Presentation on theme: "Case Presentation: Myelomeningocele Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology."— Presentation transcript:
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
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 ROS As 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 pathology Spina bifida occulta (occulta = closed) A condition involving nonfusion of the halves of the vertebral arches without disturbance of the underlying neural tissue
Neurologic pathology Lipomeningocele (lipo = fat) lipoma or fatty tumor located over the lumbosacral spine. Associated with bowel & bladder dysfunction Lipomeningocele
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 day Cranial 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 Jun;44(6): Risk factors for renal injury in patients with meningomyelocele. Arora GArora G, Narasimhan KL, Saxena AK, Kaur B, Mittal BR.Narasimhan KLSaxena AKKaur BMittal BR
Future Directions? Support MM Clinics & Research