ABSTRACT
INTRODUCTION: Limited dorsal myeloschisis (LDM) is a recently described pathological entity, characterized by a defect of the closed focal neural tube and a fibroneural pedicle connecting the cutaneous lesion to the spinal cord. PRESENTATION OF THE CASE: This case describes a 9-month-old child with a human tail and an underlying spinal dysraphism. This was represented by LDM stalk associated with a medullary lipoma, in connection with the dorsal cutaneous appendage. We also report the therapeutic proposal for this case and its clinical outcome. DISCUSSION: LDM is a distinctive clinicopathological presentation of a spinal dysraphism, associated with numerous anomalies, such as lipomyelomeningocele, tethered cord, lipoma, congenital heart disease and teratoma. In this case, surgical treatment for LDM consists of surgical resection of the appendage, untethering of the spinal cord and resection of conus medullaris lipoma in the same procedure. CONCLUSION: In this case report, we share the experience of a referral service in pediatric neurosurgery regarding clinical and radiological diagnosis, and the successful treatment of this rare type of congenital malformation. Therefore, clinicians should be aware of possible morphological variations of the skin lesion associated with LDM.
