SUMMARY: We present a variant of a split cord malformation with coexisting segmental spinal dysgenesis. Background Split cord malformations (SCMs) are uncommon congenital anomalies. Diastematomyelia: a type 1 split spinal cord malformation. Neurosurgery. MahapatraAn unusual case of split cord malformation with … Diastematomyelia is a rare abnormality of the spinal canal characterized by a split spinal cord with or without a bony or fibrous septum. She also had spina bifida occulta. 14. Type 1 and Type 2. Five out of nine patients that had preoperative bladder dysfunction were improved. Type I split cord malformation Final Diagnosis. There were more type I (n = 22) than type II (n = 15) SCM. A Type I SCM consists of two hemicords, each contained within its own dural tube and separated by a dura-sheathed rigid osseocartilaginous median septum. The patient was admitted with a hypertrichosis and hyperpigmented patch. A personal series of 131 patients with split cord malformation (SCM) operated on is presented. L5 and S1), split cord malformation type I which separated two hemi-cords, tethered cord and syringomyelia. 1993; 33:374–8. In type II SCM, the spinal cord sits within a single dural tube and is split into two independent segments for ... Posterior vertebral body fusion defect without spinal cord malformation (so-called spina bifida occulta) Forms of occult spinal dysraphism. Usually, the cord is split by some type of mesenchymal derivative such as bone or cartilage. In previous patient, authors reported a long-segment type 1 split cord malformation, with two-level split cord malformation and a single dural sac and the spur was at the lower end of split. The complete or partial division of the spinal cord is due to the presence of a septum in the central part of the spinal canal. MahapatraAn unusual case of 4 level spinal dysraphism: multiple composite type 1 and type 2 split cord malformation, dorsal myelocystocele and hydrocephalous. Background Split cord malformation (SCM) remains an important cause of tethered cord syndrome. It has reported an uncommon case a 3 year-old girl a composite split cord malformation (SCM) with two different levels of SCM type1 and one level SCM type2, tight filum and sacral dermal sinus. Normally, only the spinal cord passes through this opening. This has often (but not consistently) been referred to as diastematomyelia. A. Agarwal, A. Raheja, S.A. Borkar, A.K. 13. To add on Pangs classification, we proposed a sub classification into type A and B on basis of whether Split cord malformation (SCM) is associated with meningomyelocoele or not. The new classification recommends the term split cord malformation (SCM) for all double spinal cords. Chiari malformation type 1 is a structural abnormality of the cerebellum, the part of the brain that controls balance, and the skull. Lipomyelomeningocele and lipomeningocele. MRI of whole spine and brain was done. Sometimes there is only one protective membrane or sleeve (the dura) around both parts of the spinal cord. People with type 1 may not have any symptoms or may develop symptoms when they are teenagers or adults. Indian J Pediatr. The difference is statistically significant Split spinal cord malformation Synonyms: Diastematomyelia, SSCM, Diplomyelia, Pseudodiplomyelia, Dimyelia, Split cord malformation, SCM type 1, Split cord malformation type 1 Back to top The new classification recommends the term split cord malformation (SCM) for all double spinal cords. 160: 1180-6 . One or more skin stigmata were present in 60% cases, hypertrichosis being the most common. RESULTS: Patients with preoperative neurological symptoms (n = 21) were improved in 71.4%. The management can be further complicated by the other associated dysraphic states in the same patient. Type 2 split cord malformation. J Clin Neurosci. 2004. 1992 Sep;31(3):451-80. The malformation occurs when the lower part of the cerebellum extends below the foramen magnum (the large hole at the base of the skull which allows passage of the spinal cord), without involving the brainstem. Often, the split is caused by a thin segment of bone or cartilage that protrudes from the spinal column into the spinal cord space. Rev Neurol (Paris). The split spinal cord is a rare congenital malformation. J Pediatr Neurosci, 6 (2011), pp. A. Khandelwal, V. Tandon, A.K. split segment in Type I SCM was shorter than that in Type II SCM. The split of the cord extended from D 5 to D 11 level. This classification highlights the importance of pure or combined occurrence. The female predominance was slightly more remarkable in type I SCMs than in type II SCMs. The mean age of the patients was 7.4 years and the female to male ratio was 1.3:1. Split cord malformation (SCM) is a rare congenital anomaly. Preoperatively the patient had paraparesis and discretely moved his left foot. There were 88 girls (73 %) and 43 boys (27 %). Pang and Ersahim have also pointed on high incidence of type 1 SCM as association with MMC. In type I, the two hemicords are typically separated by a fibrous, cartilaginous, or osseous septum and reside in two separate dural tubes (Figure 7). This septum can be formed out of bone tissue or it can be cartilaginous or fibrous. Guilloton L, Allary M, Jacquin O, Billaud Y, Drouet A, Felten D. Split-cord malformation (diastematolmyelia) presenting in two adults: Case report and a review of the literature. Introduction. The series from Mahapatra et al. There was a bony spur arising from the D 11 body level without a separate dural covering but with 2 