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Case Presentation Meningomyelocele

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... = meninges + CSF Meningomyelocele = meninges + neural tissue + CSF Myelocele = neural tissue Neural Tube Defects Spina Bifida Latin split spine ... – PowerPoint PPT presentation

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Title: Case Presentation Meningomyelocele


1
Case Presentation Meningomyelocele
  • Moderator Dr. Shende
  • Presenter Dr. Puneet

www.anaesthesia.co.in anaesthesia.co.in_at_gmail.co
m
2
Case
  • Name B/o Ashma Praveen
  • Age 2 days
  • Sex Male
  • Place Delhi
  • Informant Mother

3
Chief complaints
  • Swelling in the back since birth

History of presenting illness
  • Swelling in the back since birth, not increasing
    in size, no discharge from the swelling
  • No h/o fever, running nose and laboured breathing
  • No h/o seizures, increase in head size,
    involuntary movements
  • No h/o irritability, vomiting (accepting feed
    normally)
  • No h/o bowel and bladder incontinence

4
  • Past history
  • No h/o yellowish discoloration or bluish
    discoloration of skin
  • No h/o seizures, apenic or cyanotic spells

5
  • Antenatal history
  • Primi, no h/o fever with rash during 1st
    trimester, radiation exposure during pregnancy
  • No h/o drug intake except iron and folic acid
    supplement
  • Birth history
  • Born at term by elective LSCS (short stature)
  • Cried immediately after birth, passed meconium
    and urine on D1
  • H/o moving all the four limbs
  • Birth weight 2.5 kg

6
Examination
  • Child conscious, lying quietly
  • Moving all 4 limbs
  • Afebrile
  • No pallor, icterus, cyanosis
  • No signs of dehydration
  • Anterior fontanelle full
  • Airway
  • No obvious facial deformity
  • Head size -

7
  • Vital signs
  • HR 130/min
  • RR 40/min
  • Capillary refill time lt3 seconds
  • IV line present

8
Systemic examination
  • Spine
  • Swelling in thoracic region (T8-T12)
  • 2cm in diameter, soft, cystic, compressible,
    bluish discoloration covered with normal skin
  • CNS
  • Moving all four limbs
  • Tone normal
  • CVS - S1S2 , no murmur
  • RS - B/L air entry clinically clear
  • P/A Soft

9
Provisional diagnosis
  • Term neonate with swelling in the back T8-T12
    region with no neurological deficit most
    probably unruptured meningomyelocele

10
Investigations
  • Hb 15.1 g
  • TLC 11,000
  • Platelet 5.3 x 103
  • Blood urea 38
  • Na/K 142/4.3
  • C-X-ray WNL
  • USG head No hydrocephalous
  • CT head and MRI spine

11
Proposed surgery
  • Meningomyelocele excision and repair

12
Anaesthetic considerations
  • Neonatal surgery (airway, thermoregulation)
  • Positioning (do nut for intubation, prone for
    surgery)
  • Volume status (CSF leak, blood loss)
  • Maintenance, analgesia
  • Emergence (postop monitoring)
  • Latex allergy prophylaxis

13
Preoperative management
  • Informed and written parental consent
  • NPO x 4 hours for breast milk
  • Maintenance IV fluids to continue
  • Premedication none
  • Anesthesia machine check
  • Emergency drugs
  • Monitors

14
Preoperative management (contd)
  • Induction preoxygenation, fentanyl (5µg)
    thiopentone (12.5mg) after confirming ability to
    ventilate Atracurium 1.5 mg
  • IPPV
  • Airway secured with 3.0 uncuffed ETT fixed at 9
    cm
  • Maintenance O2 N2O isoflurane, fentanyl,
    atracurium

15
Neural Tube Defects
  • Meningocele meninges CSF
  • Meningomyelocele meninges neural tissue CSF
  • Myelocele neural tissue

