seminar of pediatrics

1. Presented by- Yogesh Dengale
II MSc CON PIMS LONI

2.  Meningomyelocoele is the most severe type of SPINA
BIFIDA.
 Spina Bifida means "split spine”
 4 types of Spina Bifida are:
◦ Spina bifida occulta,
◦ Meningocele
◦ Spina bifida cystica (Myelomeningocele) and
◦ Lipomeningocele

3.  Meningomyelocoele
◦ occurs due to Failure of closure of the neural tube
during the third week of gestation
◦ abnormal differentiation of the embryonic neural
tube
 In Meningomyelocoele
A cystic swelling occurs over the site of the spinal
defect which contains meninges, nerve roots, and the
spinal cord itself which has left the vertebral canal

5.  1-2/1000 live birth
 Neural tube defects are second most common type of
birth defect
 Myelomeningocele is the most common form of
neural tube defect.
 Slightly higher in females than in males (1.2:1)
 85% occur in lumbosacral
 region

6.  Poor nutrition - Folic acid deficiency
 Genetics (People of Northern European and
Hungarian ancestry have the highest rates of the
disease)
 Chromosome abnormalities:
◦ Trisomies 13 and 18
◦ Triploidy
◦ Single gene mutations

7.  Maternal obesity, hyperthermia
 pregnancies complicated by diabetes at conception
 Drugs: valproate, carbamazepine and drugs to induce
ovulation
 Maternal exposures to fumonisins, electromagnetic
fields, hazardous waste sites, disinfection by-products
found in drinking water and pesticides

8.  Spina bifida is caused by the failure of the neural tube
to close during the first month
of embryonic development (often before the mother
knows she is pregnant).
 Under normal circumstances, the closure of the
neural tube occurs around the 23rd (rostral closure)
and 27th (caudal closure) day after fertilization.

10. Due to risk factor
Failure of NTD to close during 1st month of embryonic
development
Increase central nervous system pressure
Fails to close properly
NTD occur

11.  Both meninges and spinal cord protrude into the skin
of the back
 Leak of cerebrospinal fluid (CSF) is common
 Severe neurological deficits are common
 Risk for bacterial meningitis
 Paraplegia
 Diminished control of lower limbs, bladder and
bowel
 Hydrocephalus often accompanies
 Short stature and precocious puberty

12.  Orthopedic abnormalities (i.e., club foot, hip
dislocation)
 Bladder and bowel control problems, including
incontinence, urinary tract infections, and poor renal
function.
 Pressure sores and skin irritations
 Abnormal eye movement
 68% of children with spina bifida have
an allergy to latex
 Paralysis

13.  Scoliosis
 Back pain
 Partial or complete lack of sensation
 Weakness of the hips, legs, or feet of a newborn
 Other symptoms may include:
 Hair at the back part of the pelvis called the sacral
area
 Dimpling of the sacral area
 Difficulty swallowing, which can lead to choking.
 Hoarseness.
 Breath-holding and problems breathing during sleep.
 Below-average intelligence.

14. Primary Functional deficits System Congenital Anomalies
 Lower limb paralysis
 Sensory loss
 Bladder and bowel
dysfunction
 Cognitive dysfunction
 Cranial nerve dysfunction
◦ Ocular muscle palsies,
◦ Swallowing andeating
problems and
◦ Abnormal phonation
 Facial clefts
 Heart malformations, and
 Genitourinary tract
anomalies
 Urinary tract anomalies
◦ solitary kidney or
◦ malformed ureters

15.  Ultrasound during the second trimester
 The diagnosis of myelomeningocele is certain when 3
classic central findings are present in ultrasonography
◦ concavity of the frontal bones,
◦ ventriculomegaly, and
◦ Chiari II malformation.
 Positive screening for maternal serum alpha-
fetoprotein (AFP)
 Screening of the amniotic fluid for AFP, as well as for
the presence of acetylcholinesterase

16.  Intrauterine surgery
 Post-natal surgery:- Closure of the
Myelomeningocele is performed immediately after
birth if external CSF leakage is present and typically
within the first 24-48 hours in the absence of CSF
leakage
 Multidisciplinary interventions needed to prevent
progressive deterioration of multiple body systems
 Treatment team consists of pediatric specialists in
◦ Physical medicine and rehabilitation
◦ Neurosurgery
◦ Urology

17. ◦ Orthopedics along with
◦ Pediatric nursing
◦ Physical therapy
◦ occupational and recreational therapy
◦ psychology and
◦ medical social work

18.  Anticholinergics: - to suppress detrusor overactivity
eg. Oxybutynin chloride
 Tricyclic antidepressants, eg. Imipramine
hydrochloride
 Alpha-adrenergic antagonists: - decrease bladder
outlet resistance, increase urinary flow rate, and
improve bladder emptying eg. Terazosin

19.  Place the child in prone position.
 Cover the affected area with sterile gauze piece
dipped in normal saline.
 Maintain hydration.
 Monitor for associated defects.

20.  Catheters
 Braces
 High fiber diet
 Antibiotics may be used to treat or prevent infections
such as meningitis or urinary tract infections.

21.  Initial nursing management of the child with
myelomeningocele involves preventing trauma to the
meningeal sac and preventing infection before
surgical repair of the defect.
 Preventing Infection
 Promoting Urinary Elimination
 Assessing urinary function
 Performing clean intermittent catheterization

22.  Dietary supplementation with folic acid has been
shown to be helpful in reducing the incidence of
spina bifida. Sources of folic acid include whole
grains, fortified breakfast cereals, dried beans, leaf
vegetables and fruits.
 It is recommended that any woman considering
becoming pregnant take 0.4 mg of folic acid a day.
Pregnant women need 1 mg per day.

23.  Difficult delivery with problems resulting from a
traumatic birth, including cerebral palsy and
decreased oxygen to the brain
 Frequent urinary tract infections
 Hydrocephalus
 Loss of bowel or bladder control
 Meningitis
 Permanent weakness or paralysis of legs

24.  Many individuals with spina bifida have an
associated abnormality of the cerebellum, called
the Arnold Chiari II malformation. In affected
individuals, the back portion of the brain is displaced
from the back of the skull down into the upper neck.

25.  Specific areas of difficulty in some individuals
include planning, organizing, initiating, and working
memory. Problem-solving, abstraction, and visual
planning may also be impaired.
 Children with spina bifida and shunted hydrocephalus
have higher rates of ADHD.

26.  Individuals with spina bifida may struggle
academically, especially in the subjects
of mathematics and reading. In one study, 60% of
children with spina bifida were diagnosed with a
learning disability.

27.  Compared to typically developing children, youths
with spina bifida may have fewer friends and spend
less time with peers.