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PassPMR - 09 Pediatrics > 3- Spina Bifida > Flashcards

3- Spina Bifida Flashcards

1
Q

Explain SB to a mother πŸ”‘πŸ”‘ OSCE Dr. Haitham 🟦

Define myelodysplasia

How common is Spine Bifida?

How do we diagnose it?

List 4 different types.

A

MYELO-DYSPLASIA

Myelo = Spinal cord

Dysplasia = Abnormal growth

Group of neural tube deficits caused by congenital malformations of the vertebral column and spinal cord.

Primary neurulation is completed by about the 25th-day post-conception.

Ref: 2004 Lancet – Spina bifida.

The neural tube forms the early brain and spine. Spina bifida is when a baby’s spine and the spinal column do not develop properly in the womb, causing a gap in the spine.

Neural tube defects (NTDs) affect 0.5 to 2 per 1000 established pregnancies

Most frequent spinal cord disorder in children

Second most common disabling condition in childhood after cerebral palsy

DIAGNOSIS

  1. Elevated maternal alpha-fetoprotein (AFP) and acetylcholinesterase in the maternal serum and amniotic fluid: 13 to 15 weeks.
  2. Amniocentesis shows elevated amniotic fluid AFP: 16 to 18 weeks
  3. Ultrasound: 16 to 24 weeks (gold standard, >90% reliability)

TYPES

Spina bifida OCCULTA:

Failure of fusion of posterior elements of vertebrae.

Spina bifida CYSTICA:

  1. Meningocele: protruding sac includes meninges and spinal fluid.
  2. Myelomeningocele: protruding sac includes meninges, spinal fluid, and spinal cord.
  3. Myelocele: cystic cavity in front of the anterior wall of the spinal cord.

Cuccurollo 4th Edition Chapter 10 pg791-792

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2
Q

You are meeting 30yo female with her 1st pregnancy of child with spina bifida.

List 15 Potential complications of spina bifida πŸ”‘πŸ”‘

What are associated CNS structural abnormalities/malformations in spina bifida?

A

BRAIN & SPINAL CORD

  1. Malformations forebrain and hindbrain
  2. Intellectual function
    • Three times higher incidence of low IQ scores
    • Higher the lesion, the lower the IQ score
  3. Hydrocephalus
    • Ventriculoperitoneal shunting
    • Complications: infection and obstruction
  4. Arnold Chiari malformation Type II (ACM II)
    • Defined as downward displacement of the medulla and brainstem through the foramen magnum with associated kinking of the brainstem
    • 80-90% of Myelomeningocele
    • Hydrocephalus in 90%
  5. Central respiratory dysfunction
    • Most frequent single cause of death in myelodysplasia
    • Symptoms include stridor, central apnea, and aspiration.
  6. Impaired visual function
    • Strabismus, lateral rectus palsy, and nystagmus
  7. Precocious puberty
    • Secondary to premature activation of the hypothalamic pituitary gonadal axis from increased pressure on the hypothalamus
    • Short stature due to growth hormone deficienc
  8. Syringomyelia β†’ Central Cord
    • Pain and temperature sensory deficits
    • Loss of motor function especially of the UEs
    • Increased spasticity and hyper reflexia, and pain.
  9. Dyscoordination
    • Impaired fine hand coordination and ataxia
  10. Malformation of the urinary system
    • Renal hypoplasia; horseshoe kidney; solitary kidney; ureteral or lower tract anomalies
  11. Neurogenic bladder (UMN or LMN)
    • Urinary incontinence, Hydronephrosis, Detrusor sphincter dyssynergia
  12. Neurogenic bowel (UMN or LMN)
    • Fecal incontinence (80%) from poor rectal tone, absent cutaneous reflex response, and perianal sensory deficit
  13. Tethered cord
    • Increased weakness (55%), scoliosis (51%), pain (32%), orthopedic deformity (11%), and urologic dysfunction (6%).
  14. Splitting (divisions) of the spinal cord
  15. Benign lumbosacral tumors
  16. Skin breakdown
    • Excessive pressure, mental retardation, chronic soiling

MOTOR

  1. Osteoporosis
  2. Charcot joints
  3. Spine
    • Secondary/kyphosis to vertebral anomalies muscle weakness
  4. Hip
    • Hip dislocation and pelvic obliquity
  5. Knee
    • Flexion or extension contractures
    • Flexion contractures ≀20 degrees is well tolerated in ambulation
  6. Feet
    • Equinus, equinovarus, calcaneal, cavus, rocker-bottom feet, Rigid club foot
  7. Obesity
    • Secondary to reduced daily energy expenditure and lower metabolic rate

