Spontaneous resolution and recurrence of a Chiari I malformation and associated syringomyelia

2000 ◽  
Vol 92 (2) ◽  
pp. 207-210 ◽  
Author(s):  
John C. L. Sun ◽  
Paul Steinbok ◽  
D. Douglas Cochrane
Keyword(s):  
Chiari Malformation ◽  
Chiari I Malformation ◽  
Spontaneous Resolution ◽  
Content Type ◽  
Chiari I ◽  
Fine Print

✓ The spontaneous resolution of syringomyelia has been reported infrequently. In patients with Chiari I malformations, resolution of the syringomyelia has sometimes been associated with improvement of their malformation. The authors present a case of spontaneous resolution followed by recurrence of syringomyelia and a corresponding change in the Chiari malformation. This case is of interest in light of the theories postulated to explain spontaneous resolution of syringomyelia.

Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases

1998 ◽  
Vol 88 (2) ◽  
pp. 237-242 ◽  
Author(s):  
John L. D. Atkinson ◽  
Brian G. Weinshenker ◽  
Gary M. Miller ◽  
David G. Piepgras ◽  
Bahram Mokri
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypotension ◽  
Chiari I Malformation ◽  
Csf Leak ◽  
Cerebrospinal Fluid Leakage ◽  
Content Type ◽  
Intracranial Hypotension Syndrome ◽  
Csf Leakage ◽  
Chiari I ◽  
Fine Print

Object. Spontaneous spinal cerebrospinal fluid (CSF) leakage with development of the intracranial hypotension syndrome and acquired Chiari I malformation due to lumbar spinal CSF diversion procedures have both been well described. However, concomitant presentation of both syndromes has rarely been reported. The object of this paper is to present data in seven cases in which both syndromes were present. Three illustrative cases are reported in detail. Methods. The authors describe seven symptomatic cases of spontaneous spinal CSF leakage with chronic intracranial hypotension syndrome in which magnetic resonance (MR) images depicted dural enhancement, brain sagging, loss of CSF cisterns, and acquired Chiari I malformation. Conclusions. This subtype of intracranial hypotension syndrome probably results from chronic spinal drainage of CSF or high-flow CSF shunting and subsequent loss of brain buoyancy that results in brain settling and herniation of hindbrain structures through the foramen magnum. Of 35 cases of spontaneous spinal CSF leakage identified in the authors' practice over the last decade, MR imaging evidence of acquired Chiari I malformation has been shown in seven. Not to be confused with idiopathic Chiari I malformation, ideal therapy requires recognition of the syndrome and treatment directed to the site of the spinal CSF leak.

Spontaneous resolution of a Chiari malformation with syringomyelia

2021 ◽  
Vol 14 (6) ◽  
pp. e241789
Author(s):  
Hadleigh Cuthbert ◽  
Joshua Pepper ◽  
Rupert Price
Keyword(s):  
Natural History ◽  
Literature Review ◽  
Chiari Malformation ◽  
Surgical Intervention ◽  
Chiari I Malformation ◽  
Spontaneous Resolution ◽  
Conservative Approach ◽  
Resonance Imaging ◽  
History Of ◽  
Chiari I

The Chiari I malformation (CM-I) is characterised by overcrowding of the posterior fossa and descent of the cerebellar tonsils and is associated with syringomyelia. With the increasing availability of magnetic resonance imaging, CM-I is placing a growing burden on neurosurgical services. However, its natural history remains poorly understood, and the timing and nature of surgical intervention is controversial. We present a case of a significant, symptomatic CM-I with associated syrinx which underwent complete spontaneous resolution over a 4-year period. Spontaneous regression of Chiari malformation and syringomyelia is exceedingly rare; a literature review reveals 15 other cases and only one case which underwent complete resolution. The present case and literature review suggest a more benign natural history of CM-I and support a more conservative approach to its management. Further studies are required to determine whether any factors can predict resolution for certain patient cohorts.

Elucidating the pathophysiology of syringomyelia

1999 ◽  
Vol 91 (4) ◽  
pp. 553-562 ◽  
Author(s):  
John D. Heiss ◽  
Nicholas Patronas ◽  
Hetty L. DeVroom ◽  
Thomas Shawker ◽  
Robert Ennis ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Subarachnoid Space ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Content Type ◽  
Cine Mr ◽  
Spinal Subarachnoid Space ◽  
Chiari I ◽  
Cerebellar Tonsils ◽  
Fine Print

