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Spinal dysraphism

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https://www.readbyqxmd.com/read/28417039/a-true-human-tail-in-a-neonate-case-report-and-literature-review
#1
Mahesh K Pillai, Smitha T Nair
A true human tail is a benign vestigial caudal cutaneous structure composed of adipose, connective tissue, muscle, vessels, nerves and mechanoreceptors. A true human tail can be distinguished from a pseudotail as the latter is commonly associated with underlying spinal dysraphism, which requires specialised management. True human tails are very rare, with fewer than 40 cases reported to date. We report a healthy one-day-old male newborn who was referred to the Bharath Hospital, Kottayam, Kerala, India, in 2014 with a cutaneous appendage arising from the lumbosacral region...
February 2017: Sultan Qaboos University Medical Journal
https://www.readbyqxmd.com/read/28403928/-prenatal-diagnosis-of-neural-tube-defects-correlation-between-prenatal-and-postnatal-data
#2
L Matuszewski, E Perdriolle-Galet, I Clerc-Urmès, P Bach-Segura, O Klein, J P Masutti, O Morel
OBJECTIVES: Neural tube defects (NTD) are congenital anomalies that can cause significant neurological long-term disabilities. Theses malformations are accessible to prenatal diagnosis and quite recently, to in utero repair for some myelomeningoceles. The aim of this study was to analyse the correlation between prenatal and postnatal examinations. MATERIALS AND METHODS: A descriptive retrospective monocentric study has been conducted between January 2004 and December 2014 in a tertiary care maternity...
March 2017: J Gynecol Obstet Hum Reprod
https://www.readbyqxmd.com/read/28387433/neurosurgical-management-and-pathology-of-lumbosacral-lipomas-with-tethered-cord
#3
REVIEW
Takato Morioka, Nobuya Murakami, Takafumi Shimogawa, Nobutaka Mukae, Kimiakai Hashiguchi, Satoshi O Suzuki, Koji Iihara
Lumbosacral lipomas are the most common form of occult spinal dysraphism. The development of lumbosacral lipomas is from the premature disjunction of the neural tube from the surrounding ectoderm, leaving the neural plate open posteriorly and allowing for the infiltration of mesodermal tissue, including fatty tissue. Since lumbosacral lipomas are a common cause of spinal cord tethering that can lead to progressive neurological deficits, prophylactic neurosurgery for lumbosacral lipomas, including untethering of the spinal cord, is recommended...
April 7, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28366522/diagnostic-yield-of-lumbosacral-magnetic-resonance-imaging-requested-by-paediatric-urology-consultations
#4
M Fernández-Ibieta, J Rojas Ticona, V Villamil, M J Guirao Piñera, A López García, G Zambudio Carmona
OBJECTIVES: In the historical series, the diagnostic yield of lumbosacral magnetic resonance imaging to rule out occult spinal dysraphism (or occult myelodysplasia), requested by paediatric urology, ranged from 2% to 15%. The aim of this study was to define our cost-effectiveness in children with urinary symptoms and to define endpoints that increase the possibility of finding occult spinal dysraphism. PATIENTS AND METHODS: A screening was conducted on patients with urinary dysfunction for whom an magnetic resonance imaging was requested by the paediatric urology clinic, for persistent symptoms after treatment, voiding dysfunction or other clinical or urodynamic findings...
March 30, 2017: Actas Urologicas Españolas
https://www.readbyqxmd.com/read/28347415/congenital-and-hereditary-diseases-of-the-spinal-cord
#5
Lily L Wang, Karin S Bierbrauer
Congenital anomalies of the spinal cord can pose a diagnostic dilemma to the radiologist. Several classification systems of these anomalies exist. Antenatal ultrasound and fetal magnetic resonance imaging is playing an increasingly important role in the early diagnosis and management of patients. Understanding the underlying anatomy as well as embryology of these disorders can be valuable in correctly identifying the type of spinal cord dysraphic defect. Hereditary spinal cord diseases are rare but can be devastating...
