scholarly journals Spontaneous syrinx resolution in patient with Chiari I malformation: illustrative case

2021 ◽  
Vol 1 (26) ◽  
Author(s):  
Elizabeth Gallo ◽  
Gazanfar Rahmathulla ◽  
Dinesh Rao ◽  
Kourosh Tavanaiepour ◽  
Daryoush Tavanaiepour
Keyword(s):  
Chiari Malformation ◽  
Surgical Intervention ◽  
Chiari I Malformation ◽  
Spontaneous Resolution ◽  
Illustrative Case ◽  
Type I ◽  
Conservative Approach ◽  
Chiari Malformation Type I ◽  
Hindbrain Herniation ◽  
Chiari I

BACKGROUIND Chiari malformations include a spectrum of congenital hindbrain herniation syndromes. In patients with the most common subtype, Chiari malformation Type I, 50% to 75% develop a syrinx. The pathogenesis of syringomyelia is not well understood, with multiple theories outlined in the literature. Although the presence of a syrinx in a patient with Chiari malformation is generally accepted as an indication for surgical intervention, there are documented cases of spontaneous resolution that support a more conservative approach to management. OBSERVATIONS The authors reported a case of spontaneous resolution of a cervical syrinx in an adult with an unchanged Chiari malformation. LESSONS Given the possibility of spontaneous resolution over time, the authors believe a more conservative approach of observation with periodic surveillance, magnetic resonance imaging, and neurological examination should be considered in the management of a patient with a Chiari malformation and associated syringomyelia.

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.

scholarly journals Interlayer Dural Split Technique for Chiari I Malformation Treatment in Adult -- Technical Note

2021 ◽  
Author(s):  
Ioan Alexandru Florian ◽  
Mihaela Maria Pop ◽  
Teodora Larisa Timis ◽  
Ioan Stefan Florian
Keyword(s):  
Chiari Malformation ◽  
Chiari I Malformation ◽  
Sensory Loss ◽  
Posterior Arch ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Control Series ◽  
Suboccipital Craniectomy ◽  
Chiari I ◽  
Split Technique

Objective: To present an alternative surgical technique in treating cases of Chiari I Malformation with mild-to-moderate syringomyelia after decompressive suboccipital craniectomy: incising only the outer layer of the dura mater, then dissecting it from the inner layer without opening the latter. Methods and Results: We utilized this technique in a short series of three cases who were admitted in our department for mild symptoms such as intermittent headache and dissociated sensory loss in the upper limbs, caused by a Chiari Malformation Type I. The patients were placed in the sitting position. We performed a reduced median suboccipital craniectomy and resection of the posterior arch of C1 adapted to the level of tonsil descent, from a limited superior half to a complete resection. Afterwards, we incised the outer dural layer, while sparing the inner one. Using a fine dissector, we then split apart the outer and inner layers to the margin of the craniectomy. Through the transparency of the inner layer and the arachnoid, the cerebellum and the medulla were visible and pulsating. An autologous fascia duraplasty was then performed. The postoperative course was favorable in all cases, patients being discharged without any deficits and with complete symptom resolution. Conclusions: Interlayer dural split technique can be used effectively in treating symptomatic cases of type I Chiari malformation in adults, with mild-to-moderate syringomyelia. It is less invasive than opening the dura and possibly more effective than decompressive craniectomy and C1 laminectomy alone. This technique must be validated in a larger case-control series.

scholarly journals Acquired Chiari I malformation due to lumboperitoneal shunt: A case report and review of literature

2019 ◽  
Vol 10 ◽  
pp. 78
Author(s):  
Aslam Hentati ◽  
Mohamed Badri ◽  
Kamel Bahri ◽  
Ihsen Zammel
Keyword(s):  
Chiari Malformation ◽  
Rare Complication ◽  
Chiari I Malformation ◽  
Clinical Feature ◽  
Type I ◽  
Cerebral Magnetic Resonance Imaging ◽  
Surgical Closure ◽  
Lumboperitoneal Shunt ◽  
Cerebral Mri ◽  
Chiari I

