Gaurav Tyagi1, Prabhuraj A R2, Dhananjaya I Bhat1, Malla Bhaskara Rao1, Bhagavatula Indira Devi1. 1. Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, India. 2. Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, India. Electronic address: drprabhuraj@yahoo.co.in.
Abstract
BACKGROUND: Idiopathic spinal cord herniation (ISCH) is a treatable spinal disease. It is rare and often misdiagnosed, causing a delay in management. The etiology is multifactorial, with one of the causes being duplication of the ventral dura. The spinal cord herniates within this defect and becomes strangulated, causing neurological deficits. We report the duplication of a ventral spinal cord as an important cause of ISCH in our review. CASE DESCRIPTION: We present 2 cases of ISCH with duplication of the dura, including their relevant clinical and imaging features. The patients underwent surgical reduction of the herniated spinal cord with enlargement of the defect and placement of a dural substitute ventral to the cord. We have also reported the outcomes of the 2 patients, with an emphasis on the factors predictive of poor outcomes (i.e., long-standing symptoms, a delay in intervention, poor neurological status at presentation, and a thinned out atrophic spinal cord found during surgery). We also reviewed the available data for duplication of the dura with ISCH. CONCLUSIONS: Very few asymptomatic patients can be treated conservatively. The surgical outcomes have been favorable for symptomatic patients. Proper exposure, gentle manipulation while reducing the herniated spinal cord, enlargement of the defect, and the use of intraoperative monitoring will help limit the postoperative deficits. Duplication of the ventral dura is an important cause of ISCH. It prevents the formation of an anterior pseudomeningocele after surgery. Owing to the rarity of the disease and the lack of follow-up data with recurrence rates, it has not been possible to form clear guidelines for management.
BACKGROUND:Idiopathic spinal cord herniation (ISCH) is a treatable spinal disease. It is rare and often misdiagnosed, causing a delay in management. The etiology is multifactorial, with one of the causes being duplication of the ventral dura. The spinal cord herniates within this defect and becomes strangulated, causing neurological deficits. We report the duplication of a ventral spinal cord as an important cause of ISCH in our review. CASE DESCRIPTION: We present 2 cases of ISCH with duplication of the dura, including their relevant clinical and imaging features. The patients underwent surgical reduction of the herniated spinal cord with enlargement of the defect and placement of a dural substitute ventral to the cord. We have also reported the outcomes of the 2 patients, with an emphasis on the factors predictive of poor outcomes (i.e., long-standing symptoms, a delay in intervention, poor neurological status at presentation, and a thinned out atrophic spinal cord found during surgery). We also reviewed the available data for duplication of the dura with ISCH. CONCLUSIONS: Very few asymptomatic patients can be treated conservatively. The surgical outcomes have been favorable for symptomatic patients. Proper exposure, gentle manipulation while reducing the herniated spinal cord, enlargement of the defect, and the use of intraoperative monitoring will help limit the postoperative deficits. Duplication of the ventral dura is an important cause of ISCH. It prevents the formation of an anterior pseudomeningocele after surgery. Owing to the rarity of the disease and the lack of follow-up data with recurrence rates, it has not been possible to form clear guidelines for management.