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Tethered Cord with Intraspinal Lipoma

Case Detail

Anatomy: Brain-Spine
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 6 years ago
Updated: over 6 years ago
Tags: PEDS
Modality/Study Types: US MR
Activities:
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History

Newborn with lumbar dermal sinus.


Case Images


Diagnosis

Tethered Cord with Intraspinal Lipoma

Findings

US – Dysplastic conus medullaris is tethered at the lumboscaral junction in an intraspinal lipoma. Dermal sinus tract is present.

MR – Intraspinal fat engulfs the dysplastic cord and is continuous with the subcutaneous fat. Dermal sinus tract extends into the spinal canal superior to the lipoma.

Discussion

A dorsal dermal sinus is an epithelium-lined tract from the skin to the thecal sac. Most are located in the lumbosacral region and less often in the occipital or other midline regions. Dorsal dermal sinus is caused by incomplete separation of the superficial ectoderm from the neural ectoderm, resulting in a focal adhesion. Later the spinal cord ascends relative to the spinal canal and stretches the adhesion into a long, tubular tract. There is no genetic predominance. Dorsal dermal sinus manifests as a small midline dimple often associated with other skin anomaly (altered pigmentation, hemangioma or hypertrichosis). Since the sinus provides an avenue for infection, meningitis, epidural or subdural abscess, and myelitis may occur. A paramedian dermal sinus is often associated with an intraspinal dermoid or epidermoid cyst.

Intraspinal lipomas are classified as lipomyelocele/lipomyelomeningocele, fibrolipoma of the filum terminale, and intradural lipoma. Neurologic symptoms include sensory deficits, paresis, and bowel/bladder dysfunction. Premature separation of the superficial ectoderm from the neural ectoderm results in mesenchymal infiltration which differentiates into fat. In lipomyelocele/lipomyelomeningocele, the lipoma lies adjacent to the dysplastic spinal cord and extends into the spinal cord causing tethering. The lipoma is continuous with the subcutaneous fat and covered by intact skin. Lipomyelocele is always associated with spina bifida and often associated with segmentation anomalies of the vertebrae.

Reference

Unsinn KM, Geley T, Freund MC, et al. US of the Spinal Cord in Newborns: Spectrum of Normal Findings, Variants, Congenital Anomalies, and Acquired Diseases. RadioGraphics (2000); 20:923-938.



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