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Open access

Chiari malformation type 1: are we doing less with more? Illustrative case

Giuseppe Talamonti, Erika Ferrari, and Giuseppe D’Aliberti

BACKGROUND

Classic treatment of Chiari malformation type 1 consists of foramen magnum decompression. Selected patients may require occipitocervical fixation, transoral odontoidectomy, tonsillectomy, and so forth. Treatment standardization does not yet exist, and some patients risk being overtreated.

OBSERVATIONS

A 20-year-old man with headache and Chiari malformation type 1 underwent extradural bone decompression. One year later, he was managed with the extradural section of his filum terminale. Eighteen months later, the patient underwent monitoring of intracranial pressure, occipitocervical stabilization, transoral odontoidectomy, minimally invasive subpial tonsillectomy, and occipital cranioplasty. His headache never changed, and he progressively developed hemiparesis and swallowing and respiratory disturbances. Two years later, a new magnetic resonance imaging scan showed extended syringomyelia with scarce peritonsillar subarachnoid space. The umpteenth operation consisted of the removal of a constricting epidural scar, arachnoid dissection, total tonsillectomy, creation of a wide subarachnoid space, and dural sac augmentation. The patient’s initial postoperative course was smooth, and his headache improved. However, 8 days after surgery, the patient acutely presented with vegetative disturbances and died because of malignant brainstem edema of unknown origin.

LESSONS

The story of this patient is not so uncommon. He underwent all the possible surgical treatments rather than a timely adequate osteodural decompression. Probably, he received less with more.

Open access

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

Elizabeth Gallo, Gazanfar Rahmathulla, Dinesh Rao, Kourosh Tavanaiepour, and Daryoush Tavanaiepour

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.

Open access

Syringomyelia intermittens: highlighting the complex pathophysiology of syringomyelia. Illustrative case

Jorn Van Der Veken, Marguerite Harding, Saba Hatami, Marc Agzarian, and Nick Vrodos

BACKGROUND

Chiari Type I malformation (CM1) is a disorder recognized by caudal displacement of the cerebellar tonsils through the foramen magnum and into the cervical canal. Syringomyelia is frequently found in patients with CM1, but the pathophysiology of syringomyelia remains an enigma. As a general consensus, symptomatic patients should be treated and asymptomatic patients without a syrinx should not be treated. Mildly symptomatic patients or asymptomatic patients with a syrinx, on the other hand, pose a more challenging dilemma, as the natural evolution is uncertain. For many surgeons, the presence of a syrinx is an indication to offer surgery even if the patient is asymptomatic or mildly symptomatic.

OBSERVATIONS

The authors describe an illustrative case of a 31-year-old female with an incidental finding of a CM1 malformation and cervical syrinx in 2013. Conservative management was advocated as the patient was asymptomatic. Monitoring of the syrinx over a course of 8 years showed resolution, followed by reappearance and finally a complete resolution in 2021. A review of the literature and the possible pathophysiology is discussed.

LESSONS

The unusual course of this patient highlights the importance of guiding treatment by clinical symptoms, not radiological findings. Furthermore it reflects the complexity of the pathophysiology and the uncertain natural history of syringomyelia.

Open access

Pathophysiology and surgical treatment of spinal adhesive arachnoid pathology: patient series

Izumi Koyanagi, Yasuhiro Chiba, Genki Uemori, Hiroyuki Imamura, Masami Yoshino, and Toshimitsu Aida

BACKGROUND

Spinal adhesive arachnoid pathology is a rare cause of myelopathy. Because of rarity and variability, mechanisms of myelopathy are unknown. The authors retrospectively analyzed patients to understand pathophysiology and provide implications for surgical treatment.

OBSERVATIONS

Nineteen consecutive patients were studied. Thirteen patients had a secondary pathology due to etiological disorders such as spinal surgery or hemorrhagic events. They received arachnoid lysis (4 patients), syringo-subarachnoid (S-S) shunt (8 patients) with or without lysis, or anterior decompression. Three of them developed motor deterioration after lysis, and 6 patients needed further 8 surgeries. Another 6 patients had idiopathic pathology showing dorsal arachnoid cyst formation at the thoracic level that was surgically resected. With mean follow-up of 44.3 months, only 4 patients with the secondary pathology showed improved neurological grade, whereas all patients with idiopathic pathology showed improvement.

