SUMMARY:This case exemplifies the difficulty in differentiating cystic sacrococcygeal teratoma and terminal myelocystocele. Fetal sonography presentation and perinatal risks of sacrococcygeal teratoma and terminal myelocystocele are compared, and we emphasize the importance of obtaining fetal MR imaging to establish an accurate diagnosis.T he terminal spine presents unique imaging challenges, particularly in the case of large cystic masses. In the first case, an isolated terminal myelocystocele mimicked a cystic sacrococcygeal teratoma (SCT) on prenatal sonography. The second case of type 2 (SCT) is presented for comparison. We discuss the presentation and perinatal risks of these 2 cystic sacrococcygeal masses and illustrate surgical correction of a cystic terminal myelocystocele.
Case Reports
Index CaseA 22-year-old gravida 2, para 1 was referred to us for investigation of a fetal mass. Maternal serum alpha-fetoprotein (AFP) levels were mildly elevated, and amniocentesis results were a healthy female with normal acetylcholinesterase levels.At 30-weeks gestational age, sonography showed a cystic exophytic lower pelvic mass measuring 8 ϫ 7 ϫ 6 cm. The mass was skin-covered and septate ( Fig 1A, -B). It appeared to originate from the tip of an otherwise normal sacrum. Fetal calvaria views were normal, without Chiari II malformation. Amniotic fluid index (AFI) measured 21 cm, and fetal size was appropriate for gestational age. The size, location, and cystic composition of the mass and the perceived absence of skeletal dysraphism and Chiari II malformation supported the diagnosis of cystic SCT with complicating polyhydramnios. Fetal MR imaging was recommended to better characterize the mass; however, the patient refused due to claustrophobia. The follow-up fetal sonography at 36 weeks showed the mass had grown (10 ϫ 10 ϫ 6 cm) and the AFI had increased to 24 cm.At 37 weeks, the neonate was delivered via classic cesarean delivery without complications, weighing 3765 g with Apgar scores of 2, 5, and 8, at 1, 5, and 10 minutes, respectively. The mode of delivery was based on the prenatal diagnosis of a large SCT.Neonatal CT and MR imaging of the congenital cyst and spine revealed sacral spinal dysraphism and tethered cord (Fig 1C, -D). The diagnosis was revised to terminal myelocystocele.Surgical exploration was undertaken at 7 days of life ( Fig 1E, -F). The large cyst was entered and found to contain clear CSF in a terminal continuation of the spinal subarachnoid space. A second thinwalled cyst encountered at the base of the larger cyst represented terminal dilation of the spinal cord central canal (Fig 1G). These findings are diagnostic of terminal myelocystocele.1 Opening the second cyst revealed the true spinal canal, with lipomatous distal tethering of the cord to the dorsal and cranial aspect of the myelocystocele ( Fig 1H). Primary spinal cord untethering was undertaken, and the spinal canal was closed by using duraplasty material. Postoperatively, the neonate retained normal neurologic and urologic function....