Perrone Erin E, Jarboe Marcus D, Maher Cormac O, Berman Deborah R, Ladino-Torres Maria, Kreutzman Jeannie, Treadwell Marjorie C, Mychaliska George B
Section of Pediatric Surgery, Department of Surgery, University of Michigan, Ann Arbor, MI, USA.
Fetal Diagn Ther. 2018;43(1):72-76. doi: 10.1159/000472714. Epub 2017 May 3.
Sacrococcygeal teratoma (SCT) with intraspinal extension is rare. There is a risk of paraplegia associated with prolonged spinal cord compression. We present the case of an infant with a prenatal diagnosis of an SCT with a large intraspinal component that was causing compression of the lower spinal cord. Ultrasound at 33 weeks showed bilateral lower extremity and foot movement without hydrops or cardiac failure. Multidisciplinary decision was made to administer betamethasone and proceed with Cesarean delivery at 34 weeks. A vigorous live-born female was delivered and a multilevel laminectomy was performed at day of life 4. The pelvic resection was performed at 4 months. Pathology revealed mature teratoma. She had an uncomplicated postoperative course, is ambulatory, continent of stool, and has no evidence of recurrence. We conclude that intraspinal extension of SCT should be evaluated prenatally with ultrasound and fetal MRI. If there is concern for spinal cord compression, early delivery and urgent decompressive laminectomy may diminish the neurologic sequelae of prolonged spinal cord compression. Since these cases are rare, risks of prematurity need to be weighed against the neurologic risks. These infants should be treated with a multidisciplinary approach.
伴有脊髓内延伸的骶尾部畸胎瘤(SCT)很罕见。存在因脊髓长期受压而导致截瘫的风险。我们报告一例产前诊断为SCT且伴有巨大脊髓内成分并导致下脊髓受压的婴儿病例。孕33周时超声检查显示双侧下肢及足部有活动,无水肿或心力衰竭。经多学科讨论后给予倍他米松,并于孕34周行剖宫产。一名活力良好的女婴出生,出生后第4天行多级椎板切除术。4个月时行盆腔切除术。病理检查显示为成熟畸胎瘤。她术后恢复过程顺利,能行走,大便能自控,且无复发迹象。我们得出结论,SCT的脊髓内延伸应在产前通过超声和胎儿磁共振成像进行评估。如果担心脊髓受压,早期分娩和紧急减压性椎板切除术可能会减少脊髓长期受压导致的神经后遗症。由于这些病例罕见,需要权衡早产风险与神经风险。这些婴儿应采用多学科方法进行治疗。