CONGENITAL

ANOMALIES
By: Esperancita A. Ferrer RN
MD
 CEREBROSPINAL FLUID
A clear and colorless fluid
Produced by the choroid plexus
Ave: 100-150ml
Rate of production 500 ml/d
Function:
 Homeostastis, maintains balance w.
brain’s ECF
 Buoyancy to ↓ weight of brain on the skull &
to cushion from impact w/ bone of skull
 Drains unwanted substance
Route of CSF Production to
Clearance Choroid Plexus
↓
Lateral ventricles
↓
Interventricular foramen of Monroe
↓
3 Ventricle
rd

↓
Aqueduct of Sylvius
↓
4rth Ventricle
↓
Foramen of Luschka & Magendie
↓
Subarachnoid space
↓
Arachnoid granulations
↓
Dural sinus
↓
Venous Drainage
 Hydrocephalus
A condition of altered production,
flow or absorption of CSF.
 Abnormal increase in CSF volume of
the circulating CSF within the
intracranial cavity & by enlargement
of the head in infancy.
 Classification
 Non Communicating: obstruction in the
system between the source of CSF
production (ventricles) & the area of
reabsorption (the subarachnoid space)
 Causes:
1. Mass
2. Inflammation
3. Congenital malformation (eg.
Aqueductal stenosis)
 Classification
 Communicating: occurs when CSF is
not effectively reabsorbed through
the arachnoid villi or excessive
production of CSF – tumor or
unkown cause
Causes:
1. Subarachnoid hemorrhage
2. Scarring as a result of infection
Pathophysiologic Mechanism

EXTRAVENTRICULAR INTRAVENTICULAR
Communicating Non-Communicating

CSF accumulates
CSF not absorbed
as a result of
in the sub-
blockage in the
arachnoid villi
ventricles

Ventricular
Dilatation

Surface
Dilatation

Sutures
Splits

Increase in the
Size of Head
Clinical Manifestations
 Excessive Head
growth
 Delayed closure of
anterior fontanelle
 Fontanelle tense &
elevated above the
surface of skull
 Increased ICP
 Vomiting  Possibleseizures
 Irritability  Lethargy
 High pitched shrill  Stupor
cry  Coma
 Tense bulging
fontanelle
 Changes in VS:

↑ sys BP, ↓PR, ↓RR, ↑T

 Pupillary Changes
 Papilledema
 Alteration of ms. Tone
including clonus &
spasticity
 Delayed motor skills
 Mental retardation
 Feeding Problems
 Setting sun eyes
 Diagnostic Evaluation
 CT scan diagnostic  Ventriculography
tool of choice Air is introduced
 Opthalmoscopy directly into the
reveals papildema lateral ventricles
through trephine
 Percusion of the
openings (burr holes)
infant’s skull into the skull, X-ray
“cracked pot” films are taken
sound (Macewen’s Performed in OR
sign)
Management
 Ventriculoperitonea
l shunt
Diverts CSF
from a lateral or
the spinal
subarachnoid
space to the
peritoneal cavity
 Complication:
Occlusion,
Infection,
Malfunction
 Nursing Assessment
 HC occipitofrontal circumference
 Palpate fontanelle
 Pupillary response
 LOC
 Breathing pattern
 Feeding pattern
 Motor function, gait, coordination
 Attainment of developmental milestones
 Nursing Diagnosis
 Ineffective Cerebral Tissue Perfusion
 Imbalance Nutrition: Less than body
req’t
 Risk for Impaired Skin Integrity
 Risk for Injury
 Risk for Infection
 Nursing Interventions
MAINTAINING
CEREBRAL
PERFUSION
 Observe ↑ ICP
Brainstem herniation –
opisthotonic posturing  Sedatives CI ↑ ICP
 Observe for respiratory predisposes to
depression- sedatives hypoventilation or
administered respiratory arrest
ADEQUATE MAINTAINING SKIN
NUTRITION INTEGRITY
 Hold in semi-sitting  Prevent pressure
position w/ head sores – egg crate
supported. Allow mattress
ample time  Scalp clean & dry
burping.
 Frequent turning
 Small frequent
 Passive ROM
feedings
 After feeding- side
w/ head elevated
Spina Bifida (Spinal
dysraphia)
 Malformation of the
spine
 Posterior portion of
the laminae fails to
close
 Arrest in formation
of vertebral arches
& SC that occurs
between 4th & 6th wk
of embryogenesis
TYPES
Spina Bifida Occulta
 Defect only in vertebrae
 SC & meninges normal

Clinical Manifestations:
 Dimple in skin
 Growth of hair over
malformed vertebrae
 Foot weakness, bowel,
bladder sphincter
distrbances
Meningocele
 Meninges protrude through the
opening in the spinal canal
 Forms a cyst filled with CSF
covered w/ skin
Clinical Manifestations:
 Seen midline
 Cord & nerve roots normal
 Seldom evidence of weakness
of the legs or lack of shincter
control
Myelomengocele
 Spinal cord & cord membranes
protrude through the defect in the
laminae of the vertebral column
Clinical Manifestations:
 Loss of motor control & sensation
below level of lesion
 Bladder dysfunction- incontinence,
UTI inc emptying
 Fecal Incontinence & constipation
 Developmental disabilities
 Muskuloskeletal Deformities
 Club
Feet , dislocated hips, Kyphosis
Management:
ROM exercises
Management Nursing Assessment
 Laminectomy  Sensory motor
 Closure of lesion, response LE
removal of sac done  Ability to void
after birth spontaneously,
retention of urine,
Sx of UTI
 Usual stooling
pattern,
constipation
 Mobility
ETIOLOGY DIAGNOSTIC
 Unknown EVALUATION
 Genetic Prenatal Detection
predisposition USG, Amniocentesis
 Drugs: Valproic Diagnosis made
Acid taken during Clinically
pregnancy CT Scan MRI evaluate
 Folate deficiency further
 Nursing Diagnosis
 Risk for Impaired skin integrity r/t
impaired motor & sensory function
 Risk for Infection
 Impaired Urinary/BowelElimination
 Ineffective Tissue Perfusion: Cerebral
r/t potential hydrocephalus
Nursing Interventions
PROTECTING SKIN INTEGRITY
 Prone position w/ slips slightly flexed
 Avoid covering w/ diaper
 Observe for irritation & leakage of CSF
 Meticulous skin care
 Passive ROM
 Foam or fleece pad
 During feeding hold infant w/ elbow rotated to
avoid touching the sac or feed while in side lying
position or prone on lap
 Prone to LATEX ALLERGY: Limit or prevent direct
contact of the child to produce routinely that
contain latex. Latex products include: BP cuffs,
tourniquets, tape indwelling catheter, gloves IV
tubing injection ports
PREVENTING INFECTION
 MC caused by contamination w/ urine &
feces - keep clean
 No diaper if loc lower spine
 Apply a sterile gauze pad or sterile
moistened dressing over sac – change
frequently
 Monitor S/Sx Infection:

Oozing of fluid or pus from the sac

Fever
Irritability listlessness
Seizure
ACHIEVING CONTINENCE ACHIEVING REGULAR
 Clean intermittent self
BOWEL ELIMINATION
catheterization
 Toileting schedule
 Children 6-7 self
catheterization, younger  Stool softeners,
parents suppositories or
 Silicone Catheter enema, psyllium
 Medications: imipramine (Metamucil)
(Tofranil) ephedrine  Encourage intake
used to help children of high fiber, ↑ OFI
retain urine than
dribbling
 Used combination- dry
3-4 hrs