Spinal dysraphism: A review of neuroradiological features with embryological correlations and proposal for a new classification.
Neuroradiology ; Neuroimaging Clin N Am ; Moore K. Caffey's Pediatric Diagnostic Imaging. Philadelphia, PA: Elsevier Saunders; Sadler TW. Embryology of neural tube development. Schoenwolf GC.
Other spinal dysraphisms – PM&R KnowledgeNow
Histological and ultrastructural observations of tail bud formation in the chick embryo. Anat Rec ; Genetic studies in neural tube defects. NTD Collaborative Group. Pediatr Neurosurg ; Drolet BA. Cutaneous signs of neural tube dysraphism. Pediatr Clin North Am ; Imaging in spine and spinal cord malformations. Eur J Radiol ; Congenital Spine and Spinal Cord Malformations—pictorial review. Am J Roentgenol ;SS MR of the caudal regression syndrome: Embryologic implications.
Barkovich AJ. Congenital Anomalies of the spine. In: Paediatric Neuroimaging. Segmental spinal dysgenesis: Neuroradiologic findings with clinical and embryologic correlation. Diastematomyelia: Hemicord and meningeal sheaths; single and double arachnoid and dural tubes. Infections of the nervous system.
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Buy eBook. Buy Softcover. FAQ Policy. An intradural lipoma refers to a lipoma located along the dorsal midline that is contained within the dural sac. No open spinal dysraphism is present. Intradural lipomas are most commonly lumbosacral in location and usually present with tethered-cord syndrome, a clinical syndrome of progressive neurologic abnormalities in the setting of traction on a low-lying conus medullaris.
Fibrolipomatous thickening of the filum terminale is referred to as a filar lipoma. On imaging, a filar lipoma appears as a hyperintense strip of signal on T1-weighted MR images within a thickened filum terminale. Filar lipomas can be considered a normal variant if there is no clinical evidence of tethered-cord syndrome. Tight filum terminale is characterized by hypertrophy and shortening of the filum terminale. The filar thickening usually greater than 2 mm is usually fibrous, fatty, or cystic, and may be associated with other dysraphic myelodysplasias, including lipomyelomeningocele and diastematomyelia.
The thickened filum may also terminate in a lipoma or dermoid-epidermoid. A and B, Sagittal A and axial B T1- weighted MR images in 2-year-old boy with filar lipoma arrows , which has characteristic T1 hyperintensity and marked thickening of filum terminale. Tethered cord long arrow , thick filum and lipoma short arrows , and sacral extradural arachnoid cyst A on sagittal T1-weighted A , axial T1-weighted B , and axial T2-weighted C MR images. Complex dysraphic states—Complex dysraphic states can be divided into two categories: disorders of midline notochordal integration, which include dorsal enteric fistula, neurenteric cyst, and diastematomyelia, and disorders of notochordal formation, which include caudal agenesis and segmental spinal dysgenesis.
Disorders of midline notochordal integration: Dorsal enteric fistula and neurenteric cyst—A dorsal enteric fistula occurs when there is an abnormal connection between the skin surface and bowel. Neurenteric cysts represent a more localized form of dorsal enteric fistula.
These cysts are lined with mucin-secreting epithelium similar to the gastrointestinal tract and are typically located in the cervicothoracic spine anterior to the spinal cord. Neurenteric cyst in 3-year-old girl.
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A and B, Sagittal T2-weighted A and axial T1-weighted B MR images show bilobed neurenteric cyst arrows extending from central canal into posterior mediastinum. C, Three-dimensional CT reconstruction image shows osseous opening arrow through which neurenteric cyst passes. This opening is called the Kovalevsky canal. Terminal ventricle: Persistence of a small, ependymal lined cavity within the conus medullaris is referred to as a persistent terminal ventricle.
Key imaging features include location immediately above the filum terminale and lack of contrast enhancement, which differentiate this entity from other cystic lesions of the conus medullaris. A dermal sinus is an epithelial lined fistula that connects neural tissue or meninges to the skin surface. It occurs most frequently in the lumbosacral region and is often associated with a spinal dermoid at the level of the cauda equina or conus medullaris. Clinically, patients present with a midline dimple and may also have an associated hairy nevus, hyperpigmented patch, or capillary hemangioma.
