Abstract
Diastematomyelia is a type of closed spinal dysraphism in which there is splitting of the spinal cord. It is a rare entity that accounts for less than 3% of closed spinal dysraphisms and affects females 1.3 to 6 times more frequently than males. Lesions are usually found in the lower thoracic and upper lumbar regions. It is characterised by two hemicords separated by a bony or cartilaginous spur. In most cases, it is an isolated malformation with a favourable prognosis. However, it may be associated with other abnormalities and sonography is the imaging test par excellence for early prenatal diagnosis. We report a case of diastematomyelia diagnosed by prenatal sonography at 24 weeks’ gestation. Amniotic fluid alpha-fetoprotein (AF-AFP) was normal, while amniotic fluid acetylcholinesterase (AF-AChE) was positive. After birth, the diagnosis was confirmed with magnetic resonance imaging (MRI). The anomaly was associated with a spinal lipoma, tethered cord and dermal sinus. A review of all the cases described in the literature to date is carried out.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
Data Availability
Not applicable.
Code Availability
Not applicable.
References
Pang D, Dias MS, Ahab-Barmada M. Split cord malformation: part I: a unified theory of embryogenesis for double spinal cord malformations. Neurosurgery. 1992;31(3):451–80.
Williams RA, Barth RA. In utero sonographic recognition of diastematomyelia. AJR Am J Roentgenol. 1985;144(1):87–8.
Struben H, Visca E, Holzgreve W, Kang A, Hetzel P, Schneider J, Tercanli S. Pränataldiagnose einer Diastematomyelie und "tethered cord" - ein Fallbericht mit Literaturübersicht [Prenatal diagnosis of diastematomyelia and tethered cord - a case report and review of the literature]. Ultraschall Med. 2008;29(1):72–6.
Karaşahin KE, Gezginç K, Alanbay I, Ulubay M, Başer I. Ultrasonographic diagnosis of diastematomyelia during the 14th week of gestation. Taiwan J Obstet Gynecol. 2009;48(2):163–6.
Li SL, Luo G, Norwitz ER, Wang C, Ouyang S, Wen HX, Xia X, Yao Y. Prenatal diagnosis of diastematomyelia: a case report and review of the literature. J Clin Ultrasound. 2012;40(5):301–5.
Kutuk MS, Ozgun MT, Tas M, Poyrazoglu HG, Yikilmaz A. Prenatal diagnosis of split cord malformation by ultrasound and fetal magnetic resonance imaging: case report and review of the literature. Childs Nerv Syst. 2012;28(12):2169–72.
Turgal M, Ozyuncu O, Talim B, Yazicioglu A, Onderoglu L. Prenatal diagnosis and clinicopathologic examination of a case with diastematomyelia. Congenit Anom (Kyoto). 2013;53(4):163–5.
Sassi A, Ben Ali N, Cassart M, Van Rysselberge M. Diastématomyélie diagnostiquée en période prénatale [Diastematomyelia diagnosed prenatally]. Rev Med Brux. 2014;35(1):39–42. French
Lituania M, Tonni G, Araujo JE. First trimester diagnosis of cervico-thoracic diastematomyelia and diplomyelia using three-dimensional ultrasound. Childs Nerv Syst. 2015;31(12):2245–8.
Korostyshevskaya A, Makogon A, Savelov A, Avdeeva D, Tulupov A. Fetal diastematomyelia associated with vertebral malformation: ultrasound, MRI, and pathomorphological findings. J Med Ultrason (2001). 2015;42(4):559–63.
Wei Q, Cai A, Wang X, Wang X, Xie L. The value of prenatal ultrasonographic diagnosis of diastematomyelia. J Ultrasound Med. 2017;36(6):1129–36.
Karlı P. A case report on a very rare condition: prenatal diagnosis of diastematomyelia and its clinical significance. JOJ Case Stud. 2018;8(2):555734.
El Mhabrech H, Zrig A, Bouchahda H, et al. Second trimester diagnosis of diastematomyelia: US, CT and MRI features. Int J Pregn & Chi Birth. 2018;4(1):29–31.
SR JP, Rao A, Balu M, Rao R. Diastematomyelia differences in management of diastemetomyelia with associated abnormalities versus isolated diastemetomyelia: a case series. Int J Reprod Contracept Obstet Gynecol. 2020;9(11):4688–91.
Has R, Yuksel A, Buyukkurt S, Kalelioglu I, Tatli B. Prenatal diagnosis of diastematomyelia: presentation of eight cases and review of the literature. Ultrasound Obstet Gynecol. 2007;30(6):845–9.
Sonigo-Cohen P, Schmit P, Zerah M, Chat L, Simon I, Aubry MC, Gonzales M, Pierre-Kahn A, Brunelle F. Prenatal diagnosis of diastematomyelia. Childs Nerv Syst. 2003;19(7-8):555–60.
Vande Perre S, Guilbaud L, de Saint-Denis T, Maurice P, Lallemant-Dudek P, Maisonneuve E, et al. The myelic limited dorsal malformation: prenatal ultrasonographic characteristics of an intermediate form of dysraphism. Fetal Diagn Ther. 2021;48(9):690–700.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Ethics Approval
This case study was conducted retrospectively from data obtained for clinical purposes. We consulted extensively with the IRB of Hospital Universitario Nuestra Señora de Candelaria who determined that our study did not need ethical approval.
Consent to Publish
The authors affirm that human research participant provided informed consent for publication of the images in Figs. 1, 2, 3 and 4.
Competing Interests
The authors declare no competing interests.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Arbelo-Pérez, P., de Ganzo-Suárez, T. & de Luis-Escudero, J.F. Prenatal Diagnosis of Diastematomyelia: a Case Report and Literature Review. Reprod. Sci. 30, 3563–3567 (2023). https://doi.org/10.1007/s43032-023-01307-8
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s43032-023-01307-8