scholarly journals Surgical Management of Spinal Dysraphism: Five -year Experience in a Central Hospital

2014 ◽  
Vol 34 (1) ◽  
pp. 34-38
Author(s):  
N Banskota ◽  
R Jha ◽  
N Khadka ◽  
GR Sharma ◽  
P Bista ◽  
...  
Keyword(s):  
Surgical Management ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Clinical Presentation ◽  
Spinal Dysraphism ◽  
Cell Mass ◽  
Sensory Loss ◽  
Fetal Life ◽  
Common Location ◽  
Lower Limb Paralysis

Introduction: Spinal dysraphism is a heterogeneous group of congenital spinal anomalies resulting from defective closure of the neural tube early in fetal life and anomalous development of the caudal cell mass. Meningomyelocele is common among Neural tube defects. Patients with myelomeningocele present with a spectrum of impairments, including primary functional deficits like are lower limb paralysis, sensory loss, bladderbowel dysfunction and cognitive dysfunction. Medical, surgical management and rehabilitation have helped patients with neural tube defects to participate and be productive in mainstream society. The aims of this study were to review the clinical presentation, surgical management and their outcome in the patient with spinal dysraphism. Materials and Methods: This is a retrospective study of Forty-one cases of spinal dysraphism managed during a period of five years from January 2008 to December 2012 in Department of Neurosurgery, National Academy of Medical Sciences (NAMS), Bir Hospital. Demographic profiles, clinical presentation of patients with spinal dysraphism, associated hydocephalus, surgical management and outcome were studied. Results: Out of total 41 cases studied, male patients outnumbered female with 58.5% to 41.5%. Age ranged from 5 days to 29 years and mean age was 2.71 years whereas. Lump (97%) and paraparesis (88%) were frequent mode of presentation. Lumbar lesion (65%) was commonest followed by lumbosacral (29%). Hydrocephalus was present in 51% of cases and in 24% cases developed hydrocephalus later after repair. Total 75.6% of cases were treated with VP shunt. Conclusion: Spinal dysraphism is debilitating entity and management is challenging. Lump on back and weakness of limb are major factor for children and their parents seek medical service. Lesion in low back (lumbar and lumbosacral) were most common location. Besides repair, majority of them needed CSF diversion surgery for hydocephalus. Aim of surgical management was to prevent further deterioration, control of hydrocephalus or leak. DOI: http://dx.doi.org/10.3126/jnps.v34i1.9378 J Nepal Paediatr Soc 2014;34(1):34-38

Spina Bifida and Related Conditions

2017 ◽  
Author(s):  
Stephen L. Kinsman
Keyword(s):  
Spinal Cord ◽  
Spina Bifida ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Spinal Dysraphism ◽  
Gene Interactions ◽  
Tethered Spinal Cord ◽  
Spinal Lesion ◽  
Gene Environment ◽  
Spina Bifida Aperta

The term “spinal dysraphism” encompasses the broadest array of the conditions known as the neural tube defects. The open neural tube defects (spina bifida aperta and cystica) include both disorders of primary and/or secondary neuralation and are best defined as myelomeningocele complex (MMC) due to their protean nervous system manifestations beyond the spinal lesion. Closed spinal dysraphisms (so-called spina bifida occulta) include lipomatous lesions, forms of tethered spinal cord, sinus tracts, and forms of split spinal cord (diastematomyelia). Both genetic and environmental etiologies have been identified. Gene-environment and gene-gene interactions are also important in the pathobiology of these conditions.

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.

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.

Neural Tube Defects

2018 ◽  
Author(s):  
Jimmy Hoang ◽  
Samuel David Yanofsky
Keyword(s):  
Spina Bifida ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Congenital Abnormalities ◽  
Spinal Dysraphism ◽  
Neural Tube Closure ◽  
Increased Risk ◽  
Spina Bifida Cystica ◽  
The Brain ◽  
Is Failure

Neural tube defects (NTDs) are congenital abnormalities that arise from the neural tube failing to close. These defects can affect the brain, spine, or spinal cord and generally happen within the first month of pregnancy. Cranial dysraphism is failure of cranial neural tube closure and includes anencephaly and encephalocele. Spinal dysraphism is failure of caudal neuropore closure and includes spina bifida cystica and occulta. Myelomeningocele is a type of spina bifida cystica where the membranous sac containing neural tissue protrudes through an opening in the back. It is the most common NTD and considered a surgical emergency due to the increased risk for infection, further neurologic damage, and dehydration. Advancements in medicine have allowed for the possibility of early diagnosis and even in utero surgical intervention. This chapter focuses more exclusively on the approach and management of myelomeningocele.

