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Common Health Problems That Develop During Infancy  Invagination Intestinal obstruction

RONSAL D. RIVERA, RN, EMT, MAN


 Mesentery/lymphatic’s/blood vessels pulled into intestine
COMMON HEALTH PROBLEMS THAT DEVELOP
DURING INFANCY  Intestines become curved, sausage like
 Intussusception
 Failure to Thrive  Blood supply is cut off
 Sudden Infant Death Syndrome (SIDS)
 Colic  Swelling of the bowel
 Trisomy 21
 Cleft Palate  Hemorrhage; necrosis of involved segment
 Imperforated Anus
 Hirschprung’s Disease Complications
 Spina Bifida  Perforation
 Hydrocephalus  Peritonitis
 Otitis Media  Shock
 Meningitis  Loss of bowel
 Febrile Seizures
 Autism/ADHD Classification of Location
1. Ileocecal (most common)
INTUSSUSCEPTION  When ileum and the attached mesentery, lymphatic tissue
 most common cause of intestinal obstruction in children and blood vessels invanginate into the cecum
between ages 3 months and 6 years 2. Ileocolic
 telescoping or invagination of one portion of the bowel into  Ileum invaginates into colon
another portion which results in an obstruction to the passage 3. Colocolic
of intestinal contents  Colon invaginates into colon
 May result from hyperactive peristalsis in the proximal 4. Ileo-ileo (enteroenteric)
portion of the bowel with inactive peristalsis in the distal  Small bowel invagination into small bowel
segment
 Usually occurs at the junction of the ileum with the colon Clinical Manifestations
 Children between 3 and 12 months old or before age 2  Colicky abdominal pain that cause the child to scream and
draw the knees to the abdomen similar to the fetal position
Etiology  Vomiting of gastric contents
 Idiopathic  Bile stained fecal emesis
 Cause is unknown  Currant jelly-like stools containing blood and mucus
 Maybe related to Upper respiratory and GI virus  Hypoactive or hyperactive bowel sounds
 Hypothesized that hypertrophy of the Peyer’s patches creates  Tender distended abdomen, possibly with a palpable sausage-
a thickened segment-most common in infants shaped mass in the upper right quadrant
 Right lower quadrant empty
Lead point
 Identifiable change in the intestinal mucosa usually during Diagnostic evaluation
surgical treatment  X-ray
 Most common in children ages 2-3  Ultrasonography
 Malformations include polyps, cysts, tumors, Meckels  Color Doppler sonography
diverticulum and hematomas  use to determine whether it is reducible or not
 Children with cystic fibrosis are at risk  Absence of blood flow (color) indicates ischemia and
therefore enema reduction should be avoided
Cystic fibrosis Management
 Cystic fibrosis (also known as CF, mucoviscoidosis, or  Air or Barium enema – both for diagnosis and treatment
mucoviscidosis) is a hereditary disease that affects mainly the (Hydrostatic reduction) in reducing intussusception
exocrine (mucus) glands of the lungs, liver, pancreas, and  A surgeon should be present during the barium enema due to
intestines, causing progressive disability due to multisystem risk of perforation
failure.  A non-inflatable tube is passed into rectum; contrast enters by
gravity under fluoroscopic guidance. If air is used, it is
delivered under constant pressure

