Unveiling the Current Understanding of Idiopathic Spinal Cord Herniation: A Systematic Review.

Background: 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. Methods: 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." Results: 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. Conclusions: 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.

lVentral tethering-is the prognosis worse than in dorsal tethering in the dysraphic spine?

OBJECTIVE: To compare cases of dysraphism with ventral tethering of cord with those with dorsal tethering and to find out any differences in the outcome of surgery in them. METHODS: We collected the data of 188 consecutively operated tethered cord patients at our institute in the past 7 years and divided them into ventral tethering and dorsal tethering groups. Those that we felt had both dorsal and ventral tethering were excluded. Their preoperative clinical, radiological, and baseline neurophysiological parameters as well as postoperative clinical and radiological parameters were analyzed in a retrospective study. RESULTS: Among the 188 tethered cord patients, 52 (28%) had ventral tethering and 136 (72%) had posterior tethering. Preoperative neurodeficit and cord signal changes as well as absent baseline MEP (of any one muscle) were significantly more associated with ventral tethered cord than the dorsal tethered cord. The neurological deterioration after surgery occurred significantly in the ventral tethered cord group than in the dorsal tethered cord group. Also, the postoperative MRI had more incomplete detethering cases in the ventral group than in the dorsal tethered cord group. CONCLUSION: Ventral tethered cord is more likely to present with preoperatively neurological deficits. It should be carefully identified in the preoperative MRI, so that the intraoperative difficulties in complete detethering and postoperative deterioration can be anticipated.

Idiopathic thoracic spinal cord herniation into the vertebra progressing for 3 years.

A 43-year-old, previously healthy man experienced a decreased sensation in the left lower extremity without preceding spinal trauma. At presentation, the patient exhibited slight motor weakness in the left lower extremity, in addition to decreased pain sensation below the ipsilateral T7. Spinal magnetic resonance imaging (MRI) revealed abnormal findings consistent with idiopathic thoracic spinal cord herniation (ITSCH) at the T5/6 level. Computed tomography (CT) revealed a small vertebral erosion at the lower T5. The patient's symptoms gradually progressed over the next 3 years. MRI revealed marked lateral elongation of the cord at the T5/6 and apparent intravertebral cord herniation. The patient underwent ITSCH reduction through T5-6 laminectomies. The herniated cord was vertically long with a bulbous rostral part. Successful ITSCH reduction was achieved and the patient's postoperative course was uneventful. ITSCH is a progressive pathology that requires prompt surgical reduction. Certain ITSCHs may be complicated by intravertebral cord herniation.

Remarkable improvement of neurological deficits after surgery in patients with Idiopathic spinal cord herniations. The impact of peroperative neuromonitoring. Case reports.

Introduction: Chronic Idiopathic Spinal Cord Herniation (ISCH) is a very rare spinal cord deformation occurring predominantly in thoracic levels. ISCH lead to progressive myelopathy, spastic paraparesis and Brown Séquard syndrome. Research question: We want to hypothesize that a) the herniated segment can regain its function after untethering despite long-term and complete neurologic dysfunction. b) Intraoperative Electrophysiologic Monitoring (IOEPM) may identify intraoperative changes by monitoring specific neural pathways confirming the efficacy of the intervention in the forthcoming cases. Material & method: It is a retrospective review of data of two cases prospectively collected showing improvement of neurological deficit in cases of ISCH in thoracic levels. We describe two patients with progressive neurological deficits due to ISCH who underwent surgery using electrophysiologic monitoring and have been followed to reach remarkable clinical improvement. Results: The spastic paraparesis of the first case improved remarkably after surgery. Complete foot drop of the other case, persistent for 7 months before intervention, improved after the release of the herniated segment of the cord. Peroperative electrophysiological monitoring did not show changes during surgery. Conclusion: We want to hypothesize that the herniated segment can regain its function after untethering despite long-term and complete neurologic dysfunction. Intraoperative Electrophysiologic Monitoring (IOEPM) may confirm the efficacy of the intervention in the forthcoming cases.

