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Semester 5 PMNM Exam 10 > Spina Bifida > Flashcards

Spina Bifida Flashcards

1
Q

Embryology Refresh

A

Neural tube closes gradually and should all be closed by 28 days
Closes from middle - up and middle - down
When does not close fully from middle - down is when you see neural tube defect

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2
Q

Open NTD includes

A

Myelomeningocele (SB)

Anencephaly - neural tissue is not covered by skin and is not compatible with life

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3
Q

Closed NTD includes

A

Split spinal cord
Tethered cord
Lipomeningocele
Spina bifida occulta - covered by skin

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4
Q

Open vs. Closed NTD - which has less symptoms and complications

A

Closed

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5
Q

Spina Bifida Occulta can be

A

An incidental finding on an x-ray ; No symptoms

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6
Q

Spina bifida occulta - risk for

A

Tethered cord or other neurological issues

Did not close posteriorly

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7
Q

Sacral Dimple - concern for

A

Underlying anomaly with addition of - hair tuft, birthmark, mass, deep dimple or dimple above the cleft
These findings warrant imaging - usually an US

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8
Q

Algorithm for MRI or Neurosurgical referral

A

Other associated cutaneous finding like:

Hypertrichosis, hemangioma

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9
Q

Algorithm for US or MRI

A

Multiple dimples
Dimple diam above 5mm
Dimple more than 2.5cm above anus
Dimple outside sacrococcygeal region

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10
Q

Tethered Cord - symptoms

A

Clumsy gait
Abnormal bladder function
Foot/leg abnormality - pes cavus, limb undergrowth
Scoliosis

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11
Q

Tethered Cord - surgery

A

can prevent worsening - might provide improvement

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12
Q

Tethered Cord - why/how limb undergrowth

A

Cord is ending a lot lower than normal and you can get tension on it that damages the nerves and can lead to limb undergrowth

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13
Q

Meningocele

A

Least common form of SB
Meninges may be exposed or may have have skin covering
Neural elements are not exposed

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14
Q

Meningocele - symptoms

A

Less severe than myelomeningocele since they are covered - but still at a risk for tethered cord

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15
Q

Meningomyelocele “Spina Bifida” - how common

A

Occurs in 3.4/10,000 live births

Most common permanently disabling birth defect

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16
Q

Meningomyelocele “Spina Bifida” - cause

A 
Exact cause is unknown
Multifactorial including: 
Genetics (not a single gene defect)
Exposures (medications like anti-seizure, heat)
Nutrition (folic acid)
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17
Q

Meningomyelocele “Spina Bifida” - Folic acid

A

Incidence has decreased with the addition of folic acid to flour
Women who have had a child with a NTD are recommended to take more folic acid (4mg daily) than other pregnant women (0.4 mg)

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18
Q

Prenatal diagnosis

A

Maternal serum alpha fetoprotein (AFP) elevated

Prenatal ultrasound

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19
Q

Prenatal intervention

A

MOMS trial - showed that surgery to cover the neural elements in utero resulted in improved motor function and decreased the need for shunting
(still at risk for pre term delivery though - research still being done)

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20
Q

Immediate treatment - if defect is not closed antenatally - then what

A

Surgery is needed in the first day or two of life to close the defect and cover the neural elements
Ongoing exposure would lead to risk to CNS and further nerve damage would be possible

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21
Q

Muscles affected and sensory losses depend on

A

Level of the abnormality

Higher levels will affect all levels below

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22
Q

High lesions =

A

Above L3

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23
Q

High lesions - above L3 - High lumbar spina bifida patients will have

A

Absent quadriceps function and will need extensive orthoses

Generally will require wc for mobility outside the home

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24
Q

High lesions - above L3 - Thoracic level lesions

A

Are unusual

Would result in weak LEs as well as truncal weakness below the level of the lesion