Cuccurullo 4th Edition Chapter 10 pg795-798

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3
Q

List 4 risk factors other than folate. πŸ”‘πŸ”‘

A

RISK FACTORS

Established

  1. Maternal obesity
  2. Pregestational maternal diabetes
  3. History of previous affected pregnancy with same partner
  4. Exposure to anticonvulsant drugs (valproic acid, carbamazepine)
  5. Exposure to Agent Orange

Suspected

  1. Low vitamin B12
  2. Gestational diabetes
  3. Maternal age <25 or >35
  4. Hyperthermia
  5. Maternal diarrhea
  6. Exposure to agrochemicals and pesticides

Braddom 6th Edition Chapter 48 Myelomeningocele Table 48.1 pg 1031

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4
Q

Prophylaxis for mother with 1 child with spina bifida and wants another child? πŸ”‘πŸ”‘

A

NICE GUIDELINE (UK)

Low Risk (Usual)

400 ΞΌg or 0.4 mg/day before pregnancy and throughout the first trimester

High Risk (Hx NTD, DM, Valproic acid)

4-5 mg/day 1 to 3 months prior to conception and through the first trimester

https://www.nice.org.uk/guidance/ph11/chapter/4-recommendations#folic-acid-2

AMERICAN GUIDELINE (AAP)

4000 micrograms (4mg) one month prior to conception to end of 1st trimester.

Michael A. Alexander Pediatric Rehabilitation - 5th Edition

CANADIAN GUIDELINE (SOCG)

5 mg/day 3 months prior to conception + 3 months post conception.

Afterwards, 0.4 - 1 mg/day folic acid in a multivitamin onwards and postpartum period.

Ref: http://www.sogc.org/media/pdf/advisories/JOGC-dec-07-FOLIC.pdf

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5
Q

Mother of Spina Bifida child asking about expectation in Rolling, Crawling & Sitting πŸ”‘πŸ”‘

A

πŸ’‘ Based on SCI level, thoracic? mid lumbar? or lower lumbar?

6 MONTHS

  • Close to normal as children attain head control and hand play

9-12 MONTHS

  • Delays become obvious
  • Standing requiring adaptive equipment.

ROLLING

  • Normal baby around 3-4 months
  • Spina Bifida roll by 18 months with compensatory strategies

SITTING

  • T12 lesions allow trunk control.
  • Mid-Lumbar usually sit with some delay and increased lordosis
  • L4–L5 is spared, the child can sit normally

CRAWL

  • Mid-Lumbar usually crawl
  • L4-L5 or sacral is spared, the child can crawl normally

Cuccurullo 4th Edition Chapter 10 pg798-799

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6
Q

What are the most important determines/predictors of walking in spina bifida? πŸ”‘πŸ”‘

List 4 Other factors

A

MOST IMPORTANT

  1. Ability to achieve independent sitting (trunk control)
  2. Lesion Level
    1. Iliopsoas strength (hip flexion)
    2. Quadriceps strength (knee stabilization)

OTHER FACTORS

  1. Cognitive ability
  2. Motivation
  3. Developmental Delay
  4. Musculoskeletal complications (Scoliosis - Contracure - Spasticity)
  5. Obesity

Braddom 6th Edition Chapter 48 Spina Bifida pg1042

Cuccurollo 4th Edition Chapter 10 Peds pg800

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7
Q

The typical four goals or objectives in prescribing an orthotic device for a child with MMC are:

A
  1. To prevent deformity.
  2. To support normal joint alignment and mechanics.
  3. To control range of motion during gait.
  4. To improve function.

Braddom 6th Edition Chapter 48 Spina Bifida pg1043

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8
Q

Spina Bifida Lower Thoracic T11–T12.

Ambulation & Assistive Devices πŸ”‘πŸ”‘

A

Muscle Function

  • Intact trunk muscles: Abdominal, paraspinal, and quadratus lumborum function
  • Paralyzed lower limbs: Lack iliopsoas/hip flexion function.

Ambulation

  • WC dependent, No ambulation

Orthoses

  1. THKAFO
  2. RGO (HKAFO)

Equipments

  1. Parapodium
  2. Standing frame
  3. Wheelchair
  4. Walker or Lofstrand crutches (swing gait patter by 4 to 5 years)

Cuccurullo 4th Edition Chapter 10 pg799 Table 10-31

Braddom 6th Edition Chapter 48 Spina Bifida pg1039 Table 48.2

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9
Q

Spina Bifida High Lumbar L1-L2.