Early detection of idiopathic thoracic ventral spinal cord herniation by using imaging: A case report.

In middle-aged and older populations, clinicians often suspect lumbar spine disease when the gait is disturbed with lumbar lower extremity numbness, but spinal herniation at the thoracic level may be causal. Early detection, appropriate treatment, and minimization of complications requires understanding of characteristic magnetic resonance imaging findings of herniation.

Case report: A vertebral bone spur as an etiology for spinal cord herniation: case presentation, surgical technique, and review of the literature.

Objective: The pathophysiology of idiopathic spinal cord herniation remains unknown. However, several different factors have been postulated, such as congenital causes (ventral dura mater duplication, preexisting pseudomeningocele, or other congenital dural defects), inflammation, remote spinal trauma, or thoracic disc herniation. Herein, the diagnosis and surgical treatment of a patient with spinal cord herniation caused by an intraspinal bone spur is presented along with a relevant literature review. Case presentation: A 56-year-old male patient presented with a non-traumatic Brown-Sequard syndrome persisting for over 1 year. A magnetic resonance imaging of the spinal axis revealed a ventral spinal cord displacement in the level of T 6/7. A supplementary thin-sliced computed tomography of the spine revealed a bone spur at the same level. For neurosurgical intervention, T 6 and T 7 laminectomy was performed. The cranial and caudal end of the right paramedian ventral dural defect was visualized and enlarged. Following extradural spinal cord mobilization by denticulate ligament transection, the spinal cord was finally released. The spinal cord was rotated and the ventral closure of the dural defect was performed by continuous suture. The patient recovered from surgery without additional deficits. The patient's postoperative gait, sensory, and motor function deficits improved, and further neurological deterioration was prevented. Conclusion: Since the first description of spinal cord herniation by Wortzman et al. in 1974, approximately 260 cases have been reported in the literature. In addition to other causes, intraspinal bone spur is a possible cause of spinal cord herniation.

Thoracic Anterior Spinal Cord Herniation: Treatment and Prognosis Outcome of Seven Patients.

BACKGROUND: This study aimed to investigate the diagnosis and treatment of thoracic anterior spinal cord herniation, a rare condition. METHODS: Clinical data of 7 patients diagnosed with thoracic anterior spinal cord herniation were analyzed. All patients were diagnosed with a complete preoperative examination and scheduled for surgical treatment. In addition, regular follow-up was performed after the surgery, and the operation's efficacy was evaluated according to clinical symptoms, imaging findings, and improvement in neurologic function. RESULTS: All patients underwent spinal cord release with an anterior dural patch. Notably, no severe postoperative surgical complications were observed. All patients were followed up for 12-75 months, with an average duration of approximately 46.5 months. Post-operative pain symptoms were controlled, neurological dysfunction and related symptoms improved to varying degrees, and anterior spinal cord herniation did not recur. The modified Japanese Orthopedic Association score at the last follow-up was significantly higher than the preoperative score. CONCLUSIONS: Clinicians should avoid misdiagnosing patients with thoracic anterior spinal cord herniation with intervertebral disc herniation, arachnoid cysts, and other related diseases, and patients should undergo surgical treatment as early as possible. In addition, surgical treatment can protect the neurologic function of patients and effectively prevent the aggravation of clinical symptoms.

Cervical Spinal Cord Herniation After Laminectomy.

Following a recent cervical laminectomy, a 67-year-old female patient developed neck pain and torticollis after a coughing episode. On physical examination, manifestations of neck pain, stiffness, and 4/5 quadriparesis were noted. Magnetic resonance imaging findings indicated the presence of herniation of the cervical medulla, accompanied by cerebrospinal fluid leakage. Consequently, surgical intervention was performed to reposition the herniated cord within its original subarachnoid space, thereby averting potential recurrence. The patient experienced complete resolution of symptoms within a few days postoperatively. It is important to recognize that postoperative cervical spinal cord herniation following laminectomy is an infrequent complication that manifests with a delayed onset. Surgical repair serves as the main therapeutic modality.

