Spinal Dysraphisms: A New Anatomical–Clinicoradiological Classification

Abstract

Background Spinal dysraphisms refer to the congenital abnormalities of the spine and spinal cord due to aberrations in the processes of gastrulation, primary neurulation, and secondary neurulation. Embryology of many complex spinal dysraphisms are yet poorly understood and there is no agreeable anatomical–clinicoradiological classification with inclusion of recently documented and complex spinal dysraphisms. Aims and Objectives The main objective of this study was to review the imaging features of spinal dysraphisms with a better understanding of embryological abnormalities and propose a new classification inclusive of all complex and unusual dysraphisms based on anatomical and clinicoradiological correlation. Materials and Methods This was a retrospective single institutional observational study of 391 cases of spinal dysraphism for 10 years in our institution. Of 391 cases included in the study, 204 were males and 187 were females. Also, 123 cases belonged to the 0–6 months age group, 38 cases belonged to the 7–12 months age group, 156 belonged to the 1–5 years age group, 39 cases belonged to the 6–10 years age group, and 35 cases belonged to 10–20 years age group. Results An anatomical–clinicoradiological analysis of cases yielded a high proportion of cases of spinal lipomas, including lipomyeloceles and lipomyelomeningoceles (31.3%) and posterior myelomeningocele (14.2%). Anterior myelocoele (0.2%), sacral chordoma(0.2%), and intrasacral meningocele (0.2%) formed the least proportion of cases. A new classification was proposed based on the analysis of acquired data. Conclusion A structured approach in imaging spinal dysraphism is necessary for imaging evaluation in recent years. The proposed new classification based on clinicoradiological correlation and anatomic location is inclusive of unusual and complex dysraphisms.

Contiguous diastematomyelia with tethered cord, intradural extramedullary dermoid tumor, and lipomyelomeningocele: A unique case of spinal dysraphism

“…reporting an association with genitourinary tract abnormalities (4.1%) and dermoid cysts (3.1%), along with other associations. Diastematomyelia arises due to a defect in midline integration of the primitive notochord, such that there are two notochordal processes, each inducing the formation of separate neural plates 3 . Although diastematomyelia is commonly found to be associated with vertebral abnormalities, our patient had no such findings except for a swelling in the back along with a hairy tuft.…”

“…around 14.2% of the total cases, whereas posterior lipomyelomeningocele was found in 3.33% of cases, only 1.3% of spinal dysraphism was due to dermoid and Lumbar diastematomyelia was found in 6% of the cases. 3 To the best of our knowledge, there has not been a simultaneous concurrence of lumbar diastematomyelia, lipomyelomeningocele, and an intradural dermoid to date.…”

“…While commonly affecting the head and scalp in the pediatric population, intraspinal dermoid is extremely rare, accounting for around 1.1% of intraspinal tumors 2 . Diastematomyelia is a congenital anomaly of the spine due to defective formation of the primitive notochord, giving rise to two notochordal processes 3 . Here, we report a case of an intradural extramedullary dermoid cyst in a child with lipomyelomeningocele and the presence of a diastematomyelia with tethering of the cord, emphasizing the pathologic investigations and surgical treatment.…”

Late diagnosis of dorsolumbar lipomyelomeningocele with tethered cord in a middle aged adult: A case report from Nepal

“…Lipomyelomeningoceles are characterized by a subcutaneous fatty mass above the intergluteal crease that usually extends asymmetrically into one buttock. Because of the enlargement of the underlying subarachnoid spaces, the neural placode-lipoma interface is located outside the spinal canal, resulting in a posterior meningocele [ 6 ]. MRI scan revealed similar findings in our case.…”

Imaging spectrum of spinal dysraphism: A diagnostic challenge

“… 1 It includes a wide range of congenital anomalies resulting from aberrations in the stages of gastrulation, primary neurulation and secondary neurulation. 2 Spinal dysraphisms have a prevalence of ~1 to 3 per 1000 live births with the lumbosacral spine being the most common site. 1 Spinal dysraphisms may lead to neurological impairment of varying severity including weakness of the extremities, incontinence of bowel and bladder, and sexual dysfunction, among others.…”