Postoperative Barium Enema


 Uncommon but can occur after surgery of the abdomen and Post procedure
even the chest 1. A laxative maybe prescribed
 May be due to interrupted motility from anesthesia or direct 2. Instruct the client to increase oral fluid intake to help pass the
handling of the intestine barium
 It can also occur from placing long tubes into the bowel 3. Monitor stools for the passage of barium (stools will appear
chalky white) because barium can cause a bowel obstruction.
Pathophysiology
o Visual aids, such as a rubber glove with one finger into
Surgical reduction of intussusception may be necessary when itself, may be helpful. Reduction can be demonstrated by
radiologic reduction is unsuccessful, a pathologic lead point or filling glove with water until inverted finger resumes its
peritonitis is suspected or with multiple recurrence normal position
 Laparotomy
 Manual milking out of the intussuscepted segment from Post-operative
the distal to proximal end followed by resection of the Preventing Postoperative Complications
nonviable bowel and commonly an incidental  Monitor vital signs and general condition, notify health
appendectomy care provider of any change or unexpected trend
 Assess temperature and administer antipyretics and other
Nursing Diagnosis cooling measures. Fever is usually present from absorption
Pre-operative of bacteria through the damaged intestinal wall
 Acute pain related to paroxysmal abdominal pain, fever  Assess for abdominal tenderness, bowel sounds and
and treatments distention of abdomen. Maintain NG suction as ordered
 Risk for Decreased Fluid Volume related to vomiting  When able to take fluids, assess tolerance carefully and
 Ineffective Breathing Pattern related to abdominal advance intake slowly
distention
 Anxiety related to hospitalization, knowledge deficit of Family Education and Health Maintenance
illness, surgery, and treatments  Explain that recurrences are rare and usually within 36
hours after reduction. Review signs and symptoms with
Post-operative parents
 Risk for injury related to postoperative course  Review activity restrictions with parents (ex: positioning
on back or side, quiet play, and avoidance of water sports
Nursing Interventions until wound heals)
Pre-operative  Encourage follow-up care
Minimize pain  Provide anticipatory guidance for developmental age of
1. Observe behavior as indicator of pain; the infant may be child
irritable and very sensitive to handling or lethargic or  Encourage awareness of symptoms that require prompt
unresponsive. Handle very gently medical attention among day-care centers and other child-
2. Encourage family to participate in comfort measures. care providers (ex: paroxysmal abdominal pain, blood or
Explain cause of pain, and reassure parents as to purpose mucus in stool)
of diagnostic tests and treatments
3. Administer medications as prescribed Interventions
 Monitor for signs of perforation and shock as evidenced by
Maintaining Fluid and Electrolytes fever, increased heart rate, changes in level of
1. Monitor fluids and maintain NPO status consciousness or blood pressure and respiratory distress
2. Restrain infant as necessary for IV therapy and report immediately
3. Monitor intake and output  Antibiotics, IV fluids and decompression via nasogastric
tube
Promote Effective Breathing  Monitor the passage of normal, brown stool which
 Be alert for respiratory distress because of abdominal indicates that the intussusception has reduced itself
distention. Watch for grunting or shallow and rapid  Prepare for hydrostatic reduction as prescribed, if no signs
respiration if no shock like state of perforation or shock occur (in hydrostatic reduction,
 Insert NG tube if ordered to decompress stomach fluid is used to exert pressure on area involved to lessen,
a. Note drainage and return from irrigation diminish or rid the intestine of prolapse)
b. Irrigate at frequent intervals  Posthydrostatic reduction
 Maintain NPO status as ordered o Monitor for the return of normal bowel sounds,
a. Wet lips and perform mouth care
for the passage of barium and the characteristics
b. Give infant pacifier to suck
of the stool
 Continually reassess condition because increased pain and o Administer clear fluids and advance the diet
bloody stools may indicate perforation
gradually as prescribed
Nursing alert: Passage of one normal brown stool may occur,
FAILURE TO THRIVE
clearing the colon distal to the intussusception. Passage of more
 A sign of inadequate growth resulting from inability to obtain
than one normal brown stool may indicate that the intussusception
or use calories required for growth
has reduced itself. Report any stools immediately to the physician
 weight that falls below the 5th percentile for the child’s age
Preparing for Surgery
3 Categories (Historical category)
 Offer support to the parents during time of crisis and fear 1. Organic failure to thrive
 Children need brief, simple explanations in age-appropriate 2. Nonorganic failure to thrive
language 3. Idiopathic failure to thrive
 Offer specific teaching to parents
o Compare intussusception to a collapsible telescope or According to Pathophysiology
antenna or by drawing a picture  Inadequate caloric intake
o Incorrect formula preparation, neglect, food fads,  Child abuse or neglect
excessive juice consumption, poverty, behavioral problems  Significant dehydration
affecting eating or CNS problems affecting intake  Caretaker substance abuse or psychosis
 Outpatient management that does not result in weight gain
 Inadequate absorption Nursing Care Management
o Cystic fibrosis, celiac disease, vitamin or mineral  Provide a positive feeding environment
deficiencies, biliary atresia, or hepatic disease  Teach the parent successful feeding strategies
 Support the child and family
 Increased metabolism  Accurate assessment of the initial weight and height and
o Hyperthyroidism, congenital heart defects, or chronic daily weight as well as recording of all food intake
immunodeficiency  The nurse documents the child’s feeding behavior and the
parent-child interaction during feeding, other caregiving
 Defective utilization activities and play
o Genetic anomaly such as trisomy 21 or 18, congenital a. Nursing Child Assessment Satellite Training - an
infection, or metabolic storage diseases excellent feeding observation instrument)
b. A 25-item observational scale, the Feeding checklist-
Etiology use to observe mother- infant dyads with FTT
 Multifactorial- involves a combination of infant organic  Assess the approximate developmental age on admission
disease, dysfunctional parenting behaviors, subtle neurologic or by administering an appropriate developmental test
behavioral problems, and disturbed parent-child interactions
 Other factors Nutritional Management
 Poverty Four primary goals
 Health beliefs  Correct nutritional deficiencies and achieve appropriate
 Inadequate nutrition knowledge weight for height
 Family stress  Allow for catch-up growth
 Psychosocial factors  Restore optimum body composition
 Feeding resistance  Educate the parents of primary caregivers regarding the
 Insufficient breast milk child’s nutritional requirements and appropriate feeding

