Spinal cord herniation: a misdiagnosed and treatable cause of thoracic myelopathy

Idiopathic spinal cord herniation (ISCH), where a segment of the spinal cord has herniated through a ventral defect in the dura, is a rarely encountered cause of thoracic myelopathy. The purpose of our study was to increase the clinical awareness of this condition by presenting our experience with seven consecutive cases treated in our department since 2005. All the patients developed pronounced spastic paraparesis or Brown-Séquard syndrome for several years (mean, 4.7 years) prior to diagnosis. MRI was consistent with a transdural spinal cord herniation in the mid-thoracic region in all the cases. The patients underwent surgical reduction of the herniated spinal cord and closure of the dural defect using an artificial dural patch. At follow-up, three patients experienced considerable clinical improvement, one had slight improvement, one had transient improvement, and two were unchanged. Two of the four patients with sphincter dysfunction regained sphincter control. MRI showed realignment of the spinal cord in all the patients. ISCH is probably a more common cause of thoracic myelopathy than previously recognized. The patients usually develop progressive myelopathy for several years before the correct diagnosis is made. Early diagnosis is important in order to treat the patients before the myelopathy has become advanced.

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Operative Management of Idiopathic Spinal Cord Herniation: Case Series and Novel Technique for Repair of Recurrent Herniation

Operative Neurosurgery ◽

10.1093/ons/opy129 ◽

2018 ◽

Vol 16 (4) ◽

pp. 415-423 ◽

Cited By ~ 2

Author(s):

Randall J Hlubek ◽

David S Xu ◽

Celene B Mulholland ◽

Jourdan Gilson ◽

Nicholas Theodore ◽

...

Keyword(s):

Spinal Cord ◽

Pathological Condition ◽

Case Series ◽

Collagen Matrix ◽

Operative Management ◽

Dural Defect ◽

Thoracic Myelopathy ◽

Lower Extremity Weakness ◽

Spinal Cord Herniation ◽

Idiopathic Spinal Cord Herniation

Abstract BACKGROUND Idiopathic spinal cord herniation (ISCH) is a rare pathology of the spine defined by herniation of the spinal cord through a dural defect. OBJECTIVE To highlight the operative management of ISCH and the surgical nuances of ISCH repairs conducted at our institution. METHODS This retrospective review examines consecutive patients with ISCH who were treated surgically between January 1, 2010, and July 31, 2017, at Barrow Neurological Institute, Phoenix, Arizona. RESULTS Four patients with ISCH presented with thoracic myelopathy and lower extremity weakness during the study period. Treatment consisted of reduction of the herniated spinal cord and filling of the dural defect with a collagen-based dural regeneration matrix. In 3 patients the dural edges were covered with a collagen-matrix intradural sling, and in 1 patient they were repaired primarily with interrupted sutures. Three of the 4 patients experienced improvement in myelopathic symptoms; the fourth patient suffered neurological decline in the immediate postoperative period. CONCLUSION ISCH is a complex pathological condition likely to result in progressive myelopathy. Surgery offers patients the possibility of stabilizing the progression of the spinal cord dysfunction and perhaps restoring neurological function. However, extreme care must be taken during surgery to minimize manipulation of the fragile herniated cord.

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Spontaneous resolution of idiopathic thoracic spinal cord herniation: case report

Journal of Neurosurgery Spine ◽

10.3171/2014.12.spine14950 ◽

2015 ◽

Vol 23 (3) ◽

pp. 306-308 ◽

Cited By ~ 12

Author(s):

Nardin Samuel ◽

Christina L. Goldstein ◽

Carlo Santaguida ◽

Michael G. Fehlings

Keyword(s):

Spinal Cord ◽

Neurological Impairment ◽

Primary Treatment ◽

Spontaneous Resolution ◽

Neurological Deficits ◽

Thoracic Myelopathy ◽

Thoracic Spinal Cord ◽

Thoracic Spinal ◽

Spinal Cord Herniation ◽

Idiopathic Spinal Cord Herniation

Spinal cord herniation is a relatively rare but increasingly recognized clinical entity, with fewer than 200 cases reported in the literature to date. The etiology of this condition remains unknown, and surgery is used as the primary treatment to correct the herniation and consequent spinal cord compromise. Some patients without clinical progression have been treated with nonoperative measures, including careful follow-up and symptomatic physical therapy. To date, however, there has been no published report on the resolution of spinal cord herniation without surgical intervention. The patient in the featured case is a 58-year-old man who presented with mild thoracic myelopathy and imaging findings consistent with idiopathic spinal cord herniation. Surprisingly, updated MRI studies, obtained to better delineate the pathology, showed spontaneous resolution of the herniation. Subsequent MRI 6 months later revealed continued resolution of the previous spinal cord herniation. This is the first report of spontaneous resolution of a spinal cord herniation in the literature. At present, the treatment of this disorder is individualized, with microsurgical correction used in patients with progressive neurological impairment. The featured case highlights the potential variability in the natural history of this condition and supports considering an initial trial of nonoperative management for patients with mild, nonprogressive neurological deficits.

