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Cervical Spina Bifida without Hydrocephalus

Myelomeningocele


Presentation

  • Adult presentation of myelomeningocele is exceedingly rare. Case presentation: We present a 74 years Caucasian female was transferred to our institution for evaluation of purulent drainage around an unrepaired myelomeningocele site.[omicsonline.org]
  • On Admission POA Help "Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery, are[icd10data.com]
  • Symptoms of brain stem dysfunction, related to an Arnold Chiari malformation, also often present in infancy. Neurogenic bladder is common and often presents at birth. Early childhood Developmental delays are common.[now.aapmr.org]
  • Presentation Chiari type I [ 5 ] The presentation of this condition depends upon the severity of the herniation and associated other features.[patient.info]
  • Clinical Presentation Present since birth There is some abnormality in the local skin either a skin dimple or a local patch of hair or a naevo-lipoma or simple lipoma.[boneandspine.com]
Short Stature
  • Increased incidence of osteoporosis, associated with pathological fractures Increased incidence of obesity, short stature and precocious puberty Variable sexual dysfunction and fertility Increased incidence of depression High incidence of latex allergy[now.aapmr.org]
  • Spinal deformities Congenital – vertebrae and rib anomalies Acquired due to muscle imbalance Lower limb deformities Short Stature Cranial nerve dysfunction – Ocular muscle palsies, dysphagia and dysphonia.[boneandspine.com]
Lymphedema
  • Adulthood Obesity, metabolic syndrome, venous stasis and lymphedema are not uncommon. Bowel incontinence can be an important barrier to employment. Shoulder and wrist pain, including rotator cuff injuries are possible.[now.aapmr.org]
Precocious Puberty
  • Increased incidence of osteoporosis, associated with pathological fractures Increased incidence of obesity, short stature and precocious puberty Variable sexual dysfunction and fertility Increased incidence of depression High incidence of latex allergy[now.aapmr.org]
Pneumonia
  • Signs of brain stem dysfunction, especially in an infant, include feeding difficulty, stridor, difficulty swallowing, aspiration pneumonia and apnea. Signs of shunt malfunction include headaches, cognitive changes, sedation and nausea/vomiting.[now.aapmr.org]
  • He recorded type 2 changes in a 6 month old child who had paraplegia, a paralysed bladder, and had succumbed to pneumonia. The pons descended into the spinal canal over 6 mm. And the medulla extended to the third cervical vertebra.[jnnp.bmj.com]
  • Recurrent pneumonia from aspiration. Gradual loss of function. Presentation of types III and IV have been ignored as they are much rarer. Investigations MRI: The investigation of choice is MRI [ 8 ]. MRI now means that myelography is not required.[patient.info]
  • Inspiratory stridor Swallowing difficulties that leads to aspiration pneumonia Cyanosis an apnea. Those symptoms are directly related to brain stem malfunction.[surgicalneurologyint.com]
Flexion Contracture
  • Neurogenic bowel and bladder Increased risk for a wide variety of musculoskeletal disorders, including scoliosis, hip dislocation, flexion contractures of the hips and knees, foot abnormalities, and rotational deformities.[now.aapmr.org]
  • These patients often exhibit mild hip and knee flexion contractures and increased lumbar lordosis with variable foot and ankle deformities with various ankle and foot positions.[boneandspine.com]
  • contracture may be evident Recurvatum may be a feature at the knee, associated with talipes and claw toes in the feet These deformities may develop following birth due to unbalanced paralysis, if not evident initially Scoliosis occurs in 80% of the thoracic[orthopaedicsone.com]
Wrist Pain
  • Shoulder and wrist pain, including rotator cuff injuries are possible.[now.aapmr.org]
Sexual Dysfunction
  • Increased incidence of osteoporosis, associated with pathological fractures Increased incidence of obesity, short stature and precocious puberty Variable sexual dysfunction and fertility Increased incidence of depression High incidence of latex allergy[now.aapmr.org]
  • dysfunction, skeletal deformations social and mental impairment [ 1, 2 ].[jneuro.com]
Cognitive Deficit
  • deficits with greater difficulty with visual perceptual skills, executive functioning and attention and a relative strength in verbal skills.[now.aapmr.org]