April 2017: Seminars in Ultrasound, CT, and MR
https://www.readbyqxmd.com/read/28291421/modification-of-surgical-procedure-for-probable-limited-dorsal-myeloschisis
#6
Ji Yeoun Lee, Sangjoon Chong, Young Hun Choi, Ji Hoon Phi, Jung-Eun Cheon, Seung-Ki Kim, Sung Hye Park, In-One Kim, Kyu-Chang Wang
OBJECTIVE Since the entity limited dorsal myeloschisis (LDM) was proposed, numerous confusing clinical cases have been renamed according to the embryopathogenesis. However, clinical application of this label appears to require some clarification with regard to pathology. There have been cases in which all criteria for the diagnosis of LDM were met except for the presence of a neural component in the stalk, an entity the authors call "probable" LDM. The present study was performed to meticulously review these cases and suggest that a modified surgical strategy using limited laminectomy is sufficient to achieve the surgical goal of untethering...
February 17, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28291403/a-rat-model-of-chronic-syringomyelia-induced-by-epidural-compression-of-the-lumbar-spinal-cord
#7
Ji Yeoun Lee, Shin Won Kim, Saet Pyoul Kim, Hyeonjin Kim, Jung-Eun Cheon, Seung-Ki Kim, Sun Ha Paek, Dachling Pang, Kyu-Chang Wang
OBJECTIVE There has been no established animal model of syringomyelia associated with lumbosacral spinal lipoma. The research on the pathophysiology of syringomyelia has been focused on Chiari malformation, trauma, and inflammation. To understand the pathophysiology of syringomyelia associated with occult spinal dysraphism, a novel animal model of syringomyelia induced by chronic mechanical compression of the lumbar spinal cord was created. METHODS The model was made by epidural injection of highly concentrated paste-like kaolin solution through windows created by partial laminectomy of L-1 and L-5 vertebrae...
February 17, 2017: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/28279990/hindbrain-herniation-in-chiari-ii-malformation-on-fetal-and-postnatal-mri
#8
U D Nagaraj, K S Bierbrauer, B Zhang, J L Peiro, B M Kline-Fath
BACKGROUND AND PURPOSE: As the practice of in utero repair of myelomeningoceles becomes more prevalent, knowledge of the expected MR imaging findings has become increasingly important. Our aim was to examine neuroimaging findings with a focus on hindbrain herniation and ventricular size in fetuses with open spinal dysraphism and to compare them with postnatal imaging features in groups undergoing prenatal-versus-postnatal repair. MATERIALS AND METHODS: Single-center retrospective analysis was performed on MRIs of fetuses with open spinal dysraphism from January 2004 through July 2015 with available postnatal imaging...
March 9, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28247109/junctional-neural-tube-defect-in-a-newborn-report-of-a-fourth-case
#9
Cameron Schmidt, Vlad Voin, Joe Iwanaga, Fernando Alonso, Rod J Oskouian, Nitsa Topale, R Shane Tubbs, W Jerry Oakes
INTRODUCTION: A discontinuous, functionally disconnected spinal cord is an extremely rare finding, with only three known reports in the literature. Titled junctional neural tube defect (JNTD), this newly reported dysraphism is believed to arise from a developmental error occurring during junctional neurulation, a transitory stage of development marked by the end of primary neurulation and the beginning of secondary neurulation. Herein, we report a newborn case of JNTD. CASE REPORT: We report a newborn boy born with anorectal atresia...
February 28, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28214395/ossified-dysraphic-hamartoma-with-lipomyelocoele-lipomyelomeningocoele-a-rare-radiological-entity
#10
Sudha Bindu Tirumani, Raghavendra Prasad, Vijaya Kumari Mudunoor, Suman Chandra Aemjal, Harika Tirumani, Saurabh Rohatgi
Traditionally the presence of fat in closed spinal dysraphism has been referred to as spinal lipoma. Recent reports suggest that these spinal lesions are better described as spinal hamartomas due to the unencapsulated and infiltrating nature of the fat and presence of other heterotopic soft tissue. The presence of ossified bone in spinal hamartomas referred to as ossified dysraphic hamartoma, is extremely rare with only three case reports in literature, all associated with lipomyeloceles, none with lipomyelomeningoceles...
