Presentation
Plain radiographs are the initial imaging in patients presenting with abnormal curvatures of spine. No other central nervous system abnormality requires as systematic an approach as spinal dysraphism. [ncbi.nlm.nih.gov]
Time for evolution Variable Evaluation at Presentation Careful, complete assessment: On presentation, a patient must undergo a thorough history and physical examination. [ispn.guide]
Presenter : Dr. Charusmita Chaudhary Moderator: Dr R. K. [slideshare.net]
Presentation on theme: "Spinal Dysraphisms- Congenital Spinal Cord Abnormalities"— Presentation transcript: 1 Spinal Dysraphisms- Congenital Spinal Cord Abnormalities C Herman, J Ponting, I Wu Indiana University School of Medicine Presentation # eEdE- [slideplayer.com]
The symptoms, once present, generally continue to worsen as development continues and are more likely to be irreversible the longer they are present prior to treatment. [csfinfo.org]
Entire Body System
- Pain
Also, being diagnosed with a spinal abnormality does not mean that you will experience pain. The relationship between spinal abnormalities and pain is complicated. [laserspineinstitute.com]
At the outset, it’s important you know that congenital anomalies, per se, do not cause pain. [simplebackpain.com]
Lumbar spinal stenosis results in low back pain as well as pain or abnormal sensations in the legs, thighs, feet or buttocks, or loss of bladder and bowel control. [en.wikibooks.org]
The proceduralist then observes whether the patient experiences pain that is similar to his/her usual pain as the pressure in the affected disc is increased by injecting contrast. [spine.org]
Degenerative scoliosis is often accompanied by pain. Symptoms of sagittal imbalance range from mild discomfort to severe pain. Spinal deformities also have the capacity to interfere with the spinal cord or nerve roots. [columbiaspine.org]
- Asymptomatic
Some children are asymptomatic, and others have severe neurologic dysfunction below the lesion. Open spina bifida can be diagnosed prenatally by ultrasonography or suggested by elevated alpha-fetoprotein levels in maternal serum and amniotic fluid. [merckmanuals.com]
Some authors suggest that these findings alone should prompt further evaluation, even in asymptomatic patients, because of the association with cerebral aneurysms and subarachnoid hemorrhage. 3 Conclusion We report an interesting case of congenital absence [nature.com]
Other conditions may be asymptomatic and undetected for years. Some examples of congenital spinal conditions include: Spina Bifida: Spina Bifida can manifest in two ways, spina bifida occulta, and spina bifida overta. [laserspineinstitute.com]
Symptoms Patients with congenital vertebral anomalies may present in a variety of ways: Asymptomatic: Congenital vertebral anomalies may be discovered during the acquisition of routine radiographs for trauma or other health issues. [ispn.guide]
These lesions cause many different symptoms or can be completely asymptomatic. In infants, aside from the skin defects noted above, there may be very subtle changes in leg shape and length, especially in the feet. [csfinfo.org]
- Surgical Procedure
- MRI: - indicated prior to any planned surgical procedure to rule out spinal dysraphism, which might not be amenable to treatment once a fusion is performed; - ref: Routine use of magnetic resonance imaging in idiopathic scoliosis patients less than [wheelessonline.com]
For a detailed explanation of surgical procedures that may be required to treat a specific spinal deformity, refer to that condition’s individual page. Drs. Paul C. McCormick, Peter D. Angevine, Christopher E. Mandigo, Patrick C. [columbiaspine.org]
The surgical procedure required for the effective treatment of Diastematomyelia requires the decompression of neural elements and removal of bony spur. This may be accomplished with or without resection and repair of the duplicated dural sacs. [neurosurgerydallas.com]
This procedure is typically reserved for last resort. 134 Unfortunately for children with spina bifida, their problems do not end after the back is surgically closed and a shunt is in place. [clinicalgate.com]
He underwent another surgical procedure with removal of scar from the previous laminectomy and posterior fusion with metal placement. [en.wikibooks.org]
- Swelling
However, treatment has the capacity to reduce inflammation and swelling that has set up around and about the spinal anomaly from the cumulative physical strain caused by the anomaly. [simplebackpain.com]
The neural tube appears to close normally but is distended from the cystic swelling. The CSF may ceaselessly expand the neural canal. Prompt medical attention is mandatory. [clinicalgate.com]
