This document discusses myelomeningocele, which is a birth defect involving faulty closure of the spine and membranes around the spinal cord. It most commonly occurs in the lower back. It is often associated with other anomalies and can cause neurological problems. Prenatal screening and ultrasound can detect many cases. Treatment involves surgery after birth to repair the defect, with the goal of preventing infection and preserving nerve function. Lifelong management may be needed.

1. MyelomeningoceleMyelomeningocele
Dr.Nabaz M.MustafaDr.Nabaz M.Mustafa

2. Faulty closure of dorsal midlineFaulty closure of dorsal midline
embryological structureembryological structure

3. Anatomical structureAnatomical structure

4. Anatomical structureAnatomical structure

5. Anatomical structureAnatomical structure

6. LocationLocation
 Caudal thoracolumbar 85%Caudal thoracolumbar 85%
 Dorsal 10%Dorsal 10%
 Cervical 5%Cervical 5%

7. Associated anomalyAssociated anomaly
 Chiari II 100%Chiari II 100%
 Hydrocephalus 80-90%Hydrocephalus 80-90%
 Syrinx 40-80%Syrinx 40-80%
 Kyphosis 15%Kyphosis 15%
 CraniofacialCraniofacial
 CardiacCardiac
 RespiratoryRespiratory
 GITGIT
 GUTGUT

8. EpidemiologyEpidemiology
 Geographic UK >USAGeographic UK >USA
 Ethnic and racial white > blackEthnic and racial white > black
 Sex female > maleSex female > male
 Season winterSeason winter

9. Risk factorRisk factor
 Nutrient folic acid ,zincNutrient folic acid ,zinc
 AEA valproic acid ,tegretolAEA valproic acid ,tegretol
 DMDM
 ObeseObese
 HeatHeat
 radiationradiation
 SmokingSmoking
 CocaineCocaine
 Trisomy 13,18Trisomy 13,18

10. Prenatal diagnosisPrenatal diagnosis
Maternal serumMaternal serum
AFPAFP
16-18 weeks16-18 weeks 60-70%60-70%
accurateaccurate
Ultra soundUltra sound 20 weeks20 weeks 100%100%
AmniocentesisAmniocentesis
(AFP & AChE )(AFP & AChE )
For patient withFor patient with
previous historyprevious history
99%99%
MRIMRI

11. TreatmentTreatment

12. Fetal managementFetal management
 Intrauterine repair = controversyIntrauterine repair = controversy
 No change in neurologcal deficitNo change in neurologcal deficit
 Decrease chiari II & needs for VP shuntDecrease chiari II & needs for VP shunt
 Increase morbidity & mortality due toIncrease morbidity & mortality due to
prematurityprematurity

13. DeliveryDelivery
 Normal vaginal deliveryNormal vaginal delivery
 Caesarian sectionCaesarian section
 No significant deference but C/S betterNo significant deference but C/S better
 Decrease risk of trauma to expose neuralDecrease risk of trauma to expose neural
tissue during deliverytissue during delivery

14. Postnatal managementPostnatal management
General assessment for other organ anomalyGeneral assessment for other organ anomaly
Dressing by sterile saline moisturizing gauzeDressing by sterile saline moisturizing gauze
Nursing care in prone or on the side positionNursing care in prone or on the side position
Cranial ultrasoundCranial ultrasound
Plain X-ray if kyphosisPlain X-ray if kyphosis
prophylactic antibioticprophylactic antibiotic

15. Surgical repairingSurgical repairing
 AimAim
 Prevent CS leakPrevent CS leak
 Prevent retrograde ascending infectionPrevent retrograde ascending infection
 Preserve functioning neural tissuePreserve functioning neural tissue
 Better nursing careBetter nursing care

16. Surgical repairingSurgical repairing
 TimeTime
 Immediate if CSF leakImmediate if CSF leak
 Early within 72 hoursEarly within 72 hours
 Late 6 weeks (better to take a swap fromLate 6 weeks (better to take a swap from
palcode)palcode)

17. Surgical repairingSurgical repairing
 ContraindicationContraindication
 Paralysis at L2-3 or aboveParalysis at L2-3 or above
 Marked hydrocephalusMarked hydrocephalus
 Severe kyphosisSevere kyphosis
 Other anomaly or birth injuryOther anomaly or birth injury

18. Surgical repairingSurgical repairing

19. Postoperative managementPostoperative management
 Nursing care in prone with head downNursing care in prone with head down
 Head circumferenceHead circumference
 Obseation for chiari IIObseation for chiari II
 stridor , apnia, cyanosis,stridor , apnia, cyanosis,
choking,hypotonia,opithotonos,choking,hypotonia,opithotonos,
 Renal ultra soundRenal ultra sound

20. Postoperative complicationPostoperative complication
 InfectionInfection
 Superficial wound dehiscenceSuperficial wound dehiscence
 MeningitisMeningitis
 SepsisSepsis
 Spinal subdural abscessSpinal subdural abscess
 hydrocephalushydrocephalus

21. Other operationOther operation
 VP shunt ,revisionVP shunt ,revision
 Posterior fossa decompressionPosterior fossa decompression
 Untethering spinal cord by 5 yearsUntethering spinal cord by 5 years
 Plastic surgery :craniofacial anomaly ,trophic ulcerPlastic surgery :craniofacial anomaly ,trophic ulcer
 Orthopedic :DDH,club footOrthopedic :DDH,club foot
 UrologyUrology
 pediatric surgeon :GITpediatric surgeon :GIT
 Cardiac ,thoracic surgeonCardiac ,thoracic surgeon