January 31, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28128702/new-classification-of-spinal-lipomas-based-on-embryonic-stage
#11
Nobuhito Morota, Satoshi Ihara, Hideki Ogiwara
OBJECTIVE Spinal lipomas are generally thought to occur as a result of failed primary neurulation. However, some clinical features cannot be explained by this theory. The authors propose a novel classification of spinal lipomas based on embryonic changes seen during primary and secondary neurulation. METHODS A total of 677 patients with occult spinal dysraphism underwent 699 surgeries between August 2002 and May 2015 at the National Center for Child Health and Development and Tokyo Metropolitan Children's Medical Center...
April 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28120066/myelomeningocele-repair-surgical-management-based-on-a-30-year-experience
#12
Pier Paolo Mattogno, Luca Massimi, Giampiero Tamburrini, Paolo Frassanito, Concezio Di Rocco, Massimo Caldarelli
BACKGROUND: Myelomeningocele (MMC) is a rare but severe spinal defect resulting from a failed neurulation process. Surgical repair is a delicate procedure that needs accurate neuroanatomical knowledge and adequate surgical planning. MATERIALS AND METHODS: The authors report on the main problems of the surgical management of this type of dysraphism. The technique used for reconstruction, and the management strategies, are analyzed according to the authors' experience with more than 600 cases, in comparison with the relevant literature...
2017: Acta Neurochirurgica. Supplement
https://www.readbyqxmd.com/read/28018060/isolated-dorsal-column-dysfunction-due-to-an-intraspinal-osteolipoma-case-report-and-review-of-literature
#13
Siddharth N Aiyer, Ajoy Prasad Shetty, Rishi Kanna, Anupama Maheswaran, S Rajasekaran
Osteolipoma is a rare variant of the ubiquitous lipoma. Published literature appears in the form of isolated case reports affecting soft tissue structures in the head, neck and rarely affecting the spine. We present a unique instance of an intraspinal osteolipoma in the cervical spine, without evidence of accompanying spinal dysraphism and an atypical clinical presentation of isolated dorsal column dysfunction. We describe the clinical presentation, operative procedure and post-operative outcomes with histopathological findings of this rare entity...
October 2016: Journal of Clinical Orthopaedics and Trauma
https://www.readbyqxmd.com/read/27980290/segmental-spinal-dysgenesis-with-caudal-agenesis-in-a-holstein-calf
#14
Kaori Tokudome, Shuichi Ito, Tomoko Kato, Tohru Yanase, Tatsuo Kawarasaki, Yasuo Moritomo
A rare complex dysraphic malformation, comprising segmental spinal dysgenesis with caudal agenesis, was found in a Holstein calf that was unable to stand and was slightly short at the lumbosacral spine with taillessness. The thoracolumbar and sacrococcygeal regions of the midline axial segments showed severe deformities. In the spinal cord, the thoracolumbar region showed severe constriction with myelodysplastic changes, and the sacrococcygeal region showed dorsoventral separation with connection to a neural mass...
February 14, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/27911245/use-of-magnetic-resonance-imaging-to-detect-occult-spinal-dysraphism-in-infants
#15
Brent R O'Neill, Danielle Gallegos, Alex Herron, Claire Palmer, Nicholas V Stence, Todd C Hankinson, C Corbett Wilkinson, Michael H Handler
OBJECTIVE Cutaneous stigmata or congenital anomalies often prompt screening for occult spinal dysraphism (OSD) in asymptomatic infants. While a number of studies have examined the results of ultrasonography (US) screening, less is known about the findings when MRI is used as the primary imaging modality. The object of this study was to assess the results of MRI screening for OSD in infants. METHODS The authors undertook a retrospective review of all infants who had undergone MRI of the lumbar spine to screen for OSD over a 6-year period (September 2006-September 2012)...