き、鶏歩 stiffness1.硬直、こわばり、2.不撓性〈脊椎の〉、3.剛性 straight leg raising test (SLR test)下肢伸展挙上テスト stress fracture疲労骨折 subarachnoid spaceくも膜下腔 subclavian artery鎖骨下動脈 Sudeck's atrophy Sudeck骨萎縮 suppository坐剤 supraclavicular fossa鎖骨上窩(―じょうか) suture1.縫合[法]、2.縫合材(糸) swelling [tokyo-med.ac.jp]
He was taken to surgery where the spinal canal was opened and a large amount of swelling was found. Biopsy was sent for frozen section, which the pathologist reported was most likely a glioblastoma or astrocytoma. The wound was closed. [en.wikibooks.org]
- Enuresis
[…] or constipation, is the most common presentation of a tethering lesion, although urinary tract infection is still common in this age group as well. 77, 81, 83 As the child matures, urgency, urge incontinence, sudden or stress incontinence, new-onset enuresis [pediatrics.aappublications.org]
Urogenital
- Urinary Retention
[…] tract infection is the predominant sign in an affected infant, but urinary retention is occasionally seen. 77 Once a child is toilet trained, the onset of secondary urinary incontinence, especially in conjunction with fecal incontinence and/or constipation [pediatrics.aappublications.org]
Neurologic
- Myelopathy
NOS congenital Q06.9 Lesion (s) (nontraumatic) spinal cord G95.9 ICD-10-CM Diagnosis Code G95.9 Disease of spinal cord, unspecified 2016 2017 2018 2019 Billable/Specific Code Applicable To Myelopathy NOS congenital Q06.9 Malformation (congenital) - see [icd10data.com]
Myelopathy: Craniocervical or cervical cord compression may lead to myelopathic symptoms such as weakness, numbness, paresthesias, gait difficulty, or bladder and bowel dysfunction. An associated syringomyelia may also cause these symptoms. [ispn.guide]
Five patients had significant improvement in myelopathy and were ambulating normally, 1 had modest improvement in ambulation, and 1 remained wheelchair-bound. All patients achieved solid radiographic fusions. [en.wikibooks.org]
Many patients do not develop symptoms until the second or third decade of life. 15 This may be related to increasing instability from ligamentous laxity caused by aging, similar to that from delayed myelopathies reported after atlantoaxial dislocations [neupsykey.com]
[…] tunnel手根管 cartilage 軟骨 castギプス包帯、キャスト cauda equina馬尾 cauda equina claudication馬尾性跛行(―はこう) cerebral palsy (CP)脳性麻痺 cerebrospinal fluid (CSF)[脳髄]髄液 cervical disk herniation 頚椎椎間板ヘルニア cervical spine 頚椎、脊柱頚椎部 cervical spondylosis頚椎症 cervical spondylotic myelopathy [tokyo-med.ac.jp]
- Steppage Gait
[…] spinal canal脊柱管 spinal [canal] stenosis脊柱管狭窄[症] spinal cord 脊髄 spinal cord tumor脊髄腫瘍 spinal fusion脊椎固定[術] spinal instrumentation脊椎インストゥルメンテーション spine1.脊椎、2.脊柱、3.棘(きょく)(=spina)、4.結節 splint副子、シーネ、スプリント spondylolisthesis脊椎すべり症 spondylolysis脊椎分離症 stenosis狭窄 steppage [tokyo-med.ac.jp]
- Pyramidal Tract Signs
However microcephaly is usually associated with significant abnormalities such as pyramidal tract signs and profound learning difficulties. It is part of more than 450 syndromes listed in the Oxford Dysmorphology Database. [jnnp.bmj.com]
Workup
These patients continue to be at a very high, and maybe higher than previously thought, risk for comorbidities that can cause devastating perioperative complications if not detected preoperatively, and full MRI workups should be considered in all patients [journals.lww.com]
Workup consists of MRI of the brain and complete spine, including sagittal and axial views. [musculoskeletalkey.com]
Isolated coccygeal dimples do not require further workup or treatment. FIGURE 9 Benign coccygeal dimple within the gluteal cleft and overlying the tip of the coccyx. [pediatrics.aappublications.org]
Workup An accurate radiologic evaluation is essential. Standard posteroanterior (PA) and lateral views of the entire spine are used for the initial evaluation. [emedicine.medscape.com]
With the use of antibiotics, shunting, and early sac closure, the survival rate has increased from 20% to 85%. 61, 94, 137 Urological management Initial newborn workup should include a urological assessment. [clinicalgate.com]
Treatment
If the MRI scan shows any evidence of these malformations, a neurosurgical consultation is recommended to develop the best treatment plan. [hopkinsmedicine.org]
Master all common conditions and their treatments. Identify key facts using the "Top 100 Secrets". Review material quickly thanks to bulleted lists, tables, and short answers. Apply memory aids and "secrets" from experts in the field. [books.google.com]
[…] range of motion may be limited, no treatment is indicated. [quizlet.com]
Each patient is unique and a treatment plan is made based on the risk of curve progression during growth. The treatment options include observation, bracing, and surgical intervention. [espine.com]
