case presentation: myelomeningocele stephen confer, md ben o. donovan, md brad kropp, md dominic...
TRANSCRIPT
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Case Presentation: Myelomeningocele
Stephen Confer, MDBen O. Donovan, MD
Brad Kropp, MDDominic Frimberger, MD
University of Oklahoma Department of Urology
Section of Pediatric Urology
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Case Presentation
• NICU Consultation
• HPI : 1 day old male transferred to NICU from outside facility– No prenatal history available– Identified to have myelomeningocele– Going to OR in am with Neurosurgery
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Case Presentation
• PMHx– Denies prenatal US
– ‘Normal’ Prenatal Course
– Good Apgars
– Good amniotic fluid from birth –per report
• Social Hx– Small Town
– 21 yo Non smoker
– Denied alcohol and illicit drug usage
– Single, no children
• Medications :– ES Tylenol PRN
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Case Presentation• Family Hx
– No malignancy
• ROSAs in HPI
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Physical Examination
• Vitals signs : AFVSS
• General : NAD• GU: uncirc, bilateral testes descended• Abd : ND, no masses, no hepatosplenomegaly.
No inguinal hernia, umbilical stump is clear • Ext : No edema or cyanosis. MAE x 4• Back: large patch of irregular tissue at midline
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What would you recommend?
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General Recommendations
• Renal Ultrasound
• VCUG
• CIC times 3
• Baseline Urodynamics
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Types of Myelodysplasia*
• Spina bifida occulta
• Lipomeningocele
• Meningocele
• Myelomeningocele = Spina Bifida
*defective development of the spinal cord
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Neurologic pathologySpina bifida occulta
(occulta = closed)
A condition involving nonfusion of the halves of the vertebral arches without disturbance of the underlying neural tissue
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Neurologic pathologyLipomeningocele
(lipo = fat)
lipoma or fatty tumor located over the lumbosacral spine. Associated with bowel & bladder dysfunction
LipomeningoceleLipomeningocele
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Neurologic pathology
Meningocele (cele = sac)
Fluid-filled sac with meninges involved but neural tissue unaffected
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Types of Myelodysplasia
Myelomeningocele
or spina bifida: meninges and spinal tissue protruding through a dorsal defect in the vertebrae
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The spinal defect with myelomeningocele
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Incidence and Prevalence
• Incidence – 1/1000
• Prevalence – Increased incidence in families of Celtic and
Irish heritage (genetic or environmental?)– Increased incidence in minorities (genetic or
environmental?)– Increased incidence in families
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When do neural tube defects occur?
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Neural Tube Development
Normal embryological development Neural plate
development -18th dayCranial closure 24th
day (upper spine)Caudal closure 26th
day (lower spine)
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Preventive Care
• The United States Public Health Service recommends that: "All women of childbearing age in the United States who are capable of becoming pregnant should consume 0.4 mg of folic acid per day for the purpose of reducing their risk of having a pregnancy affected with spina bifida or other neural tube defects." Folic acid is a "B" vitamin that can be found in such foods as: cereals, broccoli, spinach, corn and others, and also as a vitamin supplement.
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Clinical Considerations
What factors contribute to neural tube defects?
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High Risk for Renal Injury
• Increasing age, evidence of hydroureteronephrosis and vesicoureteric reflux, high leak pressures, and low bladder volume define a high risk bladder in our population and predispose to renal injury in patients of myelodysplasia.
• Early referral for bladder risk assessment and management of all myelodysplasia patients is recommended. Indian Pediatr. 2007 Jun;44(6):417-20.
Risk factors for renal injury in patients with meningomyelocele.Arora G, Narasimhan KL, Saxena AK, Kaur B, Mittal BR.
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Future Directions?
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