Object. Syringomyelia causes progressive myelopathy. Most patients with syringomyelia have a Chiari I malformation of the cerebellar tonsils. Determination of the pathophysiological mechanisms underlying the progression of syringomyelia associated with the Chiari I malformation should improve strategies to halt progression of myelopathy.Methods. The authors prospectively studied 20 adult patients with both Chiari I malformation and symptomatic syringomyelia. Testing before surgery included the following: clinical examination; evaluation of anatomy by using T1-weighted magnetic resonance (MR) imaging; evaluation of the syrinx and cerebrospinal fluid (CSF) velocity and flow by using phase-contrast cine MR imaging; and evaluation of lumbar and cervical subarachnoid pressure at rest, during the Valsalva maneuver, during jugular compression, and following removal of CSF (CSF compliance measurement). During surgery, cardiac-gated ultrasonography and pressure measurements were obtained from the intracranial, cervical subarachnoid, and lumbar intrathecal spaces and syrinx. Six months after surgery, clinical examinations, MR imaging studies, and CSF pressure recordings were repeated. Clinical examinations and MR imaging studies were repeated annually. For comparison, 18 healthy volunteers underwent T1-weighted MR imaging, cine MR imaging, and cervical and lumbar subarachnoid pressure testing.Compared with healthy volunteers, before surgery, the patients had decreased anteroposterior diameters of the ventral and dorsal CSF spaces at the foramen magnum. In patients, CSF velocity at the foramen magnum was increased, but CSF flow was reduced. Transmission of intracranial pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was partially obstructed. Spinal CSF compliance was reduced, whereas cervical subarachnoid pressure and pulse pressure were increased. Syrinx fluid flowed inferiorly during systole and superiorly during diastole on cine MR imaging. At surgery, the cerebellar tonsils abruptly descended during systole and ascended during diastole, and the upper pole of the syrinx contracted in a manner synchronous with tonsillar descent and with the peak systolic cervical subarachnoid pressure wave. Following surgery, the diameter of the CSF passages at the foramen magnum increased compared with preoperative values, and the maximum flow rate of CSF across the foramen magnum during systole increased. Transmission of pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was normal and cervical subarachnoid mean pressure and pulse pressure decreased to normal. The maximum syrinx diameter decreased on MR imaging in all patients. Cine MR imaging documented reduced velocity and flow of the syrinx fluid. Clinical symptoms and signs improved or remained stable in all patients, and the tonsils resumed a normal shape.Conclusions. The progression of syringomyelia associated with Chiari I malformation is produced by the action of the cerebellar tonsils, which partially occlude the subarachnoid space at the foramen magnum and act as a piston on the partially enclosed spinal subarachnoid space. This creates enlarged cervical subarachnoid pressure waves that compress the spinal cord from without, not from within, and propagate syrinx fluid caudally with each heartbeat, which leads to syrinx progression. The disappearance of the abnormal shape and position of the tonsils after simple decompressive extraarachnoidal surgery suggests that the Chiari I malformation of the cerebellar tonsils is acquired, not congenital. Surgery limited to suboccipital craniectomy, C-1 laminectomy, and duraplasty eliminates this mechanism and eliminates syringomyelia and its progression without the risk of more invasive procedures.

Spontaneous decompression of syringomyelia: magnetic resonance imaging findings

1991 ◽  
Vol 74 (2) ◽  
pp. 283-286 ◽  
Author(s):  
Clifford R. Jack ◽  
Emre Kokmen ◽  
Burton M. Onofrio
Keyword(s):  
Magnetic Resonance ◽  
Complete Resolution ◽  
Chiari I Malformation ◽  
Minimal Change ◽  
Imaging Findings ◽  
Resonance Imaging ◽  
Content Type ◽  
Magnetic Resonance Studies ◽  
Chiari I ◽  
Fine Print

✓ The case of a 30-year-old woman with Chiari I malformation and a cervicothoracic syrinx is presented. The patient was followed clinically over a 2½-year period. Spontaneous and complete resolution of the syrinx, as documented by serial magnetic resonance studies, was accompanied by only a minimal change in objective symptomatology.

Inclination of the odontoid process in the pediatric Chiari I malformation

2003 ◽  
Vol 98 (1) ◽  
pp. 43-49 ◽  
Author(s):  
R. Shane Tubbs ◽  
John C. Wellons ◽  
Jeffrey P. Blount ◽  
Paul A. Grabb ◽  
W. Jerry Oakes
Keyword(s):  
Odontoid Process ◽  
Postoperative Outcome ◽  
Craniocervical Junction ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Magnetic Resonance Images ◽  
Posterior Aspect ◽  
Content Type ◽  
Chiari I ◽  
Fine Print

