durepair 41.7%, and EnDura 17.2%; reoperation rates were 13%, 25%, and 8.1%, respectively. Prior to adopting a different graft product, the overall complication rate was 18.1%; following the change the rate increased to 35%. Complication rates for tissue sealants were 14.8% for no sealant, 18.7% for Tisseel, and 50% for DuraSeal. Nine patients were treated with the combination of Durepair and DuraSeal and this subgroup had a 56% complication rate. Conclusions Complication rates after CM-I decompression may be dependent on the dural graft with or without the addition of tissue sealant. The complication rate at the authors' institution approximately doubled following the adoption of a different graft product. Tissue sealants used in combination with a dural substitute to augment a duraplasty may increase the risk of aseptic meningitis and/or CSF leak. The mechanism of the apparent increased inflammation with this combination remains under investigation.

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