Varshney Gaurav, Gupta Deepak K
Department of Neurosurgery, All India Institute of Medical Sciences (AIIMS), New Delhi, India.
J Pediatr Neurosci. 2020 Jul-Sep;15(3):286-289. doi: 10.4103/jpn.JPN_157_19. Epub 2020 Nov 6.
Myelocystoceles, also known as syringoceles, syringomyeloceles, and lipomeningomyelocystoceles, represent 5% of all lumbosacral skin-covered masses. Terminal myelocystocele (TMC) is a rare variant of spinal dysraphism. At times, patients with TMCs can present with a huge lumbosacral mass (giant TMC). A 14-month-old female child presented with progressive increasing swelling in the lumbosacral region (35 cm × 35 cm × 30 cm) since birth with inability to move both lower limbs. Magnetic resonance imaging revealed a dilated terminal central canal herniating through a spina bifida defect (from L4 to S2) into a huge meningocele, suggestive of a giant TMC. The girl was operated on successfully and was doing well at 6 months follow-up, although neurologically unchanged. Giant TMC is a rare entity and only a few case reports are available in literature. Herein we present a case with TMC with the largest dimensions reported till date.
脊髓脊膜囊肿,也被称为脊髓空洞囊肿、脊髓脊膜空洞囊肿和脂肪瘤型脊髓脊膜囊肿,占所有腰骶部有皮肤覆盖肿物的5%。终末脊髓脊膜囊肿(TMC)是一种罕见的脊柱裂畸形变体。有时,TMC患者可表现为巨大的腰骶部肿物(巨大TMC)。一名14个月大的女童自出生以来腰骶部区域出现进行性增大的肿胀(35厘米×35厘米×30厘米),双下肢无法活动。磁共振成像显示扩张的终末中央管通过脊柱裂缺损(从L4至S2)疝入一个巨大的脑脊膜膨出,提示为巨大TMC。该女孩手术成功,在6个月的随访中情况良好,尽管神经功能无变化。巨大TMC是一种罕见的病症,文献中仅有少数病例报告。在此我们报告一例TMC病例,并给出了迄今为止所报道的最大尺寸。