Complex spinal dysraphism: myelomenigocele associated with dorsal bony spur, split cord malformation type I, syringomyelia, lipoma and tethered cord

2019 ◽  
pp. 1-3
Author(s):  
Gautam Dutta ◽  
Ankit Shah ◽  
Manish Garg ◽  
Robin Gupta ◽  
Ghanshyam Singhal ◽  
...  
Keyword(s):  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Split Cord Malformation ◽  
Type I ◽  
Bony Spur

scholarly journals Limited Dorsal Myeloschisis with and without Type I Split Cord Malformation: Report of 3 Cases and Surgical Nuances

Medicina ◽  
2019 ◽  
Vol 55 (2) ◽  
pp. 28
Author(s):  
Yusuf Izci ◽  
Cahit Kural
Keyword(s):  
Spinal Cord ◽  
Tethered Cord ◽  
Intraoperative Neuromonitoring ◽  
The Other ◽  
Split Cord Malformation ◽  
Type I ◽  
Cystic Lesions ◽  
Rare Form ◽  
Urological Complications ◽  
Spinal Malformations

Limited dorsal myeloschisis (LDM) is a rare form of spina bifida which is characterized by a fibroneural stalk between the inner part of the skin and the spinal cord. It may be associated with split cord malformation (SCM). Diagnosis and management of this complex malformation is challenging. We presented 3 different cases of LDM. Two of them were associated with Type I SCM and the other had no associated malformation. All of them were evaluated radiologically just after the birth and underwent surgical treatment under intraoperative neuromonitoring. They discharged without any complication. Newborns with spinal cystic lesions should be carefully evaluated for spinal malformations after the birth and treated surgically as soon as possible in order to prevent neurological and urological complications secondary to tethered cord syndrome. Surgical technique in LDM-SCM patients is quite different than the patients with solitary LDM.

scholarly journals Spinal Dysraphism of a Baby – A Case Report

2016 ◽  
Vol 5 (1) ◽  
pp. 42-45
Author(s):  
Md Abu Taher ◽  
Jafreen Sultana ◽  
Md Mofazzal Sharif ◽  
Md Mohit Ul Alam ◽  
Md Towhidur Rahman ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Final Diagnosis ◽  
Type I ◽  
Mri Findings ◽  
College Hospital ◽  
X Ray ◽  
Imaging Department ◽  
Systemic Examination

A 8 months old male child reported to paediatric OPD of Shaheed Suhrawardy Medical College Hospital with the complaints of small swelling in the back and tilting of body on one side during sitting and standing posture. His antenatal period was unremarkable, post natal period revealed delayed crying and immunization was not completed. Local examination showed a small hairy patch in lower dorsal region. General and systemic examination revealed no significant abnormality. Consulting physician advised to do X- ray of dorso- lumbar spine (both views), Ultrasonography (USG) of the swelling and FNAC from the swelling. X- ray report concluded as spinal dysraphism with widening of spinal canal. Ultrasonography showed dural ectasia. No malignant or granulomatous cells were found in FNAC. For confirmation of plain x- ray and USG findings, the patient’s parent was advised to do MRI of dorsolumbar spine in Radiology and Imaging Department of BIRDEM hospital. MRI findings were compatible with Diastematomyelia with tethered cord as evidenced by two unequal hemicord separated by signal void cleft and low insertion of spinal cord at L4-L5 level. As MRI is less sensitive regarding bone , CT scan was done and CT revealed grossly abnormal vertebral bodies with an osseous spur traversing the entire canal dividing the canal into two halves. Spinal cord was also divided into two unequal cord. Considering X-ray, USG, CT and MRI, final Diagnosis was made as Type I Diastematomyelia with Tethered cord.Birdem Med J 2015; 5(1): 42-45

scholarly journals Type I split cord malformation and tethered cord syndrome in an adult patient: A case report and literature review

