Congenital ventral thoracic spinal cord herniation (original) (raw)
Related papers
Idiopathic Ventral Spinal Cord Herniation: An Illustrative Case and Literature Review
The Iranian Journal of Neurosurgery, 2023
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.
Spine Journal, 2011
BACKGROUND CONTEXT: Idiopathic spinal cord herniation (ISCH) is a rare cause of progressive myelopathy. Preoperative diagnosis can be made with magnetic resonance imaging (MRI). Many surgical techniques have been applied by various authors, and ISCH is usually reversible by surgical treatment. PURPOSE: To present a case of ISCH in two separate zones at two thoracic levels. To our knowledge, this is the first such case to be published in English literature. We also discuss the clinical findings, surgical procedures, and surgical outcomes for other previously reported cases of ISCH in the literature. STUDY DESIGN: Case report. METHODS: A 52-year-old woman with bilateral lower extremity weakness underwent thoracic MRI, which revealed transdural spinal cord herniation at two separate zones, namely, the T4-T5 and T5-T6 intervertebral disc levels. RESULTS: During surgery, the spinal cord was reduced, the two separate dural defects were connected, and the new single defect was restored then reinforced with a thin layer of fascial graft. The posterior dural defect was then closed with interrupted stitches. The patient's neurologic status was characterized by no changing of the preoperative motor status. Follow-up MRI scans showed that the cord was replaced in the dural sac and showed cord hyperintensity in the herniation levels. The patient could move with a cane at the sixth month postoperatively. CONCLUSIONS: Idiopathic spinal cord herniation is a rare clinical condition that should be considered in the differential diagnosis of paraplegia. Although progression of neurologic deficits can be very slow, reduction of the spinal cord and repair of the defect are crucial to stop or reverse the deterioration. The outcome for patients who initially have Brown-S equard syndrome is significantly better than for patients who presented with spastic paralysis. To our knowledge, this case study represents the first reported instance in which two separate anterior dural defects caused two levels of anterior spinal cord herniation. Ó
Idiopathic Ventral Spinal Cord Herniation: An Increasingly Recognized Cause of Thoracic Myelopathy
Journal of Central Nervous System Disease, 2014
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.
Thoracal Herniation of the Spinal Cord
Herniation of the spinal cord through a dural defect is a rather rare deformity and very easily misdiagnosed as retromedullary occult intraspinal arachnoid cyst or meningocele. The possible origin of the dural defect can be traumatic, iatrogenic or unknown, so in these cases, congenital with great probability. On the thoracal part of the spinal column it shows a rather characteristic and misleading appearance. The anomaly leads to progressive Brown-Sequard syndrome, and the case history can be extremely long. Surgical repair of the dural defect results in improvement, or even complete recovery, if performed in time. These are the facts that emphasize the importance of early diagnosis.
Idiopathic Spinal Cord Herniation: Case Report and Literature Review
Acta Neurochirurgica, 2001
We report one case of spontaneous thoracic spinal cord herniation presenting with a progressive spastic paraparesis for 4 years in a 55 years old man. From preoperative MRI, showing a ventrally displaced atrophic spinal cord at T2±T3 level, a dorsal intradural arachnoid cyst was suspected. At operation, after a 3 level laminectomy, no arachnoid cyst was found and spinal cord herniation into a meningeal diverticulum was con®rmed. The herniated myelon was replaced intradurally and the lumen of the diverticulum was ®lled with Te¯on= settled with ®brin glue to prevent recurrence. Postoperatively some neurological recovery was achieved.
Surgical neurology international, 2014
Spinal cord herniation was first described in 1974. It generally occurs in middle-aged adults in the thoracic spine. Symptoms typically include back pain and progressive paraparesis characterized by Brown-Séquard syndrome. Surgical reduction of the hernia improves the attendant symptoms and signs, even in patients with longstanding deficits. A 66-year-old female with back pain for 7 years, accompanied by paresthesias and a progressive paraparesis, underwent a thoracic MRI which documented a ventral spinal cord herniation at the T4 level. Following a laminectomy, with reduction of the hernia and ventral dural repair, the patient improved. Herniation of the thoracic cord, documented on MR, may produce symptomatic paraparesis which may resolve following laminectomy with ventral dural repair.
Spinal Cord Herniation: Why Anterior Thoracic?
Journal of Neurology and Neuroscience, 2015
Background: Spinal cord herniation and thoracic anterior adhesion syndrome make up the two extremes of a rare condition; characterized by anterior dural adhesion or protrusion of the spinal cord through the arachnoideal and dural membrane into the extradural space, respectively. Summary: We present the main features of the condition by our case series and forward a hypothesis for the consistent anterior, mid-thoracic localization. We surmise the role of an anterior pulling force by the Hofmann (meningo-vertebral) ligaments; acting when the physiologic thoracic kyphosis suddenly increases. The traction may tear the anterior dura; resulting in a dural defect; it allows the nipping/ protrusion of the spinal cord. Key messages: Because the spinal cord compression syndrome caused by adhesion or herniation of the spinal cord may be surgically treatable; the recognition of the condition is essential. The pathogenetic traction effect of the Hofmann ligament in the affected level may have surgical an prognostic implications.
International journal of spine surgery, 2014
Idiopathic 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. Single case report. A 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. The present case indicates that adhesions around the dura mater ca...
AJNR. American journal of neuroradiology, 2012
tSCH in the absence of spinal trauma or surgery is a rare disorder for which numerous mechanisms have been proposed. Here, we have conducted an analysis of images in all published reports of idiopathic tSCH and identified evidence supporting a pathogenesis in which anterior dural erosion at thoracic levels generates a CSF leak that pushes adjacent spinal tissue to tamponade the dural defect, causing progressive myelopathy. Additionally, we describe a case of tSCH in which postural headache was a significant symptom before myelopathy. This finding suggests that tSCH pathogenesis may be related to spontaneous intracranial hypotension. Published imaging from all available prior case reports in the scientific literature was reviewed to determine whether tSCH occurred at the disk or bone level. The presence of EDF, HNP, or an osteophyte in the spinal canal was determined from review of published images. Additionally, 3 previously unreported cases from the teaching files of our department...