Object. The quantitative analysis of odontoid process angulation has had scant attention in the Chiari I malformation population. In this study the authors sought to elucidate the correlation between posterior angulation of the odontoid process and patients with Chiari I malformation. Methods. Magnetic resonance images of the craniocervical junction obtained in 100 children with Chiari I malformation and in 50 children with normal intracranial anatomy (controls) were analyzed. Specific attention was focused on measuring the degree of angulation of the odontoid process and assigning a score to the various degrees. Postoperative outcome following posterior cranial fossa decompression was then correlated to grades of angulation. Other measurements included midsagittal lengths of the foramen magnum and basiocciput, the authors' institutions' previously documented pB—C2 line (a line drawn perpendicular to one drawn between the basion and the posterior aspect of the C-2 body), level of the obex from a midpoint of the McRae line, and the extent of tonsillar herniation. Higher grades of odontoid angulation (retroflexion) were found to be more frequently associated with syringomyelia and particularly holocord syringes. Higher grades of angulation were more common in female patients and were often found to have obices that were caudally displaced greater than three standard deviations below normal. Conclusions. These results not only confirm prior reports of an increased incidence of a retroflexed odontoid process in Chiari I malformation but quantitatively define grades of inclination. Grades of angulation were not found to correlate with postoperative outcome. It is the authors' hopes that these data add to our current limited understanding of the mechanisms involved in hindbrain herniation.

Tonsillectomy without craniectomy for the management of infantile Chiari I malformation

2002 ◽  
Vol 97 (5) ◽  
pp. 1018-1022 ◽  
Author(s):  
Jorge A. Lazareff ◽  
Marcelo Galarza ◽  
Tooraj Gravori ◽  
Theodore J. Spinks
Keyword(s):  
Failure To Thrive ◽  
Vocal Cord Palsy ◽  
Chiari I Malformation ◽  
Cortical Atrophy ◽  
Limb Weakness ◽  
Content Type ◽  
Time Period ◽  
Chiari I ◽  
Cerebellar Tonsils ◽  
Fine Print

Object. The authors report their experience with 15 pediatric patients who underwent resection or shrinkage of the cerebellar tonsils without craniectomy or laminectomy, for the management of Chiari I malformation. Methods. The procedure was performed in six boys and nine girls with a mean age of 10 years. Thirteen patients presented with the congenital form of this disorder and two patients with Chiari I malformation caused by lumboperitoneal shunting. Clinical complaints included headaches (seven patients), scoliosis (four patients), numbness of the extremities (four patients), and upper-limb weakness (two patients). Two patients presented with failure to thrive and one with vocal cord palsy. Eight patients (six girls and two boys) had syringomyelia. The patients' symptoms had developed within a mean time period of 21 months (range 1–70 months). In all patients the cerebellar tonsils were exposed through a dura mater—arachnoid incision at the occipitoatlantal space. In seven patients the tonsils were resected and in the remaining eight patients the tonsils were shrunk by coagulating their surfaces. All patients improved postoperatively. Gliosis with cortical atrophy was observed in the resected neural tissue. Syringomyelia was reduced in seven of eight patients. The mean length of the follow-up period was 7 months. Conclusions. Removal of herniated cerebellar tonsils can be sufficient for alleviating symptoms in patients with Chiari I malformations.

Chiari I malformation: a rare cause of noncommunicating hydrocephalus treated by third ventriculostomy

2001 ◽  
Vol 95 (5) ◽  
pp. 783-790 ◽  
Author(s):  
Philippe Decq ◽  
Caroline Le Guérinel ◽  
Jean-Christophe Sol ◽  
Pierre Brugières ◽  
Michel Djindjian ◽  
...  
Keyword(s):  
Third Ventricle ◽  
Chiari I Malformation ◽  
Third Ventriculostomy ◽  
Transverse Diameter ◽  
Content Type ◽  
The Third ◽  
Chiari I ◽  
Noncommunicating Hydrocephalus ◽  
Fine Print