Idiopathic spinal cord herniation at the cervicothoracic junction level presenting with unilateral sensory symptoms.

Idiopathic spinal cord herniation (ISCH) most commonly occurs through a ventral dural defect at the midthoracic levels with a predilection to affect middle-aged females. It can have various presentations, the most common of which are Brown-Séquard syndrome and spastic paraparesis. Due to its rarity in clinical practice, the diagnosis of ISCH can be challenging to physicians unfamiliar with this entity. We report an exceedingly rare case of ISCH at the C7-T1 intervertebral disc level in a 44-year-old male presenting with eight months of isolated unilateral sensory symptoms. The diagnosis was made based on the findings on the patient's magnetic resonance imaging of the spinal cord, including the presence of an extradural cerebrospinal fluid collection. Surgical reduction of the herniated segment and patching of the dural defect resulted in a remarkable clinical improvement beginning in the immediate postoperative period. Follow-up MRIs showed no sign of reherniation, and the patient remained asymptomatic after one year of follow-up. Early diagnosis and surgical intervention led to an excellent early outcome in this case. However, long-term follow-up is necessary to monitor for reherniation and relapse of the symptoms in ISCH patients.

Spontaneous-Onset Delayed Spinal Arachnoiditis With Dorsal Cord Herniation in a 29-Year-Old Paraplegic Patient: A Case Report.

Spinal adhesive arachnoiditis is a rare occurrence with a diverse etiology. The clinical picture is not universal, and varying degrees of neurodeficit have been mentioned. Spontaneous spinal cord herniation or idiopathic spinal cord herniation occurs due to displacement of the cord through a dural or arachnoid defect. We report a case of a 29-year-old male paraplegic patient with a nontraumatic spinal cord injury (SCI) following surgery for an intradural extramedullary lesion at T10-T11 level who developed loss of truncal balance after two years of the index surgery. After a thorough clinical examination and MRI as well as other investigations, the patient was diagnosed as having spontaneous-onset delayed spinal arachnoiditis with dorsal cord herniation through the laminectomy window with effacement of neural tissue and ascending edema up to T6 level. A new-onset weakness or the development of an ascending loss of sensory level with a loss of truncal balance should alarm the therapist about some new pathology happening at the cord level in patients with SCI. In this regard, spinal adhesive arachnoiditis with or without cord herniation should always be suspected in a paraplegic patient with delayed-onset deterioration of neurology. Differential diagnoses like arachnoid web and arachnoid cysts should also be kept in mind.

A Rare Case of Idiopathic Spinal Cord Herniation Treated by DuraGen® Collagen Matrix Graft.

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.

[A case of idiopathic spinal cord herniation with incomplete Brown-Séquard syndrome].

A 50-year-old man was referred to our hospital with myelitis associated with a 10-months history of progressive muscle weakness in the left leg. Neurological examinations demonstrated diffuse muscle weakness of the left leg, touch hypoesthesia of the right leg, reduced pain sensation below the right nipple, left pyramidal sign, and urinary incontinence. On the basis of thoracic spinal MRI and thoracic CT myelography, revealing anterior displacement of the spinal cord and enlargement of the posterior subarachnoid space at the Th4 vertebral level, we diagnosed the patient as having idiopathic spinal cord herniation with incomplete Brown-Séquard syndrome. After microsurgical release of the spinal cord and subsequent covering of the anterior dural defect with an artificial dura mater, the symptoms improved without progression. Clinicians should consider spinal cord herniation as a cause of slowly progressive thoracic myelopathy with Brown-Séquard syndrome.

