Adult cervicothoracic lipomyelomeningocele
Nancy Abu-Bonsrah1, Taylor E Purvis1, C Rory Goodwin1
1Department of Neurosurgery, The Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205, USA.
Insights
Lipomyelomeningocele (LMM) can cause tethered cord syndrome (TCS) in adults, presenting unusually in the cervicothoracic spine. Early diagnosis and surgical untethering are crucial for preventing severe neurological decline and improving outcomes.
Area of Science:
- Neuroscience
- Neurosurgery
- Developmental Biology
Background:
- Lipomyelomeningocele (LMM) is a congenital spinal malformation often associated with tethered cord syndrome (TCS).
- TCS typically manifests in childhood with lumbosacral symptoms including neurological deficits and bladder dysfunction.
- Early surgical untethering is the standard treatment for symptomatic pediatric cases.
Observation:
- An adult woman presented with a cervicothoracic LMM, a rare location for this condition.
- Initial symptoms included a subcutaneous lump, pain, and progressive neurological decline.
- Despite subtotal resection, the patient experienced severe, long-term neurological deterioration requiring multiple surgeries.
Findings:
- This case highlights the potential for LMM to present atypically in adults with cervicothoracic involvement.
- Delayed diagnosis and intervention in adult LMM can lead to irreversible neurological damage.
- Recurrence and progressive tethering may necessitate complex, multi-stage surgical management.
Implications:
- Emphasizes the importance of considering LMM in the differential diagnosis of adult spinal cord issues.
- Underscores the need for heightened clinical suspicion and advanced imaging for cervicothoracic spinal anomalies.
- Suggests that comprehensive surgical strategies and long-term follow-up are vital for managing adult LMM and TCS.
Abstract:
Lipomyelomeningocele (LMM) as a cause of tethered cord syndrome (TCS) commonly presents in childhood in the lumbosacral spine. Patients frequently present with cutaneous manifestations, progressive neurological deterioration, bladder dysfunction, and intractable pain. Early surgical intervention with untethering is recommended for symptomatic patients. We report an unusual case of a woman who presented with a subcutaneous lump, pain, and neurological decline found to have a cervicothoracic LMM. The patient underwent laminectomy and subtotal resection of the mass; seventeen years later she was confined to a wheelchair with severe neurological decline ultimately requiring three additional attempts at surgical excision and repair. This case emphasizes the need for early recognition of and intervention in adult patients with LMM.