Diagnostic Evaluation SUDDEN INFANT DEATH SYNDROME (SIDS)


 Weight is below the accepted standard (5th percentile)  Sudden death of an infant under 1 year of age that
 Low height and weight- chronic malnutrition remains unexplained after a complete postmortem
 Dietary history examination, including an investigation of the death scene
 Physical assessment and a review of the case history.
 Death occurs during sleep periods but not necessarily at
 Developmental assessment
night
 Family assessment
 Most frequently affects infants from 2 mos. to 4 mos. of
age; less danger during first month; rare after 12 mos.
Therapeutic Management
 Incidence is higher in males
 Goal: reversing the cause of the growth failure  Higher incidence in winter: June and July and low-income
 Malnutrition – provide sufficient calories to support groups
“catch-up” growth
 Multidisciplinary team approach- physician, nurse, Etiology
dietitian, child-life specialist, occupational therapist,  Cause is unknown but may be related to a brainstem
pediatric feeding specialist, and social worker or mental abnormality in the neurologic regulation of
health professional cardiorespiratory control
 Efforts are made to relieve any additional stresses on the o Prolonged sleep apnea
family by offering referrals to welfare agencies or o Increased frequency of brief inspiratory pauses
supplemental food programs
o Excessive periodic breathing
 Family therapy
o Impaired arousal responsiveness to increased
 Temporary placements of the child in a foster home
carbon dioxide or decreased oxygen
 Behavior modification aimed at mealtime rituals
Maternal factors
Provide nurturing to infant
 Unwed mothers
 Ensure a warm, loving environment through holding,
 Younger mothers
cuddling and physical contact
 Multiparous with shorter between-
 Limit number of persons interacting with infant; primary
pregnancy intervals
nursing preferred
 Cigarette-smoking
 Spend time talking to infant and building a trusting
 Not utilizing health care facilities
relationship
 Maintain as much eye-to-eye contact as possible
 Genetic predisposition
Hospitalization  Maternal smoking, prenatally and postnatally
 Evidence of severe acute malnutrition
o Decreased in the infant’s ability to arouse to  trapped gas in the digestive tract (traditional cause)
auditory stimuli in mothers who smoked  variable gut flora, which includes a lack of Lactobacillus
prenatally acidophilus
o Increased nicotine concentrations in lung  is a combination of a baby’s sensitive temperament, the
tissue were found in children who died environment, and its immature nervous system that makes
from SIDS him/her cry easily and without control.
 Poor prenatal care and low maternal age  mild gastroesophageal reflux
 Cosleeping or bed sharing with an adult or older children  Lactose intolerance or lactose overload
 Prone sleeping – may cause oropharyngeal obstruction or  the onset of melatonin production by the pineal gland
affect thermal balance or arousal state (which does not begin until 12 weeks of age, about the
 Side-lying position (position in supine) time colic seems to disappear),
 Soft beddings – waterbeds, sheepskins, beanbags, pillows  circadian rhythms,
or quilts (avoid)  smoking and stress of the mother in the third trimester.

