Presentation
A summary of the management of a 54-year-old woman with recent clinical symptomatology related to an undiagnosed split-cord malformation is presented with accompanying literature review. [ncbi.nlm.nih.gov]
They advised that if the imaging appears to show otherwise, a second spur (present in about 5% of patients with diastematomyelia) is likely to be present. [en.wikipedia.org]
Entire Body System
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Soft Tissue Mass
Characteristic findings are localised widening of the posterior ossification centres with a central echogenic focus at the point of widening, visible on coronal scanning, and absence of a posterior defect or soft tissue mass on transverse scanning. [ncbi.nlm.nih.gov]
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Localized Pain
The treatment should be dependent on local pain intensity (which is often not directly associated with the disorder) and on the degree of neurological dysfunction. 5. 1. [ncbi.nlm.nih.gov]
Skin
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Cutaneous Manifestation
Early recognition of these cutaneous manifestations by the plastic surgeon, evaluation with an MRI scan, and consultation with a pediatric neurosurgeon can prevent delayed diagnosis of this surgically correctable anomaly and irreversible neurologic sequelae [ncbi.nlm.nih.gov]
The diagnosis is dependent on presentation of orthopedic syndromes, cutaneous manifestations, neurological deficits, congenital scoliosis and spina bifida in X-ray examination, a detailed evaluation of the spinal cord by myelography, CT myelography and [ci.nii.ac.jp]
Musculoskeletal
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Leg Weakness
The authors describe a 42-year-old man who presented with pain and distal left-leg weakness as well as neurogenic claudication for 1 month. [ncbi.nlm.nih.gov]
Leg weakness Pain in lower back Scoliosis or curve in spine Bladder and bowel incontinence Associated anomalies with Diastematomyelia Most of the patients of Diastematomyelia also have following associated anomalies: Meningocele Neurenteric cyst Dermoid [chennaibrainandspine.com]
Children may develop leg weakness, lower back pain, or incontinence. Foot and spinal deformities such as scoliosis may also occur. [wisegeek.com]
Presenting symptoms include: leg weakness low back pain scoliosis incontinence Patients with diastematomyelia also frequently have other associated anomalies including: meningocele neurenteric cyst dermoid cyst clubfoot spinal cord lipoma hemangioma overlying [radiopaedia.org]
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Neurogenic Claudication
The authors describe a 42-year-old man who presented with pain and distal left-leg weakness as well as neurogenic claudication for 1 month. [ncbi.nlm.nih.gov]
Kaminker, R, Fabry, J: Split cord malformation with diastematomyelia presenting as neurogenic claudication in an adult. Spine 25: 2269 – 2271, 2000. Google Scholar 15. [journals.sagepub.com]
Psychiatrical
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Denial
For a patient with a congenital spine deformity, a diastematomyelia, a tight filum terminale, and a congenital stenosis, denial of magnetic resonance imaging evaluation and appropriate surgery cannot be justified. [ncbi.nlm.nih.gov]
Urogenital
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Dysuria
A 26-year-old primigravida at 14 weeks' gestation was presented to our outpatient department with a complaint of dysuria. [ncbi.nlm.nih.gov]
Neurologic
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Neurologic Manifestation
We believe that resection of the spur should be performed in patients who have progressive neurological manifestations. [ncbi.nlm.nih.gov]
Treatment [ edit ] Surgery Surgical intervention is warranted in patients who present with new onset neurological signs and symptoms or have a history of progressive neurological manifestations which can be related to this abnormality. [en.wikipedia.org]
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Confusion
This variant is potentially confused with arachnoiditis or thecal scarring in the lumbo-sacral sac. [ncbi.nlm.nih.gov]
The more I think about this, the more confused I get! Has anyone heard of this happening, or did the doctors just pretend to remove the spur when I was little?! [spinabifidaconnection.com]