LESSONS

The idiopathic pathology was the localized dorsal arachnoid adhesion that responded to surgical treatment. The secondary pathology produced disturbed venous circulation of the spinal cord by extensive adhesions. Lysis of the thickened fibrous membrane with preservation of thin arachnoid over the spinal veins may provide safe decompression. S-S shunt was effective if the syrinx extended to the level of normal subarachnoid space.

Open access

Syrinx regression after correction of iatrogenic kyphotic deformity: illustrative case

Robert Y. North, Timothy J. Yee, Michael J. Strong, Yamaan S. Saadeh, Hugh J. L. Garton, and Paul Park

BACKGROUND

Syringomyelia has a long-established association with pediatric scoliosis, but few data exist on the relationship of syringomyelia to pediatric kyphotic deformities.

OBSERVATIONS

This report reviewed a unique case of rapid and sustained regression of syringomyelia in a 13-year-old girl after surgical correction of iatrogenic kyphotic deformity.

LESSONS

In cases of syringomyelia associated with acquired spinal deformity, treatment of deformity to resolve an associated subarachnoid block should be considered because it may obviate the need for direct treatment of syrinx.

Open access

Management of failed Chiari decompression and intrasyringeal hemorrhage in Noonan syndrome: illustrative cases

Cody J. Falls, Paul S. Page, Garret P. Greeneway, Daniel K. Resnick, and James A. Stadler III

BACKGROUND

Noonan syndrome (NS) is a rare genetic RASopathy with multisystem implications. The disorder is typically characterized by short stature, distinctive facial features, intellectual disability, developmental delay, chest deformity, and congenital heart disease. NS may be inherited or arise secondary to spontaneous mutations of genes in the Ras/mitogen activated protein kinase signaling pathways.

OBSERVATIONS

Numerous case reports exist detailing the association between NS and Chiari I malformation (CM-I), although this relationship has not been fully established. Patients with NS who present with CM-I requiring operation have shown high rates reoperation for failed decompression. The authors reported two patients with NS, CM-I, and syringomyelia who had prior posterior fossa decompressions without syrinx improvement. Both patients received reoperation with successful outcomes.

LESSONS

The authors highlighted the association between NS and CM-I and raised awareness that patients with these disorders may be at higher risk for failed posterior fossa decompression, necessitating reoperation.

Open access

Cervical cerebrospinal fluid venous fistula with syringomyelia treated with suboccipital decompression: illustrative case

Chia-Jung Hsieh, Lu-Ting Kuo, Dar-Ming Lai, and Abel Po-Hao Huang

BACKGROUND

Cerebrospinal fluid (CSF) venous fistulas are a recently discovered and underdiagnosed cause of spontaneous spinal CSF leak, which may lead to spontaneous intracranial hypotension. Most cases occur in the thoracic spine, and only 2 cases were reported in the cervical spine. Treatments include the epidural blood patch, fibrin glue injection, and surgical ligation of the fistula.

OBSERVATIONS

The authors report the treatment of a C6–7 CSF venous fistula, for which direct ligation was not feasible, with suboccipital decompression, leading to the complete resolution of the symptoms. Based on the clinical course and outcome in our patient, the authors summarize the previous theory and propose a hypothesis for the pathophysiology of headache and other symptoms in patients with CSF venous fistulas.

LESSONS

The symptoms of CSF venous fistulas may be linked not only to intracranial hypotension but also to the altered CSF dynamics induced by tonsillar herniation. Suboccipital decompression should be considered as a potential treatment option, especially in patients with Valsalva-induced headache who show a poor response to surgical ligation, patients in whom surgical ligation is not feasible, and patients with foramen magnum obstruction. Further investigation of the pathophysiology of CSF venous fistulas is warranted and should be performed in the future.