Infants at risk for SIDS Treatment


 Preterm infants who continue to have apnea at the time of  Many babies are soothed by gentle bouncing or rocking,
hospital discharge which leads to a parasympathetic response, endorphin
 Siblings of two or more victims production, and therefore calming
 Infants with certain types of diseases or conditions such as  simethicone, which treats trapped gas
central hypoventilation  probiotics such as Lactobacillus reuteri, intended to reduce
gas
Assessments  soothing measures, such as pacifiers, listening to white
 Child is apneic, blue and lifeless noise and rocking, are often effective in calming the baby
 Frothy blood-tinged fluid is in the nose and mouth during crying periods
 Child may be found in any position, but typically is  Some parents take turns holding the baby upright (which
found in a disheveled bed, with blankets over the head, may reduce the pain and crying) to enable the other parent
and huddled in a corner to catch up on sleep.
 Child may appear to have been clutching bedding  light massage to static pressure could easily and most often
 Diaper may be wet and full of stool does relieve the spasm.

Prevention IMPERFORATED ANUS


 Infants should be placed in the supine position for sleep  Anal agenesis
 Soft moldable mattresses and bedding such as pillows  A congenital abnormality in the formation of the anorectal
or quilts, should not be used for bedding canal or in the location of the anus resulting in the rectum
 Stuffed animals should be removed from the crib while ending blindly
the infant is sleeping  A fistula or severe narrowing of the anal canal
 Discourage bed sharing Assessment
 Avoid overheating during sleep  Assess patency of anal opening with small finger or soft
catheter if the following symptoms are present
Nursing Care Management o No meconium stool within 24 hours
1. Educate families about the risk of prone sleeping position o Green-tinged urine
in infants from birth to 6 months of age, the use of o Progressive abdominal distention
appropriate bedding surfaces, and the dangers of cosleeping
Implementation
COLIC  Maintain NPO status when anomaly is diagnosed
 Baby colic  Monitor IVs
 also known as infant colic, three-month colic, infantile  Check v/s frequently and hydration
colic and colic  Maintain temp by using isolette or radiant warmer
 is a condition in which an otherwise healthy baby cries or
screams frequently and for extended periods without any Surgery
discernible reason.  Perineal anoplasty, which creates an anal opening for
 typically appears three weeks after birth and almost passage of stool for low lesion
invariably disappears, often very suddenly, before the baby  Colostomy for high-lesion (1st step)
is three to four months old.  anal repair, colon/rectum anastomosis, possible colostomy
 It is more common in bottle-fed babies, but also occurs in closure (2nd step)
breast-fed infants. The crying frequently occurs during a  Third step is colostomy closure if defects is complex and
specific period of the day, often in the early evening. more healing time is needed
 common rule of thumb is to consider a baby "colicky" if he  Posterior sagittal anorectoplasty,
or she cries intensely more than three days a week, for  Also known as Pena procedure (after the surgeon
more than three hours, for more than three weeks in a who developed it).
month.  The rectum is “pulled down” and sewn into a
newly made anal opening in the perineum
 Most surgeons wait 4 to 6 months to complete it
Causes to allow for growth
o Enlarge the nipples
Postoperative Care o Stimulate the suck reflex
 Prevent infection of operative site o Swallow
 Provide colostomy care o Rest to allow the child to finish swallowing
 Check for return of peristalsis so that oral feedings may be what has been placed in the mouth
started  Encourage the parents to express their feelings about the
disorder
CLEFT PALATE  Encourage parental bonding with the child, including
 A birth defect in which the primary and secondary palatine holding the child and calling the child by name
plates-openings between nose and roof of mouth- fail to
close properly Surgical Treatment
 Hereditary Criteria of Surgery:
 Maybe Rule of 10s" coined by surgeons Wilhelmmesen and
o Clefted soft Musgrave in 1969
o Clefted hard palate 1. The child is at least 10 weeks of age;
o Cleft that infrequently involves the nose 2. Weighs at least 10 pounds, and
3. Has at least 10g hemoglobin)
 Cleft lip(cheiloschisis)
 cleft palate (palatoschisis), Surgical Treatment
 Cleft- a fissure or an opening  Millard repair procedure
o Pioneered by Ralph Millard
Etiology o Rotation-advancement procedure for cleft lip repair
 Genetic o Designed to create a softer, more natural-looking lip
 Chromosomal abnormalities o Rotates the tissue and creates a "Z" shaped scar
 Hereditary instead. The "Z" shape gives the tissue more
 Teratogenic factors elasticity, resulting in greater flexibility and
 Environmental factors restoration of the Cupid's bow
o Exposure to radiation Movement of the flaps; flap A is moved between B
o Rubella virus and C. C is rotated slightly while B is pushed down.
Note how the Cupid’s bow is created

