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

Case Presentation: Myelomeningocele

Stephen Confer, MDBen O. Donovan, MD

Brad Kropp, MDDominic Frimberger, MD

University of Oklahoma Department of Urology

Section of Pediatric Urology

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

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

Case Presentation• Family Hx

– No malignancy

• ROSAs in HPI

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

What would you recommend?

General Recommendations

• Renal Ultrasound

• VCUG

• CIC times 3

• Baseline Urodynamics

Types of Myelodysplasia*

• Spina bifida occulta

• Lipomeningocele

• Meningocele

• Myelomeningocele = Spina Bifida

*defective development of the spinal cord

Neurologic pathologySpina bifida occulta

(occulta = closed)

A condition involving nonfusion of the halves of the vertebral arches without disturbance of the underlying neural tissue

Neurologic pathologyLipomeningocele

(lipo = fat)

lipoma or fatty tumor located over the lumbosacral spine. Associated with bowel & bladder dysfunction

LipomeningoceleLipomeningocele

Neurologic pathology

Meningocele (cele = sac)

Fluid-filled sac with meninges involved but neural tissue unaffected

Types of Myelodysplasia

Myelomeningocele

or spina bifida: meninges and spinal tissue protruding through a dorsal defect in the vertebrae

The spinal defect with myelomeningocele

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

When do neural tube defects occur?

Neural Tube Development

Normal embryological development Neural plate

development -18th dayCranial closure 24th

day (upper spine)Caudal closure 26th

day (lower spine)

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.

Clinical Considerations

What factors contribute to neural tube defects?

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.

Future Directions?

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