Treatment can improve symptoms and help prevent further neurological deterioration. [cedars-sinai.edu]
Prognosis
In addition, in cases where the natural history indicates a poor prognosis, orthotic treatment is contraindicated. [emedicine.medscape.com]
Which pattern has the worst prognosis and is an indication for surgery. Review Topic QID: 881 ML 1 Select Answer to see Preferred Response PREFERRED RESPONSE 5 (OBQ05.116) A 2-year-old girl presents to the office for evaluation of spinal deformity. [orthobullets.com]
Their prognosis is usually poor but with proper treatment, they can survive until adulthood. Modalities of treatment for the different conditions are surgery, therapy and medication. [brighthub.com]
Prognosis When your child's congenital spine anomaly is first diagnosed, no one will know exactly how much the spine deformity will progress as the child grows. There are some clues, however. [srs.org]
The careful assessment of patients with these abnormalities is important in order to provide an accurate prognosis and genetic counselling. [jnnp.bmj.com]
Etiology
Furthermore, congenital deformities of the bony spine often present in the context of defined congenital syndromes and associations such as those of genetic origin (Jarcho−Levin, multiple pterygium), those with unconfirmed etiology (Goldenhar syndrome [link.springer.com]
Large deformities can result due to the number of years of growth remaining. [16] Epidemiology /Etiology The etiology of congenital spine deformities varies greatly because there are many different types. [physio-pedia.com]
Incidence, etiology, and economic impact Statistics about the incidence of spina bifida vary considerably in different parts of the world. [clinicalgate.com]
Retethering can occur, and if new symptoms suggest this as an etiology, reoperation is indicated. [musculoskeletalkey.com]
Their etiology is not known, but it has been postulated that it may be similar to intraspinal lipomas, where dorsal mesenchyme is able to enter the neural tube secondary to premature disjunction and is subsequently induced to form these hamartomatous [radiologykey.com]
Epidemiology
Introduction Congenital scoliosis is the failure of normal vertebral development during 4th to 6th week of gestation caused by developmental defect in the formation of the mesenchymal anlage Epidemiology incidence prevalence in general population estimated [orthobullets.com]
Large deformities can result due to the number of years of growth remaining. [16] Epidemiology /Etiology The etiology of congenital spine deformities varies greatly because there are many different types. [physio-pedia.com]
Epidemiology Neural tube defects (NTDs) are among the most common congenital abnormalities but prevalence varies between countries and races. [jnnp.bmj.com]
Pathophysiology
Stay current on the latest standards in medical care thanks to extensive updates, including new chapters on Spinal Cord Stimulation and Implantable Drug Delivery Systems, Special surgical Techniques for the Growing Spine, Pathophysiology of Degenerative [books.google.com]
Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome. Neurosurg Focus. 2010;29:E9. Hertzler DA, DePowell JJ, Stevenson CB, Mangano FT. [rarediseases.org]
Closed spinal dysraphisms: neural tissue covered by skin With subcutaneous mass Without subcutaneous mass Simple dysraphic states Complex dysraphic states (including segmentation anomalies) 43 Sources Adzick NS: Fetal myelomeningocele: natural history, pathophysiology [slideplayer.com]
Normal Embryology and the Pathophysiology of Spinal Cord Tethering Dysraphic CNS malformations arise during the second, third, and fourth weeks of human embryogenesis, generally referred to as the period of neurulation. [pediatrics.aappublications.org]
Prevention
Current fortification programs are preventing about 22,000 cases, or 9% of the estimated folic acid–preventable spina bifida and anencephaly cases. 15 Genetic considerations, such as an Rh blood type, a specific gene type (HLA-B27), an X-linked gene, [clinicalgate.com]
Management of an Unrepaired Newborn Closure is usually performed 24 to 48 hours after birth to prevent ventriculitis. [musculoskeletalkey.com]
Early surgical intervention is vital to prevent the worsening of the curvature as the patient grows. To view video of imaging navigation for a complex scoliosis procedure, click on the image below. [espine.com]
Such a bar prevents the spine from growing on one side after a child is born. This results in a spinal curve that increases as a child grows. [orthoinfo.aaos.org]
Some birth defects, especially those related to environmental factors, can be prevented by avoiding alcohol, smoking, potent drugs and exposure to radiation and infections, and by maintaining good and proper nutrition. [brighthub.com]