Assessment .
 Cleft lip: slight notch to a complete separation from the
floor of the nose
 Cleft palate: nasal distortion, midline or bilateral cleft and
variable extension from the uvula and soft and hard palate
 Difficulty in swallowing
 Increase in upper respiratory infections
The blue lines indicate incisions.

Surgical Treatment
 Cheiloplasty
o Lip reduction - the process of surgically reducing
the size of the lip or lips in order to reduce the
appearance of abnormally large or protruding lips
and the process of forming an artificial tip or part
of the lips by using a piece of healthy tissue from
some neighboring part

Postoperative Interventions
Interventions  Cleft Lip
 Assess the ability to suck, swallow, handle normal o A lip protector device or Logan bar may be taped
secretions, and breathe without distress securely to the cheeks to prevent trauma to the suture
 Assess fluid and calorie intake daily line
o Position the child on the side lateral to the repair or
 Monitor daily weight
on the back
 Modify feeding techniques: plan to use specialized feeding
o Avoid prone position to prevent rubbing of the
techniques, obturators and special nipples and feeders
 Hold the child in an upright position and direct the formula surgical site on the matter
to the side and back of the mouth to prevent aspiration o After feeding, cleanse the suture line of formular or
 Feed small amounts gradually and burp frequently serosanguineous drainage with a cotton-tipped swab
dipped in normal saline
 Position on the side after feeding
o Apply antibiotic ointment to the site as prescribed
 Keep suction equipment and a bulb syringe at the bedside
o Elbow restraints should be used to prevent the infant
 Teach the parents special feeding or suctioning techniques
from injuring or traumatizing the surgical site
o ESSR Method
-Soft elbow or jacket restraints to keep the child
from touching the repair site Treatment
-Remove restraints at least every 2 hours to assess  Relieve constipation with stool softeners and rectal
skin integrity and to allow for exercising the arms irrigations
 Monitor for signs and symptoms of infections at the  Surgery- surgical removal (resection) of the abnormal
surgical site section of the colon, followed by reanastomosis
 Keep the surgical site clean and dry a. temporary colostomy – neonatal period
 relieve obstruction and allow the normally
Cleft Palate Repair innervated, dilated bowel to return to its normal
 The child is allowed to lie on the abdomen size
 Feedings are resumed by bottle, breast or cup b. Complete surgical repair
 Oral packing may be secured to brush his or her teeth o Done when child weighs 9 kg (20lpbs)
 Instruct the parents to avoid offering hard food items to the o Pull through procedure to excise portions of the
child such as toast or cookies bowel
 Repairs the colon by connecting the
Other Interventions functioning portion of the bowel to the anus.
 Avoid contact with sharp objects near the surgical site  The typical method for treating
 Avoid the use of oral suction or placing objects in the Hirschsprung’s in younger patients.
mouth such as tongue depressor, thermometer, straws, o Colostomy is closed.
spoons, forks, or pacifiers
 Provide analgesic for pain
 Instruct the parents to monitor for signs of infection at the The Swenson, Soave, Duhamel, and Boley procedures all
surgical site, such as redness, swelling or drainage vary slightly from each other:
 Encourage the parents to hold the child  The Swenson procedure leaves a small portion of the
 Initiate appropriate referrals for speech impairment or diseased bowel.
language-based learning difficulties  The Soave procedure leaves the outer wall of the
colon unaltered. The Boley procedure is just a small
modification of the Soave procedure. The term
HIRSCHPRUNG’S DISEASE "Soave-Boley" procedure is sometimes used.
 Congenital aganglionosis or aganglionic megacolon;  The Duhamel procedure uses a surgical stapler to
congenital aganglionic megacolon, connect the good and bad bowel.
 Congenital disorder of the colon in which certain nerve
cells, known as ganglion cells, are absent, causing chronic Interventions: Medical Management
constipation.  Maintain low-fiber ; high-calorie, high-protein diet;
 Results in mechanical obstruction because of inadequate parenteral nutrition
motility in an intestinal segment  Stool softeners
 The lack of ganglion cells is in the myenteric plexus,  Daily rectal irrigations with normal saline to promote
which is responsible for moving food in the intestine adequate elimination and prevent obstruction

