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CASECASE
The pediatrician is called to the delivery The pediatrician is called to the delivery room because an infant is born with a room because an infant is born with a defect in the lumbosacral area.defect in the lumbosacral area.
This is what the baby presentedThis is what the baby presented
MYELOMENINGOCELEMYELOMENINGOCELE
Cases from Hospital Manuel de Jesus Rivera “La Mascota” Cases from Hospital Manuel de Jesus Rivera “La Mascota”
National Children’s Hospital Managua, NicaraguaNational Children’s Hospital Managua, Nicaragua
IMELDA MEDINA, MDIMELDA MEDINA, MD
MYELOMENINGOCELEMYELOMENINGOCELE
Neural tube defectNeural tube defect
The most severe form of dysraphism The most severe form of dysraphism involving the vertebral column.involving the vertebral column.
MYELOMENINGOCELEMYELOMENINGOCELE
EtiologyEtiology
UnknownUnknown
It is thought that agents such as drugs, It is thought that agents such as drugs, radiation, malnutrition (Specially folic acid radiation, malnutrition (Specially folic acid deficiency during prenatal period), and deficiency during prenatal period), and genetics have a role in adversely affecting genetics have a role in adversely affecting normal CNS development.normal CNS development.
MYELOMENINGOCELEMYELOMENINGOCELEClinical PresentationClinical Presentation
Defect in the Lumbosacral regionDefect in the Lumbosacral region Bowel and bladder incontinenceBowel and bladder incontinence Physical Exam: Physical Exam:
Flaccid paralysis of lower extremitiesFlaccid paralysis of lower extremities Absent deep tendon reflexes, lack of response to Absent deep tendon reflexes, lack of response to
touch or paintouch or pain Postural abnormalities including clubfoot or subluxed Postural abnormalities including clubfoot or subluxed
hips may be present.hips may be present. 80% develop hydrocephalus associated with Type II 80% develop hydrocephalus associated with Type II
Chiari defect.Chiari defect.
MYELOMENINGOCELEMYELOMENINGOCELEPrenatal Diagnostic StudiesPrenatal Diagnostic Studies
Prenatal screening of AFP (Alpha-Prenatal screening of AFP (Alpha-Fetoprotein acetylcholinesterase) at 16-18 Fetoprotein acetylcholinesterase) at 16-18 weeks.weeks.
AFP is excreted from the fetus and leaks into the amniotic AFP is excreted from the fetus and leaks into the amniotic fluid when there is failure of the neural tube to close.fluid when there is failure of the neural tube to close.
MYELOMENINGOCELEMYELOMENINGOCELETreatmentTreatment
Prenatal Folic acid and vitamins, adequate Prenatal Folic acid and vitamins, adequate nutrition and Ob/Gyn carenutrition and Ob/Gyn care
Multidisciplinary care needed (Pediatrics, Multidisciplinary care needed (Pediatrics, Neurology, Urology, Neurosurgery, Neurology, Urology, Neurosurgery, Occupational/Physical therapists, etc).Occupational/Physical therapists, etc).
Surgical repair of Lumbosacral defect and Surgical repair of Lumbosacral defect and ventriculo-peritoneal shunt for those who ventriculo-peritoneal shunt for those who develop hydrocephalus. develop hydrocephalus.
MYELOMENINGOCELEMYELOMENINGOCELE
ComplicationsComplications
Potential Herniation of the medulla and cerebellar Potential Herniation of the medulla and cerebellar tonsils through the foramen magnum secondary to tonsils through the foramen magnum secondary to Chiari II malformationChiari II malformation
Monitor for clinical presentation: Feeding problems, drooling, Monitor for clinical presentation: Feeding problems, drooling, choking, DEATH if left untreated.choking, DEATH if left untreated.
Infections: Meningitis, UTI’sInfections: Meningitis, UTI’s Treat with IV antibioticsTreat with IV antibiotics Prevent UTI’s with frequent catheterizationsPrevent UTI’s with frequent catheterizations
MYELOMENINGOCELEMYELOMENINGOCELE
MYELOMENINGOCELEMYELOMENINGOCELEClub FootClub Foot
MYELOMENINGOCELEMYELOMENINGOCELEHydrocephalusHydrocephalus
MYELOMENINGOCELEMYELOMENINGOCELEVentriculomegalyVentriculomegaly
MYELOMENINGOCELEMYELOMENINGOCELEVentriculomegalyVentriculomegaly
Ventricular PunctureVentricular Puncture
Hospital Manuel de Jesus Rivera “La Mascota” Hospital Manuel de Jesus Rivera “La Mascota” Marina Morales, MD; Miguel Angel Guevara, MDMarina Morales, MD; Miguel Angel Guevara, MD
Ventricular PunctureVentricular Puncture
Hospital Manuel de Jesus Rivera “La Mascota” Hospital Manuel de Jesus Rivera “La Mascota” Marina Morales, MD; Miguel Angel Guevara, MDMarina Morales, MD; Miguel Angel Guevara, MD
BIBLIOGRAPHYBIBLIOGRAPHY
9.9.Nelson’s Textbook of Pediatrics.Nelson’s Textbook of Pediatrics. Berhrman RE, Berhrman RE, Kliegman R, Jensen Hal, W.B. Saunders Co, Kliegman R, Jensen Hal, W.B. Saunders Co, Philadelphia, 2000, 16th ed. P 1804-1806.Philadelphia, 2000, 16th ed. P 1804-1806.
Hospital Manuel de Jesus Rivera “La Mascota.” Neonatal Hospital Manuel de Jesus Rivera “La Mascota.” Neonatal Intensive Care Unit Department, Isolation Unit. Photos Intensive Care Unit Department, Isolation Unit. Photos taken by Imelda Medina, MD with informed consent taken by Imelda Medina, MD with informed consent from parents and faculty at the Institution. March-May from parents and faculty at the Institution. March-May 20052005
THE ENDTHE END