2019 ◽  
Vol 10 ◽  
pp. 90
Author(s):  
Erin N. D’Agostino ◽  
Daniel R. Calnan ◽  
Vyacheslav I. Makler ◽  
Imad Khan ◽  
John H. Kanter ◽  
...  
Keyword(s):  
Conservative Therapy ◽  
Tethered Cord ◽  
Tethered Cord Syndrome ◽  
Leg Pain ◽  
Split Cord Malformation ◽  
Type I ◽  
Review Of Literature ◽  
History Of ◽  
Magnetic Resonance Imaging Mri

Background: In a split cord malformation (SCM), the spinal cord is divided longitudinally into two distinct hemicords that later rejoin. This can result in a tethered cord syndrome (TCS). Rarely, TCS secondary to SCM presents in adulthood. Here, we present an adult female with Type I SCM resulting in TCS and a review of literature. Case Description: A 57-year-old female with a history of spina bifida occulta presented with a 2-year history of worsening back and left leg pain, difficulty with ambulation, and intermittent urinary incontinence; she had not responded to conservative therapy. Magnetic resonance imaging (MRI) revealed a tethered cord secondary to lumbar type I SCM. The patient underwent an L1–S1 laminectomy for resection of the bony septum with cord detethering. At 2-month follow-up, the patient had improvement in her motor symptoms and less pain. In literature, 25 cases of adult-onset surgically managed SCM with TCS were identified (between 1936 and 2018). Patients averaged 37 years of age at the time of diagnosis, and 56% were female. Conclusion: TCS can present secondary to SCM in adulthood and is characterized predominantly by back and leg pain.

scholarly journals A rare case of thoracic myelocystocele associated with type 1 split cord malformation with low lying tethered cord, dorsal syrinx and sacral agenesis: Pentad finding

2015 ◽  
Vol 6 (01) ◽  
pp. 087-090 ◽  
Author(s):  
Dipanker Singh Mankotia ◽  
Guru Dutta Satyarthee ◽  
Bhawani Shankar Sharma
Keyword(s):  
Spina Bifida ◽  
Surgical Management ◽  
Rare Case ◽  
Clinical Presentation ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Split Cord Malformation ◽  
Rare Form ◽  
Sacral Agenesis

ABSTRACTMyelocystocele is a rare form of spinal dysraphism. Thoracic myelocystocele is still rarer. The occurrence of thoracic myelocystocele associated with type-1 split cord malformation, low lying tethered cord, dorsal syrinx and spina bifida is extremely rare. Clinical presentation of such a rare case and an early surgical management is discussed briefly.

Is It Better to Resect a Bony Spur Before Corrective Surgery for Congenital Scoliosis with Type I Split Cord Malformation?

2019 ◽  
Vol 125 ◽  
pp. e1151-e1159 ◽  
Author(s):  
Ningning Yang ◽  
Ming Luo ◽  
Yi Yu ◽  
Jie Wang ◽  
Lei Xia ◽  
...  
Keyword(s):  
Congenital Scoliosis ◽  
Split Cord Malformation ◽  
Type I ◽  
Corrective Surgery ◽  
Bony Spur

scholarly journals Contiguous triple spinal dysraphism associated with Chiari malformation Type II and hydrocephalus: an embryological conundrum between the unified theory of Pang and the unified theory of McLone

2016 ◽  
Vol 17 (1) ◽  
pp. 103-106 ◽  
Author(s):  
Sivashanmugam Dhandapani ◽  
Anirudh Srinivasan
Keyword(s):  
Neural Tube ◽  
Neural Tube Defects ◽  
Chiari Malformation ◽  
Spinal Dysraphism ◽  
Split Cord Malformation ◽  
Type I ◽  
Unified Theory ◽  
Type Ii ◽  
Small Posterior Fossa ◽  
Closure Theory

Triple spinal dysraphism is extremely rare. There are published reports of multiple discrete neural tube defects with intervening normal segments that are explained by the multisite closure theory of primary neurulation, having an association with Chiari malformation Type II consistent with the unified theory of McLone. The authors report on a 1-year-old child with contiguous myelomeningocele and lipomyelomeningocele centered on Type I split cord malformation with Chiari malformation Type II and hydrocephalus. This composite anomaly is probably due to select abnormalities of the neurenteric canal during gastrulation, with a contiguous cascading impact on both dysjunction of the neural tube and closure of the neuropore, resulting in a small posterior fossa, probably bringing the unified theory of McLone closer to the unified theory of Pang.

