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]
Entire Body System
- Developmental Disorder
This defect often co-occurs with other developmental disorders of the skull or the atlanto-occipital junction. The course may be benign or aggressive. 2. [ncbi.nlm.nih.gov]
Developmental disorders of paediatric spine. RCNA 1991; 29: 4-5. 5. Bruberg JA, Latchaw RC, Emanuel K et al. Magnetic resonance imaging in occult spinal dysraphism. RSNA 1988; 26: 181-205 6. Anand AK, Kuchner E, James R. [ijri.org]
- 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]
- Dysostosis
Myelomeningocele are frequently associated with Chiari malformation More Details [3] diastematomyelia (up to 45% in some reported series) [4] and other congenital vertebral defects such as spondylocostal dysostosis, [5] Jarcho-Levin syndrome, Klippel-Feil [pediatricneurosciences.com]
Musculoskeletal
- Lower Limb Muscle Atrophy
Only Type 1 patients presented with unilateral lower-limb muscle atrophy and malformations of the lower limb. [online.boneandjoint.org.uk]
Neurologic
- Quadriplegia
The Health Impacts The impact of diastematomyelia upon the quality of life (QoL) is almost similar, if not more, as of other chronic neurological diseases like quadriplegia and epilepsy. [ncbi.nlm.nih.gov]
- Motor Symptoms
The female patient noted sensory and motor symptoms at 49 years of age, four months before hospitalization. [ncbi.nlm.nih.gov]
- Hyperreflexia
Sensorimotor deficits, hyperreflexia, and a positive Babinski reflex in the right lower extremity were demonstrated on examination. [ncbi.nlm.nih.gov]
- Radiculitis
The thoracic cord is more frequently involved, followed by lumbar and cervical cords. [4] There are three stages of tubercular radiculomyelitis namely radiculitis, arachnoiditis and adhesive arachnoiditis. [5] Our case was in the third stage of tubercular [saudijhealthsci.org]
- Flaccid Paralysis
On neurological examination there was complete flaccid paralysis of right lower extremity with normal anal tone. On local examination, swelling was soft, cystic, and tensile with positive fluid thrill. [pediatricneurosciences.com]
Workup
Although cases of diastematomyelia have been previously reported, fully documented approaches by both prenatal and postnatal diagnostic workup are rare in the literature. [ncbi.nlm.nih.gov]
Treatment
Cold packs were applied over the treatment area and surrounding area before and after treatment to decrease pain. Sessions were repeated at 4-8 weekly intervals. [e-ijd.org]
What Are Some Common Treatments? Treatment does not aim to "reattach" the halves of the split spinal cord. However, treatment varies depending on the symptoms and severity of the malformation. [nervous-system-diseases.com]
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]
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]
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
He also provides updated information about the etiology, evaluation, and treatment of this interesting group of disorders. [medlink.com]
They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]
[…] 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]
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]