22. PrognosisPrognosis
 PowerPower
 Lesion at D12 :paraplegicLesion at D12 :paraplegic
 Lesion at L3 :compatible with erect posture and walking with orthosisLesion at L3 :compatible with erect posture and walking with orthosis
 SphincterSphincter
 90% have neurogenic bladder90% have neurogenic bladder
 85% have fecal contenent85% have fecal contenent
 Needs intermittent catheterization or medicationNeeds intermittent catheterization or medication
 Dribbling urine with crying or movement is badDribbling urine with crying or movement is bad
 If one leg is normal ,normal sphincter will be expectedIf one leg is normal ,normal sphincter will be expected
 IntelligenceIntelligence
 75-80% normal75-80% normal
 MortalityMortality
 15% die within first 6 months even with aggressive treatment15% die within first 6 months even with aggressive treatment

23. PreventionPrevention
 Folic acidFolic acid
 3 months before conception till end of first3 months before conception till end of first
trimester (0.4mg)trimester (0.4mg)
 If has previous history (4mg)If has previous history (4mg)
 Genetic counselingGenetic counseling

24. lipomyelomeningocelelipomyelomeningocele

25. DefinitionDefinition
 Lipoma of the conus medullarisLipoma of the conus medullaris
 A form of occult spinal dysraphism (OSD)A form of occult spinal dysraphism (OSD)
in which a subcutaneous fibrofatty massin which a subcutaneous fibrofatty mass
 penitrating the dorsolumbar fascia,penitrating the dorsolumbar fascia,
 causes a spinal laminar defect,causes a spinal laminar defect,
 displaces the dura, anddisplaces the dura, and
 infiltrates and tethers the spinal cordinfiltrates and tethers the spinal cord

27. SubtypeSubtype
 Dorsal Variant •Dorsal Variant •
 Lipoma pass through a fascial defect and attaches directly to the dorsalLipoma pass through a fascial defect and attaches directly to the dorsal
surface of conus medullaris; •surface of conus medullaris; •
 nerve roots emerge from the ventral or lateral surface of the neuralnerve roots emerge from the ventral or lateral surface of the neural
tissue and lie in the subarachnoid space.tissue and lie in the subarachnoid space.
 Caudal VariantCaudal Variant
 Lipoma attach to caudal end of conusLipoma attach to caudal end of conus
 It replace terminal filumIt replace terminal filum
 nerve roots do pass through lipoma.nerve roots do pass through lipoma.
 Many of these nerve roots are thought to be non-functionalMany of these nerve roots are thought to be non-functional
 Transitional Form •Transitional Form •
 Has elements of both dorsal and caudal variants.Has elements of both dorsal and caudal variants.
 Viable nerve roots pass through significant amounts of lipoma beforeViable nerve roots pass through significant amounts of lipoma before
exiting.exiting.

29. Embryo logicallyEmbryo logically
 Day 25-48Day 25-48

30. Clinical findingsClinical findings
 Cutaneous stigma on the midline of the back:Cutaneous stigma on the midline of the back:
 Atretic meningoceleAtretic meningocele
 dermal sinusdermal sinus
 hemangioma, portwine stain,hemangioma, portwine stain,
 hypertrichosis,hypertrichosis,
 Skin tagSkin tag
 pigmentary noevus,pigmentary noevus,
 coccygeal dimplecoccygeal dimple
 Other malformation :CNS .GUT,GIT,limbsOther malformation :CNS .GUT,GIT,limbs

32. Neurological symptomsNeurological symptoms
 Due toDue to
 mass compressing neural structuremass compressing neural structure
 Tethering of cordTethering of cord
 Usually slowly progressive over a period ofUsually slowly progressive over a period of
yearsyears
 Rarely acute which could be due to hyperflexionRarely acute which could be due to hyperflexion
or lithotomy positionor lithotomy position

33. Presentation according to agePresentation according to age
 InfantInfant
 cutaneous anomaliescutaneous anomalies
 Older childrenOlder children
 orthopedic, neuroenic,urogenic symptomsorthopedic, neuroenic,urogenic symptoms
 AdultAdult
 painpain

34. investigationinvestigation
 PrenatalPrenatal
 ultra soundultra sound
 PostnatalPostnatal
 MRIMRI
 Urodynamic study for preop.evaluation and postopUrodynamic study for preop.evaluation and postop
follow upfollow up
 CT.scan if suspect bone abnormalityCT.scan if suspect bone abnormality

35. surgerysurgery
 TimeTime
 first 6 months of agefirst 6 months of age
 AimsAims
 Untethering the cordUntethering the cord
 Debulking of intramedulary massDebulking of intramedulary mass
 Reconstruction of spinal cord and thecal sacReconstruction of spinal cord and thecal sac

36. Intraoperative neuro monitoringIntraoperative neuro monitoring
 ControversyControversy
 Because untethering is based on anatomy notBecause untethering is based on anatomy not
neurophysiologyneurophysiology
 Somatosensory evoked potentialsSomatosensory evoked potentials
 Bladder and extensor anal sphincterBladder and extensor anal sphincter
manometrymanometry
 EMGEMG

37. complicationcomplication
 CSf leakCSf leak
 psudomeningocelepsudomeningocele
 InfectionInfection
 New neurological deficitNew neurological deficit

38. Out comeOut come
Poor result with increasing agePoor result with increasing age
Foot deformityFoot deformity
Stabilized orStabilized or
Worsen after surgery due to muscle weaknessWorsen after surgery due to muscle weakness
or imbalanceor imbalance
Retethering by scar 15 %Retethering by scar 15 %