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Congenital Spinal Cord Anomaly

Spinal Cord Anomalies


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]
  • Presenter : Dr. Charusmita Chaudhary Moderator: Dr R. K.[slideshare.net]
  • This situation is uncommon, but when typical symptoms are present, surgical exploration is justified.[musculoskeletalkey.com]
  • Injection Techniques for Discogenic Pain -Minimally Invasive Techniques for Thoracolumbar Deformity -Spinal Cord Herniation and Spontaneous Cerebrospinal Fluid Leak -MIS Versus Open Spine Surgery Extensive revisions to many of the existing chapters present[books.google.com]
Enuresis
  • […] 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]
Goiter
  • […] disorders (255.2) congenital disorders of steroid metabolism (255.2) 759.2 Anomalies of other endocrine glands Absent parathyroid gland Accessory thyroid gland Persistent thyroglossal or thyrolingual duct Thyroglossal (duct) cyst Excludes: congenital: goiter[theodora.com]
Inspiratory Stridor
  • Type II lesions typically manifest in infancy with inspiratory stridor owing to vocal cord paralysis, weakness in feeding, bradycardia, apnea, or “blue spells.”[musculoskeletalkey.com]
Recurrent Vomiting
  • This is a formidable procedure, however, and recurrent vomiting may occur from irritation or compression of the brainstem at the obex.[musculoskeletalkey.com]
Oscillopsia
  • Symptoms include lower cranial nerve palsies, vertigo, oscillopsia, truncal ataxia, headache, and syncope in association with the Valsalva maneuver.[musculoskeletalkey.com]
Truncal Ataxia
  • Symptoms include lower cranial nerve palsies, vertigo, oscillopsia, truncal ataxia, headache, and syncope in association with the Valsalva maneuver.[musculoskeletalkey.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

  • 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]
  • Fatal urologic or cardiac anomalies may be evident that would favor nonoperative treatment.[musculoskeletalkey.com]
  • DISCLAIMER : All material available on eHealthMe.com is for informational purposes only, and is not a substitute for medical advice, diagnosis, or treatment provided by a qualified healthcare provider.[ehealthme.com]
  • If the MRI scan shows any evidence of these malformations, a neurosurgical consultation is recommended to develop the best treatment plan.[hopkinsmedicine.org]

Prognosis

  • In addition, in cases where the natural history indicates a poor prognosis, orthotic treatment is contraindicated.[emedicine.medscape.com]
  • Despite the high spinal level, the actual spinal cord involvement is often minor, and the prognosis is good.[musculoskeletalkey.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]

Etiology

  • Retethering can occur, and if new symptoms suggest this as an etiology, reoperation is indicated.[musculoskeletalkey.com]
  • 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]
  • 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]
  • Incidence, etiology, and economic impact Statistics about the incidence of spina bifida vary considerably in different parts of the world.[clinicalgate.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]
Sex distribution
Age distribution

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

  • Management of an Unrepaired Newborn Closure is usually performed 24 to 48 hours after birth to prevent ventriculitis.[musculoskeletalkey.com]
  • 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]
  • 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]
  • 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]

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