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the newborn at risk: congenital disorders

QuestionAnswer
absence of a normal body opening or the abnormal closure of a body passage. atresia
an abnormal opening between the right and left atria. atrial septal defect
pertaining to both sides (e.g. bilateral cleft lip involves both sides of the lip) bilateral
shortness of the head brachycephaly
chord-like anomaly that extends from the scrotum to the penis; pulls the penis downward in an arc. chordee
constriction or narrowing of the aortic arch or the descending aorta usually adjacent to the ligamentum arteriosum coarctation of the aorta
abnormal fetal development of the acetabubulum that may or may not cause dislocation the hip; if the malformed acetabulum permits dislocation, head of femur displaces up and back-might be hard to recognize in early infancy congenital hip dysplasia
this condition is a result of impaired pumping capability of the heart. congestive heart failure (CHF)
this condition, may appear in the first year of life in infants with conditions such as large ventricular septal defects, coarctation of the aorta, and other defects that place an increased workload on the ventricles. congestive heart failure (CHF)
congenital heart disease that causes right-to-left shunting of blood in the heart (may occur ta the atrial, ventricular, or aortic level) results in depletion of O2 saturation of the peripheral arterial blood= 85% or less. cyanotic heart disease
condition in which the opening of the urinary meatus is located abnormally on the dorsal(upper) surface of the glans penis. epispadias
recessive hereditary metabolic disorder in which the enzyme necessary for converting galactose into glucose is missing; generally appears normal at birth but experiences difficulties after ingesting milk. galactosemia
abnormal protrusion of part of an organ through a weak spot or other abnormal opening in a body wall. hernia
condition that occurs when the opening to the urethra is on the ventral(under) surface of the glans. hypospadias
low body temperature; and when caring for the newborn its important to remember this can happen quickly because of the immature thermo-regulation their body hypothermia
congenital disorder in which the rectal pouch ends blindly above the anus and there is no anal orfice imperforate anus
in tetralogy of Fallot; the aorta shifts to the right over the opening in the ventricular septum so that blood from both right and left ventricles is pumped into the aorta overriding aorta
recessive hereditary defect of metabolism that results in a congenital disease caused by a defect in the enzyme that normally changes the essential amino acid, phenylalanine, into tyrosine; if no treated: can result in severe MR. phenylketonuria (PKU)
narrowing of the opening between the right ventricle and the pulmonary artery that decreased blood flow to the lungs pulmonary stenosis
increase in thickness of the myocardium of the right ventricle right ventricular hypertrophy
failure of the posterior lamina of the vertebrae to close; leaves an opening through which the spinal meninges and spinal cord may protrude spina bifida
excessive in number (more than the usual number of teeth) supernumerary
clubfoot with plantar flexion talipes equinovarus
grouping of heart defects (4 defects); pulmonary stenosis, ventricular septal defect, overriding aorta, right ventricular hypertrophy. tetralogy of Fallot
occurs when the aorta arises from the right ventricle instead of the left, and the pulmonary artery arises from the left ventricle instead of the right transposition of the great arteries
one sided (e.g. cleft lip: only one side of the lip is cleft) unilateral
abnormal opening in the septum of the heart between the ventricles; allows blood to pass directly from the left to right side of the heart; is the most common intracardiac defect. ventricular septal defect
plastic tubing implanted into the cerebral ventricle passing under the skin to the cardiac atrium; provides drainage for excessive cerebrospinal fluid. ventriculoatrial shunting
plastic tubing implanted into the cerebral ventricle passingunder the skin to the peritoneal cavity, providing drainage for excessive cerebrospinal fluid; excessive tubing can be inserted with child's growth. ventriculoperitoneal shunting
Your client and her husband are told that their baby has a congenital anomaly, they are both very upset and feel bad for feeling this way-what is appropriate to say/do? assure the parents that their response is normal and that they are entitled to honest answers to their question about their newborn's condition.
congenital anomalies or malformation can affect what systems of the newborn's body? (5 specifically) cardiovascular, GI, GU, CNS, skeletal systems.
A nursing student says to you just after helping deliver a 7lb boy, "At least he doesn't have any congenital anomalies." What do you say to her? some anomalies can be apparent at birth (e.g. cleft lip or sever neural tube defects) but others may not be noticeable until we perform a complete physical of the newborn.
