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Developmental Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James H. Tonsgard, Nikolas Mata-Machado
An occult spinal dysraphism is spina bifida occulta associated with: Fibrous bands causing distortion of the cord.Intraspinal lipoma.Dermoid or epidermoid cyst.Fibrolipoma, subcutaneous lipoma.Diastematomyelia.Commonly results in the tethered cord syndrome (TCS).
Paper 3
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
This is a congenital cord malformation causing a sagittal division of the spinal cord splitting it into two hemicords which usually join together again more caudally. Each hemicord has its own central canal, dorsal horn and ventral horn. This is in contrast to diplomyelia which is complete cord duplication. In diastematomyelia the lower thoracic and upper lumbar levels are most commonly affected. The condition is almost always symptomatic. Patients frequently have scoliosis and can present secondary to its association with a tethered cord with lower limb neurology and bowel and bladder dysfunction. Radiological signs include a widened interpedicular distance and vertebral anomalies. On MRI the cord can be seen splitting and there is sometimes a fibrous or bony spur between the two hemicords.
Skeletal Embryology and Limb Growth
Published in Manoj Ramachandran, Tom Nunn, Basic Orthopaedic Sciences, 2018
Rick Brown, Anish Sanghrajka, Deborah Eastwood
Diastematomyelia is a longitudinal splitting of the spinal cord by a bony or fibrocartilaginous spicule arising from the vertebral body, which is believed to be a remnant of the early connection to the primitive gut or amniotic cavity.
Complex spinal dysraphism: myelomenigocele associated with dorsal bony spur, split cord malformation type I, syringomyelia, lipoma and tethered cord
Published in British Journal of Neurosurgery, 2023
Gautam Dutta, Ankit Shah, Manish Garg, Robin Gupta, Ghanshyam Singhal, Daljit Singh, Anita Jagetia, Hukum Singh, Arvind Srivastava, Ravindra Saran
Magnetic resonance imaging (MRI) of the neuraxis was obtained which revealed open posterior elements of L1 and L2 vertebrae with a well-defined fluid signal intensity cystic lesion of size 5 by 4 by 3 cm with few neural elements in the midline of lower dorsal spine communicating with spinal subarachnoid space via a narrow neck extending through dehiscent posterior elements. The spinal cord was seen to split into two hemicords at L1–L2 vertebral level for a short segment suggestive of diastematomyelia. Syringomyelia was noted proximal to diastematomyelia extending proximally up to the D10 vertebral level along with a small intraspinal lipoma at the L2-L3 vertebral level on right posterolateral aspect measuring 1.5 by 1.8 cm. The cord was low-lying and tethered to the thickened dura protruding into the canal. (Figure 1 (A–E)). Computed tomography (CT) of the neuraxis revealed sagittal clefting of cord due to dorsal osteocartilaginous spur at L1–L2 level (Figure 2(A) and (B)). There was no associated hydrocephalus or any evidence of Arnold-Chiari malformations.
Congenital Spinal Lipomatous Malformations. Part 1. Spinal Lipomas, Lipomyeloceles, and Lipomyelomeningoceles
Published in Fetal and Pediatric Pathology, 2020
A directly- or indirectly-related tethered spinal cord is common among patients with congenital spinal lipomatous malformations, and this can be the clinical setting that brings the patient to a surgeon. Associated diastematomyelia, hydromyelia, or a syrinx can be encountered. The patients may have dislocation of the hips, clubfeet, polydactyly, scoliosis, kyphosis, perineal anomaly, sacral agenesis, the Currarino syndrome (triad of sacral agenesis, presacral meningocele, and anorectal anomaly), upper gastrointestinal atresia, renal or cardiac anomalies, intraspinal arachnoidal cysts, epidermoid or dermoid cysts, a benign sacrococcygeal teratoma, or neurocutaneous melanosis [21, 24, 54, 62, 64, 73, 112–121].
Spinal dysraphism in a 63-year-old multipara faun-tailed woman
Published in British Journal of Neurosurgery, 2020
Sofia Kamakh Asaad, Anne-Sofie Caroline Jensen, Carsten Reidies Bjarkam
A thoracolumbar MRI revealed spina bifida occulta at level L1/L2 (Figure 1(D)) corresponding to the hairy stigma (Figure 1(A)). Spinal cord abnormalities were visualised from Th10 to L3 (Figures 1(B–F)). The sagittal image (Figure 1(F)) revealed a tethered cord as the conus medullaris ended at level L2/L3 (Figures 1(E,F)). At level Th12/L1 axial images (Figure 1(C)) revealed two hemicords (diastematomyelia) in a single dural sac without a fibrous component, thus, a split spinal cord malformation (SSCM) type II. Cranially and caudally, the SSCM was surrounded by syrinx formation of the spinal cord (Figures 1(B,D,F)).