Ambulation & Assistive Devices πŸ”‘πŸ”‘

A

Muscle Function

  • L1: Weak Iliopsoas
  • L2: Fair Iliopsoas, sartorius, and adductors

Mobility

  • Limited household ambulation
  • WC dependent for long distance

Orthosis

  1. RGO (HKAFO)
  2. Scott-Craig orthoses (Bilateral KAFOs)

Equipments

  1. Wheelchair
  2. Walker (achieved by 4 to 5 years)
  3. Forearm crutches

https://www.youtube.com/watch?v=VoDVZjAoh7M

https://www.youtube.com/watch?v=Q4oWlINIrAg

Cuccurullo 4th Edition Chapter 10 pg799 Table 10-31

Braddom 6th Edition Chapter 48 Spina Bifida pg1039 Table 48.2

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10
Q

Spina Bifida Mid Lumbar L3-L4. Ambulation & Assistive Devices πŸ”‘πŸ”‘

A

Muscle Function

  • Partial lumbar plexus (L2-L4)
    1. Strong knee extension β†’ Community
    2. L3 Weak quadriceps & hamstring β†’ Buckling
    3. L4 Strong quadriceps, Weak hamstring β†’ unable to flex knee
  • Missing sacral plexus (L4-S2)
    1. Lack hip abduction

Mobility

  • Household ambulation (forearm crutches)
  • Limited community ambulation (WC for outdoor)

Orthosis

  • KAFO: Weak Quadriceps ≀ 3/5, ↑ risk of buckling
  • AFO: Strong Quadriceps 4/5, no risk of buckling

Equipments

  1. Wheelchair
  2. Walker
  3. Forearm crutches

Cuccurullo 4th Edition Chapter 10 pg799-800 Table 10-31

Braddom 6th Edition Chapter 48 Spina Bifida pg1039 Table 48.2

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11
Q

Spina Bifida Low Lumbar L5. Ambulation & Assistive Devices πŸ”‘

A

Muscle Function:

  • Intact Lumbar Plexus L2-L4
    1. L1-2: Strong hip flexion (iliopsoas)
    2. L3: Strong knee extension (quadriceps)
    3. L4: Strong ankle dorsiflexion & inversion (tibialis anterior)
  • Partial Sacral Plexus L4-S2
    1. L5: Strong medial and lateral hamstrings, Tibialis posterior & hip abduction
    2. S1: Lack active hip extension and ankle plantar flexion

Mobility

  • Community ambulation with forearm crutches
  • Wheelchair for long-distance ambulation

Orthosis

  1. AFO β€œL5 = foot-drop”

Equipments

  1. Wheelchair
  2. Forearm crutches

Cuccurullo 4th Edition Chapter 10 pg799-800 Table 10-31

Braddom 6th Edition Chapter 48 Spina Bifida pg1039 Table 48.2

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12
Q

Spina Bifida Sacral S2-S3. Ambulation & Assistive Devices

A

Muscle Function

  • S1
    • Normal ankle dorsiflexion, inversion, eversion
    • Moderate ankle plantar flexion
    • Moderate hip extension
  • S2-S3 Gastrocnemius and soleus

Mobility

  • Community ambulation
  • Partial reliance on crutch or cane in adulthood for long distances

Orthosis

  • Supramalleolar foot orthosis
  • Foot orthosis

Equipments

  1. Crutch or cane

Cuccurullo 4th Edition Chapter 10 pg799-800 Table 10-31

Braddom 6th Edition Chapter 48 Spina Bifida pg1039 Table 48.2

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13
Q

Most frequent single cause of death in myelodysplasia. πŸ”‘πŸ”‘

A

Central respiratory dysfunction

Symptoms include stridor, central apnea, and aspiration.

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14
Q

What are the leading factors affecting morbidity and mortality in individuals with myelomeningocele? πŸ”‘πŸ”‘ EXAM 🟦

A

πŸ’‘ Water everywhere: brain, spinal cord, kidney.

  1. Hydrocephalus
  2. Hydromyelia
  3. Tethered cord
  4. Renal failure
  5. Latex hypersensitivity
  6. Pressure Ulcer
  7. Obesity

PMR Secrets 2nd Edition pg425

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15
Q

Spina Bifida, Types & Definition. What is the clinical relevant? πŸ”‘πŸ”‘

A

Spina bifida OCCULTA:

  • Failure of fusion of posterior elements of vertebrae.

