Batista Cezar-Junior Auricelio, Faquini Igor Vilela, Frank Kauê, Euripedes Almondes S Lemos Luiz, de Carvalho Eduardo Vieira, Almeida Nivaldo S, Azevedo-Filho Hildo Rocha Cirne
Instituto de Medicina Integral Professor Fernando Figueira (IMIP), Recife, Brazil.
Neurosurgery Department of the Hospital da Restauração Gov. Paulo Guerra, Recife, Brazil.
Int J Surg Case Rep. 2020;71:303-306. doi: 10.1016/j.ijscr.2020.05.021. Epub 2020 May 21.
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.
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.
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.
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.
局限性脊髓脊膜膨出（LDM）是一种最近才被描述的病理实体，其特征为闭合的局灶性神经管存在缺陷，以及有一个纤维神经蒂将皮肤病变与脊髓相连。
本病例描述了一名9个月大的患有人类尾巴及潜在脊髓发育异常的儿童。表现为与髓内脂肪瘤相关的LDM蒂，与背部皮肤附属器相连。我们还报告了该病例的治疗方案及其临床结果。
LDM是脊髓发育异常的一种独特临床病理表现，与多种异常情况相关，如脂肪瘤型脊髓脊膜膨出、脊髓栓系、脂肪瘤、先天性心脏病和畸胎瘤。在本病例中，LDM的手术治疗包括在同一手术过程中切除附属器、松解脊髓以及切除圆锥脂肪瘤。
在本病例报告中，我们分享了小儿神经外科转诊服务在临床和放射学诊断以及成功治疗这种罕见先天性畸形方面的经验。因此，临床医生应意识到与LDM相关的皮肤病变可能存在的形态学变异。