Surgical repair is of great importance because the fistulous connection between neural tissue and the skin surface can result in infectious complications such as meningitis and abscess. A and B, Sagittal T2-weighted A and sagittal T1-weighted contrast-enhanced B MR images in month-old boy show persistent terminal ventricle as cystic structure arrows at inferior aspect of conus medullaris, which does not enhance.
A and B, Sagittal schematic A and sagittal T2-weighted MR image B in 9- year-old girl show intradural dermoid stars with tract extending from central canal to skin surface black arrows. Note tenting of dural sac at origin of dermal sinus white arrows. C, Axial T2-weighted MR image from same patient as in B shows posterior location of hyperintense dermoid arrow. Thoracic dermal sinus black arrows and dermoid cyst white arrows on sagittal T1-weighted A , sagittal T2- weighted B , and axial T1-weighted C MR images.
Thoracic dermal sinus posterior black and white arrows in A, and B and cyst with enhancing abscess anterior white arrows in B with cord edema on sagittal T2-weighted A and gadolinium-enhanced T1- weighted B MR images. Disorders of midline notochordal integration: Diastematomyelia—Separation of the spinal cord into two hemicords is referred to as diastematomyelia. The two hemicords are usually symmetric, although the length of separation is variable. There are two types of diastematomyelia. In type 1, the two hemicords are located within individual dural tubes separated by an osseous or cartilaginous septum.
In type 2, there is a single dural tube containing two hemicords, sometimes with an intervening fibrous septum. Diastematomyelia can present clinically with scoliosis and tethered-cord syndrome. Type 1 diastematomyelia. Type 2 diastematomyelia. A—C, Sagittal T1-weighted A , coronal T1-weighted B , and axial T2-weighted C MR images in 9-year-old girl show splitting of distal cord into two hemicords white arrows, B and C within single dural tube, which is characteristic for type 2 diastematomyelia.
Incidental filum lipoma black arrows, A and B is present as well. Lumbar diastematomyelia with tethered hemicords long arrows , no septum, and a lipoma short arrow on sagittal T1-weighted A and axial T2-weighted B MR images. Disorders of notochordal formation: Caudal agenesis—Caudal agenesis refers to total or partial agenesis of the spinal Column and may be associated with the following: anal imperforation, genital anomalies, renal dysplasia or aplasia, pulmonary hypoplasia, or limb abnormalities.
Caudal agenesis can be categorized into two types. In type 1, there is a high position and abrupt termination of the conus medullaris. In type 2, there is a low position and tethering of the conus medullaris. Segmental spinal dysgenesis—The clinical—radiologic definition of segmental spinal dysgenesis includes several entities: segmental agenesis or dysgenesis of the thoracic or lumbar spine, segmental abnormality of the spinal cord or nerve roots, congenital paraparesis or paraplegia, and congenital lower limb deformities.
Three-dimensional CT reconstructions can be helpful in showing various vertebral segmentation anomalies Conus medullaris is high in position and wedge shaped arrow due to abrupt termination. These findings are characteristic of type 1 caudal agenesis. Distal cord syrinx arrowhead is present as well. Cord ending at a higher level at DL1 with 'wedge' shaped termination.
No cord tethering. No terminal cord cyst or syrinx. Imaging diagnosis : Caudal Regression, Group I. Teratoma - Genetics A teratoma is a disordered mass of cells which exhibit varying degrees of resemblance to normal tissues and structures. Disrupted or incomplete twinning versus uncontrolled stem cell?
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Segmental Spinal Dysgenesis–“Redefined”
Fetus in fetus. Other teratomas - arise from stem cells Congenital anomalies of the vertebral column. Schematic drawing depicting the development of normal and abnormal vertebral bodies. Asomia Congenital absence of the body of L2 Hypoplastic posterior elements are present arrow. Left hemivertebra involving T Butterfly vertebra. Block vertebra.