Spina bifida CYSTICA:

  1. Meningocele: protruding sac includes meninges and spinal fluid.
  2. Myelomeningocele: protruding sac includes meninges, spinal fluid, and spinal cord.
  3. Myelocele: cystic cavity in front of anterior wall of spinal cord.

Cuccurollo 4th Edition Chapter 10 pg793

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16
Q

Spina Bifida Occulta

Definition

How common is it?

List 4 Dermatological findings in Spina Bifida

What are some clinical findings?

A

Spina Bifida Occulta

  1. Failure of fusion of the posterior elements of the vertebral body (usually L or LS region).
  2. A normal variant in 5-10% of the population.

SKIN

  1. Dermal sinus (Tunnel)
  2. Sacral dimple (Hole)
  3. Pigmented nevus
  4. Hirsute patch
  5. Angioma (Blood)

CNS

  1. Normal neurological exam.
  2. Sacral lipoma – rare.
  3. Tethered cord – rare.
  4. Occasional bowel and bladder symptoms.
  5. No hydrocephalus.
  6. No Chiari malformation.

Cuccurullo 4th Edition Chapter 10 pg793

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17
Q

How prevalent is latex allergy in spina bifida?

A

20-65% prevalence in spina bifida.

Avoid all latex products in all spina bifida throughout life, as can develop later.

Ref: Braddom pg 1282.

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18
Q

List 4 Dermatological findings in Spina Bifida. Mention the type of SB. πŸ”‘

A
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19
Q

Meningocele. What is it? What location of spine does it affect mainly?

How common is it? Is there a risk of hydrocephalus?

A

DEFINITION

Bony defect of POSTERIOR ELEMENTS of vertebral body with herniation of meninges & CSF (no spinal cord).

LOCATIONS

75% LS, rest thoracic or sacral, rarely cervical.

COMMON

<10% of spina bifida cystica.

CLINICAL:

  1. +/- skin covering, incomplete coverage leads to CSF leak.
  2. normal neurological exam.
  3. no: hydrocephalus or chiari malformation.
  4. need to follow.

Ref: Cuccurullo pg 782.

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20
Q

Myelomeningocele πŸ”‘πŸ”‘ What is it: What location of spine does it affect mainly?

What are the clinical findings? Is there a risk of hydrocephalus?

A

DEFINITION

Bony defect of posterior elements of vertebral body with herniation of meninges and neural elements.

LOCATION

75% LS, rest thoracic or sacral, rarely cervical.

CLINICAL (ACM ll, Hydro & SCI)

  1. Arnold Chiari II malformation.
  2. Hydrocephalus (>90%).
  3. Sensory deficits.
  4. Motor paralysis.
  5. Neurogenic bowel and bladder.

Ref: Cuccurullo pg 782.

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21
Q

Myelocele. Definition?

Where does myelocele rank in terms of severity in spina bifida cystica?

A

Failure of fusion of posterior elements of spine with cystic cavity in front of the anterior wall of the spinal cord (neural elements herniate out of defect).

Most severe form.

Ref: http://emedicine.medscape.com/article/311113-overview

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22
Q

Spina Bifida. List 4 complications in spinal cord. πŸ”‘πŸ”‘

A
  1. Diastematomyelia, diplomyelia, split cord
  2. Tethering
  3. Thick, short film terminale
  4. Lumbosacral tumors (lipoma, fibrolipoma, epidermoid cyst)

Michael A. Alexander Pediatric Rehabilitation 5th Edition Chapter 15 Spina Bifida pg380

23
Q

Spina Bifida. List 4 complications in brain stem. πŸ”‘πŸ”‘

A
  1. Arnold chiari malformation ll
  2. Syringobulbia
  3. Hypoplasia or dysgenesis
  4. Hemorrhage, ischemic necrosis

Michael A. Alexander Pediatric Rehabilitation 5th Edition Chapter 15 Spina Bifida pg380

24
Q

Spina Bifida. List 2 complications in cerebellum. πŸ”‘πŸ”‘ EXAM 2019-2020

A
  1. Arnold chiari malformation ll
  2. Cerebellar dysplasia

Michael A. Alexander Pediatric Rehabilitation 5th Edition Chapter 15 Spina Bifida pg380