scholarly journals To Document The Clinical Presentation and Outcome of Open Neural Tube Defect at Teaching Hospital, Dera Ghazi Khan

2020 ◽  
Vol 24 (1) ◽  
Author(s):  
IQBAL AHMAD ◽  
MALIK LIAQAT ALI JALAL ◽  
TEHMINA NAWAZ ◽  
SAMIA SAEED
Keyword(s):  
Folic Acid ◽  
Prenatal Diagnosis ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Clinical Presentation ◽  
Csf Leak ◽  
Ultra Sound ◽  
X Rays ◽  
Increased Risk ◽  
Made In

Objectives: To study clinical presentation and to determine outcome of open Neural Tube Defects at ourrespected institution. We took a review of all cases of open neural tube defects seen at the Neurosurgery Unit of hospital in the last years to document their medical patterns, assess their neonatal outcome.Materials and Methods: This retrospective study included 74 patients, admitted to the Department ofNeurosurgery, D. G. Khan Medical College and Hospital, Dera Ghazi Khan. The procedure for history takingand clinical examination was completed to measure Folic Acid Intake in first trimester, ultra sound abdomen,pre-natal diagnosis, hydrocephalous, CSF leak from swelling, sphincter involvement, weakness of lower limb,previous baby affected, type & location of open neural tube defects along the cranio-vertebral axis. Spine X-rays,neurosonograms, MRIs were carried out to find associated irregularities and complications if exist.Results: Out of 74 children born with open neural tube defects, 9 (12%) mothers received antenatal care only, 7(9%) get folic acid regularly. Prenatal diagnosis was made in 11 (14.8%) mothers whose antenatal abdominalultrasound was done. The most common type of myelomeningocele was lumbosacral 58 (79%). Forty-four(59.4%) babies have hydrocephalous while five babies were microcephaly. Increased risk of rupturedmyelomeningocele was linked with vaginal delivery. Sphincter disturbance and limb paralysis was present in 35(47%) and 32 (43.7%) of babies respectively. The mortality was in 12% patients.Conclusion: We suggest that efforts should be made in this respect to prenatal diagnosis of such lesions to getbetter neonatal outcome

scholarly journals Infl uence of selenium deficiency on neural tube defects

2014 ◽  
Vol 6 (2) ◽  
pp. 62-65
Author(s):  
Rajeev Vats
Keyword(s):  
Blood Serum ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Venous Blood ◽  
Cell Mass ◽  
Selenium Deficiency ◽  
Prenatal Development ◽  
Maternal Blood ◽  
Selenium Status ◽  
Medical Sciences

Objective: As for the role of selenium on human fetal development, a little data is available in literature.  The purpose of this manuscript was to study the influence of selenium on neural tube defects. This study will be helpful in planning strategies for prevention of neural   tube defects.Methodology: After collection of the venous blood the same was immediately centrifuged and after immediate centrifugation, the clear serum was transferred to deionized plastic vials, stored and frozen at –20º C until determination of the analysis was carried out. The selenium levels as ng/ml were determined on GBC 932 spectrophotometer by fluorometery.Results: The mean maternal blood serum and cell mass concentrations in NTD group (306.4 ± 10.95 ng/ml, 192.44 ± 6.12 ng/ml, 165.8 ± 16.99 ng/g respectively) were significantly lower than those of control mothers (363.75 ± 17.1 ng/ml, 242.34 ± 15.7 ng/ml, 260.0 ± 20.57 respectively).  A significant decrease in concentration of selenium in newborns with NTD (298.4 ± 12.3 ng/ml, 96.3 ± 7.15 ng/ml, 139.8 ± 27.5 ng/g respectively) as compared with healthy babies (358.1 ± 16.11 ng/ml, 122.44 ± 6.03 ng/ml, 268.6 ± 31.37 ng/g respectively).Conclusion: Selenium deficiency in mothers during pregnancy thought to be one of the factors responsible for NTDs.  However, the lowered selenium concentrations in blood, serum and cell mass can be secondary cause of an abnormal pregnancy and didn’t contribute to its production.  More investigations on selenium status in mothers during antenatal period, especially in prenatal development and antenatal selenium status including normal babies and NTD babies are required.DOI: http://dx.doi.org/10.3126/ajms.v6i2.11151Asian Journal of Medical Sciences Vol.6(2) 2015 62-65    

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