scholarly journals Secondary Chiari malformation due to enlarged spinal arachnoid villi–like structure: illustrative case

2021 ◽  
Vol 1 (2) ◽  
Author(s):  
Izumi Koyanagi ◽  
Yasuhiro Chiba ◽  
Hiroyuki Imamura ◽  
Masami Yoshino ◽  
Toshimitsu Aida

BACKGROUNDSecondary Chiari malformation can be caused by various disorders associated with cerebrospinal fluid (CSF) leakage at the spinal level. In this report, the authors describe a rare case of secondary Chiari malformation caused by excessive CSF absorption through the enlarged spinal arachnoid villi–like structure.OBSERVATIONSA 20-year-old woman presented with progressive severe headache and posterior neck pain. Magnetic resonance imaging showed tonsillar herniation and decreased subarachnoid space around the spinal cord. A hypointense signal area was observed in the ventral spinal canal on a T2-weighted image. An axial image revealed multiple small, arachnoid cyst–like structures at the right T1 nerve root sleeve. Direct surgery revealed that the cyst-like structures were continuous with the arachnoid membrane and protruded into the abnormally large epidural venous sinus. The cyst-like structures were resected, and the dural sleeve was repaired using fascia. The patient showed good improvement of symptoms after surgery.LESSONSExcessive CSF absorption through the enlarged spinal arachnoid villi–like structure can cause secondary Chiari malformation. Neurosurgeons should be aware of this unusual mechanism of CSF leakage. Simple posterior fossa decompression will be ineffective or even harmful.

2020 ◽  
Vol 26 (1) ◽  
pp. 53-59 ◽  
Author(s):  
Jennifer M. Strahle ◽  
Rukayat Taiwo ◽  
Christine Averill ◽  
James Torner ◽  
Jordan I. Gewirtz ◽  
...  

OBJECTIVEIn patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.METHODSA large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.RESULTSOf 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.CONCLUSIONSIn this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii448-iii448
Author(s):  
Jorge Luis Ramírez-Melo ◽  
Regina M Navarro-Martin del Campo ◽  
Manuel D Martinez-Albarran ◽  
Fernando Sánchez-Zubieta ◽  
Ana L Orozco-Alvarado ◽  
...  

Abstract BACKGROUND Primary central nervous system lymphoma (PCNSL) are very rare in children. CLINICAL CASE: An 11-year-old male presented with a 2 months history with myoclonic movements in the upper right limb, and a sudden frontal headache, gait disturbance due to right hemiparesis and an ipsilateral convulsive episode. Upon admission he had critical condition, with hypertensive skull syndrome, Glasgow of 12, Karnofsky 40%, right hemiparesis, swallowing disorder, facial paralysis, and loss of photo motor reflex and unilateral amaurosis. A CT and MRI showed a huge tumor mass in the left tempo-parietal region, infiltrating the white matter and shifting the midline. A Tumor biopsy was done, and reported diffuse small cell non-Hodgkin lymphoma of high-grade, Burkitt type. Systemic lymphoma workup was negative. He received six cycles of chemotherapy based on high dose methotrexate, rituximab and triple intrathecal.After the second cycle an ophthalmologic evaluation was done, and found infiltration to the right retina, for which 6 cycles of intra vitreous chemotherapy with methotrexate were applied, he showed an excellent response, and recovered all his neurological functions except that right hemianopia persist. Control MRI showed partial response at 2nd cycle and complete response after the 4th cycle. No Radiation was performed. CONCLUSION This report highlights the fact that pediatric PCNSL may be effectively treated by a combination of HDMTX and rituximab-based chemoimmunotherapy without irradiation. Lack of awareness of this rare entity may lead to extense resections of brain, and potential permanent secuelae that were avoided in this illustrative case.