Expanding the indications for measurement of objective functional impairment in spine surgery: A pilot study of four patients with diseases affecting the spinal cord.

•The TUG test and the 6WT are feasible to apply in ambulatory patients with spinal cord affection.•The objective measurement of functional impairment resembled the functional status of four patients in this pilot study.•The TUG test and the 6WT may detect and quantify functional decline and/or improvement resulting from spinal cord affection.•Modern smartphone app-based technology may help to accurately quantify impairment, which is key for decision-making and evaluating the efficacy of interventions.

Dural repair with fat patch for idiopathic spinal cord herniation: operative technique and a review of seven cases.

Background: Idiopathic ventral thoracic spinal cord herniation is a rare disease presented with progressive myelopathy or Brown Séquard syndrome, causing neurological deficits. There is no consensus on etiology and surgical strategy. The purpose of the present study is to report the case series using fat patch for the repair of the ventral dural defect with clinical follow up. Methods: A retrospective review of all cases of idiopathic spinal cord herniation (ISCH) at our institution was performed between January 2017 and June 2021. Clinical data were reviewed including patients' demographic information, symptoms, imaging, operative details, perioperative and postoperative courses, and clinical outcomes, and complications. Japanese Orthopedic Association (JOA) score was calculated preoperatively and postoperatively for the comparison of outcomes. Results: A total of 7 patients were included. Fat patch was applied in all cases, and artificial dural patch was also used in 2 cases. Average operating time and estimated blood loss were 3 hours and 24 minutes and 88.6 mL, respectively. Five of 7 patients improved and 2 patients remained unchanged during follow up (average, 23.4 months; range, 9-42 months). The mean recovery rate (RR) of JOA score was 17.9%. One patient experienced cerebrospinal fluid (CSF) leakage, and 1 patient suffered from surgical related spinal canal stenosis. Conclusions: Surgical treatment using fat patch is an effective strategy for the ventral dural defect repair of ISCH.

Progressive superficial siderosis from Chronic CSF leak as a long-term complication of cervical anterior corpectomy: A case report and review of the literature.

Background: Superficial siderosis of the central nervous system (SSCNS) is a rare progressive neurological disorder resulting from chronic subarachnoid hemorrhage and subsequent subpial hemosiderin deposition. A prolonged cerebrospinal fluid (CSF) leak is a known cause of SSCNS. We present a novel case where progressive SSCNS resulted from a chronic CSF leak related to an anterior cervical corpectomy. Case Description: A 73-year-old man presented with gait ataxia and progressive hearing loss. Thirteen years before, he had undergone a combined anterior-posterior cervical decompression for symptomatic ossification of the posterior longitudinal ligament (OPLL). The presenting MR imaging showed extensive superficial siderosis and focal spinal cord herniation at the site of a ventral dural defect at the corpectomy site. A CT myelogram showed extensive CSF leakage into the corpectomy surgical site and a communicating pseudomeningocele in the anterior neck. Conclusion: This is the first reported case of progressive SSCNS as a long-term complication of an anterior cervical corpectomy for OPLL. Clinicians should be aware of SSCNS secondary to a chronic CSF leak in patients with a prior corpectomy.

Ventral Spinal Cord Herniation Causing Spinal Intradural Hematoma and Subarachnoid Hemorrhage: A Case Report.

Ventral spinal cord herniation is a rare pathology, caused by a dural defect, that leads to progressive myelopathy. The true prevalence of ventral spinal cord herniation is unknown largely because of underdiagnosis due to its nonspecific symptoms. Though there are theories that attempt to describe how these dural defects are formed, the true causes of these defects are unknown. In this case report, we present a case of a 29-year-old female who had an idiopathic ventral spinal cord herniation causing an intradural hematoma and subarachnoid hemorrhage. This is the first reported case of spinal cord herniation causing hemorrhage.

Case Report: Idiopathic Spinal Cord Herniation: An Overlooked and Frequently Misdiagnosed Entity.