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25
Mid lumbar lesions =
L3 and L4
26
Mid lumbar lesions: L3 and L4
Preserved quad function with diminished glut med and max Trendelenburg gait Community ambulation with forearm crutches, AFOs Often wc for mobility for longer distances
27
High sacral lesions
Weak PFs AFOs Ambulation maintained
28
Low sacral lesions
Normal strength at ankle and above Possibly weakness in intrinsic foot mm Normal gait
29
Lesions at all levels can affect
brain and spinal cord as well as other organ systems These individuals will have normal cognition but their brain is usually abnormal in some way which puts them at risk for other things
30
Brain - what can happen
Hydrocephalus Chiari II malformation Cognition Seizures
31
Brain - Hydrocephalus
Present in 25% at birth | Up to 60% by 12 months of age will require a shunt
32
Brain - Chiari II malformation
Cerebellar tonsils downwardly displaced into spinal canal | Abnormality of the pons and medulla
33
Brain - cognition
Usually normal but specific learning disabilities and difficulty with executive function and attn are common
34
Brain - seizures
in up to 20% of patients
35
Ventriculoperitone AL Shunt (VP Shunt)
``` Surgically placed (usually in infancy) Can malfunction and would result in life threatening acutely increased intracranial pressure ```
36
Ventriculoperitone AL Shunt (VP Shunt) - s/s of malformation
Lethargy, irritability, HA, vomit | Might be unique to patient so if parent says something about their s/s, trust them!
37
Ventriculoperitone AL Shunt (VP Shunt) - infection - s/s
Usually occurs in first month of being placed or after a change in it Fever, HA, Vomit, Neck stiffness
38
Chiarai II Malformation
Almost always present in SB to a variable degree Associated callosal dysgenesis and migration defects - likely to cause learning issues Cbm and Brainstem coming down into spinal canal
39
Chiarai II Malformation - can result in
Brainstem dysfunction (hypoventilation, apnea), Swallowing dysfunction, Aspiration, Sudden Death
40
Chiarai II Malformation - tx
``` If needed (15-35% of patients will need tx) - Surgical decompression of the upper spinal cord Some will have it without symptoms ```
41
Tethered cord - who is at risk for tethering
Children with SB as well as with deep/abnormal sacral dimples Radiographically all kinds with SB have tethering - management will depend on symptoms Symptomatic kids will require a surgical release
42
Tethered cord - warning signs
Back or leg pain Decreasing strength/increasing tone Change/decline in BB function New or progressing scoliosis or foot deformities
43
GI complications
``` Sphincter dysfunction Abnormal bowel motility Incontinence of stool Bowel regimens are needed Surgical procedures (cecostomy) might be needed to flush ```
44
Puberty
Precocious Girls with SB have avg. menarche at age 10.9-11.4 (avg. is usually 12.7) Early puberty can result in short stature as the growth plates close early
45
Orthopedic issues - spine
Scoliosis is common | Related to neuromuscular abnormalities
46
Orthopedic issues - spine - how common is scoliosis
Up to 90% of patients with high lesions have significant scoliosis - decreasing incidence with the lower levels
47
Orthopedic issues - spine - tx for the scoliosis
Nonoperative management is preferred | Recovery would be difficult for SB patients with increased risk for wound infection, implant failure, loss of function
48
Orthopedic issues - hip
Strength imbalances affect stability in the hip and can lead to dislocation, subluxation and/or contracture
49
Orthopedic issues - hip - goal
To maintain ROM and strength via therapy Surgical tx is used only for pts with joint issues causing problems with established ambulation and to release contracture (if wc anyways though, likely will not get surgery)
50
Orthopedic issues - feet
95% of babies with SB have foot deformities | Calcaneous, clubfoot, vertical talus, cavovarus)
51
Orthopedic issues - feet - clubfoot can be treated with
Serial casting as it would be in babies without SB | Efficacy is less and recurrence is common though
52
Ortho issues - feet - goal
THat it can be braced and can wear a shoe for ambulation
53
Kidney/Bladder dysfunction
Neurogenic bladder is very common May not empty completely May not be continent UTIs are common and so is Asymptomatic bacteruria
54
Kidney/Bladder - Treatment is aimed at
Continence and avoiding distension/pressure in the bladder which can lead to further kidney damage
55
Kidney/Bladder - Treatment options
``` Clean intermittent catheterization Medications to decrease bladder tone Surgical procedures (appendicovesicostomy/Mitrofanoff operation) ```
56
Skin
Sensation is not normal below level of lesion | Pts must be monitored for pressure injury due to immobility, fit of adaptive equipment, and orthotics
57
Latex allergy
Present in up to 50% of SB patients - until routine avoidance was recommended from birth Avoid latex at all times in all SB patients
58
Red flags - consider a VP shunt malformation
Behavior changes/Lethargy Vomiting Headache These would all lead you to consider a VP shunt malformation
59
Red flags - consider tethered cord
Gait/strength/tone change Progressive or new MSK deformation or back pain Change in BB function These would all lead you to consider tethered cord
60
Red Flags - priority
There can be overlap in symptoms | SHUNT always takes priority since it can be life threatening
61
What type of care is ideal
MULTIDISCIPLINARY!!! | Plenty of significant chronic health issues involving a lot of systems
62
Outcomes
75% survive into young adulthood 82% ind. in ADLs 32% employed
63
Outcomes - causes of early death include
Chiari II malformation | Shunt malformation

Semester 5 PMNM Exam 10 (6 decks)

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