1. Case Presentation: Myelomeningocele
Stephen Confer, MD
Ben O. Donovan, MD
Brad Kropp, MD
Dominic Frimberger, MD
University of Oklahoma
Department of Urology
Section of Pediatric Urology

2. 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

4. Case Presentation PMHx Social Hx Medications : 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

5. Case Presentation
Family Hx
No malignancy
ROS
As in HPI

6. 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

8. What would you recommend?

9. General Recommendations
Renal Ultrasound
VCUG
CIC times 3
Baseline Urodynamics

10. Types of Myelodysplasia*
Spina bifida occulta
Lipomeningocele
Meningocele
Myelomeningocele = Spina Bifida
*defective development of the spinal cord

11. Neurologic pathology Spina bifida occulta (occulta = closed)
A condition involving nonfusion of the halves of the vertebral arches without disturbance of the underlying neural tissue

12. Neurologic pathology Lipomeningocele (lipo = fat)
lipoma or fatty tumor located over the lumbosacral spine. Associated with bowel & bladder dysfunction
Lipomeningocele

13. Neurologic pathology Meningocele (cele = sac)
Fluid-filled sac with meninges involved but neural tissue unaffected

14. Types of Myelodysplasia
Myelomeningocele
or spina bifida: meninges and spinal tissue protruding through a dorsal defect in the vertebrae

15. The spinal defect with myelomeningocele

16. Incidence and Prevalence
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

17. When do neural tube defects occur?

18. Neural Tube Development
Normal embryological development
Neural plate development -18th day
Cranial closure 24th day (upper spine)
Caudal closure 26th day (lower spine)

19. 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.

20. Clinical Considerations
What factors contribute to neural tube defects?

21. 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 Jun;44(6):
Risk factors for renal injury in patients with meningomyelocele.
Arora G, Narasimhan KL, Saxena AK, Kaur B, Mittal BR.

22. Future Directions?
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