25
Q

Spina Bifida. List 2 complications in ventricular system. πŸ”‘

A
  1. Hydrocephalus
  2. Aqueductal stenosis, forking, atresias

Michael A. Alexander Pediatric Rehabilitation 5th Edition Chapter 15 Spina Bifida pg380

26
Q

Spina Bifida. List 2 complications in forebrain

A
  1. Polymicrogyria (Too many small gyri)
  2. Dysgenesis of corpus callosum
  3. Agenesis of olfactory bulbs and tracts
  4. Thalamic fusion

Michael A. Alexander Pediatric Rehabilitation 5th Edition Chapter 15 Spina Bifida pg380

27
Q

List 2 eye complications in SB

A
  1. Strabismus
  2. Lateral rectus palsy
  3. Nystagmus
28
Q

Intellectual function in SB

A
  1. Low IQ scores (higher the lesion, the lower the IQ score)
  2. Concentration and the attention deficit
  3. Repeated CNS complications with meningitis β†’ significant cognitive deficits.
  4. β€œCocktail party personality” represents children with good verbal ability that creates the impression of higher intellectual functioning than is found on formal testing.
29
Q

What is Arnold Chiari Malformation? πŸ”‘ Dr. Haitham

A

Arnold Chiari Malformation Type l

  • Downward displacement of the medulla and brainstem through the foramen magnum
  • Syringomyelia

Arnold Chiari Malformation Type ll

  • Downward displacement of the medulla and brainstem through the foramen magnum with associated kinking of the brainstem (medulla)
  • Hydrocephalus
  • Syringomyelia
  • Associated with myelomeningocele
30
Q

Arnold Chiari Malformation Type 2. List 4 MRI Findings πŸ”‘

A
  1. Hydrocephalus
  2. Fourth ventricle enlargement, easily identified on MRI studies
  3. Compression on brain stem
  4. Inferior displacement of cerebellar tonsils into the spinal canal
31
Q

Signs and Symptoms or Arnold Chiari ll Malformation πŸ”‘πŸ”‘

A
  1. Neck: Torticollis
  2. Cerebellum: Ataxia & Nystagmus
  3. Brainstem
    • Diplopia
    • Dysphagia
  4. Cardiorespiratory
    • Stridor
    • Vocal cord paralysis
    • Intermittent obstructive or central apnea
    • Bradycardia
  5. Spinal Cord: Upper extremity weakness, spasticity

Braddom 6th Edition Chapter 48 Spina Bifida pg1035

32
Q

Arnold Chiari Malformation. List 3 Poor Prognostic Factors.

A

πŸ’‘ High Mortality β†’ Central respiratory dysfunction

Symptoms include stridor, central apnea, and aspiration.

  1. Bilateral vocal cord paralysis
  2. Prolonged apnea
  3. Severe neurogenic dysphagia
33
Q

Hydrocephalus vs Raised intracranial pressure

What is structural abnormality responsible for the upper gaze paralysis?

A

Hydrocephalus

  1. Macrocrania (abnormal increase in the size of the skull)
  2. Bulging fontanelle
  3. Suture diastasis (abnormal widening of the skull sutures)
  4. Enlarged scalp veins
  5. Positive Macewen sign: β€œCracked pot” sound on percussion
  6. Abducent Palsy

Raised intracranial pressure

  1. Sunset eyes / Vertical gaze palsy
    1. Lesion in dorsal midbrain affecting supranuclear pathway: MLF or posterior commissure
  2. Blurred vision.
  3. Recurrent vomiting
  4. Irritability
  5. Decline in intellectual performance
  6. Gradual drowsiness
  7. Respiratory arrest
  8. Coma and death

Braddom 6th Edition Chapter 48 Spina Bifida pg1034

34
Q

List 5 Shunt complications πŸ”‘πŸ”‘

A
  1. Shunt/shunt tract infections.
  2. Blockage
  3. Shunt system failure.
  4. Ventricular catheter malfunction.
  5. Ventricular end/shunt displacement.
  6. Cerebrospinal fluid (CSF) leak from abdominal wound.
  7. Peritoneal catheter/peritoneal end malfunction.
  8. Extrusion of peritoneal catheter through anus.
  9. Colon perforation.
  10. Vaginal performation
  11. CSF pseudocysts peritoneal cavity.
  12. Extrusion of peritoneal catheter from neck, chest, abdominal scar and through umbilicus.

Ref: Afr J Paediatr Surg. 2012 Jan-Apr;9(1):32-9

Ref: PEDIATRICS Vol. 54 No. 6 December 1, 1974 pp. 791 -796.

