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

2019 ◽  
Vol 12 (5) ◽  
pp. e227847 ◽  
Author(s):  
D Gomez-Amarillo ◽  
C Garcia-Baena ◽  
D Volcinschi-Moros ◽  
F Hakim

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.

2016 ◽  
Vol 37 (03) ◽  
pp. 280-283
Author(s):  
Rogerio Sacco ◽  
Ricardo Gepp ◽  
Marco Quiroga ◽  
Henrique Souza ◽  
Vitor Aguiar ◽  
...  

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.


2017 ◽  
Vol 26 (3) ◽  
pp. 384-387 ◽  
Author(s):  
Pedro David Delgado-López ◽  
Cecilia Gil-Polo ◽  
Vicente Martín-Velasco ◽  
Javier Martín-Alonso ◽  
Ana María Galacho-Harriero ◽  
...  

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.


2014 ◽  
Vol 6 ◽  
pp. JCNSD.S16180 ◽  
Author(s):  
Jon Berg-Johnsen ◽  
Eivind Ilstad ◽  
Frode Kolstad ◽  
Mark Züchner ◽  
Jarle Sundseth

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.


2018 ◽  
Vol 16 (4) ◽  
pp. 415-423 ◽  
Author(s):  
Randall J Hlubek ◽  
David S Xu ◽  
Celene B Mulholland ◽  
Jourdan Gilson ◽  
Nicholas Theodore ◽  
...  

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.


2006 ◽  
Vol 4 (1) ◽  
pp. 10-19 ◽  
Author(s):  
Giulio Maira ◽  
Luca Denaro ◽  
Francesco Doglietto ◽  
Annunziato Mangiola ◽  
Cesare Colosimo

✓ Idiopathic spinal cord herniation (ISCH) is a rare, although increasingly recognized, cause of myelopathy. It is the result of an anterior dural defect in the thoracic spine through which the spinal cord herniates. Surgical restoration of the herniated cord to its normal position is usually followed by significant improvement in patients' clinical status. Differing surgical techniques have been used to manage the dural defect. In this report the authors discuss the cases of five patients (four women and one man) with ISCH treated during a 13-year period. Clinical and imaging findings in each patient are reported. Two different surgical techniques were used to treat this condition: dural defect enlargement in two cases and dural patch secured with stitches in three. The intra-and postoperative findings are discussed in relation to the two surgical techniques. Based on the results and complications in these five cases, the authors now believe that ISCH should be treated, when feasible, by using a dural patch to close the dural defect at the site of the herniation.


2002 ◽  
Vol 97 (3) ◽  
pp. 369-374 ◽  
Author(s):  
Giuseppe M. V. Barbagallo ◽  
Laurence A. G. Marshman ◽  
Carl Hardwidge ◽  
Richard W. Gullan

✓ The authors present two cases of thoracic idiopathic spinal cord herniation (TISCH) occurring at the vertebral body (VB) level in whom adequate surgical reduction failed to reverse symptoms. In the second case, in which TISCH occurred into a VB cavity, presentation was atypical (subacute spinal cord syndrome) and there was persistent postoperative deterioration. In both cases, adequate surgical reduction was achieved via a posterior midthoracic laminectomy, and reduction was maintained by closure of the anterior dural defect by using prosthetic material. Thoracic idiopathic spinal cord herniation occurring at a VB level may be technically well treated by surgical reduction, but the outcome appears less predictable. Herniation that occurs directly into a VB cavity may form a distinct subgroup in which the presentation is atypical and the prognosis worse.


1998 ◽  
Vol 88 (2) ◽  
pp. 331-335 ◽  
Author(s):  
Shigeru Miyake ◽  
Norihiko Tamaki ◽  
Tatsuya Nagashima ◽  
Hiromitsu Kurata ◽  
Takahiro Eguchi ◽  
...  

✓ The authors describe two occurrences of idiopathic spinal cord herniation, an entity that has been reported previously in only 11 cases. The patients described in this report presented in midlife with Brown—Séquard syndrome. Computerized tomography myelography and magnetic resonance (MR) imaging showed ventral displacement of the spinal cord with no apparent dorsal mass. Intraspinal cerebrospinal fluid (CSF) flow studies in which phase-contrast pulse sequence cine MR imaging was used displayed a normal pattern dorsal to the spinal cord. Percutaneous intrathecal endoscopic observation did not disclose dorsal intradural cysts, but ventral adhesions were seen between the spinal cord and the dura. Microsurgical intradural exploration revealed ventrolateral herniation of the cord and a ventral root through a dural defect into an epidural arachnoid cyst. The adhesions were released, the cord was repositioned intradurally, and the dural defect was patched. The patients showed gradual improvement postsurgery, as did most of the patients in the previously reported cases. The CSF flow and endoscopic studies were found to be particularly informative, and dural patching with surgical membrane is recommended.


