Department of Radiology, Hospital de Clínicas da Universidade Federal do Paraná, Curitiba, Paraná, Brazil.
Department of Radiology, Hospital de Clínicas da Universidade Federal do Paraná, Curitiba, Paraná, Brazil.
World Neurosurg. 2021 Mar;147:8-10. doi: 10.1016/j.wneu.2020.11.135. Epub 2020 Dec 19.
Terminal myelocystocele is a rare type of neural tube malformation, consisting of a skin-covered lumbosacral mass, highly associated with other complex abdominal malformations within the OIES complex (omphalocele, imperforate anus, exstrophy of the cloaca and spinal defects). We present a case of a premature female with an extensive lumbosacral mass at birth, as well as an omphalocele, cloacal exstrophy, renal abnormalities, and sacral agenesis. Lumbar magnetic resonance imaging revealed a meningocele sac herniating through the bone defects and containing a syringocele sac. Advanced imaging techniques showed turbulent cerebrospinal fluid flow. At control, 4 weeks later, the defect doubled in size. The myelocystocele sac was evacuated and closed, and the patient persisted with paraparesis. The role of cerebrospinal fluid flow analysis is well established in Chiari-type malformations, in which turbulent flow within the syrinx is related to a better outcome after surgery. It is possible that the same principle could be applied to other spinal malformations, as shown in this case of terminal myelocystocele.
终丝脊髓脊膜膨出是一种罕见的神经管畸形，由一个覆盖皮肤的腰骶部肿块组成，与 OIES 复合体（脐膨出、肛门闭锁、泄殖腔外翻和脊柱缺陷）内的其他复杂腹部畸形高度相关。我们报告了一例女性早产儿，出生时即有广泛的腰骶部肿块，同时还有脐膨出、泄殖腔外翻、肾脏异常和骶骨发育不全。腰椎磁共振成像显示脑膜膨出囊通过骨缺损疝出，其中包含一个脊髓空洞囊。高级影像学技术显示脑脊液流动紊乱。4 周后复查时，缺损大小增加了一倍。对终丝脊髓脊膜膨出囊进行了排空和闭合，患者仍存在下肢轻瘫。脑脊液流动分析在 Chiari 型畸形中的作用已得到充分证实，其中脊髓空洞内的湍流与手术后更好的结果相关。该病例表明，同样的原理可能适用于其他脊柱畸形。