35
Q

List 6 Possible symptoms of Shunt malfunction πŸ”‘πŸ”‘

A

Braddom 6th Edition Chapter 48 Spina Bifida pg1035 Box 48.2

36
Q

Recovered SB patient c/o upper limb weakness. Diagnosis, Examination & Risk factors. πŸ”‘πŸ”‘

A

SYRINGOMYELIA

  • Fluid-filled central cavity (syrinx) in the spinal cord parenchyma lined with glial cells. Usually occurs in the cervical spine.
  • β€œCommunicating” syringomyelia/syringobulbia refers to cavities with direct communication with the fourth ventricle.
  • Hydromyelia is a dilatation of the central canal by cerebrospinal fluid (CSF) and may be included within the definition of syringomyelia.

CENTRAL CORD SYNDROME

  1. Deficits in pain and temperature sensation (Lateral Spinothalamic Tract)
  2. Preserved light touch, position and vibration sensation (Dorsal Column)
  3. Weakness of hands, Increased spasticity and hyperreflexia (Corticospinal Tract)

CAUSES OF SYRINX

  1. Spina bifida
  2. Spinal cord injury (SCI)
  3. Spinal trauma
  4. Spine tumors
  5. Idiopathic syringomyelia
37
Q

Syringomyelia vs Hydromyelia

A

πŸ’‘ Treat hydrocephalus and other causes of abnormal CSF flow.

Syringomyelia = Sac filled with water = Central Cord

  1. Pain and temperature sensory deficits cape like
  2. Weakness, atrophy, and often fasciculations and hyporeflexia of the hands and arms
  3. Light touch and position and vibration sensation are not affected.
  4. Later, spastic leg weakness develops.

Hydromyelia = Wide spinal canal filled with water = SCI

  1. Rapidly progressive scoliosis
  2. Upper or lower extremities weakness
  3. Decreased coordination
  4. Spasticity.

Ref: Braddom 1281

38
Q

List 6 Symptoms of Tethered cord syndrome πŸ”‘

A

UMN SYNDROME & SCI

  1. Sensory: Pain (32%)
  2. Tone: Increasing spasticity.
  3. Motor
    1. Weakness (54%)
    2. Worsening gait (54%)
    3. Scoliosis (51%)
  4. Bowel & Bladder: Urologic dysfunction (6%)
  5. Special: Scoliosis (51%) & Orthopedic deformity (11%)

Cuccurullo 4th Edition Chapter 10 pg795

Braddom 6th Edition Chapter 48 Spina Bifida pg1036

39
Q

List 4 Causes of Tethered cord syndrome πŸ”‘πŸ”‘ MOCK Dr. Haitham

A

ABNORMAL SPINAL CORD

  1. Spina bifida (myelomeningocele)
  2. Lipomyelomeningocele
  3. Tight filum terminale
  4. Diastematomyelia (split cord)

ABNORMAL STRUCTURES

  1. Postsurgical adhesions
  2. Lipoma

Braddom 6th Edition Chapter 48 Spina Bifida pg1036

Alexander matthews pg 206.

40
Q

Bladder Dysfunction in Spina Bifida: Malformation, Complication & Follow up. πŸ”‘

A

NEUROANATOMY

  • T10–L2: Sympathetic adrenergic innervation
  • S2–S4: Parasympathetic cholinergic innervation
  • S2–S5: Somatic innervation through pudendal plexus

MALFORMATIONS

  1. Solitary kidney (One)
  2. Renal hypoplasia (small)
  3. Horseshoe kidney (fused)
  4. Ureteral or lower tract anomalies

TYPES

  1. Thoracic Lesion β†’ Hypertonic (spastic) bladder
  2. Lumbar Lesion β†’ Detrusor sphincter dyssynergia (50%)
  3. Sacral Lesion β†’ Hypotonic (flaccid) bladder

COMPLICATIONS

  1. Urinary incontinence (95%) β€œFailure to store”
  2. Hypoactive badder wall β€œFailure to pass”
  3. Detrusor sphincter dyssynergia (DSD)
  4. Recurrent UTIs (incomplete empty and urine stasis)
  5. Vesicoureteral reflux
  6. Hydronephrosis

INVESTIGATIONS

  1. Postvoid residual volume
  2. Renal US is used to define anatomy in infancy (2 weeks old)
  3. Voiding cystourethrogram

Cuccurullo 4th Edition Chapter 10 pg796

Braddom 6th Edition Chapter 48 Spina Bifida pg1034

41
Q

Bladder Dysfunction in Spina Bifida: TreatmentπŸ”‘πŸ”‘

Spina bifida, list 3 urologic surgical procedures to treat neurogenic bladder.