February 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/27884878/comparison-of-3-different-types-of-spinal-arteriovenous-shunts-below-the-conus-in-clinical-presentation-radiologic-findings-and-outcomes
#16
T Hong, J E Park, F Ling, K G terBrugge, M Tymianski, H Q Zhang, T Krings
BACKGROUND AND PURPOSE: Spinal arteriovenous shunts below the conus constitute 3 types of lesions, which have previously been mainly described in case reports, given their rarity, and are sometimes misdiagnosed. The purpose of this study was to describe the features of each type and compare these types as to epidemiologic features, clinical and radiologic presentations, treatment, and outcomes in a consecutive series of 48 cases. MATERIALS AND METHODS: The prospectively collected data bases of 2 referral centers for spinal vascular lesions were retrospectively reviewed...
November 24, 2016: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/27857788/ultrasonography-and-magnetic-resonance-imaging-evaluation-of-pediatric-spinal-anomalies
#17
Dhaval Durlabhbhai Dhingani, Deb Kumar Boruah, Hemonta Kumar Dutta, Rudra Kanta Gogoi
CONTEXT: Spinal dysraphisms are congenital abnormalities of the spine due to imperfect fusion of midline mesenchymal, bony and neural structures. Imaging plays a vital role in their evaluation as significant portion of patients may present with concurrent anomalies that need to be corrected simultaneously to avoid repeat surgeries. AIMS: The aims of the study were to evaluate Spinal dysraphisms using USG and MRI and to correlate imaging findings with operative findings in patients undergoing surgery...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27818449/association-of-obesity-bmi-and-hispanic-ethnicity-on-ambulatory-status-in-children-with-spinal-dysraphism-followed-near-the-california-mexico-border
#18
Michelle L McDonald, Andy Huang, James A Proudfoot, Joan T Le, George J Chiang, Ruth A Bush
PURPOSE: Evaluate the relationship between body mass index (BMI), overweight status (OW), or obesity (OB) and ambulatory status in a predominantly Hispanic population of children with spinal dysraphism (SD). METHODS: Retrospective data were extracted from records of 272 children and youth aged 0-24 years with a diagnosis of SD. Body mass index (BMI) and OW / OB rates were calculated for children 0-3 years, 4-11 years, and adolescents older than 11. RESULTS: Ethnicity was predominantly Hispanic (65...
2016: Journal of Health Care for the Poor and Underserved
https://www.readbyqxmd.com/read/27796548/unjoined-primary-and-secondary-neural-tubes-junctional-neural-tube-defect-a-new-form-of-spinal-dysraphism-caused-by-disturbance-of-junctional-neurulation
#19
Sebastian Eibach, Greg Moes, Yong Jin Hou, John Zovickian, Dachling Pang
INTRODUCTION: Primary and secondary neurulation are the two known processes that form the central neuraxis of vertebrates. Human phenotypes of neural tube defects (NTDs) mostly fall into two corresponding categories consistent with the two types of developmental sequence: primary NTD features an open skin defect, an exposed, unclosed neural plate (hence an open neural tube defect, or ONTD), and an unformed or poorly formed secondary neural tube, and secondary NTD with no skin abnormality (hence a closed NTD) and a malformed conus caudal to a well-developed primary neural tube...
October 29, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27790529/successful-pregnancy-outcome-in-an-operated-case-of-lipomeningomyocele-a-rare-case
#20
Vineet Vashistha Mishra, Sakshi Nanda, Rohina Aggarwal, Tanvir
Lipomeningomyocele is one of the types of occult spinal dysraphism associated with tethered cord syndrome, which is characterised by neurodeficit symptomatology due to stretch- induced functional disorder of the spinal cord with its caudal part attached by an in elastic tissue. There is stretching of conus medullaris and nerve roots due to disproportionate growth between vertebral column and spinal cord, resulting in various neurological deficits. Its incidence is approximately 1 in 4000 births in the USA, with a slightly higher female preponderance, although its true incidence is not known...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
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