Spina Bifida

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Spina Bifida, Meningemyelocele

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Spina Bifida Meningomyelocele: 

Spina Bifida Meningomyelocele Amanda Jamieson and Jennifer Harris February 21, 2013

General Description: 

General Description Spina Bifida is an incomplete closure of one or more of the neural arches during spinal formation. Most often occurs in the lumbo -sacral region 3 types: spina bifida occulta , meningocele , myelomeningocele 1

Spina Bifida Occulta: 

Spina Bifida Occulta Spinal Cord and Meninges remain within vertebral column 2 Least severe form Usually asymptomatic: rarely occurs with neurological deficits Detectable only by radiograph: 15% of healthy people have SBO and do not know it 3 Image depicting small tuft of hair common with Spina Bifida Occulta

Meningocele: 

Meningocele Meninges and cerebral spinal fluid extrude through the defect in the neural arches forming a sac, which is covered by normal skin 1,2 Spinal cord remains within spinal canal. Neurological symptoms typically absent at birth Corrected by surgery, typically results in minimal residual deficits 4

Meningomyelocele: 

Meningomyelocele Spinal cord and nerve roots lie outside the vertebral column 1,2 Most severe form Multiple surgeries required for survival 2 Serious neurological deficits

Meningomyelocele Signs and Symptoms: 

Meningomyelocele Signs and Symptoms Foot and hip deformities, knee contractures, scoliosis and other spinal deformities Often accompanied by hydrocephalus 1,4 Bowel/bladder incontinence Motor and sensory loss in LE Spasticity Latex allergy

Meningomyelocele Incidence and Prevalence: 

Meningomyelocele Incidence and Prevalence 1 in every 1000 children born in the United States is born with Spina Bifida 4 Meningomyelocele is more common than Meningocele 2 Females affected 14% more than males 5

Diagnosis/Prognosis: 

Diagnosis/Prognosis Diagnosed prenatally with fetal ultrasound, MRI and alpha fetoprotein screening 6,7,8 Diagnosed after birth with x-ray, CT scan and MRI 6 Approximately 90% survive into adulthood 4 Image above: Ultrasound Image to Left: Prenatal Ultrasound

Impact on Function: 

Impact on Function 80% have normal IQ scores and 75% are able to take part in sports and recreational activity 4 Ambulation is likely achieved with an assistive device 6 Urinary tract infections and fecal incontinence can greatly impact function later in life 4 Early intervention such as corrective surgery and physical therapy can greatly improve function by addressing neurological and orthopedic impairments that may impede ADL’s .

Imaging : 

Imaging Pre-natal Diagnosis Post-natal Diagnosis Spina Bifida is typically diagnosed with fetal ultrasound and MRI Ultrasound is used initially to detect the deformity MRI provides additional and more detailed anatomical information 8 Spina Bifida is detected using x-ray, MRI or CT scan MRI and CT scan provide a more clear view of the spine, however there is no gold standard method of imaging to diagnosis Spina Bifida

Radiograph Image: 

Radiograph Image Findings: No closure of posterior portion of S1 Unremarkable soft tissue findings Biomechanical and Physical Impairments: Likely no physical impairments due to lack of soft tissue findings Appears to be no extrusion of meningeal sac, CSF or spinal cord Reprinted from Wikipedia 9

MRI Image: 

MRI Image Findings: Herniation of meningeal sac through the posterior defect Formation of subcutaneous swelling Biomechanical and Physical Impairments: Dysfunction appears to be at L5/S1 level Decreased hamstring, gluteus medius and minimus strength Ambulation possible with use of ankle foot orthosis Reprinted from Journal of Pediatric Surgery 10

Questions: 

Questions Other than the spine, what other anatomical location warrants imaging in patients diagnosed with Spina Bifida Meningocele or Meningomyelocele ? If your patient who is pregnant came to you reporting that her physician found elevated levels of alpha fetoprotein at her latest check up and recommended a pre-natal MRI, what would be your response to your patient?

References: 

References Salter, RB. Textbook of Disorders and Injuries of the Musculoskeletal System. Third Edition. Philadelphia. Lippincott Wiliams & Wilkins. 1999. Northrup H, Volcik KA. Spina bifida and other neural tube defects. Curt Probl Pediatr . 2000; 30: 317-32. Liptak GS. What Is Spina Bifida? Spina Bifida Association. http://www.spinabifidaassociation.org/site/c.evKRI7OXIoJ8H/b.8277225/k.5A79/What_is_Spina_Bifida.htm . Assessed February 17, 2013. Emil Vernarec , ed. Ellen Barker, Michael Saulino , Angela Caristo . A new age for childhood diseases: Spina bifida. RN. 2002; 12:33. Shin M, Besser LM, Siffel C, Kucik JE, Shaw GM, Lu C, Correa A, and the Congenital Anomaly Multistate Prevalence and Survival Collaborative. Prevalence of Spina Bifida Among Children and Adolescents in 10 Regions in the United States. Pediatrics. 2010 [ e -published July 12, 2010]. Cunniff C. Prenatal screening and diagnosis for pediatricians. Pediatrics. 2004; 114 (3): 889-894. Spina Bifida Fact Sheet. National Institute of Neurological Disorders and Stroke. March 2007. Accessed February 18, 2013. http://www.ninds.nih.gov/disorders/spina_bifida/detail_spina_bifida.htm Aaronson OS, Hernanz -Schulman M, Bruner JP, Reed GW, Tulipan NB. Myelomeningocele : prenatal evaluation – comparison between transabdonimal US and MR imaging. Radiology. 2003; 227: 839-843. Spina Bifida. Wikipedia. Febuary 15, 2013. Accessed February 19, 2012. http://es.wikipedia.org/wiki/Archivo:Spina_Bifida_pelvis_X-ray.jpg Parelkar SV et al. Midline mature teratoma inside a ruptured meningomyeocele of lumbosacral region, a case report. J Pediatr Surg . 2012; 47(10):1934-193