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

Abstract

BackgroundIdiopathic 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 descriptionA 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.ConclusionPreoperative 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.

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

“…Some ISCH patients have neurological symptoms. Yang et al 3 . reported on 96 men and 156 women with a mean age of 51.6 ± 12.1 years (range: 20–78 years), with ISCH at a thoracic level (central [T5–T8]: 66.0%; upper [T1–T4]: 30.0%; and lower [T9–T12]: 4.0%), and with a mean preoperative symptom duration of 4.2 ± 4.5 years (range: 0.1–32 years).…”

“…As ISCH is more common in middle‐aged and older patients and mainly presents with lower‐extremity symptoms, lumbar degenerative spinal disease is typically suspected, as in the present case. As lumbar spine degeneration is frequently observed on lumbar spine MRI in this population, ISCH is underdiagnosed and is often misdiagnosed as lumbar degenerative spinal cord disease 3,4 . Differential diagnoses of ISCH are dorsal arachnoid cyst, astrocytoma, disc herniation, extradural compression, and transverse myelitis 3 …”

“…As lumbar spine degeneration is frequently observed on lumbar spine MRI in this population, ISCH is underdiagnosed and is often misdiagnosed as lumbar degenerative spinal cord disease 3,4 . Differential diagnoses of ISCH are dorsal arachnoid cyst, astrocytoma, disc herniation, extradural compression, and transverse myelitis 3 …”

“…2 ISCH is usually more common in older and middle-aged patients with lower extremity symptoms and is often misdiagnosed and mistreated as a lumbar spine disorder. [3][4][5] Here, we present a surgical video and characteristic pre-and postoperative images of a patient with T6/7-level ISCH, who was treated with surgical repair of the dural defects. This case report aims to address the diagnostic issues of this disease.…”

“…ISCH is usually more common in older and middle‐aged patients with lower extremity symptoms and is often misdiagnosed and mistreated as a lumbar spine disorder 3–5 …”