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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


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.


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.


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


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

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