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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
Q

Mid lumbar lesions =

A

L3 and L4

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26
Q

Mid lumbar lesions: L3 and L4

A

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
Q

High sacral lesions

A

Weak PFs
AFOs
Ambulation maintained

28
Q

Low sacral lesions

A

Normal strength at ankle and above
Possibly weakness in intrinsic foot mm
Normal gait

29
Q

Lesions at all levels can affect

A

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
Q

Brain - what can happen

A

Hydrocephalus
Chiari II malformation
Cognition
Seizures

31
Q

Brain - Hydrocephalus

A

Present in 25% at birth

Up to 60% by 12 months of age will require a shunt

32
Q

Brain - Chiari II malformation

A

Cerebellar tonsils downwardly displaced into spinal canal

Abnormality of the pons and medulla

33
Q

Brain - cognition

A

Usually normal but specific learning disabilities and difficulty with executive function and attn are common

34
Q

Brain - seizures

A

in up to 20% of patients

35
Q

Ventriculoperitone AL Shunt (VP Shunt)

A 
Surgically placed (usually in infancy)
Can malfunction and would result in life threatening acutely increased intracranial pressure
36
Q

Ventriculoperitone AL Shunt (VP Shunt) - s/s of malformation

A

Lethargy, irritability, HA, vomit

Might be unique to patient so if parent says something about their s/s, trust them!

37
Q

Ventriculoperitone AL Shunt (VP Shunt) - infection - s/s

A

Usually occurs in first month of being placed or after a change in it
Fever, HA, Vomit, Neck stiffness

38
Q

Chiarai II Malformation

A

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
Q

Chiarai II Malformation - can result in

A

Brainstem dysfunction (hypoventilation, apnea), Swallowing dysfunction, Aspiration, Sudden Death

40
Q

Chiarai II Malformation - tx

A 
If needed (15-35% of patients will need tx) - Surgical decompression of the upper spinal cord
Some will have it without symptoms
41
Q

Tethered cord - who is at risk for tethering

A

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
Q

Tethered cord - warning signs

A

Back or leg pain
Decreasing strength/increasing tone
Change/decline in BB function
New or progressing scoliosis or foot deformities

43
Q

GI complications

A 
Sphincter dysfunction
Abnormal bowel motility
Incontinence of stool 
Bowel regimens are needed
Surgical procedures (cecostomy) might be needed to flush
44
Q

Puberty

A

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
Q

Orthopedic issues - spine

A

Scoliosis is common

Related to neuromuscular abnormalities

46
Q

Orthopedic issues - spine - how common is scoliosis

A

Up to 90% of patients with high lesions have significant scoliosis - decreasing incidence with the lower levels

47
Q

Orthopedic issues - spine - tx for the scoliosis

A

Nonoperative management is preferred

Recovery would be difficult for SB patients with increased risk for wound infection, implant failure, loss of function

48
Q

Orthopedic issues - hip

A

Strength imbalances affect stability in the hip and can lead to dislocation, subluxation and/or contracture

49
Q

Orthopedic issues - hip - goal

A

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
Q

Orthopedic issues - feet

A

95% of babies with SB have foot deformities

Calcaneous, clubfoot, vertical talus, cavovarus)

51
Q

Orthopedic issues - feet - clubfoot can be treated with

A

Serial casting as it would be in babies without SB

Efficacy is less and recurrence is common though

52
Q

Ortho issues - feet - goal

A

THat it can be braced and can wear a shoe for ambulation

53
Q

Kidney/Bladder dysfunction

A

Neurogenic bladder is very common
May not empty completely
May not be continent
UTIs are common and so is Asymptomatic bacteruria

54
Q

Kidney/Bladder - Treatment is aimed at

A

Continence and avoiding distension/pressure in the bladder which can lead to further kidney damage

55
Q

Kidney/Bladder - Treatment options

A 
Clean intermittent catheterization
Medications to decrease bladder tone
Surgical procedures (appendicovesicostomy/Mitrofanoff operation)
56
Q

Skin

A

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
Q

Latex allergy

A

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
Q

Red flags - consider a VP shunt malformation

A

Behavior changes/Lethargy
Vomiting
Headache
These would all lead you to consider a VP shunt malformation

59
Q

Red flags - consider tethered cord

A

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
Q

Red Flags - priority

A

There can be overlap in symptoms

SHUNT always takes priority since it can be life threatening

61
Q

What type of care is ideal

A

MULTIDISCIPLINARY!!!

Plenty of significant chronic health issues involving a lot of systems

62
Q

Outcomes

A

75% survive into young adulthood
82% ind. in ADLs
32% employed

63
Q

Outcomes - causes of early death include

A

Chiari II malformation

Shunt malformation

Semester 5 PMNM Exam 10 (6 decks)

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