Background: The Type I malformations are supposed to be the result of mesodermal defects that create a congenitally small posterior fossa. However, Chiari malformation could be also “iatrogenic” and then called “acquired” Chiari I malformation. In this study, the authors report the clinical feature of a patient who developed a Chiari I malformation after lumboperitoneal shunt. Case Description: A 35-year-old woman has been suffering from idiopathic intracranial hypertension and rhinorrhea due to an anterior skull base defect. A valveless lumboperitoneal shunt followed by surgical closure of the defect was performed. Six months later, she suffered from major continuous occipital headaches. The neurological examination found a mild cerebellar gait ataxia and cerebellar dysarthria. The cerebral magnetic resonance imaging (MRI) showed a ptosis of the cerebellar tonsils and a disappearance of the cisterna magna; there was no syringomyelia. This herniation was not present before shunt was performed. A replacement of the lumboperitoneal shunt with a pressure-regulated valve chamber was performed. After a 1-year follow-up, the patient reports a marked decrease of the headache as well as the ataxia, and the last cerebral MRI showed resolution of the Chiari I malformation. Conclusions: Symptomatic acquired Chiari malformation with or without syringomyelia as a delayed complication after lumbar shunting is a rare complication, particularly reported in the pediatric population, but could also occur to adult patients. Treating these patients by correcting the shunt’s valve could be enough, but should be monitored, as it may fail to resolve the Chiari malformation even years after treatment.

Advanced Chiari malformation Type I with marked spinal cord atrophy

2005 ◽  
Vol 3 (4) ◽  
pp. 324-327 ◽  
Author(s):  
Yoshiro Ito ◽  
Koji Tsuboi ◽  
Hiroyoshi Akutsu ◽  
Satoshi Ihara ◽  
Akira Matsumura
Keyword(s):  
Spinal Cord ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Type I ◽  
Foramen Magnum Decompression ◽  
Spinal Cord Atrophy ◽  
Chiari Malformation Type I ◽  
Content Type ◽  
Neurological Improvement ◽  
Chiari I

✓ The authors discuss the results obtained in patients who underwent foramen magnum decompression for longstanding advanced Chiari I malformation in which marked spinal cord atrophy was present. This 50-year-old woman presented with progressive quadriparesis and sensory disorders. Magnetic resonance imaging revealed the descent of cerebellar tonsils and medulla associated with remarkable C1—L2 spinal cord atrophy. After a C-1 laminectomy—based foramen magnum decompression, arachnoid dissection and duraplasty were undertaken. These procedures resulted in remarkable neurological improvement, even after 40 years of clinical progression. Spinal cord atrophy may be caused by chronic pressure of entrapped cerebrospinal fluid in the spinal canal.

Spontaneous resolution of a 13-mm Chiari malformation Type I in relation to differential growth of the posterior fossa volume

2009 ◽  
Vol 3 (2) ◽  
pp. 110-114 ◽  
Author(s):  
Ben Waldau ◽  
Leahthan F. Domeshek ◽  
Fawn A. Leigh ◽  
Kristian C. Lum ◽  
Herbert E. Fuchs ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Normal Development ◽  
Spontaneous Resolution ◽  
Type I ◽  
Normal Range ◽  
Differential Growth ◽  
Morphometric Measurements ◽  
Chiari Malformation Type I ◽  
Time Period

The case of a 3-year-old patient with tuberous sclerosis and a 13-mm Chiari malformation Type I that spontaneously disappeared over the course of 4 years is presented. Using morphometric measurements of the posterior fossa and cerebellum in this patient, the authors show that the volume of the posterior fossa at the time of initial evaluation was consistent with that reported as normal in the literature (180.24 cm3; normal volume 132–198 cm3). Moreover, the patient showed a normal rate of growth of his posterior fossa over the period of observation (201.05 cm3; normal range 153–230 cm3). Cerebellar volumes were found to increase only minimally during this time period, which is compatible with observations in healthy controls. The posterior fossa volume, on the other hand, was shown to increase significantly more than that of the cerebellum (p = 0.0185). This differential growth may permit the tonsils to ascend back up into the posterior fossa. Therefore, pediatric patients with normal posterior fossa volumes and normal development may have a spontaneous resolution of their asymptomatic Chiari malformation Type I.