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A Case of Ventral Spinal Cord Herniation from a Chronic Dural-pleural Fistula Resulting in Thoracic Myelopathy

Cureus ◽

10.7759/cureus.6123 ◽

2019 ◽

Author(s):

Ilyas Eli ◽

Jian Guan ◽

Michael Karsy ◽

Marcus D Mazur ◽

Andrew Dailey

Keyword(s):

Spinal Cord ◽

Thoracic Myelopathy ◽

Spinal Cord Herniation ◽

Ventral Spinal Cord

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Thoracic ventral spinal cord herniation with progressive myelopathy – A case report and review of the literature

Surgical Neurology International ◽

10.25259/sni_496_2021 ◽

2021 ◽

Vol 12 ◽

pp. 382

Author(s):

Taylor Anne Wilson ◽

Ramachandran Pillai Promod Kumar ◽

Emmanuel Omosor

Keyword(s):

Spinal Cord ◽

Neurological Deficits ◽

Case Description ◽

Review Of The Literature ◽

Imaging Studies ◽

Thoracic Myelopathy ◽

Progressive Myelopathy ◽

Spinal Cord Herniation ◽

Idiopathic Spinal Cord Herniation ◽

Ventral Spinal Cord

Background: Idiopathic spinal cord herniation (ISCH) is a rare, underrecognized, and often misdiagnosed entity of unclear pathogenesis that typically presents as a slowly progressive thoracic myelopathy. There are less than 200 such cases reported in the literature. ISCH diagnosis and treatment are often delayed contributing to greater fixed neurological deficits, often leading to costly, unnecessary imaging studies, and inappropriate surgery. Case Description: Here, a 48-year-old female presented with trauma-induced ISCH characterized by gradually worsening lower extremity myelopathy. Conclusion: Idiopathic spinal cord herniation (ISCH) is rare, often underdiagnosed posttraumatic myelopathy that, when accurately diagnosed and treated, can result in good outcomes.

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Thoracic idiopathic spinal cord herniation in a young patient: a diagnostic and therapeutic challenge

BMJ Case Reports ◽

10.1136/bcr-2018-227847 ◽

2019 ◽

Vol 12 (5) ◽

pp. e227847 ◽

Cited By ~ 1

Author(s):

D Gomez-Amarillo ◽

C Garcia-Baena ◽

D Volcinschi-Moros ◽

F Hakim

Keyword(s):

Spinal Cord ◽

Posterior Approach ◽

Dural Defect ◽

Thoracic Myelopathy ◽

Postoperative Mri ◽

Younger Age ◽

Spinal Cord Herniation ◽

Idiopathic Spinal Cord Herniation ◽

Brown Sequard Syndrome

Idiopathic Spinal Cord Herniation (ISCH) is considered to be a rare cause of Thoracic Myelopathy. It is secondary to the gliding of the Spinal Cord through an anterior dural defect, without a completely defined cause. We present a case of ISCH which, even though was in its usual location, developed in a woman at a younger age than expected. The patient was 20 years old when diagnosed with Brown-Séquard Syndrome. MRI showed herniation at T4-T5 level, which was corrected using a posterior approach to expose the dural defect, reduce the herniation and place a heterologous graft. Postoperatively, neurological function improved, and adequate reduction was seen on imaging. Given the reports of recurrence and deterioration that have been seen after 18 months, follow-up was prolonged for a total of 2 years. We consider postoperative MRI performance important to establish the degree of reduction and alignment of the Spinal Cord.

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Idiopathic Spinal Cord Herniation—Case Report

Arquivos Brasileiros de Neurocirurgia Brazilian Neurosurgery ◽

10.1055/s-0036-1581084 ◽

2016 ◽

Vol 37 (03) ◽

pp. 280-283

Author(s):

Rogerio Sacco ◽

Ricardo Gepp ◽

Marco Quiroga ◽

Henrique Souza ◽

Vitor Aguiar ◽

...