16
(No Transcript)
17
Neural Tube Defects
  • Spina Bifida Latin split spine
  • Failure in closure of the neural tube during
    early embryologic development
  • Trilaminar embryo day 16-17 gestation
  • Endoderm ? gut
  • Mesoderm ? muscle and skeletal system
  • Ectoderm ? skin nervous tissue
  • Neural tube formation days 18-27
  • Neural folds fuse in midline days 22-23
  • Disjunction of superficial ectoderm from neural
    tube
  • Failure of any step ? dysraphia ? maldevelopment
    of the neural structures and adjacent mesodermal
    structures

18
Neural Tube Defects
19
Meningomyelocele
20
Etiology
  • Nutritional deficiency / Metabolic defect
  • Folate 0.4mg/day US Public Health Service
  • Decrease risk of NTD by 70
  • Teratogens
  • Valproic acid, carbamezapine, cytochalasins,
    calcium channel blockers
  • Genetics
  • Lower risk in blacks(13000), increased risk
    caucasian (11000)
  • Increased risk if previous child with NTD
  • 1 affected child risk2-5
  • 2 affected children risk 10-15
  • Non-Mendelian transmission
  • Point mutations in folate receptor genes C677G,
    A127T
  • MTHF and CT677 mutations ? abnormal maternal
    folate metabolism

21
Diagnosis
  • Pre-natal ultrasound 18wk GA
  • Inter-operator variability
  • Sensitivity increases with risk of NTD
  • 60 low risk, 89 high risk
  • a-Fetoprotein levels
  • Sensitivity 64
  • Non-specific
  • Combined 86 sensitivity
  • Br J Obstet Gynaecol. 1995 May102(5)370-6.

22
MMC Effects by Organ System
  • CNS
  • Open defect
  • ? CSF leak
  • ? High infection risk
  • ? Damage to exposed neural tissue
  • Depends on level of lesion
  • Flaccid paralysis below lesion
  • Sensory deficits
  • Bladder / Bowel dysfunction
  • Hydrocephalus (15 at primary closure ? 90 post
    closure)
  • VP shunting
  • Arnold-Chiari Type II malformation
  • Small posterior fossa
  • Caudal displacement of cerebellum, medulla, and
    pons into cervical canal

23
Arnold-Chiari Malformation Type II
  • 80 incidence with MMC
  • 20 develop symptoms of brainstem dysfunction
  • Dysphagia
  • Poor feeding
  • Recurrent Aspiration
  • Vocal cord paralysis
  • Stridor
  • Apnea
  • Abnormal responses to hypoxia, hypercapnea
  • BEWARE extreme head flexion ? brainstem
    compression

24
  • Pulmonary
  • Sequellae of recurrent aspiration
  • Possible restrictive pulmonary function
    post-closure
  • Kyphoscoliosis
  • CVS
  • Congenital cardiac defects 40, FgtM
  • Secundum ASD most common then VSD
  • GI
  • Neurogenic bowel dysfunction / incontinence

25
  • GU
  • Bladder extrophy
  • Neurogenic bladder dysfunction
  • Urinary incontinence/obstruction
  • Recurrent UTIs/pyelonephritis
  • Bladder dysfunction ? chronic vesico-ureteric
    reflux
  • Renal dysfunction ? ESRD
  • MSK
  • Kyphoscoliosis
  • Clubfoot
  • Hip dislocation
  • Flexion Contractures
  • IMMUNE
  • High risk population for latex allergy

26
Latex Allergy
  • Tosi et al. 1993 J Pediatr Orthop reported 40
    serologic evidence of sensitivity
  • Pittman et al. 1995 Pediatr Neuro Surg 50 IgE
    latex antibodies
  • Exact cause unknown
  • Sensitization due to early and repeated exposure
    with many medical procedures
  • Spectrum from localized dermatitis,
    conjunctivitis, rhinorrhea, to anaphylactic shock

27
  • Latex free medical care
  • Medic Alert bracelet if sensitized
  • High suspicion for latex allergy and anaphylaxis

28
  • Pre-Op Assessment
  • Pre-natal hx, method of delivery, complications
  • Gestational Age
  • Medications antibiotics re meningitis
  • Family history
  • Physical Exam
  • Vital signs
  • Neurologic deficits
  • Associated anomalies
  • Volume status ?fluid deficit
  • Vascular access adequate?
  • Investigations
  • CBC, Lytes, Cr, ABG
  • Echocardiogram?
  • Pre-Medication
  • SBE prophylaxis?

