A Unique Case of Concomitant Intraspinal Epidermoid Cyst and Lipoma Associated with Tethered Cord Syndrome

Patients presenting with pain in lower back and paresthesia in lower limbs may have tethered cord causing the symptomology. Tethered cord may be associated with intradural tumors causing symptoms due to progressive increase in size. Association of tethered cord with single intradural tumor is a common occurrence but, to date, only one case has been reported of tethered cord associated with intradural lipoma and epidermoid cyst. The authors would like to present a similar case in a middle-aged patient.

Combined Type 1 and 2 split cord malformations, kyphoscoliosis, tethered cord, and a lipoma

Background: Split cord malformations (SCMs) are uncommon congenital anomalies. They can be divided into Type 1 (bony septum and two separate dural sheaths) and Type 2 (fibrous septum and a single dural sheath).[1,2,4] Rarely,SCM can be associated with orthopedic anomalies (mostly seen in older children), such as scoliosis, followed by kyphosis, and/or both.[1-3] Conversely, patients with congenital scoliosis have 40% risk of congenital underlying neural deformities, with 16.3% attributed to SCM.[1,4] Those with thoracic or lumbosacral SCM may have congenital tethering lesions, predominantly consisting of intradural lipomas.[3,4] Surgery is optimally performed in two stages: first, removal of the spinal septum and untethering of the cord, and second, correction of the kyphosis, scoliosis, and/or both.[1-5] Case Description: In this video, a 44-year-old female initially presented after having progressively developed kyphoscoliosis since childhood. Three months before presentation, she had developed increased left lower extremity pain with hypoesthesia followed 1 month later by the onset of the left lower extremity monoplegia (0/5). The computed tomography and magnetic resonance imaging studies revealed a SCM at the L3 level with bony and membranous septum, accompanied by an intradural lipoma, and tethered cord. She underwent a two- stage procedure; first, removal of the bony and membranous septum, resection of the intradural lipoma, and untethering of the cord; second, she had correction of the kyphoscoliosis. Postoperatively, although the pain and sensory deficits improved, the left lower extremity monoplegia remained. Conclusion: SCM is a rare cause of spinal deformity. If left untreated, the associated neurological deficits may progress. Treatment should include a two-staged approach; first, the bony and membranous septum should be removed followed by lipoma resection and untethering the cord with adequate cord decompression, while second, a fusion may be performed to address attendant kyphoscoliosis. Keywords: Intradural lipoma, Kyphoscoliosis, Split cord malformation, Tethered cord

Syringomyelia in children with closed spinal dysraphism: long-term outcomes after surgical intervention

OBJECTIVEThe nature of the relationship between spinal cord syrinx and tethered cord is not well known. It is unclear if surgical cord untethering results in resolution or improvement of an associated syrinx. The objective of this study was to report the response of spinal cord syrinx to surgical cord untethering.METHODSThe authors retrospectively reviewed all patients with a syrinx and tethered cord who presented to a single institution over an 11-year interval. Patients with open neural tube defects were excluded. Thirty-one patients were identified, 25 of whom had both clinical and imaging follow-up after surgery. Patients were grouped according to etiology of the tethered cord. Clinical outcomes and syrinx characteristics were recorded.RESULTSOf the 25 patients with tethered cord, 68% (n = 17) were male. The average age at presentation was 2.5 years (0–10.1 years) and age at surgery was 3.7 years (range 1 day to 17 years). Etiologies of tethered cord were lipomyelomeningocele (n = 8), thickened/fatty filum (n = 7), intradural lipoma (n = 5), myelocystocele (n = 2), meningocele (n = 2), and diastematomyelia (n = 1). Twenty-three of the patients underwent primary untethering, whereas 2 patients had received untethering previously at another institution. The average syrinx length and width prior to surgery were 4.81 vertebral levels (SD 4.35) and 5.19 mm (SD 2.55 mm), respectively. Conus level ranged from L1 to S3. Patients were followed for an average of 8.4 years (1.35–15.85 years). Overall there was no significant change in syrinx length or width postoperatively; the average syrinx length increased by 0.86 vertebral levels (SD 4.36) and width decreased by 0.72 mm (SD 2.94 mm). Seven of 25 patients had improvement in at least one presenting symptom, including scoliosis, weakness, bowel/bladder dysfunction, and pain. Eight patients had stable presenting symptoms. Six patients were asymptomatic and 5 patients had new or worsening symptoms, which included scoliosis, pain, or sensory changes.CONCLUSIONSAlthough some syrinxes improved after surgery for tethered cord, radiological improvement was not consistent and did not appear to be associated with change in clinical symptoms. The decision to surgically untether a cord should be focused on the clinical symptoms and not the presence of a syrinx alone. Further studies are needed to confirm this finding.

Intradural Lipoma at the Craniocervical Junction Presenting with Progressing Hemiparesis: A Case Report

Intradural spinal lipomas are rare in an adult population. They are mostly asymptomatic and usually associated with spinal dysraphism in a pediatric population. We report a rare case of spinal lipoma without dysraphism and with progressing hemiparesis. A 60-year-old woman had incidental lipoma at the craniocervical junction observed for more than 5 years. Recently, she developed right-sided hemiparesis and sensory disturbance. Radiological studies revealed a large lipoma compressing the dorsal medulla and C1–C2 spinal cord. Standard midline suboccipital craniotomy and C1 laminectomy were performed, and the lipoma was removed subtotally. The lipoma showed severe adhesion to the dorsal medulla and C1 spinal cord; therefore, the excision was limited as internal debulking. Her neurological deficit subsided within 6 months after the decompressive surgery. Considering the benign nature of lipoma, internal decompression is a reasonable management for this lesion.