A

πŸ’‘ Culture and sensitivities are done initially at 6-week intervals but can be lengthened to 6-month intervals if symptom free.

BEHAVOURAL

  • CredΓ© maneuver should not be used in these patients, it can cause vesicoureteral reflux
  • Fluid = 100ml/kg for 10kg β†’ 50ml/kg for next 10kg β†’ 20ml/kg for 3rd 10kg.
  • Capacity <12yrs = (age +1) x 30

COLLECTING DEVICE

  1. Clean Intermittent Catheterization (CIC)
    • Independent self-catheterization may be achieved at the age of 5 to 6 years
  2. Condom catheters (LMN type)
    • No Vesicoureteral reflux
    • No Large residual volumes

PHARMACOLOGICAL

  1. Anticholinergics
    • Decrease detrusor contractions and enlarge bladder storage capacity
  2. Alpha-adrenergic agents
    • Increase outflow resistance
  3. Botulinum toxin
  4. Antibiotics are not prescribed for positive urine cultures alone.

SURGICAL

  1. Bladder augmentation using the ileum or colon
  2. Suprapubic vesicostomy
  3. Artificial urinary sphincter

Cuccurullo 4th Edition Chapter 10 pg797

Braddom 6th Edition Chapter 48 Spina Bifida pg1036-1037

42
Q

List 2 surgical & 2 non-surgical methods to increase bladder capacity πŸ”‘πŸ”‘

A

NON-SURGICAL

  1. Anticholinergic medications
  2. Botulinum toxin

SURGICAL

  1. Bladder augmentation
  2. Artificial sphincter placement
43
Q

List 4 risk factors of hydronephrosis and 2 ways of management πŸ”‘

A

RISK FOR HYDRONEPHROSIS

  1. Detrusor sphincter dyssynergia
  2. Outflow obstruction (↑ outlet resistance)
  3. Vesicoureteral reflux
  4. Enlarged bladder

MANAGEMENT

  1. Anticholinergic to increase capacity
  2. Clean intermittent catheterization (CIC)
44
Q

List 4 side effects of anticholenergic πŸ”‘

A
45
Q

Spina Bifida: Bowel Dysfunction, List 2 factors for outcome, Management πŸ”‘πŸ”‘

A

SPHINCTER TONE

  • Normal reflex arc in lesions above L2
  • Intact spinal reflex arc can maintain sphincter tone despite absent rectal sensation.

REFLEXS

  1. Recto-colonic defecation reflex: left colonic contraction upon rectal distension
  2. Recto anal inhibitory reflex: relaxation of the anal canal following distention of the rectum.

COMPLICATIONS

  1. Fecal incontinence (80%) from poor rectal tone
  2. Absent cutaneous reflex response
  3. Perianal sensory deficit

PRESENCE = FECAL CONTINENCE

  1. Bulbocavernosus reflex
  2. Anocutaneous reflex (anal wink) reflex

MANAGEMENT

Behavioral

  1. Peristalsis and gastrocolic reflexes are usually intact
    • Timed bowel program initiated by 2 to 3 years
  2. Digital rectal stimulation (UMN Bowel) β†’ intact spinal reflex arc
  3. Manual removal (UMN & LMN Bowel)
  4. Optimize fluid and fiber intake, goal is Bristol Scale 4

Collecting Device

  1. Transanal irrigation system (TAIS)
  2. Anal Plug for LMN Bowel
  3. Suppository insertor and rectal stimulation devices

Pharma

  1. Stool softeners (Docusate sodium; Dulcolax, Colace)
  2. Bulk additives
  3. Suppositories (Glycrine and Bisacodyl)
  4. Sodium Phosphate Enemas.

Surgical

  1. MACE: Antegrade continence enema
  2. Colostomy

Cuccurullo 4th Edition Chapter 10 pg796

Braddom 6th Edition Chapter 48 Spina Bifida pg1037-1038

46
Q

List 6 MSK complications of pediatric SCI

List 6 Musculoskeletal complications associated with spina bifida.

