Dysraphic spinal cord lipoma

Dysraphic spinal cord lipoma, also known as spinal lipoma associated with spinal dysraphism, is a congenital malformation in which a fatty mass (lipoma) is abnormally attached to or incorporated within the spinal cord, arising from a defect in neural tube closure during embryonic development. This condition falls within the spectrum of closed spinal dysraphism (occult spinal dysraphism), meaning the overlying skin is typically intact, unlike open neural tube defects such as myelomeningocele. The lipoma may be classified based on its anatomical relationship to the spinal cord and may include lipomyelomeningocele, lipomyelocele, or intradural lipoma subtypes. The condition primarily affects the nervous system and musculoskeletal system. The lipoma tethers the spinal cord, restricting its normal movement within the spinal canal, which can lead to progressive neurological deterioration as the child grows. Key clinical features include lower limb weakness or asymmetry, sensory deficits, bladder and bowel dysfunction (neurogenic bladder and bowel), foot deformities (such as clubfoot or cavus foot), and back pain. A subcutaneous fatty mass or skin stigmata (such as a dimple, hairy patch, or skin tag) in the lumbosacral region is often the first visible sign, frequently noted at birth or in early childhood. Some patients may remain asymptomatic for years before symptoms develop due to progressive cord tethering. Management typically involves neurosurgical evaluation and, in many cases, prophylactic or therapeutic surgical untethering of the spinal cord to prevent or halt neurological decline. Surgery aims to debulk the lipoma and release the tethered cord, though complete removal is often not possible due to the intimate relationship between the lipoma and neural tissue. Urological assessment and management of neurogenic bladder are important components of care. Orthopedic interventions may be needed for associated skeletal deformities. Long-term follow-up is essential, as re-tethering can occur after surgery, and some patients may require repeat procedures. The decision to operate on asymptomatic patients remains a topic of ongoing clinical discussion.

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