Idiopathic anterior herniation of the thoracic cord: a review.
Idiopathic spinal cord herniation (ISCH) is a rare condition caused by a defect in the dura mater, resulting in ventral displacement of the spinal cord. Its etiology is not fully understood, but it mainly affects middle-aged women and manifests as progressive myelopathy. Surgical treatment is the best option to avoid neurological worsening. This report presents a case of spinal cord herniation in a 45-year-old man, complaining of numbness for 4 months, with paraparesis that progressed to gait disorder. This condition was diagnosed by magnetic resonance imaging and computed tomography. The patient underwent T2 to T3 laminectomy, hernia reduction, and duroplasty, with successful resolution of the condition.
- Research Article
1
- 10.18926/amo/64124
- Dec 1, 2022
- Acta medica Okayama
We report a rare case of idiopathic spinal cord herniation (ISCH) with a history of cerebrospinal fluid (CSF) leakage. ISCH is a protrusion of the spinal cord through a dural defect. Thin constructive interference in steady-state (CISS) images clearly demonstrated the herniated cord in the present case. The myelopathy worsened and the patient underwent surgery for reduction of herniated spinal cord; the dural defect was filled by placing collagen matrix graft (DuraGen®) between the inner and outer dural layers. The patient's symptoms have improved without relapse for 8 months since surgery. This method may be a good surgical option for cases of spinal cord herniation.
- Research Article
2
- 10.1159/000323798
- Feb 23, 2011
- Neurodegenerative Diseases
Background: Idiopathic spinal cord herniation (SCH) is a rare and often misdiagnosed condition characterized by displacement of the spinal cord through an anterior defect of the dural sac. This condition determines continuous focal trauma of the spinal cord, causing slowly progressive myelopathy. The peculiar MR scan findings, particularly sagittal T<sub>2</sub>-weighted images, allow its recognition. Objective: Herein, we report 3 cases of SCH and suggest a possible association with trauma-related neurodegenerative or chronic inflammatory diseases. Methods: We reviewed the clinical files of all patients admitted to our hospital with a history and signs of progressive myelopathy (paraparesis and/or lower extremity hypoesthesia) who were diagnosed with SCH by MRI. Results: We found 3 female patients (37, 69 and 68 years of age) with a diagnosis of SCH. Two of them presented with concomitant, relatively rare disabling neurological diseases, namely amyotrophic lateral sclerosis and multiple sclerosis. The third patient underwent surgery and gradually recovered over 6 months. Conclusions: Persistent central nervous system trauma due to idiopathic SCH might provide an unexpected hint about the pathogenesis of amyotrophic lateral sclerosis and multiple sclerosis.
- Research Article
8
- 10.1016/j.wneu.2017.01.078
- Feb 1, 2017
- World Neurosurgery
Spinal Cord Herniation After Cervical Corpectomy with Cerebrospinal Fluid Leak: Case Report and Review of the Literature.
- Research Article
- 10.1016/j.ijscr.2025.111394
- Jun 1, 2025
- International journal of surgery case reports
A successful surgical management of spinal cord herniation in a patient with old thoracic spine fracture: a case report from Syria.
- Research Article
23
- 10.1016/j.spinee.2011.07.003
- Aug 1, 2011
- The Spine Journal
Idiopathic spinal cord herniation at two separate zones of the thoracic spine: the first reported case and literature review
- Research Article
2
- 10.22603/ssrr.2023-0102
- May 27, 2024
- Spine Surgery and Related Research
Idiopathic spinal cord herniation (ISCH) is a rare condition that is characterized by ventral herniation of the spinal cord through a defect in the dura mater into the epidural space, with no identifiable cause. ISCH is frequently underdiagnosed, and the information available in case reports is limited. To provide an overview of the clinical manifestations and diagnosis of this condition, this study aims to conduct a review of reported cases of ISCH. A literature review was carried out using seven databases. The search was conducted using the keywords "Idiopathic spinal cord herniation" OR "Idiopathic Ventral Spinal Cord Herniation" AND "Case report" OR "case series." A total of 92 relevant papers reporting 224 cases, besides the index case, were determined. Of the cases, 58.5% were females and the mean age was 50.7 (SD 13.2) years. Symptoms, diagnoses, and outcomes were similar between genders. The most common clinical signs included motor symptoms (82.6%), instability (61.3%), hypoesthesia (59.2%), and disturbance of thermal sensitivity (47.3%). Brown-Séquard syndrome was observed in 27.2% of the cases, and surgical treatment was employed in 89.7% of the cases. ISCH is a pathology that is principally treated with surgical approach. This study provides valuable insights into the clinical manifestations and diagnosis of ISCH, which can aid in the early recognition and treatment of this rare condition.