scholarly journals Spinal dysraphism controversies: AIIMS experiences and contribution

2012 ◽  
Vol 01 (01) ◽  
pp. 004-008 ◽  
Author(s):  
A.K. Mahapatra
Keyword(s):  
Surgical Approach ◽  
Spinal Dysraphism ◽  
Large Series ◽  
Split Cord Malformation ◽  
Type I ◽  
Type Ii ◽  
Composite Type

Abstract A large series of split cord malformation (SCM). Over the last 22 years, we have operated more than 1500 patients of SD, of which over 450 are (SCM), and 300 are with various lipomatus malformations. About 55% type II and 45% type I SCM. A separate sub-classification of type I SCM (a,b,c and d), is presented which alter the surgical approach and influence the results. Overall improvement following surgery in patients with SCM was observed in 94%. Fifty percent patients improved and 44% remained stable. However, deterioration was noticed in 6%, mostly with composite type of SCM. A paradigm for management of SCM is provided taking into consideration also the author’s large experience.

scholarly journals Extraspinal Type I Dural Arteriovenous Fistula with a Lumbosacral Lipomyelomeningocele: A Case Report and Review of the Literature

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Khaled M. Krisht ◽  
Michael Karsy ◽  
Wilson Z. Ray ◽  
Andrew T. Dailey
Keyword(s):  
Arteriovenous Fistula ◽  
Dural Arteriovenous Fistula ◽  
Vascular Malformations ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Endovascular Embolization ◽  
Rapid Decline ◽  
Type I ◽  
Av Fistula ◽  
Spinal Vascular Malformations

Seven cases of adult spinal vascular malformations presenting in conjunction with spinal dysraphism have been reported in the literature. Two of these involved male patients with a combined dural arteriovenous fistula (DAVF) and lipomyelomeningocele. The authors present the third case of a patient with an extraspinal DAVF and associated lipomyelomeningocele in a lumbosacral location. A 58-year-old woman with rapid decline in bilateral motor function 10 years after a prior L4-5 laminectomy and cord detethering for diagnosed tethered cord underwent magnetic resonance imaging showing evidence of persistent cord tethering and a lipomyelomeningocele. Diagnostic spinal angiogram showed a DAVF with arterial feeders from bilateral sacral and the right internal iliac arteries. The patient underwent Onyx embolization of both feeding right and left lateral sacral arteries. At 6-month follow-up, MRI revealed decreased flow voids and new collateralized supply to the DAVF. The patient underwent successful lipomyelomeningocele exploration, resection, AV fistula ligation, and cord detethering. This report discusses management of this patient as well as the importance of endovascular embolization followed by microsurgery for the treatment of cases with combined vascular and dysraphic anomalies.

Prioritizing neurosurgical education for pediatricians: results of a survey of pediatric neurosurgeons

2009 ◽  
Vol 4 (4) ◽  
pp. 309-316 ◽  
Author(s):  
Philipp R. Aldana ◽  
Paul Steinbok
Keyword(s):  
Head Injury ◽  
American Association ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Spastic Cerebral Palsy ◽  
Type I ◽  
Occult Spinal Dysraphism ◽  
Neurosurgical Patients ◽  
Joint Section ◽  
Neurological Surgery

