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Clin Med Fall 2015 > Myelodysplasia test 3 > Flashcards

Myelodysplasia test 3 Flashcards

1
Q

Myelodysplasia general definition

A

-defective development of any part (especially lower segments) of the spinal cord ~ thoracic, lumbar,sacral
-congenital neural tube defect (NTD)
(lack closure of tubes and deformities of skeletal structures)

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2
Q

Meningomyelocoele

A
  • defective closure of the neural tube

- incomplete development of the neural arches

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3
Q

Pathophysiology

A

-degree of resulting dysfunction is related to the anatomical level of the defect
-usually present w/ loss of neurological function (sensory and motor) below the level of the lesion
(children born w/ spina bifida likely have hydrocephalus as well)

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4
Q

Eitology

A

Multifactorial: possible environmental and genetic causes:

  • folic acid deficiency*( impt in first 6 weeks)
  • lower SES
  • teratogenic agents
  • hyperthermia during early pregnancy( fever, hot tub)
  • Vitamin A deficiency
  • Rh factor
  • Alcohol ingestion
  • genetic link* ; parents w/ one child w/ myelodysplasia have a 50 times higher chance of having another sibling affected)
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5
Q

Pathophysiogy

A

-embryo(20 days after conception) neural groove–> neural crest–> closure of neural tube
-day 23 completely closed except for hole at top-> brain, and hole at bottom-> spinal cord
-also errors in development of vertebral architecture
(lack of closure of vertebral arches)

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6
Q

Incidence

A

neural tube defects occur in 1-2 cases per 1,000 live births

  • 6,000 to 10,000 children born each yr in US
  • females >males
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7
Q

diagnosis

A
  • prenatal detection
    • ultrasound scanning is able to detect NTD prenatally
    • Serum alpha-fetoprotein(AFP) testing ( produced by infants at 10-13 weeks, w/ Meningomyelocoele-increased AFP, in circulatory system ->placenta->mother)
  • Prental diagnosis-> lanned cesarean section to avoid trauma to neural sac during vaginal birth
  • post natal detection- observation( except occult)
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8
Q

Myelodysplasia-Types

A
  • Occulta (hidden, not visible)
  • Aperta( cystica)
  • myleomenigocele
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9
Q

Occulta

A
  • failure of one or more vertebral arches to meet and fuse in 3rd month of development (cord lacks pot. protection)
  • spinal cord and meninges are unharmed and remain in the vertebral canal
  • midline over area
  • bony defect covered by skin
  • most common in the lumbosacaral region
  • no disturbance in neurological or musculoskeletal function
  • may present w/ depression or dimple, cafe au lait spot, soft fatty deposit or tuft of hair (fawns beard)
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10
Q

Aperta-Cystica

A
  • neural tube and vertebral arches fail to close appropriately
  • there is cystic protrusion of material through defective arches
  • 2 types:
    • meningocele (herniation of meningeal sac)
    • myleomeningocele ( herniation of meninges, cord and nerves in sac)
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11
Q

Myelodysplasia-meningocele

A

-protrusion of meninges and CSF into cystic sac
-spinal cord remains within vertebral column
-may exhibit some abnormalities but less common
(minor losses in sensation/strength (vary w/ levels_typically none)

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12
Q

Myelodysplasia- Myelomenigocele

A

-protrusion of both spinal cord and meninges into cystic sac
-can be open or closed
-closed: covered w/ a combination of skin and membranes
( meningoceles and closed myelomeningoceles are most common at the thoracic and lumbosacral areas)
-Open: nerve roots and spinal cord may be exposed with dura and skin at edge of lesion
(2/3rds of open type occur at T-L junction, increased risk of infection or further damage(open to environment)

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13
Q

myelodysplaasia other types

A
  • lipoma: fatty tumor on spinal cord- within tissue( likely not visible, arches closed, pain/motor/sensory changes over time)
  • myelocystocele: cystic like tumor of spinal cored( fluid filled)( likely not visible, arches closed, pain/motor /sensory changes over time)
  • anencephaley-failure of closure of cranial end of neural tube ( some brain tissue may be evident but forebrain is usually absent, lack of sustained life)
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14
Q

