Can Spina Bifida Occulta Become Symptomatic in Adulthood
Global Spine J. 2012 Jun; ii(2): 115–118.
Symptomatic Lumbar Disc Protrusion Causing Progressive Myelopathy in a Low-Lying Cord
Shreya Srinivas
1Section of Trauma and Orthopaedics, Northern Deanery, Cumberland Infirmary, Carlisle, Cumbria, United Kingdom
Rohit Shetty
2Department of Trauma and Orthopaedics, Whittington Infirmary NHS Trust, London, United Kingdom
Iona Collins
3Department of Trauma and Orthopaedics, Abertawe Bro Morgannwg NHS Trust, Swansea, Wales, United kingdom
Received 2011 Aug 20; Accepted 2011 Dec fifteen.
Abstract
Low-lying cord is an uncommon entity, and cord compression due lumbar disc disease is rarely encountered. We discuss our experience with a case of lumbar cord pinch secondary to a big disc protrusion, which caused myelopathy in a low-lying/tethered cord. A 77-yr-erstwhile adult female with known spina bifida occulta presented with 6-week history of severe low dorsum pain and progressive paraparesis. Magnetic resonance imaging showed a low-lying tethered cord and a large disc prolapse at L2/3 causing string pinch with associated syringomyelia. Medical comorbidities precluded her from inductive decompression, and therefore a posterior decompression was performed. She recovered full motor power in her lower limbs and could eventually walk unaided. She had a deep wound infection, which was successfully treated with debridement, negative force per unit area therapy (vacuum-assisted closure pump), and antibiotics. Six months after surgery, her Oswestry Disability Index improved from 55% preoperatively to 20%. Posterior spinal string decompression for this condition has been successful in our case, and we believe that the lumbar lordosis may accept helped indirectly decompress the spinal cord by posterior decompression alone.
Keywords: low-lying cord, tethered string, decompression, spina bifida, myelopathy
Spinal cord usually ends above or at the level of inferior aspect of L2 vertebral body in 95% of cases. Low-lying cord is usually associated with diverse forms of spinal dysraphism. It presents in childhood due to longitudinal traction that results in tethering of the cord.1 Rarely, patients progress to adulthood with no neurological symptoms. In virtually of these cases, spinal degenerative conditions or precipitating factors such equally trauma exacerbate neurological symptoms, and patients can and so present with diverse forms of neurological arrears.ii
Tethered cord syndrome (TCS) is considered a clinical entity in patients in the presence of a low-lying cord, thickened filum terminale, or on occasion a lipomatous lesion seen on radiological imaging. TCS in adults is usually associated with spina bifida occulta and can crusade pregnant morbidity if non diagnosed and treated early.3
We nowadays the case of an elderly woman with known spina bifida occulta, who presented with progressive back hurting and paraparesis of relatively short duration.
She had radiological testify of lumbar disc herniation causing cord compression and a tethered cord. Nosotros discuss the possible pathophysiology of disc illness causing cord compression in the presence of a tethered cord/depression-lying cord and the role of decompressive surgery to possibly resolve these symptoms.
Case Report
A 77-year-onetime woman with known spina bifida occulta presented to the emergency section with a 6-week history of severe low back pain and progressive paraparesis. She was able to walk only two to three steps and only with ii helpers. She denied whatever bowel or bladder dysfunction.
She was previously asymptomatic and was aware of a hairy patch in the lower back since birth. Her medical comorbidities included diabetes mellitus (type II), hypertension, increased body mass alphabetize, and mitral regurgitation.
Neurological test of the lower limbs revealed proximal lower limb weakness of MRC (Medical Research Council) grade three in L2, L3 myotomes and grade 4 in L4, L5, and S1 myotomes. Sensory exam was normal. Reflexes were brisk with upgoing plantars and three beats of clonus in the correct pes.
Subsequent investigation with magnetic resonance imaging of the whole spine showed a low-lying tethered string and a large disc prolapse at L2/three causing string compression with associated syringomyelia (Figs. 1, two, and 3).
Sagittal view T1-weighted image.
Sagittal view T2-weighted image.
The initial operative plan was to perform an anterior decompression, which may have entailed a corpectomy with os graft and fusion. However, her extensive medical comorbidities increased the risk of perioperative morbidity. Consideration was given for posterior approach with the possibility of increased adventure of wound infection due to the large lumbar pit and hairy patch, overlying the planned surgical wound. As posterior approach presented a significantly lower take chances to the patient overall, a posterior decompression with a standard midline incision and laminectomy was performed at L2/3 under general anesthesia.
Post-obit posterior decompression, the patient recovered full motor power in her lower limbs, and she could eventually walk unaided. In the early postoperative period, she had a deep wound infection, which was successfully treated with debridement, negative pressure therapy (vacuum-assisted closure pump), and antibiotics.
Follow-up in outpatient dispensary half-dozen months afterward surgery, she was walking unaided and the wound had healed satisfactorily. Oswestry Disability Index showed an comeback from 55% preoperatively to twenty%. Her upper motor neuron signs seen preoperatively had also resolved.
Discussion
TCS was described in last three decades and was previously associated only with the pediatric population.iv Now, however, there is good show that it can occur even in adults and is much more than common than previously idea.1
Adults with TCS are considered to vest to two groups: those who were healthy in childhood but symptoms adult in adulthood or those who had static neurological deficits or skeletal deformities that were diagnosed in babyhood but remained well until the onset of new and progressive neurological deficits in adulthood.three v Other classifications included grouping them on basis of extent of spinal dysraphism6 or surgical outcome post-obit detethering of the cord.7
Information technology has been shown that adults commonly present with varying neurological symptoms usually following a precipitating cistron such every bit coughing, bending, or strenuous concrete action. It is thought that the mechanical longitudinal traction as a result of tethered cord causes these symptoms.
Many clinical serial have found that these patients can present with a gradual, insidious onset of back pain, nondermatomal leg hurting, burning pain in the buttocks and perineal region, and bowel and/or float dysfunction.one ii 4 7 They can mimic symptoms of lumbar disc disease or spinal stenosis. In that location are some reports of symptoms being aggravated due to disc disease.eight
The recommended treatment is to detether the cord and excise any lesion.i 2 3 Though the role of surgery is controversial, it has been shown that surgical detethering in the presence of neurological deterioration has been of some do good. Motor weakness and pain seem to resolve though the return of sensory deficit, and render of bladder function varies.4 Some surgeons have fifty-fifty questioned the part of detethering in the absence of symptoms and whether information technology would alter the natural history of TCS.nine
Our hypothesis is that the neurological arrears in this patient was likely secondary to the prolapsed disc. Owing to the natural lordosis of lumbar spine, posterior decompressive surgery may accept allowed the spinal string and neural elements to fall back and therefore relieved inductive cord pinch. Upper motor neuron signs and myelopathy are not ordinarily seen with lumbar disc prolapse when the string is in the normal position. However, in the presence of low-lying cord, this could effect in signs of cord compression with resulting myelopathy or upper motor neuron signs. This is the first case we know of that describes the management of lumbar cord compression with progressive neurological deficit. Posterior decompression appears to have provided a satisfactory outcome in this case.
Disclosures
Shreya Srinivas, None
Rohit Shetty, None
Iona Collins, None
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Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3864496/
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