1 - Department of Neuroradiology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
2 - Department of Neuroradiology, Hospital D. Estefânia, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
3 - Department of Anatomical Pathology, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
4 - Department of Neurosurgery, Hospital D. Estefânia, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
- Neuropediatrics. 2019 Nov 21. DOI: 10.1055/s-0039-3399530
Case Report: A 6-year-old girl presented to our hospital with lower extremity weakness associated with paresthesia, with 6 months duration. The neurologic examination revealed lower-limb grade IV motor weakness (slightly more severe on the right side), hyperreflexia, and clonus of the right foot. She had no other neurological deficits, including sphincter dysfunction. A magnetic resonance imaging revealed a dorsal intradural intramedullary cystic mass. She underwent surgical resection, with good clinical outcome. The histopathologic examination revealed a neuroenteric cyst. Neuroenteric cysts are rare congenital lesions accounting for 0.7 to 1.3% of spinal tumors; they result from abnormal partitioning of the embryonic notochordal plate and the endoderm, during the thirdweek of embryogenesis.These lesions are typically intradural extramedullary lesions, with intra-axial lesions being extremely rare. Neurological symptoms usually present around the second or third decades of life, with generally size-dependent myelopathy. Surgical treatment is typically required, with total surgical resection being the primary goal; subtotal excision is often associated with tumor recurrence.
Palavras Chave: Neuroenteric cyst; spinal cord; children; MRI