1999 ◽  
Vol 7 (5) ◽  
pp. E8 ◽  
Author(s):  
Shigeru Miyake ◽  
Norihiko Tamaki ◽  
Tatsuya Nagashima ◽  
Hiromitsu Kurata ◽  
Takahiro Eguchi ◽  
...  

The authors describe two occurrences of idiopathic spinal cord herniation, an entity that has been reported previously in only 11 cases. The patients described in this report presented in midlife with Brown-Séquard syndrome. Computerized tomography myelography and magnetic resonance (MR) imaging showed ventral displacement of the spinal cord with no apparent dorsal mass. Intraspinal cerebrospinal fluid (CSF) flow studies in which phase-contrast pulse sequence cine MR imaging was used displayed a normal pattern dorsal to the spinal cord. Percutaneous intrathecal endoscopic observation did not disclose dorsal intradural cysts, but ventral adhesions were seen between the spinal cord and the dura. Microsurgical intradural exploration revealed ventrolateral herniation of the cord and a ventral root through a dural defect into an epidural arachnoid cyst. The adhesions were released, the cord was repositioned intradurally, and the dural defect was patched. The patients showed gradual improvement postsurgery, as did most of the patients in the previously reported cases. The CSF flow and endoscopic studies were found to be particularly informative, and dural patching with surgical membrane is recommended.


2012 ◽  
Vol 16 (2) ◽  
pp. 114-126 ◽  
Author(s):  
Klaus Novak ◽  
Georg Widhalm ◽  
Adauri Bueno de Camargo ◽  
Noel Perin ◽  
George Jallo ◽  
...  

Object Thoracic idiopathic spinal cord herniation (TISCH) is a rare neurological disorder characterized by an incarceration of the spinal cord at the site of a ventral dural defect. The disorder is associated with clinical signs of progressive thoracic myelopathy. Surgery can withhold the natural clinical course, but surgical repair of the dural defect bears a significant risk of additional postoperative motor deficits, including permanent paraplegia. Intraoperative online information about the functional integrity of the spinal cord and warning signs about acute functional impairment of motor pathways could contribute to a lower risk of permanent postoperative motor deficit. Motor evoked potential (MEP) monitoring can instantly and reliably detect dysfunction of motor pathways in the spinal cord. The authors have applied MEPs during intraoperative neurophysiological monitoring (IOM) for surgical repair of TISCH and have correlated the results of IOM with its influence on the surgical procedure and with the functional postoperative outcome. Methods The authors retrospectively reviewed the intraoperative neurophysiological data and clinical records of 4 patients who underwent surgical treatment for TISCH in 3 institutions where IOM, including somatosensory evoked potentials and MEPs, is routinely used for spinal cord surgery. In all 4 patients the spinal cord was reduced from a posterior approach and the dural defect was repaired using a dural graft. Results Motor evoked potential monitoring was feasible in all patients. Significant intraoperative changes of MEPs were observed in 2 patients. The changes were detected within seconds after manipulation of the spinal cord. Monitoring of MEPs led to immediate revision of the placement of the dural graft in one case and to temporary cessation of the release of the incarcerated spinal cord in the other. Changes occurred selectively in MEPs and were reversible. In both patients, transient changes in intraoperative MEPs correlated with a reversible postoperative motor deficit. Patients without significant changes in somatosensory evoked potentials and MEPs demonstrated no additional neurological deficit postoperatively and showed improvement of motor function during follow-up. Conclusions Surgical repair of the dural defect is effected by release and reduction of the spinal cord and insertion of dural substitute over the dural defect. Careful monitoring of the functional integrity of spinal cord long tracts during surgical manipulation of the cord can detect surgically induced impairment. The authors' documentation of acute loss of MEPs that correlated with reversible postoperative motor deficit substantiates the necessity of IOM including continuous monitoring of MEPs for the surgical treatment of TISCH.


2015 ◽  
Vol 23 (3) ◽  
pp. 306-308 ◽  
Author(s):  
Nardin Samuel ◽  
Christina L. Goldstein ◽  
Carlo Santaguida ◽  
Michael G. Fehlings

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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