1
CHAPTER 14
STUDY GUIDE: THE NEWBORN WITH A PERINATAL INJURIES
OR CONGENITAL MALFORMATION
- Defect present at birth involves the skeletal system; limbs may be missing,
malformed or duplicated
- Some abnormalities (congenital hip dysplasia) are more subtle and the nurse
must be alert to detect them
- Inborn errors of metabolism includes number of inherited diseases that
affects body chemistry (there may be absence or deficiency of a substance
usually an enzyme that is necessary for cell metabolism); cystic fibrosis and
phenylketonuria (PKU)
- In Disorder of the blood, there is a reduced or missing blood
component or an ability of a component to function adequately; sickle
cell disease, thalassemia, and hemophilia.
- Chromosomal abnormalities, involves some type of intellectual
impairment and other are incompatible with life; down syndrome, Turner’s
syndrome or Klinefelter’s syndrome may have impaired physical growth
and sexual development
- Perinatal injuries have many causes and are seen in various form, the most
common of which is premature birth; infections, drugs, maternal disorders,
abnormalities unique to pregnancy (Rh disease, difficult labor or delivery,
premature birth)
MALFORMATION PRESENT AT BIRTH
NERVOUS SYSTEM
Neural Tube Defects:
- Failure of neural tube closure at either the cranial (top) or the caudal
(lower) end of the spinal cord; results in hydrocephalus and spina bifida
Hydrocephalus:
● Increase of CSF within the ventricles of the brian, which causes
pressure changes in the brain and an increase in the head size
● Caused by imbalance between the production and absorption of
CSF or improper formation of the ventricles
● It can be congenital or acquired
● Commonly acquired by an obstruction such as tumor or a sequela of
infection (encephalitis or meningitis)
or perinatal hemorrhage
● Classified by either noncommunicating (obstructive) or communicating
● Noncommunicating hydrocephalus results from the obstruction of
CSF flow from the ventricles of the brian to the subarachnoid space;
Communicating hydrocephalus when CSF is not obstructed in the
, 2
ventricles but is adequately reabsorbed in the subarachnoid space
Manifestations:
● Increase in head size
● If hydrocephalus occurs in utero, the enlarged head will necessitate a
cesarean section delivery
● At birth the head enlarges rapidly and the fontanelles bulge; the cranial
sutures separate to accommodate the enlarging mass; the scalp is shiny
and the veins are dilated
● In advanced cases, the pupils of the eyes may appear to be downward
and the sclera maybe seen above the pupils
● Arnold-Chiari malformation: the 4th ventricle with brain stem protrude
through cervical canal
● The infant is helpless and lethargic; the body becomes thin and the
muscle tone of the extremities is often poor; the cry is shrill and high
pitched; irritability, vomiting and anorexia are present and convulsion
may
occur
● When hydrocephalus occur in older child, the head cannot enlarge bc
the cranial sutures are fused; therefore headache is the predominant
symptom with cognitive slowing, personality changes, spasticity and
other neurological signs
Diagnosis:
, 3
● Transillumination: the inspection of a cavity or an organ by
passing a light through its walls; for visualizing fluid
● A flashlight with a sponge rubber collar is held tightly against the
infant’s head in a dark room; a small ring of light is normal, but a large
halo effect is not
● The child head is measured daily
● Echoencephalography CT scanning and MRI are used to visualize the
enlarged ventricles and to identify the area of obstruction
● A ventricular tap or puncture may be performed using sterile technique,
to determine the pressure and to drain CSF; a specimen is labeled and
sent to the laboratory for analysis
Treatment:
● MED: Acetazolamide and Furosemide (both diuretic) to reduce the
production of CSF
● Most often surgery is needed; the surgeon bypass or shunt the point of
obstruction; the CSF may thus be carried to another area of the body,
where it is absorbed and finally excreted; this is accomplished by
inserting special tubing, which is replaced at interval as the child grows;
this procedure is known as ventriculoperitoneal shunt which allows the
excess fluid to drain into the peritoneal cavity where it is absorbed
● Complication of shunts are usually mechanical (kinking or plugging of
tubing) or infections; the shunt act as a focal spot for infection and may
need to be removed if infection persists
Preoperative Nursing Care:
● Surgery correction can be accomplished in utero via fetal surgery or
shortly after birth to prevent brain damage caused by increased
intracranial pressure
● Those who don’t undergo surgery; as the child grows he or she may
be barely able to raise the head; mental development is delayed, lack
of appetite, a tendency to vomit easily and poor resistance to
infections
● The position of infant must be changed frequently to prevent hypostatic
pneumonia and pressure sores
● Hypostatic pneumonia occurs when the circulation of the blood in the
lungs is poor and the infant remains in one position too long; more
prevalent in infant who are poorly nourished or weak or who have
debilitating disease
● Infants head should always be supported when turning the infant; to turn
the infant in bed, the weight of head is borne in the palm of one hand and
the head and body are rotated together to prevent a strain on the neck;
when the infant is lifted from the crib, the head must be supported by the
nurse’s arm and chest
● The tissues of head, ears and bony prominences tend to break down; a
CHAPTER 14
STUDY GUIDE: THE NEWBORN WITH A PERINATAL INJURIES
OR CONGENITAL MALFORMATION
- Defect present at birth involves the skeletal system; limbs may be missing,
malformed or duplicated
- Some abnormalities (congenital hip dysplasia) are more subtle and the nurse
must be alert to detect them
- Inborn errors of metabolism includes number of inherited diseases that
affects body chemistry (there may be absence or deficiency of a substance
usually an enzyme that is necessary for cell metabolism); cystic fibrosis and
phenylketonuria (PKU)
- In Disorder of the blood, there is a reduced or missing blood
component or an ability of a component to function adequately; sickle
cell disease, thalassemia, and hemophilia.