A

MSK

  • Spasticity (UMN), Muscle atrophy (LMN & UMN), Pressure ulcers

SPINE

  • Scoliosis, Kyphosis, Lordosis

HIP

  • Hip subluxation, Dislocation, Dysplasia

ANKLE

  • Flexion or Extension contractures

FOOT

  • Equinus, equinovarus, cavus, rocker bottom deformity, rigid clubfoot

JOINT

  • Contractures, Avascular Necrosis, Septic arthritis

BONE

  • Osteoporosis / osteopenia, Fragility / pathologic fractures, Heterotopic ossification (HO)

Ref: First principles.

Ref: Braddom pg 1285-86

47
Q

Scoliosis in Spina Bifida.

Causes? Predictors? πŸ”‘πŸ”‘

What to think about should there be rapid progression? πŸ”‘πŸ”‘

A

CAUSES

  1. Congenital
    • Vertebral body abnormality
    • Congenital kyphosis
    • Diastomatomyelia
  2. Neuromuscular
    • Weakness & non-ambulation
    • Hydromyelia / syringomyelia

PREDICTORS FOR SCOLIOSIS

  1. Motor level (90% of thoracic SB have scoliosis).
  2. Ambulatory status.
  3. Last intact lamina arch.

RAPID PROGRESSION

  1. Tethered cord
  2. Hydromyelia / syringomyelia

Ref: Braddom pg 1285.

48
Q

List 4 hip complications in SB. πŸ”‘πŸ”‘

A
  1. Hip flexion deformity
  2. Hip flexor spasticity
  3. Hips subluxation
  4. Hip dislocations (unopposed hip flexors and adductors)
  5. Anterior pelvic tilt
  6. Increases lumbar lordosis
  7. Interfere with ambulation

Braddom 6th Edition Chapter 48 Spina Bifida pg1040-1041

49
Q

List 2 knee complications in SB & management.

A

COMPLICATIONS

  1. Knee flexion or extension contractures.
  2. Valgus knee stress during gait

MANAGEMENT

  1. Crutches
  2. Orthotics (AFO & KAFO)

VALGUS KNEE STRESS

  1. Lateral trunk sway
  2. Weak hip abductors
  3. Internal hip rotation
  4. Increased knee flexion
  5. External tibial torsion
  6. Valgus foot deformity.

Braddom 6th Edition Chapter 48 Spina Bifida pg1040

50
Q

List 2 knee complications in SB & management.

A

COMPLICATIONS

  1. Knee flexion or extension contractures.
  2. Valgus knee stress during gait

MANAGEMENT

  1. Crutches
  2. Orthotics (AFO & KAFO)

VALGUS KNEE STRESS

  1. Lateral trunk sway
  2. Weak hip abductors
  3. Internal hip rotation
  4. Increased knee flexion
  5. External tibial torsion
  6. Valgus foot deformity.

Braddom 6th Edition Chapter 48 Spina Bifida pg1040

51
Q

List 2 risk factors/casues for feet deformities. List 2 common findings.

A

RISK FACTORS / CAUSES

  1. Lack of intrauterine movement
  2. Muscle imbalance across the ankle
  3. Lack of sensation and autonomic instability can lead to secondary skin problems and poor wound healing

FINDINGS

  1. Equinus contracture
    • Splinting and passive manipulation
    • Tendo Achilles lengthening
  2. Pes cavus
    • Loss of intrinsic muscle function
    • Foot orthoses or custom-molded shoes
  3. Valgus deformities
    • Poor orthotic tolerance because of pressure over the medial malleolus or the head of the navicular bone
  4. Clubfeet 50%
    • Serial casting is the first line of treatment (Ponseti method)
    • Surgical correction after bearing weight because crawling will lead to clubfeet
  5. Vertical talus
  6. Calcaneal deformities
    • Solid AFOs

Braddom 6th Edition Chapter 48 Spina Bifida pg1041

52
Q

Risk factors for pathological fracture, Invx, DDx & Management.

A

Risk Factors

  1. Immobilization
  2. Muscle weakness
  3. Osteopenia
  4. Insensate extremities
  5. Contractures

Investigations

  1. Nuclear bone scan
  2. Computed tomography scan (CT scan)
  3. Magnetic resonance imaging (MRI)

DDx

  1. Cellulitis
  2. Osteomyelitis
53
Q

Child with spina bifida and a heel ulcer - treatment? πŸ”‘πŸ”‘

A

NUTRITION

  1. Intrinsic factors (nutrition to avoid anemia, increase protein and caloric intake).

PRESURE

  1. Offload heel (seating issues, wheelchair issues, offloading boots, etc).

ULCER

  1. Keep eschar intact and dry.

Ref: First principles.

PassPMR - 09 Pediatrics (5 decks)

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