cords. Much confusion still exists concerning the pathological definitions and clinical significance of double spinal cord malformations. Conclusion. Neurosurgery. Defined as two hemicords, each with its own central canal and surrounding pia, each within a separate dural tube separated by a dural-sheathed rigid osseocartilaginous (bony) median septum. One hundred thirty-two patients had type I SCM and 71 had type II SCM. Split cord malformation (SCM) is a rare congenital anomaly, in which the cord is split over a portion of its length to form double dural tubes (SCM type 1) or two hemicords in a single dural sheath (SCM type 2). Type I Split Cord Malformation. [Google Scholar] 34. Cranial and cervical MRI showed Chi-ari I and corpus callosum hypoplasia. The split lengths of the 19 Type I lesions (including the one case of double bone spurs and tripartite cord, in which the two split lengths were identical) varied between 1 and 7 vertebral levels, with a mean of 2.97. The term is used to describe a developmental malformation of the spinal cord that is characterized by a splitting of the cord into at least two independent segments. Background: Split cord malformation (SCM) remains an important cause of tethered cord syndrome. We report the rare finding of a split cord malformation in a young girl. In the SSCM Type II, both parts of the spinal cord are surrounded by a common dura tube. 58-61. Chiari malformation Type I Type 1 happens when the lower part of the cerebellum (called the cerebellar tonsils) extends into the foramen magnum. DIASTEMATOMYELIA or split cord malformation is a rare phenomenon that may be discovered on magnetic resonance imaging of the spine. The excision of bony spur in type 1 split cord malformation is challenging due to the intricate pathologic anatomy. Type 1—which may not cause symptoms—is the most common form of CM. The split spinal cord malformation (SSCM) is an uncommon congenital malformation of the vertebral canal in which parts of the spinal cord are longitudinally duplicated. It is the most common type. In SSCM Type I, each spinal cord has its own dura tube. The presence of bony spurs seen in type 1 SCM are usually partial and unequivocally are found attached to the dorsal surface of the vertebral body. 14. Frequently with overlying cutaneous and/or subcutaneous abnormalities. Warder DE, Oakes WJ. These images are from the case of a 29-yr-old woman with back pain without neurological symptoms. Tethered cord syndrome and the conus in a normal position. Management of split cord malformation in children: the Lyon experience. There was no hydrocephalus. DISCUSSION. Long segment bony spur in split cord malformation Type 1. Surgical management of adult Type 1 split cord malformation. CT myelography showed the left hemicord with a small remnant of subarachnoid space running through an intravertebral cleft in a spine anomaly. Diastematomyelia (occasionally diastomyelia) is a congenital disorder in which a part of the spinal cord is split, usually at the level of the upper lumbar vertebra.. Diastematomyelia is a rare congenital anomaly that results in the "splitting" of the spinal cord in a longitudinal direction.Females are affected much more commonly than males. Split cord malformation (diastematomyelia): a complex type of spinal dysraphism in which the spinal cord splits lengthwise into two distinct cords. Split cord malformation is well documented and reported in various case series and reports in the literature. Report of two cases with literature review. 2018; 52:119–21. Type I split cord malformation References [1] Pang D, Dias MS, Ahab-Barmada M. Split cord malformation: Part I: A unified theory of embryogenesis for double spinal cord malformations. A Type II SCM consists of two hemicords housed in a single dural tube separated by a nonrigid, fibrous median septum. A Type I SCM consists of two hemicords, each contained within its own dural tube and separated by a dura-sheathed rigid osseocartilaginous median septum. Compared with Type II SCM, patients with Type I SCM presented with higher incidence of kyphotic deformity, more extensive and complicated vertebral anomalies, and more complex rib anomalies. Whereas, in Type II split cord malformations both the hemicords lie withina single, non-duplicated, dural tube[2]. It is also known as the split cord malformation and a type of spinal dysraphism (spina bifida occulta). The dorsolumbar and lumbar regions were the most common sites. Type 1. Wolf AL, Tubman DE, Seljeskog EL. Type 1.5 split cord malformation? The differential diagnosis includes dimyelia (complete spinal cord duplication) and diplomyelia (the presence of an accessory spinal cord). Age, gender, symptoms and signs, radiological and operative findings, complications, associated anomalies, outcome, and pathological specimens were analyzed. The split lengths of the 18 single Type II lesions varied between 0.5 and 2 vertebral levels, with a mean of 1.17. 84: 246-8. 2017. Type 1 and Type 2. Figure A is an axial T2-weighted image at the T12 level demonstrating diastematomyelia with two hemicords sharing a single … The term is from the Greek "diastema" (cleft) and "myelos" (marrow or medulla). In type I, the two hemicords are typically separated by a fibrous, cartilaginous, or osseous septum and reside in two separate dural tubes (Figure 7). View Record in Scopus Google Scholar. Whereas, in Type II split cord malformations both the hemicords lie withina single, non-duplicated, dural tube[2]. 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