Open access

Spinal cord stimulator for the treatment of central neuropathic pain secondary to cervical syringomyelia: illustrative case

Bryan A. Schatmeyer, Rakan Dodin, Michael Kinsman, and David Garcia

BACKGROUND

Central neuropathic pain (CNP) of the cervical and/or thoracic spinal cord has many etiologies, both natural and iatrogenic. Frequently, CNP is medically refractory and requires surgical treatment to modulate the perception of pain. Spinal cord stimulation is a modality commonly used in adults to treat this type of refractory pain; however, it is rarely used in the pediatric population.

OBSERVATIONS

The authors reported a case involving a common pediatric condition, Chiari malformation type I with syrinx, that led to a debilitating complex regional pain syndrome. The associated life-altering pain was successfully alleviated following placement of a spinal cord stimulator.

LESSONS

CNP, or the syndromic manifestations of the pain (complex regional pain syndrome), can alter an individual’s life in dramatic ways. Spinal cord stimulator placement in carefully selected pediatric patients should be considered in these difficult pain treatment paradigms.

Open access

Coexisting ossification of the posterior longitudinal ligament, intramedullary hemangioblastoma, and syringomyelia of the cervical spine: illustrative case

Chi-Ruei Li, Cheng-Ying Lee, Wen-Yu Cheng, Hsin-Ni Li, Chih-Hsiang Liao, and Chiung-Chyi Shen

BACKGROUND

Ossification of the posterior longitudinal ligament (OPLL) is a rare but potentially devastating cause of severe spinal cord compression and degenerative cervical myelopathy. Because OPLL is rarely accompanied by prominent syringomyelia, when both are observed, other causes of syringomyelia should be considered. Simultaneous presentation of OPLL and hemangioblastoma of the cervical spine is a rare encounter and has never been reported in the English-language literature.

OBSERVATIONS

The authors present a case of a 64-year-old man with muscle weakness of the right upper limb and worsening dysesthesia of the right thumb and index finger. Noncontrast magnetic resonance imaging (MRI) of the cervical spine from another institution revealed OPLL from the C2 to C6 levels with severe spinal cord compression and prominent syringomyelia. Repeated MRI with contrast showed an intramedullary tumor, about 11 mm in diameter, at the right posterior aspect of the C4 level. The authors performed laminectomies from C1 to C6 with posterolateral fusion and removed the C4 tumor. Pathohistological examination of the tumor demonstrated hemangioblastoma.

LESSONS

Careful evaluation of the preoperative imaging study is extremely important in surgical decision making. Although rare, concomitant cervical hemangioblastoma should be listed in the differential diagnosis when OPLL is accompanied with prominent syringomyelia.

Open access

Spontaneous improvement in syringomyelia in a patient with Chiari 1 malformation: illustrative case

Oyku Ozturk, Emetullah Cındıl, Hakan Emmez, Pelin Kuzucu, and Emrah Celtıkcı

BACKGROUND

“Chiari malformation” refers to a spectrum of hindbrain abnormalities characterized by impaired cerebrospinal fluid circulation through the foramen magnum. Syringomyelia is frequently found in patients with Chiari malformation type 1. Although many theories have suggested how cerebrospinal fluid enters and makes the cystic cavity in the spinal cord, the pathogenesis of syringomyelia remains controversial. This report documents a case with spontaneous resolution of syringomyelia followed up by 3-year serial magnetic resonance imaging (MRI). These kinds of cases support a more conservative approach.

OBSERVATIONS

A 59-year-old female presented to the authors’ clinic in June 2019 with a history of Chiari malformation type 1. This symptomatic patient has been followed up with serial MRI. When the last MRI was performed in August 2022, compared with previous imaging, resolution of the syringomyelia was recognized.

LESSONS

Because the natural evolution of mildly symptomatic/asymptomatic patients with syringes is unclear, these patients pose a treatment dilemma. Although surgical intervention is a widely accepted therapeutic method, a more conservative approach can be considered in cases with spontaneous resolution. Especially for patients without progressive symptoms, the surgical approach should not be considered as the first step. In view of relapses, follow-up with periodic neurological examinations and radiological imaging is preferrable.