Volumetric analysis of the posterior cranial fossa in a family with four generations of the Chiari malformation Type I

2008 ◽  
Vol 1 (1) ◽  
pp. 21-24 ◽  
Author(s):  
R. Shane Tubbs ◽  
Mark Hill ◽  
Marios Loukas ◽  
Mohammadali M. Shoja ◽  
W. Jerry Oakes
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Familial Aggregation ◽  
Volumetric Analysis ◽  
Posterior Cranial Fossa ◽  
Preoperative Imaging ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Hindbrain Herniation ◽  
Cranial Fossa

Object Many authors have concluded that the Chiari malformation Type I (CM-I) is due to a smaller than normal posterior cranial fossa. In order to establish this smaller geometry as the cause of hindbrain herniation in a family, the authors of this paper performed volumetric analysis in a family found to have this malformation documented in 4 generations. Methods Members from this family found to have a CM-I by imaging underwent volumetric analysis of their posterior cranial fossa using the Cavalieri method. Results No member of this family found to have CM-I on preoperative imaging had a posterior fossa that was significantly smaller than that of age-matched controls. Conclusions The results of this study demonstrate that not all patients with a CM-I will have a reduced posterior cranial fossa volume. Although the mechanism for the development of hindbrain herniation in this cohort is unknown, this manifestation can be seen in multiple generations of a familial aggregation with normal posterior fossa capacity.

Urgent surgical intervention in pediatric patients with Chiari malformation Type I

2007 ◽  
Vol 107 (1) ◽  
pp. 49-52 ◽  
Author(s):  
John C. Wellons ◽  
R. Shane Tubbs ◽  
Cuong J. Bui ◽  
Paul A. Grabb ◽  
W. Jerry Oakes
Keyword(s):  
Chiari Malformation ◽  
Pediatric Patients ◽  
Surgical Intervention ◽  
Type I ◽  
Chiari Malformation Type I

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.

Spontaneous resolution of syringomyelia and Chiari malformation Type I in a patient with cerebrospinal fluid otorrhea

2006 ◽  
Vol 105 (5) ◽  
pp. 769-771 ◽  
Author(s):  
Nicholas D. Coppa ◽  
H. Jeffrey Kim ◽  
Kevin M. McGrail
Keyword(s):  
Cerebrospinal Fluid ◽  
Chiari Malformation ◽  
Spontaneous Resolution ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Simultaneous Development ◽  
Cerebrospinal Fluid Otorrhea

✓ The spontaneous resolution of syringomyelia in the setting of a Chiari malformation Type I (CM-I) has been reported infrequently. Several theories about the pathogenesis and spontaneous resolution of syringomyelia associated with CM-I have been proposed. The authors present the case of a patient with spontaneous resolution of a CM-I and syringomyelia coinciding with the development of cerebrospinal fluid (CSF) otorrhea. Although cases of spontaneous resolution of syringomyelia have been reported, this is the first reported case of spontaneous resolution of syringomyelia and a CM-I associated with the simultaneous development of CSF otorrhea.

scholarly journals Imaging Findings in Chiari I Malformation with Syringomyelia in a Case of Charcot Shoulder

2011 ◽  
Vol 1 ◽  
pp. 46 ◽  
Author(s):  
Shantanu Kumar ◽  
Vineet Sharma ◽  
Santosh Kumar ◽  
Sonal Jain
Keyword(s):  
Shoulder Joint ◽  
Fluid Collection ◽  
Joint Space ◽  
Chiari I Malformation ◽  
Type I ◽  
Neuropathic Arthropathy ◽  
Chiari Malformation Type I ◽  
Lateral Dislocation ◽  
Chiari I ◽  
The Right

Neuropathic arthropathy of the shoulder is reported in only 5% of cases. Here, we report a rare case of neuropathic arthropathy of the shoulder, secondary to Chiari malformation Type I with associated syringomyelia, that remained undetected for four years. A 38-year-old female presented to our Department with a swelling over the right shoulder that had persisted for four years. X-ray of the joint showed destruction of the head of the right humerus, with typical blunt amputated appearance of the bone and increased joint space. Magnetic resonance imaging showed destruction and lateral dislocation of the head of the humerus. Large amount of fluid collection was seen in and around the right shoulder joint. Neuropathic osteoarthropathy can be defined as bone and joint changes that occurs secondary to loss of sensation. In our case, neuropathic shoulder joint was secondary to syringomyelia associated with Chiari I malformation.

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