Background: Idiopathic spinal cord herniation is an extremely rare entity that is characterized by protrusion of the spinal cord through a defect in the ventral dura. Due to the paucity of enough clinical evidence, the treatment and prognosis of idiopathic spinal cord herniation are still elusive. Herein, we reported a case of idiopathic spinal cord herniation occurring at the C7-T1 levels that was treated by surgical reduction. Case description: A 44-year-old Chinese woman presented with a 5-year history of numbness and weakness in the bilateral lower limbs. Spinal magnetic resonance imaging demonstrated ventral displacement of the spinal cord at the C7-T1 levels, and there seemed to be a cuneiform space-occupying lesion dorsal to the spinal cord. A diagnosis of the spinal intradural extramedullary tumor was suspected. An exploratory operation was performed via a posterior midline approach. Intraoperatively, we found a defect in the ventral dura through which the spinal cord herniated to the epidural space. After the herniated parenchyma was returned, an artificial dura matter was used to repair the defect. The postoperative course was uneventful. After a 3-month follow-up, the lower-extremity weakness was significantly improved, and there was no recurrence of the spinal cord herniation. Conclusion: Preoperative diagnosis of idiopathic spinal cord herniation is exceedingly challenging. Surgical reduction of the herniated spinal cord with the repair of the dural defect is an effective approach for the treatment of this rare disorder, and the surgical outcome is favorable.

Multifocal myxopapillary ependymoma and iatrogenic spinal cord herniation: Management options and lessons learned.

Although myxopapillary ependymoma is a fairly common tumour of the lumbosacral spine, primary multi-focal myxopapillary ependymoma is a rare variant. Drop metastasis and leptomeningeal spread in the craniospinal axis is seen more frequently in the paediatric population, although it is unusual in adults. Surgical resection of the primary lesion remains the standard treatment. As per the authors' knowledge, to-date there is only one prior case in literature reporting iatrogenic spinal cord herniation with indentation after surgery for thoracolumbar spinal tumour. Here, we are discussing an unusual case of primary multi-focal ependymoma in a 16-year-old Asian boy, with drop metastasis and lepto-meningeal disease, who developed iatrogenic spinal cord herniation after the first surgery for the primary tumour. He presented to the Shaukat Khanum Memorial Cancer Hospital & Research Centre (SKMCH & RC), Lahore, after his first surgery. He underwent the definitive corrective surgery at SKMCH & RC where he was managed further. We discuss the management options for this patient and the lessons learned along the way.

Scalpel Sign in Spine Pathology: Presentation in 3 Different Rare Diagnoses.

BACKGROUND: The scalpel sign is a radiological finding observed on sagittal magnetic resonance imaging and computed tomography myelography corresponding to an indentation in the dorsal aspect of the spinal cord resembling a surgical scalpel blade. It is said to be a pathognomonic imaging discovery linked to dorsal arachnoid webs. However, other spine-related conditions may mimic dorsal arachnoid webs on magnetic resonance imaging, such as spinal arachnoid cysts or ventral spinal cord herniation, leading to misdiagnosis. METHODS: A retrospective review was performed of cases involving 3 different diagnoses at our institution in the last 5 years that shared in common the characteristic focal dorsal indentation of the spinal cord. RESULTS: Of 7 cases identified, all but 1 were treated and confirmed intraoperatively. All lesions were located at the dorsal spinal cord. Magnetic resonance imaging was the study of choice for evaluation. Clinical manifestations included back pain and lower extremity numbness and weakness together with compressive myelopathy signs and urinary symptoms. Mean follow-up was 16.8 months with satisfactory postoperative results. CONCLUSIONS: Isolated radiological presentation of the scalpel sign is not sufficient to distinguish between dorsal arachnoid webs, arachnoid cysts, and ventral herniation of the spine. However, awareness of its importance is relevant for accurate curative surgical planning.