2021 ◽  
Vol 11 (3) ◽  
pp. 299
Author(s):  
Yeong Jin Kim ◽  
Tae-Young Jung ◽  
In-Young Kim ◽  
Shin Jung ◽  
Kyung-Sub Moon

Postoperative complications after brain tumor surgery occur occasionally and it is important for clinicians to know how to properly manage each complication. Here, we described a rare case of late-onset, subdural fluid collection localized at the resection cavity that caused motor weakness after convexity meningioma resection, requiring differentiation from an abscess, to help clinicians determine treatment strategies. A 58-year-old right-handed female was admitted to the hospital with a headache and posterior neck pain. Brain computed tomography (CT) scans and magnetic resonance (MR) images showed a homogeneously enhanced, calcified, and multi-lobulated mass adjacent to the right motor strip without perilesional edema. The patient underwent surgery without incident or residual deficit and was discharged from the hospital in good condition. Six weeks after surgery, the patient complained of left arm monoparesis without infection-related symptoms. Brain imaging studies showed a localized fluid collection in the resection cavity with an enhanced margin and perilesional edema. Diffusion restriction was not detected. After three months of conservative treatment without surgery or antibiotics, she recovered from the neurologic deficits, and brain imaging studies showed the spontaneous regression of the fluid collection and perilesional edema. Late-onset, localized fluid collection at the resection cavity, which is similar to an abscess, could occur three to eight weeks after meningioma resection. When there are predisposing factors, including blood components and hemostatic materials in the surgical cavity, it is important for clinicians to understand this type of complication and choose conservative management as a feasible strategy.


2011 ◽  
Vol 21 (6) ◽  
pp. 1143-1150 ◽  
Author(s):  
Tao Wu ◽  
Zezhang Zhu ◽  
Jian Jiang ◽  
Xin Zheng ◽  
Xu Sun ◽  
...  

2001 ◽  
Vol 95 (1) ◽  
pp. 115-118 ◽  
Author(s):  
Ralf Weigel ◽  
Michael Rittmann ◽  
Joachim K. Krauss

✓ The authors report on a 31-year-old man with spontaneous craniocervical osseous fusion secondary to cervical dystonia (CD). After an 8-year history of severe CD, the patient developed a fixed rotation of his head to the right. Three-dimensional computerized tomography reconstructions revealed rotation and fixation of the occiput and C-1 relative to C-2, which was similar to that seen in atlantoaxial rotatory fixation. There was abnormal ossification of the odontoid facet joints and ligaments. Additional ossification was observed in the cervical soft tissue bridging the lateral mass of C-1 and the occiput. The patient underwent partial myectomy of the dystonic left sternocleidomastoid muscle and selective posterior ramisectomy of the right posterior neck muscles; postoperatively he experienced relief of his neck pain. In patients with CD refractory to conservative treatment, the appropriate timing of surgical treatment is important.


2008 ◽  
Vol 2 (1) ◽  
pp. 42-49 ◽  
Author(s):  
Susan R. Durham ◽  
Kristina Fjeld-Olenec

Object Surgery for Chiari malformation Type I (CM-I) is one of the most common neurosurgical procedures performed in children, although there is clearly no consensus among practitioners about which surgical method is preferred. The objective of this meta-analysis was to compare the outcome of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression without duraplasty (PFD) for the treatment of CM-I in children. Methods The authors searched Medline–Ovid, The Cochrane Library, and the conference proceedings of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons (2000–2007) for studies meeting the following inclusion criteria: 1) surgical treatment of CM-I; 2) surgical techniques of PFD and PFDD being reported in a single cohort; and 3) patient age < 18 years. Results Five retrospective and 2 prospective cohort studies involving a total of 582 patients met the criteria for inclusion in the meta-analysis. Of the 582 patients, 316 were treated with PFDD and 266 were treated with PFD alone. Patient age ranged from 6 months to 18 years. Patients undergoing PFDD had a significantly lower reoperation rate (2.1 vs 12.6%, risk ratio [RR] 0.23, 95% confidence interval [CI] 0.08–0.69) and a higher rate of cerebrospinal fluid–related complications (18.5 vs 1.8%, RR 7.64, 95% CI 2.53–23.09) than those undergoing PFD. No significant differences in either clinical improvement (78.6 vs 64.6%, RR 1.23, 95% CI 0.95–1.59) or syringomyelia decrease (87.0 vs 56.3%, RR 1.43, 95% CI 0.91–2.25) were noted between PFDD and PFD. Conclusions Posterior fossa decompression with duraplasty is associated with a lower risk of reoperation than PFD but a greater risk for cerebrospinal fluid–related complications. There was no significant difference between the 2 operative techniques with respect to clinical improvement or decrease in syringomyelia.


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