Object. Hydrocephalus associated with Chiari I malformation is a rare entity related to an obstruction in the flow of cerebrospinal fluid (CSF) in the foramen of Magendie. Like all forms of noncommunicating hydrocephalus, it can be treated by endoscopic third ventriculostomy (ETV). The object of this study is to report a series of five cases of hydrocephalus associated with Chiari I malformation and to evaluate the use of ETV in the treatment of this anomaly. Methods. Five patients (four women and one man with a mean age of 29.6 years) underwent ETV for hydrocephalus associated with Chiari I malformation between April 1991 and February 1997. All patients had presented with paroxysmal headaches, which in two cases were associated with visual disorders. All patients had also presented with hydrocephalus (mean transverse diameter of the third ventricle 12.79 mm; mean sagittal diameter of the fourth ventricle 18.27 mm) with a mean herniation of the cerebellar tonsils at 13.75 mm below the basion—opisthion line. Surgery was performed in all patients by using a rigid endoscope. No complications occurred either during or after the procedure, except in one patient who experienced a wound infection that was treated by antibiotic medications. The mean duration of follow up in this study was 50.39 months. Four patients became completely asymptomatic and remained stable throughout the follow-up period. One patient required an additional third ventriculostomy after 1 year, due to secondary closure, and has remained stable since that time. Postoperative magnetic resonance images demonstrated a significant reduction in the extent of hydrocephalus in all patients (mean transverse diameter of the third ventricle 6.9 mm [p = 0.0035]; mean sagittal diameter of the fourth ventricle 10.32 mm [p = 0.007]), with a mean ascent of the cerebellar tonsils from 13.75 mm below the basion—opisthion line to 7.76 mm below it (p = 0.01). In addition, CSF flow was identified on either side of the orifice of the third ventriculostomy in all patients postoperatively. Conclusions. Results in this series confirm the efficacy of ETV in the treatment of hydrocephalus associated with Chiari I malformation. It is a reliable, minimally invasive technique that also provides a better understanding of the pathophysiology of this malformation.

Posterior atlantooccipital membrane for duraplasty

2002 ◽  
Vol 97 (2) ◽  
pp. 266-268 ◽  
Author(s):  
R. Shane Tubbs ◽  
John C. Wellons ◽  
Jeffrey P. Blount ◽  
W. Jerry Oakes
Keyword(s):  
Foreign Body ◽  
Postoperative Complications ◽  
Pediatric Patients ◽  
Chiari I Malformation ◽  
Posterior Cranial Fossa ◽  
Content Type ◽  
Chiari I ◽  
Cranial Fossa ◽  
Fine Print

✓ The authors describe the use of autogenetic posterior atlantooccipital (PAO) membrane for duraplasty following after posterior cranial fossa surgery. The PAO membrane is routinely exposed for procedures of the posterior cranial fossa and merely needs to be dissected free of the underlying dura mater. Recently this membrane was obtained in several pediatric patients following procedures of the posterior cranial fossa such as duraplasty in case of Chiari I malformation. No postoperative complications were found at 6-month follow-up examination. The advantages of this intervention include less manipulation of muscle and fascia than that involved in other procedures and, therefore, seemingly less postoperative pain and the negation of issues inherent with foreign-body graft sources. The authors believe this structure to be of use as a dural substitute in small dural openings of the posterior cranial fossa.

Percutaneous thecoperitoneal shunt for syringomyelia

1991 ◽  
Vol 74 (5) ◽  
pp. 827-831 ◽  
Author(s):  
Umesh S. Vengsarkar ◽  
Venilal G. Panchal ◽  
Parimal D. Tripathi ◽  
Sushil V. Patkar ◽  
Alok Agarwal ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Clinical Improvement ◽  
Clinical Presentation ◽  
Simple Procedure ◽  
Chiari I Malformation ◽  
Magnetic Resonance Images ◽  
Content Type ◽  
The Third ◽  
Chiari I ◽  
Fine Print

✓ Between January and April, 1990, three consecutive cases of syringomyelia were treated by percutaneous placement of thecoperitoneal shunts. Two of these patients had undergone craniovertebral decompression earlier at other centers and the third was treated primarily by a thecoperitoneal shunt. In each case, the syrinx was associated with Chiari I malformation, although the clinical presentation was due to a myelopathy. All three patients obtained unequivocal benefit from this simple procedure. Postoperative magnetic resonance images showed considerable shrinkage of the cysts corresponding with clinical improvement.

“Acquired” Chiari I malformation

1994 ◽  
Vol 80 (6) ◽  
pp. 1099-1102 ◽  
Author(s):  
Paul P. Huang ◽  
Shlomo Constantini
Keyword(s):  
Magnetic Resonance ◽  
Mr Imaging ◽  
Chiari I Malformation ◽  
Congenital Defect ◽  
Content Type ◽  
Chiari I ◽  
Gait Abnormalities ◽  
Cerebellar Tonsils ◽  
Tonsillar Descent ◽  
Fine Print

✓ Tonsillar descent of the cerebellum in Chiari I malformations is often considered a congenital defect. A patient is presented in whom magnetic resonance (MR) imaging revealed normally positioned cerebellar tonsils; however, 1 year later MR imaging was repeated for evaluation of gait abnormalities and showed descent of the cerebellar tonsils. This case illustrates worsening symptoms with progressive descent of the cerebellar tonsils and suggests that Chiari I malformations can evolve postnatally.

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