Postpartum paraparesis from spinal neurofibroma.

BACKGROUND CONTEXT

One hallmark of neurofibromatosis Type 1 (NF1) is the presence of multiple neurofibromas, which are hormonally responsive and may undergo rapid growth during periods of hormonal surge. Although spinal neurofibromas occur in up to 40% of patients with NF1, they rarely cause neurologic sequelae, especially in the young.

PURPOSE

To document a unique case of acute postpartum paraparesis in a young woman because of an extradural neurofibroma compressing the conus medullaris and discuss the possible hormonal influences related to rapid growth of this otherwise "benign" nerve sheath tumor. This case demonstrates the importance of closely monitoring patients with known NF1 for neurologic symptoms, especially during pregnancy and the postpartum period. We also seek to describe the management of this patient, which resulted in an excellent outcome.

STUDY DESIGN

Case report and literature review.

METHODS

Detailed history was obtained from the patient and her family members. All medical records, imaging studies, histopathological findings, and pertinent literature were reviewed.

RESULTS

After evaluating a 26-year-old postpartum woman with NF1 for paraparesis, magnetic resonance imaging of the lumbar spine revealed a large heterogeneously enhancing mass at the conus medullaris. She subsequently underwent resection of the mass via laminectomy. Postoperatively, the patient exhibited marked improvement in weakness and was able to ambulate with assistance 2 days later. Histopathological examination of the mass revealed a neurofibroma with strong expression of estrogen and progesterone receptors.

CONCLUSIONS

This patient had a spinal neurofibroma, which likely grew in size within the peripartum period, causing a neurologic emergency. The rapid growth of the neurofibroma may have been the result of hormonal influence of estrogen or progesterone or both. To the best of our knowledge, rapid paraparesis caused by a spinal neurofibroma has never been reported in a peripartum setting. Laminectomy with complete tumor resection is the preferred treatment for spinal neurofibromas causing acute neurologic symptoms.