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Dysesthesia
A 44-year old woman, presented with progressive lower limb muscle weakness, gait disturbances and dysesthesias in the trunk and lower extremity. [ncbi.nlm.nih.gov]
She also complained of dysesthesia over the lateral aspect of her right thigh. Neurologic examination revealed hyperactive reflexes of her right lower extremity and diminished response to fine touch and pressure. [ajronline.org]
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Hyperreflexia
Sensorimotor deficits, hyperreflexia, and a positive Babinski reflex in the right lower extremity were demonstrated on examination. [ncbi.nlm.nih.gov]
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Motor Symptoms
The female patient noted sensory and motor symptoms at 49 years of age, four months before hospitalization. [ncbi.nlm.nih.gov]
Workup
Her initial workup revealed a lumbosacral dermoid sinus associated with diastematomyelia and a tethered cord. Therefore, a surgical repair to correct the anomaly was performed. [ncbi.nlm.nih.gov]
Treatment
Surgical resection of the septum was one of the possibilities, but because of the limited clinical impact, a conservative treatment was followed. [ncbi.nlm.nih.gov]
Post-myelographic CT scanning provides individualized detailed maps that enable surgical treatment of cervical diastematomyelia, first performed in 1983. [1] [2] Observation Asymptomatic patients do not require surgical treatment. [en.wikipedia.org]
Prognosis
We present eight cases of diastematomyelia diagnosed prenatally in our institution as well as a review of the literature in order to determine the prognosis of isolated cases of this very unusual condition. [ncbi.nlm.nih.gov]
Etiology
[…] peripheral neurological deficit. [1] Diastematomyelia is a rare congenital spinal cord abnormality characterized by longitudinal "splitting up" of the spinal cord into two. [2] We present a unique case of chronic adhesive arachnoiditis of tubercular etiology [saudijhealthsci.org]
They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]
He also provides updated information about the etiology, evaluation, and treatment of this interesting group of disorders. [medlink.com]
The precise etiology is not known. [nature.com]
Epidemiology
Introduction A fibrous, cartilagenous, or osseous bar creating a longitudinal cleft in the spinal cord if the cord does not reunite distally to the spur, it is considered a diplomyelia (true duplication of the cord) Epidemiology demographics typically [orthobullets.com]
Neurology and Neurosurgery Czech and Slovak Ophthalmology Czech and Slovak Psychiatry Czech Geriatric Review Czech Gynaecology Czech Rheumatology Czech Dental Journal Czech Urology Czech-Slovak Dermatology Czecho-Slovak Pathology Czech-Slovak Pediatrics Epidemiology [prolekare.cz]
American Journal of Epidemiology 153: 961 – 968, 2002. Google Scholar 11. Hughes, L, McCarthy, R: Segmental spinal dysgenesis: A report of three cases. J Pediatr. Orthop 18: 227 – 232, 1998. Google Scholar 12. [journals.sagepub.com]
Epidemiological studies have demonstrated multifactorial influences underlying NTDs. Variations in incidence have been seen across geographical variation, ethnic groups, socioeconomic status, and genetic predisposition. [emedicine.medscape.com]
Pathophysiology
Pathophysiologic and clinical aspects as well as the radiological characteristics of these pathologies are dicussed and the importance of the early diagnosis is emphasized. Keywords : diastematomyelia; spinal dysraphism; spinal tumor. [scielo.br]
Pathophysiology [ edit ] Diastematomyelia is a " dysraphic state " of unknown embryonic origin, but is probably initiated by an accessory neurenteric canal (an additional embryonic spinal canal.) [en.wikipedia.org]
Various precipitating factors have been implicated in their pathophysiology including trauma, spinal canal compression, and strong stretching factors. All these factors contribute to a neurologic deficit in an already compromised neural canal. [ajronline.org]
Prevention
This technique may be useful to prevent ventral retethering in diastematomyelia. [ncbi.nlm.nih.gov]
[…] which can develop. 25 In order to prevent the serious sequelae of this disease, all physicians must be familiar with the disease entity. [jamanetwork.com]