List 2 CNS defects spina bifida and hydrocephalus
a bony defect that occurs without soft-tissue involvement; asymptomatic and presents no problems; a dimple in the skin or tuft of hair over the site may indicate its presence-if noticed at all. spina bifida occulta
part of the spinal meninges protrudes though the bony defect and forms a cystic sac, no nerve roots, no no paralysis or sensory loss below the lesion; however, the sac may rupture or perforate CSP and cause meningitis-surgical removal indicated. spina bifida meningocele
a protrusion of the spinal cord and meninges, with nerve roots embedded in the wall of the cyst; partical paralysis or complete paralysis below the lesion can occur. spina bifida myelomeningocele
the three types of spina bifida are? spina bifida occulta; spina bifida meningocele; and spina bifida myelomeningocele.
this is the term used to designate the two protruding spina bifidas. spina bifida cystica
you notice in the chart that Mrs. Smith is coming in for a MSAFP test to test for the presence of AFP, you can guess that your patient is between how many weeks gestation? 13-15 weeks
The parents of a newborn are told that their newborn has spina bifida myelomeningocele; after the initial shock, they begin asking questions: "So, after his surgery to close the opening he will be cured?" What MUST the nurse stress to the parents? there is no cure for spina bifida and that a child with spina bifida will require years of ongoing follow-ups and therapy.
when performing the newborn exam with a newborn with spina bifida cystica, what 2 neurologic observations must be checked; and what one thing must the nurse take great care in doing during the exam? measure head circumference and check fontanelles; observe movement and response to stimuli; and prevent injury to the sac
Preoperative goals for the newborn with myelomingocele would be to keep a dressing on the sac, including a plastic covering over the dressing-what kind of dressing will this be and how often(if any) will she change it. it will be a moistened, warm sterile solution dressing (usually sterile NS) and will be changed every 2hr.
why is it important to change the dressing on the sac of a myelomingocele newborn before surgery? to keep it from drying out and possibly cracking open-keeping it from infection.
the myelomingocele newborn will be kept in what position both before and after surgery (until complete healing of surgical site). prone position (on belly)
With a low defect, the nurse has her work cut out for her, trying to keep fecal material from nearing the sac, to keep infection out; the student nurse says"but they have their diaper what's the problem?" You as the nurse should say? wearing a diaper is not advisable for the low defect, even if the sphincter muscles are involved and cause continual loose stool-you MUST create a barrier.
Knowing the newborn with a low defect can suffer from leakage of stool and urine that's continual and irritation and skin breakdown is high what is the most important implementation must the nurse do? scrupulous perineal care
If the newborn with spina bifida has a loss of motion; the nurse with perform ROM with the newborn's hips abducted and legs in neutral, massage his knees and other bony prominences with lotion regularly and pad and protect these bony prominences while mai contracture of the lower extremeties
With the family that just found out that their newborn has spina bifida, what one thing must the nurse AVOID not doing, that could exaggerate their feeling of loss and depression? do NOT avoid this family-give privacy if needed but don't avoid.
If the physician gives permission-what might the mother/father be allowed to do, in order to bond more so with their spina bifida baby chest-to-chest hold
How should the nurse teach the parents of the spina bifida newborn to feed their newborn after surgery. to hold the newborn's head, neck, and chest in one hand slightly raised.
Even though the child with spina bifida will have many long-term problems what must the nurse stress to the parents and family to remember about their newborn's intelligence? their intelligence is not affected; and many will grow into productive young adults who may live independently.
condition characterized by an excess of CSF within the ventricular and subarachnoid spaces of the cranial cavity. hydrocephalus
this type of congenital hydrocephalus is caused by an obstruction that occurs and CSF isn't able to pass between the ventricles and the spinal cord. noncommunicating congenital hydrocephalus
the type of hydrocephalus that has no obstruction of the free flow of CSF exists between the ventricles and the spinal theca; rather the condition is caused by defective absorption of CSF, increasing pressure on the brain or spinal cord. communicating hydrocephalus
The RN asks you, the student nurse, what is one of the most common causes of noncommunicating hydrocephalus; and you can tell her it is the narrowing of what? the aqueduct of Sylvius
As you help the RN with the newborn with congenital hydrocephalus, you as the student nurse knows that congenital hydrocephalus is most likely caused by what 2 types hydrocephalus? obstructive or noncommunicating type
hydrocephalus may or may not be noticed at birth...true/false true...it may be evident at birth or take up to a few weeks or months of life
all hydrocephalus are congenital...true/false false...some can occur during childhood.
non-congenital hydrocephalus can result from what 3 things? neoplasm, head injury, infection such as meningitis.
what might be the explanation of why some newborns fail to show the usual symptoms of brain pressure with hydrocephalus it occurred early in life while the skull was still soft and pliable.
Created by: gracekimes