Object Pediatricians play a vital role in the diagnosis and initial treatment of children with pediatric neurosurgical disease. Exposure of pediatrics residents to neurosurgical diseases during training is inconsistent and is usually quite limited. After residency, opportunities for pediatricians' education on neurosurgical topics are few and fall mainly on pediatric neurosurgeons. The American Association of Neurological Surgery/Congress of Neurological Surgeons Joint Section on Pediatric Neurological Surgery Committee on Education undertook a survey of practicing pediatric neurosurgeons to determine whether focused education of practicing pediatricians might lead to better patient outcomes for children with a sampling of common pediatric neurosurgical conditions. Methods An Internet-based 40-item survey was administered to practicing pediatric neurosurgeons from the US and Canada identified from the roster of the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Section of Pediatric Neurological Surgery. Survey topics included craniosynostosis and plagiocephaly, occult spinal dysraphism and tethered cord, hydrocephalus and endoscopic third ventriculostomy, Chiari malformation Type I, mild or minor head injury, spastic cerebral palsy, and brain tumors. Most questions pertained to diagnosis, initial medical treatment, and referral. Results One hundred three (38%) of the 273 practicing pediatric neurosurgeons completed the survey. Two-thirds of the respondents had completed a pediatric neurosurgery fellowship, and two-thirds were in academic practice. Eighty-two percent of the respondents agreed that the care of pediatric neurosurgical patients could be improved with further education of pediatricians. In the opinion of the respondents, the 3 disease topics in greatest need of educational effort were craniosynostosis and plagiocephaly, occult spinal dysraphism and tethered cord, and hydrocephalus. Head injury and spasticity were given the lowest priorities. Conclusions This survey identified what practicing pediatric neurosurgeons perceive to be the most important knowledge deficits of their colleagues in pediatrics. These perceptions may not necessarily be congruent with the perceptions of practicing pediatricians themselves; nevertheless, the data from this survey may serve to inform conversations between neurosurgeons and planners of continuing medical education for pediatricians, pediatrics residency program directors, and medical school pediatrics faculty.

scholarly journals Syrinx location and size according to etiology: identification of Chiari-associated syrinx

2015 ◽  
Vol 16 (1) ◽  
pp. 21-29 ◽  
Author(s):  
Jennifer Strahle ◽  
Karin M. Muraszko ◽  
Hugh J. L. Garton ◽  
Brandon W. Smith ◽  
Jordan Starr ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Spine ◽  
Chiari Malformation ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Spinal Tumors ◽  
Cerebellar Tonsil ◽  
Type I ◽  
Chiari Malformation Type I ◽  
The Brain

OBJECT Syrinx size and location within the spinal cord may differ based on etiology or associated conditions of the brain and spine. These differences have not been clearly defined. METHODS All patients with a syrinx were identified from 14,118 patients undergoing brain or cervical spine imaging at a single institution over an 11-year interval. Syrinx width, length, and location in the spinal cord were recorded. Patients were grouped according to associated brain and spine conditions including Chiari malformation Type I (CM-I), secondary CM (2°CM), Chiari malformation Type 0 (CM-0), tethered cord, other closed dysraphism, and spinal tumors. Syringes not associated with any known brain or spinal cord condition were considered idiopathic. Syrinx characteristics were compared between groups. RESULTS A total of 271 patients with a syrinx were identified. The most common associated condition was CM-I (occurring in 117 patients [43.2%]), followed by spinal dysraphism (20 [7.4%]), tumor (15 [5.5%]), and tethered cord (13 [4.8%]). Eighty-three patients (30.6%) did not have any associated condition of the brain or spinal cord and their syringes were considered idiopathic. Syringes in patients with CM-I were wide (7.8 ± 3.9 mm) compared with idiopathic syringes (3.9 ± 1.0, p < 0.0001) and those associated with tethered cord (4.2 ± 0.9, p < 0.01). When considering CM-I–associated and idiopathic syringes, the authors found that CM-I–associated syringes were more likely to have their cranial extent in the cervical spine (88%), compared with idiopathic syringes (43%; p < 0.0001). The combination of syrinx width greater than 5 mm and cranial extent in the cervical spine had 99% specificity (95% CI 0.92–0.99) for CM-I–associated syrinx. CONCLUSIONS Syrinx morphology differs according to syrinx etiology. The combination of width greater than 5 mm and cranial extent in the cervical spine is highly specific for CM-I–associated syringes. This may have relevance when determining the clinical significance of syringes in patients with low cerebellar tonsil position.

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