- Research Article
96
- 10.1016/j.surneu.2003.10.030
- Jul 14, 2004
- Surgical Neurology
Idiopathic spinal cord herniation: a new theory of pathogenesis
- Research Article
- 10.32598/irjns.9.22
- Dec 6, 2023
- Iranian Journal of Neurosurgery
Background and Importance: Idiopathic spinal cord herniation (ISCH), or spontaneous spinal cord herniation, is a rare but serious condition that can cause progressive myelopathy and irreversible neurological deficits if left untreated. The condition is marked by the gradual herniation of the spinal cord through a ventral defect in the dura, leading to compromised blood flow and neurological deficits. Common symptoms include Brown-Séquard syndrome or asymmetrical paraparesis. Treatment options typically focus on reducing the strangulated spinal cord and closing the dural defect with a synthetic patch. Case Presentation: We present the case of an adult woman with progressive asymmetrical weakness of the lower limbs compatible with spastic paraparesis. Thoracic magnetic resonance imaging (MRI) revealed characteristic features of ISCH at the T3-T4 level. Intraoperative neurophysiological monitoring was used during the surgical intervention, which involved a 3-level laminectomy, dura opening, excision of the dentate ligament, and reduction of the cord across the dural defect. The defect was then filled with an autogenous piece of muscle, followed by the closure of the defect with an artificial dural patch and dural closure. At the 6-month follow-up, the patient showed favorable improvement. Conclusion: Patients with slowly progressive paraparesis or Brown-Séquard syndrome should consider the possibility of ISCH as a potential cause, despite its rarity. In symptomatic cases, the preferred treatment option often involves reducing the incarcerated spinal cord followed by covering the dural defect.
- Research Article
11
- 10.14444/1029
- Jan 1, 2014
- International Journal of Spine Surgery
BackgroundIdiopathic spinal cord herniation (ISCH) is a rare condition and its pathogenesis remains unclear. The purpose of this case report is to present an ISCH case with dorsal subarachnoid septum suggesting the pathogenesis of ISCH being adhesions from preexisting inflammation.MethodsSingle case report.ResultsA 60-year-old woman presented with Brown-Séquard syndrome below the level of T6. Magnetic resonance imaging revealed the thoracic spinal cord was displaced ventrally, and the dorsal subarachnoid space was enlarged and had a septum between the spinal cord and dura mater. Intraoperatively, the dorsal dura mater was seen to be adherent and the subarachnoid septum was identified after durotomy. The inner layer defect of the duplicated dura mater was found in the ventral dura mater, through which the spinal cord had herniated. After releasing the septum, the adhesions around the dura mater, and the hiatus, the spinal cord was reduced.ConclusionsThe present case indicates that adhesions around the dura mater can be the pathogenesis of ISCH.
- Research Article
14
- 10.1093/ons/opy244
- Jul 1, 2019
- Operative Neurosurgery
Idiopathic ventral thoracic spinal cord herniation is a rare condition that usually presents with progressive myelopathy or Brown-Séquard syndrome. More than 100 cases have been reported with significant variance in surgical treatment strategies and likewise, significant variance in patient outcomes. Although laminectomy has often been used, to date, there is no consensus regarding the optimal surgical approach or strategy for ventral dural repair. To report and illustrate a novel approach to repair the ventral dural defect with more than 2 yr of clinical follow-up. The specific approach and graft used are both detailed. A retrospective chart review of all known cases of idiopathic spinal cord herniation at the Cleveland Clinic over the last 15 yr was performed. Postoperative outcome scores (including the Japanese Orthopedic Association score, European Myelopathy score, and Nurick) were calculated preoperatively and postoperatively. A total of 5 patients were identified. Four of five patients improved clinically after surgery and 1 patient remained unchanged at last follow-up (average 23.2 mo, range 12-60 mo). There were no complications. All patients had postoperative magnetic resonance imaging demonstrating realignment of the spinal cord and no recurrence of tethering. A unilateral dorsolateral, transpedicular approach combined with laminectomy provides excellent exposure for ventral or ventrolateral dural defects associated with idiopathic spinal cord herniation and minimizes spinal cord manipulation. A collagen matrix graft used as an onlay between the spinal cord and ventral dural defect is a safe and effective option for ventral dural repair.