Treatment

  • Astoundingly she never proceeded to get a life threatening infection and, besides her urostomy, she had no other surgical treatments. The treatment of myelomeningocele has evolved dramatically over the last half century.[omicsonline.org]
  • REHABILITATION MANAGEMENT AND TREATMENTS Available or current treatment guidelines A widely established, research-based guideline for MM does not exist.[now.aapmr.org]
  • With surgery and other forms of treatment, many people with spina bifida live into adulthood.In a milder form of the condition, called spina bifida occulta, the bones of the spinal column are abnormally formed, but the nerves of the spinal cord usually[icdlist.com]
  • Treatments for Hydrocephalus If hydrocephalus is not creating symptoms, or if it is due to temporary obstruction, treatment may consist of monitoring the condition instead of intervening. In most cases, though, hydrocephalus is treated with surgery.[princetonbrainandspine.com]

Prognosis

  • Traditionally, MM patients that received no surgical intervention have a dismal prognosis.[omicsonline.org]
  • Treatment and prognosis The overall prognosis is variable depending on the type and extent. For an isolated spina bifida, the recurrence risk range for future pregnancies is thought to be around 0.3-5% 12-13.[radiopaedia.org]
  • The prognosis otherwise depends on severity of the malformation, associated neurological abnormalities and complications. There has been some improvement in prognosis with improved diagnostic and surgical techniques.[patient.info]
  • One needs to be cautious about providing too optimistic a prognosis with respect to neurologic dysfunction based on a neonatal examination and to recognize that the appearance of neurologic deficits may not indicate deterioration.[ncbi.nlm.nih.gov]
  • Prognosis varies by the level of cord involvement and the number and severity of associated anomalies.[merckmanuals.com]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • Etiology MM is a result of failure of the normal development of the neural tube, which typically starts on day 16 and is complete by day 28.[now.aapmr.org]
  • […] body system manifestations due to the underlying etiology.[icd10data.com]
  • The etiology is often multifactorial with both genetic and environmental factors considered to play a role. Intake of adequate amounts of folic acid plays a protective role. Classification There are many ways of classifying a spina bifida.[radiopaedia.org]
  • The etiology in most cases of spina bifida is multifactorial.[boneandspine.com]

Epidemiology

  • Epidemiology including risk factors and primary prevention The birth prevalence of MM is 2:10,000 live births. 1 Most cases of MM occur sporadically and are felt to have a multifactorial etiology.[now.aapmr.org]
  • Epidemiology Chiari type I Retrospective investigation of brain MRIs has found a prevalence of 1 in 1,280 individuals [ 5 ]. It tends to present earlier if there is syringomyelia.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • Normal Physiology and Pathophysiology of Hydrocephalus Cerebrospinal fluid (CSF) is a liquid that normally surrounds the brain and spinal cord, serving to cushion the nervous tissue as well as wash away metabolic byproducts.[cns.org]

Prevention

  • - 0% Emergent - ED Care Needed - Preventable/Avoidable - 0% Emergent - ED Care Needed - Not Preventable/Avoidable - 0% Primary diagnosis of injury 0% Primary diagnosis of mental health problems 0% Primary diagnosis of substance abuse 0% Primary diagnosis[medicbind.com]
  • Measurement of Patient Outcomes In 2008 the Centers for Disease Control and Prevention, in partnership with the Spina Bifida Association, launched the National Spina Bifida Registry.[now.aapmr.org]
  • Prevention is the best approach.[hawaii.edu]
  • These malformations can be prevented with the technique of MMC closure [ 10, 11 ].[jneuro.com]
  • If the myelomeningocele is leaking CSF, antibiotics are started to prevent meningitis.[merckmanuals.com]

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