- Chromosomal abnormalities, involves some type of intellectual
impairment and other are incompatible with life; down syndrome, Turner’s
syndrome or Klinefelter’s syndrome may have impaired physical growth
and sexual development
- Perinatal injuries have many causes and are seen in various form, the most
common of which is premature birth; infections, drugs, maternal disorders,
abnormalities unique to pregnancy (Rh disease, difficult labor or delivery,
premature birth)
MALFORMATION PRESENT AT BIRTH
NERVOUS SYSTEM
Neural Tube Defects:
- Failure of neural tube closure at either the cranial (top) or the caudal
(lower) end of the spinal cord; results in hydrocephalus and spina bifida
Hydrocephalus:
● Increase of CSF within the ventricles of the brian, which causes
pressure changes in the brain and an increase in the head size
● Caused by imbalance between the production and absorption of
CSF or improper formation of the ventricles
● It can be congenital or acquired
● Commonly acquired by an obstruction such as tumor or a sequela of
infection (encephalitis or meningitis)
or perinatal hemorrhage
● Classified by either noncommunicating (obstructive) or communicating
● Noncommunicating hydrocephalus results from the obstruction of
CSF flow from the ventricles of the brian to the subarachnoid space;
Communicating hydrocephalus when CSF is not obstructed in the
, 2
ventricles but is adequately reabsorbed in the subarachnoid space
Manifestations:
● Increase in head size
● If hydrocephalus occurs in utero, the enlarged head will necessitate a
cesarean section delivery
● At birth the head enlarges rapidly and the fontanelles bulge; the cranial
sutures separate to accommodate the enlarging mass; the scalp is shiny
and the veins are dilated
● In advanced cases, the pupils of the eyes may appear to be downward
and the sclera maybe seen above the pupils
● Arnold-Chiari malformation: the 4th ventricle with brain stem protrude
through cervical canal
● The infant is helpless and lethargic; the body becomes thin and the
muscle tone of the extremities is often poor; the cry is shrill and high
pitched; irritability, vomiting and anorexia are present and convulsion
may
occur
● When hydrocephalus occur in older child, the head cannot enlarge bc
the cranial sutures are fused; therefore headache is the predominant
symptom with cognitive slowing, personality changes, spasticity and
other neurological signs
Diagnosis:
, 3
● Transillumination: the inspection of a cavity or an organ by
passing a light through its walls; for visualizing fluid
● A flashlight with a sponge rubber collar is held tightly against the
infant’s head in a dark room; a small ring of light is normal, but a large
halo effect is not
● The child head is measured daily
● Echoencephalography CT scanning and MRI are used to visualize the
enlarged ventricles and to identify the area of obstruction
● A ventricular tap or puncture may be performed using sterile technique,
to determine the pressure and to drain CSF; a specimen is labeled and
sent to the laboratory for analysis
Treatment:
● MED: Acetazolamide and Furosemide (both diuretic) to reduce the
production of CSF
● Most often surgery is needed; the surgeon bypass or shunt the point of
obstruction; the CSF may thus be carried to another area of the body,
where it is absorbed and finally excreted; this is accomplished by
inserting special tubing, which is replaced at interval as the child grows;
this procedure is known as ventriculoperitoneal shunt which allows the
excess fluid to drain into the peritoneal cavity where it is absorbed
● Complication of shunts are usually mechanical (kinking or plugging of
tubing) or infections; the shunt act as a focal spot for infection and may
need to be removed if infection persists
Preoperative Nursing Care:
● Surgery correction can be accomplished in utero via fetal surgery or
shortly after birth to prevent brain damage caused by increased
intracranial pressure
● Those who don’t undergo surgery; as the child grows he or she may
be barely able to raise the head; mental development is delayed, lack
of appetite, a tendency to vomit easily and poor resistance to
infections
● The position of infant must be changed frequently to prevent hypostatic
pneumonia and pressure sores
● Hypostatic pneumonia occurs when the circulation of the blood in the
lungs is poor and the infant remains in one position too long; more
prevalent in infant who are poorly nourished or weak or who have
debilitating disease
● Infants head should always be supported when turning the infant; to turn
the infant in bed, the weight of head is borne in the palm of one hand and
the head and body are rotated together to prevent a strain on the neck;
when the infant is lifted from the crib, the head must be supported by the
nurse’s arm and chest
● The tissues of head, ears and bony prominences tend to break down; a