- Research Article
- 10.1227/ons.0000000000001584
- Apr 28, 2025
- Operative neurosurgery (Hagerstown, Md.)
Idiopathic spinal cord herniation through a defect in the ventral dura mater is rare and typically results in progressive myelopathy. Various surgical procedures to release the tethered spinal cord can prevent the progression of myelopathy; however, the optimal procedure has not yet been established. We describe techniques using endoscopic assistance to minimize spinal cord manipulation. A 60-year-old woman presented with Brown-Séquard syndrome. Magnetic resonance imaging demonstrated ventral displacement of the spinal cord at T3-4. Right T2, T3, T4, and T5 hemilaminectomies and T4 pediculectomy were performed. After paramedian durotomy and transection of the dentate ligament, we identified a defect in the inner layer of the dura mater ventrally and found the spinal cord incarcerated in a pocket between the inner and outer layers. The spinal cord was adherent to the dura at the caudal end of the defect. The defect was extended caudally on the right under microscopic observation. On the left, which could not be visualized under the microscope, the adhesions were dissected under endoscopic guidance. After complete spinal cord untethering, the defect was closed using collagen matrix. The patient's motor weakness fully recovered, and she was walking independently at the time of discharge. Endoscopic assistance for release of thoracic spinal cord herniation is useful for minimizing intraoperative spinal cord manipulation.
- Research Article
24
- 10.1227/neu.0000000000000437
- Sep 1, 2014
- Operative Neurosurgery
Idiopathic spinal cord herniation (ISCH) is an uncommon condition located predominantly in the thoracic spine and often associated with a remote history of a major traumatic injury. ISCH has an incompletely described presentation and unknown etiology. There is no consensus on the treatment algorithm and surgical technique, and there are few data on clinical outcomes. In this case series and technical report, we describe the atypical myelopathy presentation, remote history of traumatic injury, radiographic progression, treatment, and outcomes of 5 patients treated at Washington University for symptomatic ISCH. A video showing surgical repair is presented. In contrast to classic compressive myelopathy symptomatology, ISCH patients presented with an atypical myelopathy, characterized by asymmetric motor and sensory deficits and early-onset urinary incontinence. Clinical deterioration correlated with progressive spinal cord displacement and herniation observed on yearly spinal imaging in a patient imaged serially because of multiple sclerosis. Finally, compared with compressive myelopathy in the thoracic spine, surgical treatment of ISCH led to rapid improvement despite a long duration of symptoms. Symptomatic ISCH presents with atypical myelopathy and slow temporal progression and can be successfully managed with surgical repair.
- Research Article
4
- 10.25259/sni_496_2021
- Aug 3, 2021
- Surgical neurology international
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.
- Research Article
26
- 10.1227/01.neu.0000249195.76527.61
- Jan 1, 2007
- Neurosurgery
OBJECTIVE Posttraumatic spinal cord herniation is a rare condition. We describe a case of spinal cord herniation into an associated pseudomeningocele after a brachial plexus avulsion injury. CLINICAL PRESENTATION A 33-year-old man began to develop progressive Horner's syndrome 14 years after a brachial plexus avulsion injury. At a clinical presentation 17 years after that injury, sensory disturbance and a unilateral pyramidal sign were also evident. In addition to myelography and computed tomographic myelography findings, coronal magnetic resonance imaging scans clearly demonstrated herniation of the spinal cord into a large pseudomeningocele inside the C7–T1 intervertebral foramen. Another pseudomeningocele inside the T1–T2 intervertebral foramen was also noted. INTERVENTION The patient underwent a C6–T2 laminectomy, during which the spinal cord was found to be herniated through a dural defect into a pseudomeningocele at the C8 root level, and a second dural defect was also shown, with an arachnoid outpouching that included an avulsed T1 root. The spinal cord herniation was reduced and the dural defects were repaired. After surgery, the patient showed no significant neurological changes, and his condition stabilized. CONCLUSION Brachial plexus root avulsions may result in the formation of pseudomeningoceles and can lead to spinal cord herniation. Coronal magnetic resonance imaging is useful to demonstrate spinal cord herniation as well as pseudomeningoceles. Surgical treatment is recommended for such cases with progressive symptoms to prevent further deterioration.
- Research Article
11
- 10.1016/j.wneu.2019.08.099
- Aug 23, 2019
- World Neurosurgery
Failure of Surgery in Idiopathic Spinal Cord Herniation: Case Report and Review of the Literature
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