Idiopathic Ventral Spinal Cord Herniation: An Increasingly Recognized Cause of Thoracic Myelopathy

Abstract

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.

Idiopathic Spinal Cord Herniation—Case Report

“…Spinal cord herniation is probably a more frequent cause of myelopathy than what is currently diagnosed. 5,6 Due to the rarity and the atypical presentation of spinal cord herniation, its diagnosis and treatment may be delayed. 7,8 The presence of a dural defect is the primary pathophysiological factor in the development of this condition, but its etiology remains unknown.…”

“…Most publications on spinal cord herniation are isolated descriptions or series consisting of a few cases. [4][5][6] Currently, 200 cases are found in the literature. 9 Surgical treatment is described by several authors as a way to reverse the resulting myelopathy of this clinical condition.…”

Double trouble!!! An unusual presentation of cervical cord herniation and medial end clavicle non-union in a single patient

“…It refers to the herniation of the spinal cord through a ventral dural defect, typically in the mid-thoracic spinal column2 and is a rarely reported clinical entity that may be classified on the basis of aetiology—spontaneously, post-traumatically or iatrogenically 3. Since its first report in 1974, approximately 180 cases of SCH have been reported in the literature4 with majority of them being located in the thoracic region. Few cases of SCH in the cervical region have been reported, but all of them are iatrogenic in aetiology5–7 with no case of post-traumatic aetiology being documented until.…”

Spinal cord herniation repair with microstaples: case report

“…[1][2][3][4]9,12 Although its etiology remains unclear, ISCH has been suggested to be either congenital or somehow due to trauma. [1][2][3][4]9,12 This condition is characterized by a ventral displacement of the spinal cord through a dural defect, usually at the thoracic level. ISCH produces progressive myelopathy and presents as spastic paraparesis or as a Brown-Séquard syndrome, although some patients may be asymptomatic.…”

“…ISCH produces progressive myelopathy and presents as spastic paraparesis or as a Brown-Séquard syndrome, although some patients may be asymptomatic. [1][2][3][4]9,12 MRI demonstrates a ventral C-shaped kinking of the thoracic cord on sagittal views. Moreover, cerebrospinal fluid flow in the subarachnoid space posterior to the herniated spinal cord is visible on phase-contrast pulse cine MRI, which helps to distinguish it from an arachnoid cyst.…”