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Paediatrics > Ch16 Primary Spinal Anomalies > Flashcards

Ch16 Primary Spinal Anomalies Flashcards

1
Q

What is the typical location of a spinal arachnoid cyst?

A

Almost always dorsal. If ventral then think of neurenteric cyst or arachnoiditis

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2
Q

What are the treatment options for arachnoid cysts?

A

Drainage, resection, fenestration and shunting (cysto-peritoneal)

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3
Q

What is spina bifida occulta?

A

Congenital absence of the spinous process +/- other posterior elements with no exposure of the meninges or neural tissue. Incidence = 10%

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4
Q

What is a meningocele?

A

Herniation of the meninges but not neural diffuse through spinal defect

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5
Q

What is a myelomeningocele?

A

Herniation of the meninges and neural tissue through a spinal defect

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6
Q

When does the cranial neuropore close?

A

Day 25

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7
Q

When does the caudal neuropore close?

A

Day 28

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8
Q

What is the incidence of spina bifida (myelomeningocele)?

A

1 in 1,000

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9
Q

What lowers the incidence of myelomeningocele?

A

Folate supplementation

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10
Q

What is the incidence of hydrocephalus with myelomeningocele?

A

80%. Note closure of the defect converts a latent hydrocephalus to a active one as there is no other route for CSF egress

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11
Q

What allergy is commonly found in patients with myelomeningocele?

A

Latex allergy in 75%

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12
Q

What does the MOMS study show?

A

Intrauterine closure of myelomeningocele reduces incidence of hydrocephalus and type 2 chiari.

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13
Q

How do you manage a fetus with spina bifida?

A

Measure size of defect
If ruptured then start abx
Cover with sterile dressing to prevent dessication
Lie patient on stomach to prevent pressure on MM
Closure within 24 hours

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14
Q

Following surgical closure of a myelomeningocele what are good prognostic signs?

A 
Spontaneous movement of the LLs
No chiari 2 (if present check for stridor and apnoeas)
No HCP (do head USS)
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15
Q

Why do patients with sacral myelomeningocele get clawing of the feet?

A

As the foot intrinsics are supplied by S1-3

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16
Q

What other anomalies are associated with myelomeningoceles?

A

Pulmonary immaturity
Bladder dysfunction (need catheterization)
Scoliosis
Hip and knee deformities

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17
Q

What are the key steps of a myelomeningocele repair?

A
  1. Free the placode from the dura to avoid tethering and release the filum terminale
  2. Re-approximate the placode by suturing the pia
  3. Water-tight dural closure
  4. Facia and skin closure
18
Q

What happens if there are any epithelial remnants on the placode?

A

Dermoid cysts arise

19
Q

What is the cause of a CSF leak after a myelomeningocele repair?

A

Hydrocephalus

20
Q

What is the most important factor to rule out when a patient with a myelomeningocele deteriorates?

A

Shunt malfunction

21
Q

What is the benefit of detethering the cord during myelomeningocele repair?

A

May improve scoliosis

22
Q

What is the main cause of mortality in patients with myelomeningocele?

A

Complications associated with the Chiari 2 malformation - aspiration / respiratory arrest etc and shunt failure

23
Q

What are the 3 important spinal dysraphisms associated with lipoma?

A

Intradural lipoma
Lipomyelomeningocele
Lipoma of the filum terminale
These all occur due to early (premature) dysjunction

24
Q

What is the embryological cause of myelomeningoceles?

A

Non-dysjunction (which is part of primary neurulation)

25
Q

What proportion of myelomeningocele patients become ambulatory?

A

50-80% with bracing

26
Q

What are the cutaneous stigmata associated with spina bifida?

A

Fatty pads, port-wine stains, hair tuft, dermal sinus opening or skin appendages

27
Q

What embyrological process causes a dermal sinus?

A

Failure of dysjunction

28
Q

What types of cysts are associated with dermal sinuses?

A

Epidermoid or Dermoid depending on the contents

29
Q

What is the difference between an epidermoid and a dermoid?

A

Epidermoids contain epithelium, Dermoids contain skin and hair.

30
Q

When should dermal sinuses be excised surgically?

A

When above the lumbosacral region; Coccyx sinuses do not need to be treated unless infections occur.

31
Q

What is the management of dermal sinuses?

A

Surgical exploration and full excision prior to neurological deficit or infection (as they cause recurrent meningitis)

32
Q

What is Klippel-Feil syndrome?

A

Congenital fusion of two or more cervical vertebrae

33
Q

What causes Klippel-Feil syndrome?

A

Failure of the normal segmentation of cervical somites between weeks 3-8.

34
Q

What is the clinical triad of Klippel-Feil syndrome?

A
  1. Low posterior hair line
  2. Short neck
  3. Limited neck movement (affects rotation more)
35
Q

What is Sprengel’s deformity?

A

Raised scapula due to failure to descend to normal position (assoc with Klippel-Feil)

36
Q

What is tethered cord syndrome?

A

Abnormally low conus (below L2) - assoc with short thickened filum (>2 mm) or intradural lipoma

37
Q

What are the causes of worsening symptoms with MM?

A

Shunt faliure! Consider tethered cord if painful and syringomyelia if painless

38
Q

How do tethered cords present?

A

May be asymptomatic, pain, foot deformities, scoliosis, leg weakness, urological symptoms and cutaneous stigmata

39
Q

How can the filum be differentiated from a nerve root?

A

Tortuous vessel on the surface of the filum and white appearance.

40
Q

What are the types of spilt cord malformation?

A

Type 1 - two hemicords, each with its own central canal, pia and dura. Have a bony septum. Treated by untethering the cord after removing any bony septums and reconstituting a single tube.

Type 2 - two hemicords within a single dural tube separated by a fibrous medial septum

41
Q

What is the Cannon’s classification for congenital nerve root anomalies?

A

1 - conjoined roots (2 nerve roots within a common dural sheath)
2 - 2 nerve roots exiting through the same foramen
3 - Adjacent nerve roots are separated by an anastomosis

42
Q

What is Ecchordosis physaliphora?

A

Notochordal remnant found in 1-2% of autopsies in the retroclival region. Focal gelatinous mass. Indistinguiable from chrodomas.

Paediatrics (13 decks)

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