Lumbar Diastomatomyelia with Syringomyelia: A Case Report (original) (raw)
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Terminal Syringomyelia: Is It As Innocent As It Seems?-Case Report
Neurologia medico-chirurgica, 2004
A 5-day-old newborn girl presented with cutaneous signs of hypertrichosis, skin dimple, and subcutaneous lipoma in the lumbar region. Magnetic resonance (MR) imaging of the thoracolumbar spine demonstrated spinal dermal sinus, type II split cord malformation, and tethered cord. A small terminal syringeal cavity was also present. Five months after the diagnosis, weakness in dorsal flexion of the bilateral feet and toes was detected. Surgery to untether the cord was performed. Her deficits resolved completely. Nearly 4 years later, she was admitted with weakness in dorsal flexion of the right foot and toe. MR imaging of the spine showed holocord syringomyelia, postoperative changes, and tethered cord. No Chiari malformation was seen. Syringosubarachnoid shunt placement was performed. Terminal syringomyelia is common in cases of occult spinal dysraphism, but the natural history and management remain unclear. Progression, regression, or persistence of terminal syringeal cavity can occur whether untethering is performed or not. Progression of the terminal syrinx occurred after tethered cord release and the enlargement of the cavity involved the entire spinal cord in the present case. Terminal syringomyelia requires close follow up for a long time even in asymptomatic cases. Surgical treatment should be considered in symptomatic cases.
Idiopathic syringomyelia: case report and review of the literature
Advances in Functional and Reparative Neurosurgery
Syringomyelia is an uncommon disease that is caused most often by type I Chiari malformation, which develops in the hindbrain, and less frequently by other factors which are not limited to the hindbrain, including trauma, infection, or scoliosis. Idiopathic syringomyelia is rare. We present in this article a patient with idiopathic syringomyelia characterized by hypoesthesia and progressive weakness in the left lower limb. Decompression was attempted by means of laminectomy and a syringoarachnoid shunt. Motor, sensory, and bladder functions were monitored by the change in Japanese Orthopedic Association scores, which increased from 10 points preoperatively to 14 points 30 days postoperatively. This case demonstrates the effectiveness of surgical decompression in a patient with remarkable neurological deficit.
Spinal arteriovenous malformation associated with syringomyelia
Journal of Neurosurgery: Spine, 2009
The authors describe 4 cases of syringomyelia-associated spinal cord arteriovenous malformation (AVM). All cases were managed with embolization of the AVM. Treatments were aimed to stabilize the AVM itself and not directed toward the syrinx. In 3 of the 4 cases the syringomyelia resolved after treatment. Reports concerning AVM as a cause of syringomyelia is very scarce and lacks posttreatment clinical information. In light of the clinical course and imaging findings, the authors propose a theory that venous hypertension in the spinal cord is the trigger for the development of syringomyelia, which may reverse after AVM treatment.
Familial syringomyelia: case report and review of the literature
Surgical neurology, 1995
BACKGROUND: Syringomyelia is an uncommon disease of the spinal cord, occurring sporadically. However, rare familial cases with autosomal dominant or recessive inheritance patterns are reported and their incidence quoted as approximately 2%. Only one previous ...
Fetal Diastematomyelia: Rare Case
IOSR Journal of Dental and Medical Sciences, 2012
A ten-month-old infant with Diastematomyelia, as a very rare variety of spinal dysraphism is presented in this article Routine USG in a 36 weeks pregnant woman revealed a spinal abnormality of the fetus in thoracolumbar [D12-L1] regions. At birth, examination of the newborn showed a small midline swelling and bluish discoloration of skin and scoliosis in the lumbar region. There was associated umbilical hernia.no neurological deficit was noted. Postnatally 8 month plane radiograph showed multiple deformed dorsal vertebrae and mild scoliosis.MR imaging revealed diastematomyelia was seen at thoracolumbar [D12-L1] regions. It was type I with a bony spur measuring 1.0 X0.5 cm the two hemicords were asymmetrical. There was no cord tethering. The conus ended at the L4 level.. There was associated thoracolumbar spina bifida measuring 1.6 cm with a small meningocele measuring 2.5 X 0.6 cm. multilevel syringohydromelia and multiple deformed dorsal vertebrae and mild scoliosis of dorsal spine was seen. Thus what makes this case different is inspite of having so many microanatomical defects, there is no neurologic deficit. So its advisible to do corrective surgery to avoid further complicatiions-thethered cord syndrome before the baby starts to take steps.
Journal of Neurosurgery: Spine, 2005
✓ In the present review the author describes the different types of syringomyelia that originate from abnormalities at the level of the spinal cord rather than at the craniovertebral junction. These include posttraumatic and postinflammatory syringomyelia, as well as syringomyelia associated with arachnoid cysts and spinal cord tumors. The diagnosis and the principles of managing these lesions are discussed, notably resection of the entity restricting cerebrospinal fluid flow. Placement of a shunt into the syrinx cavity is reserved for patients in whom other procedures have failed or who are not candidates for other procedures.
Split Cord Syndrome (Type 1 Diastematomyelia): A Case Report
Annals of International Medical and Dental Research, 2016
Spinal dysraphism can present in various ways in the form of severity. Split cord is one of the presentations where two hemi cords are separated either by a fibrous septum or calcific spur. This may be accompanied with different other vertebral anomalies like block vertebra, hemi vertebra or spina bifida. We present a 8-years old girl who presented with weakness and gait disturbances since she started walking. She also had a tuft of hair on the back in the lumbar region. She underwent computerized tomography (CT) and magnetic resonance imaging (MRI) and was diagnosed as diastematomyelia with other associated anomalies.
Prenatal and postnatal evaluation for syringomyelia in patients with spinal dysraphism
Journal of neurosurgery. Pediatrics, 2014
Syringomyelia can be diagnosed in isolation but is more commonly found in the presence of craniocervical junction anomalies or spinal dysraphism. The origin of syringomyelia has been hypothesized to be either congenital or acquired. The purpose of this study was to determine the incidence of syringomyelia within the fetal and postnatal population with neural tube defects (NTDs). A review was performed of the authors' fetal MRI database of pregnancies with imaging between March 2004 and November 2011 for evaluation of an intrauterine anomaly detected via prenatal ultrasonography. Those cases with an NTD were then selected and a chart review was performed of all prenatal and postnatal imaging as well as available clinical history. A total of 2362 fetal MRI examinations were performed, and 109 of these were patients with an NTD. Of the 2362 studies reviewed, 2 cases of fetal syringomyelia were identified. Both fetal syrinxes were identified in fetuses with CSF flow disturbances (1 ...