29
  • Latex free anesthetic gloves, foleys,
    stopcocks, medication stoppers
  • Vascular Access maintenance blood products
    PRN
  • Monitoring routine, bladder catheter /-
    arterial line / CVC PRN
  • Warm Room, warming blanket
  • Positioning
  • Supine or lateral for induction, airway
    management
  • Donut for protection of defect from pressure,
    trauma if supine
  • Prone for repair
  • Induction
  • Inhalational or IV
  • Succinylcholine for intubation not associated
    with ?K
  • Endotracheal intubation armoured ETT

30
  • Maintenance
  • Avoid long acting muscle relaxants neural
    element identification
  • Ventilation may develop restrictive defect with
    large closure
  • Volume status
  • Deficits maintenance fluid
  • Blood loss
  • Replace CSF loss with NS
  • Emergence
  • Confirm adequacy of analgesia
  • Confirm adequate spontaneous ventilation
  • Beware vocal cord paralysis, stridor
  • Be prepared for emergent re-intubation
  • Disposition
  • ICU setting for post-op monitoring - apneas
  • Prone or lateral position
  • Monitoring for CSF leak, infection, hydrocephalus

31
Spinal Anesthesia for Closure of MMC
  • Viscomi et al. Anes Analg 199581492-5
  • Case series
  • n14 neonatal lumbo-sacral MMC closures
  • Prone positioning with chest roll
  • Hyperbaric tetracaine 0.5 injected at caudal
    border
  • Supplemental intrathecal tetracaine infused by
    surgeon PRN for crying related to surgical
    manipulation
  • Fussy infants given pacifier /- midazolam for
    sedation 9/14
  • Post-op apnea monitoring x 36 hrs with impedance
    monitor
  • Results
  • Spinal anesthesia 100 successful
  • Supplemental spinal anesthesia required in 50 _at_
    93/-14 minutes
  • 2 post-op respiratory events correlated to dose
    of midazolam
  • Considerations
  • Contraindicated with local infection
  • Uncontrolled neonatal airway in prone position

32
Primary Closure of MMC
  • Emergency neonatal surgery
  • Goal closure within 24-72hr of delivery
  • Surgical Goals
  • Close dural defect and stop CSF leak
  • Prevent infection
  • Preserve neural function
  • Prevent secondary tethering of spinal cord
  • Surgical Technique
  • Separate neural placode
  • Dissection of the dura
  • Approximation of tissues in midline
  • Closure of dural sac and skin defect (may
    require rotational flap if the defect is large)

33
MMC Other Surgical Procedures
  • VP Shunting
  • Hydrocephalus 15 at primary closure
  • 80-90 post-closure
  • Frequent revisions through childhood
  • Deterioration in neurological condition must
    determine ?ICP vs. brain-stem compression
  • Hindbrain decompression
  • 20 develop symptoms of brainstem dysfunction
  • Dysphagia, poor feeding, choking, aspiration,
    apnea
  • Posterior fossa decompression
  • Genitourinary procedures
  • High incidence of bladder extrophy
  • Vesicoureteral reflux 2 neurogenic bladder
  • Urethral dilation
  • Vesicostomy
  • Orthopedic Revisions
  • Club foot
  • Hip dislocation
  • Spinal kyphosis

34
Fetal Surgery for MMC Closure
  • Animal studies suggest in utero closure of MMC
    defect may
  • mitigate neurological damage
  • decreased incidence of hydrocephalus
  • Two Hit Hypothesis
  • First Hit failure of neuralation ? open defect
  • Second Hit amniotic fluid induced spinal cord
    injury CSF leak through neural tube defect

35
Fetal Surgery for MMC Closure
  • Fetal risks
  • Mortality 4
  • Increased risk of
  • Oligohydramnios
  • Pre-term delivery
  • Low birth weight
  • Maternal risks
  • Uterine rupture
  • Placental abruption
  • Post-op bowel obstruction

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