Clinical Pic of Occulta

A
  • usually does not cause neurological dysfunction

- occasional disturbances in bowel and bladder function or foot function (depends on level, most common in L-S level)

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15
Q

Clinical Picture of Myelomeningocele

A

-motor dysfunction: myotomal loss
-sensory loss: dermatomal loss
- skeletal deformity: lack of formation of arches(not complete vertebrae-hemi)
-hydrocephalus: 90% develop over time
-MR: low percentage
-LD: high percentage
-motor function prognosis-depends on level of lesion
-pt presents as if he or she has a dx & presentation consistent w/ a pt who has a SCI
(not recovery of functions~compensations)

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16
Q

medical management of Myelodysplasia

A

team of physcians and healthcare providers

  • surgical repair; prenatal and postnatal
  • postnatal
    • closure usually within 72 hrs
    • place neural tissue into vertebral canal
    • cover and repair vertebral defect
    • achieve a flat , watertight closure of the thecal sac ( no leaking of CSF)
17
Q

medical management of Myelodysplasia- surgical outcomes

A
  • 10% of infants recover after surgery and are d/c w/out further medical complications
  • 90% begin to develop hydrocephalus from the next few days to weeks
  • can develop tethered cord( if scared down) later as a result and could experience similar signs and symptoms as Arnold Chiari- also would require surgery( cord unable to move within canal)
18
Q

Hydrocephalus

A

abnormal accumulation of CSF in cranial vault caused by:
- overproduction of CSF
-failure of absorption of CSF
-obstruction in normal flow of CSF through brain and spinal cord
accumulation of CSF causes increase ICP, can lead to cerebral damage & celular death
(increased fluid within ventricles(dilation of ventricles))

19
Q

signs and symptoms of hydrocephalus

A
  • full, bulging, tense soft soft (fontanel) on top of the child’s head
  • large prominent veins on the scalp
  • “setting sun sign”-child appears to only look down, whites of the eyes are obvious above the colored portion of the eyes
  • behavioral changes( irritability, lethargy)
  • high pitched cry
  • seizures
  • vomiting or change in appetite
20
Q

Management of Hydrocephalus

A
  • shunt thin tube with a small pump attached which diverts CSF from lateral ventricles to a location where the fluid can be managed
  • ventriculoperitoneal is most common, ventriculo-atrial also possible
  • need permanent solution (pump fluid out)
  • often goes behind ear (protected/out of the way)
  • over time shunt may need to be revised due to growth or clogged/kinked tube or malfunction of shunt
21
Q

Shunt malfunction: Signs and Symptoms

A
  • firm fontanels
  • listlessness, drowsiness,irritability
  • vomiting, change in appetite
  • increasing head circumference
  • swelling along trunk( where tube is in place due to infection)
  • distribance of bowel or bladder patterns
  • seizures
  • older child/adult; headaches, blurring vision, seizures, decrease in school performance, decrease in sensory and motor functions
22
Q

Arnold Chiari Malfunction

A

-defect in formation of brainstem
-hindbrain is displaced through the foramen magnum
-frequently contributes to the development of hydrocephalus
(brainstem/ CN symptoms, life threatening, CSF unable to circulate properly to cord, may not be dx’d until adult, can occur with tetheredcord)

23
Q

Signs of Arnold Chiari Malfunction

A

hindbrain dysfunction and cranial nerve involvement

  • feeding difficulties
  • choking
  • pooling of secretions
  • repeated aspirations, apnea, neck pain, vocal cord paralysis
  • stridor breathing (noisy breathing)
  • UE weakness, spasticity, weakness, incoordination
24
Q

Signs and Symptoms of tethered cord

A
  • scoliosis (sudden onset)
  • increased spasticity (babinski, mm stretch reflex)
  • increased asymmterical postures or movements
  • altered gait pattern( if affecting LE innervation)
  • decreased UE coordination
  • changes in mm. strength at or below lesion
  • back pain

Clin Med Fall 2015 (10 decks)

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