Keyword(s):

Spinal Cord ◽

Lower Limbs ◽

Surgical Trauma ◽

Dural Defect ◽

Thoracic Myelopathy ◽

Radiological Investigation ◽

Postoperative Mri ◽

Spinal Cord Herniation ◽

History Of ◽

Idiopathic Spinal Cord Herniation

AbstractIdiopathic spinal cord herniation is a rare cause of progressive myelopathy, especially in the absence of a history of spinal or surgical trauma. The radiological diagnosis is made through a myelography or an MRI exam. The spinal cord is pushed anteriorly, buffering the dural defect and leading in most cases to Brown-Séquard syndrome. The present study describes the case of a male patient with a clinical picture of progressive thoracic myelopathy. In the clinical and radiological investigation, an idiopathic spinal cord herniation on the chest level was identified. During the surgery, the spinal cord was reduced to the natural site, taking its usual elliptical shape, and the dural defect was repaired with a dural substitute. The numbness of the patient improved, and the shocks in the lower limbs disappeared. A postoperative MRI confirmed the surgical reduction of the herniation and the restoration of the anterior cerebrospinal fluid (CSF) column to the spinal cord. The authors describe the clinical, radiological, intraoperative, and postoperative evolution.

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Repair of Thoracic Spinal Cord Herniation: 2-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opy409 ◽

2019 ◽

Vol 17 (4) ◽

pp. E161-E161

Author(s):

Siri Sahib S Khalsa ◽

Yamaan S Saadeh ◽

Brandon W Smith ◽

Jacob R Joseph ◽

Mark E Oppenlander

Keyword(s):

Spinal Cord ◽

Lower Extremities ◽

Urinary Symptoms ◽

Dural Defect ◽

Thoracic Myelopathy ◽

Thoracic Spinal Cord ◽

Thoracic Spinal ◽

Spinal Cord Herniation ◽

Patient Consent ◽

Progressive Weakness

Abstract Spinal cord herniation is an uncommon surgically treatable cause of thoracic myelopathy and progressive paraplegia. The thoracic spinal cord focally protrudes through a defect in the dura, resulting in progressive weakness, numbness, and spasticity affecting the lower extremities, in addition to possible urinary symptoms. In this video, we present the case of a 69-yr-old female who presented with 3 yr of progressive thoracic myelopathy due to a thoracic spinal cord herniation at T4-T5. We demonstrate the surgical steps to lyse arachnoid webs, mobilize the spinal cord, reduce the spinal cord herniation, and repair the dural defect. Appropriate patient consent was obtained.

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Spinal cord herniation repair with microstaples: case report

Journal of Neurosurgery Spine ◽

10.3171/2016.8.spine16318 ◽

2017 ◽

Vol 26 (3) ◽

pp. 384-387 ◽

Cited By ~ 5

Author(s):

Pedro David Delgado-López ◽

Cecilia Gil-Polo ◽

Vicente Martín-Velasco ◽

Javier Martín-Alonso ◽

Ana María Galacho-Harriero ◽

...

Keyword(s):

Spinal Cord ◽

Spastic Paraparesis ◽

Dural Defect ◽

Thoracic Myelopathy ◽

Dural Closure ◽

Spinal Cord Herniation ◽

Surgical Options ◽

Mri Study ◽

Idiopathic Spinal Cord Herniation

Idiopathic spinal cord herniation (ISCH) is a relatively rare and frequently misdiagnosed condition. It preferentially affects women and causes progressive thoracic myelopathy that presents as a Brown-Séquard syndrome or as spastic paraparesis. Although its etiology and pathogenesis are controversial, ISCH is characterized by the presence of an anterior dural defect that allows the incarceration of a segment of the cord. Typically, a C-shaped ventral displacement and kinking of the cord are visible on sagittal MRI. Surgery aimed at stopping or reversing myelopathic symptoms is usually recommended for symptomatic patients. Surgical options include reduction of the hernia and direct suturing, or enlargement of the dural defect, with or without patching. Suturing under the cord in a very tight space can be troublesome and may lead to neurological deterioration. The authors present the case of a symptomatic ISCH in which nonpenetrating titanium microstaples were used to close the dural defect after cord reduction. The patient experienced a good outcome, and the follow-up MRI study showed adequate cord repositioning and stability of the suture. The use of microstaples, which allows for an easier and faster dural closure than conventional suturing, is a novel technical adjunct that has not been previously reported for this condition. In addition, microstaples produce minimal metallic artifact that does not hinder the quality of follow-up MR images.

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