Etiology Surgical Management: Preoperative Interventions


 Familial congenital defect  Assess bowel function
 May occur with other anomalies such as Down Syndrome  Administer bowel preparation
and genitourinary abnormalities  Maintain NPO preparations
 Monitor hydration and fluid and electrolyte status; provide
Assessment fluids IV
1. Rectal biopsy – absence of ganglionic cells  Administer antibiotics: Metronidazole (Flagyl) to clear the
2. Newborn infants bowel of bacteria
o Failure to pass meconium stool  I and O
o Refusal to suck  Daily weight
o Abdominal distention  Measure abdominal girth daily
o Bile-stained vomitus  Avoid taking temp rectally
3. Children  Monitor for respiratory distress
o Failure to gain weight and delayed growth
o Abdominal distention Surgical Management: Postoperative Interventions
o Vomiting  Monitor V/S
o Constipation alternating with diarrhea  Measure abdominal girth and PRN
o Ribbon-like and foul-smelling stools  Assess the surgical site for redness, swelling, and drainage
 Assess the stoma if present for bleeding or skin breakdown
Complication (red and moist)
1. Enterocolitis  Assess the anal area for the presence of stool, redness or
o Fever discharge
o Severe explosive watery diarrhea  Maintain NPO status until bowel sounds return or flatus is
o Severe prostration passed usually within 48-72 hours
o GI bleeding
 Maintain the NG tube to allow intermittent suction until  Facial Features
peristalsis return o Short palpebral features
 Maintain IV fluid until the child tolerates appropriate oral o Hypoplastic or smooth philtrum (vertical ridge in
intake, advancing the diet from clear liquids to regular as upper lip)
tolerated o Thinned upper lip (Vermilion)
 Assess for dehydration and fluid overload o Short, upturned nose
 Monitor I and O o Hypoplastic maxilla
 Daily weight o Micrognathia or prognathia in adolescence
 Assess for pain and provide comfort measures o Retrognathia in infancy
 Provide instructions on colostomy care  Neurologic
 Teach the appropriate diet and the need for adequate fluid o Mental retardation
intake o Motor retardation
o Microcephaly
o Poor coordination
o Hypotonia
o Hearing disorders
FETAL-ALCOHOL SYNDROME (FAS)  Behavior
 Alcohol-related birth defects (ARBD) o Irritability (infancy)
 With characteristic facial and associated physical features o Hyperactivity (child)
attributed to excessive ingestion of alcohol by the mother  Growth
during pregnancy o Disproportionately low weight to height
 Infants may not initially display the dysmorphic facial o Prenatal growth retardation
features; but will be more well defined with increasing age o Persistent postnatal growth lag
during childhood

Diagnosis of FAS (CDCP)


 Growth restriction both prenatal and postnatal
 Mildfacial dysmorphic facial features
 CNS involvement (structural, neuorologic or functional
abnormality)
 Major of goal of nursing care: prevention of these
disorders through provision of adequate prenatal care for
the expectant mother and precautions regarding exposure
to potentially harmful infections
 FAE(Fetal Alcohol Effects) – behavioral and psychosocial
problems without any facial dysmorphism and growth Nursing Care Management
retardation  Monitor weight gain, analyzing feeding behaviors, and
 FAS – recognized as the leading cause of mental devising strategies to promote nutrition intake
retardation  All women should be counseled regarding the risks of
 Alcohol (ethanol and ethyl aclohol) alcohol to the fetus
 Interferes with normal fetal development  All women should be counseled to stop smoking at least 3
 Effects on fetal brain are permanent mos. before they plan to conceive
 Moderate use of alcohol during pregnancy may TRISOMY21
cause long-term postnatal difficulties including  Down Syndrome
impaired maternal-infant attachment  Due to presence of an extra chromosome, number 21
 Fetal abnormalities are not related to the amount of the  Usually the result of nondisjunction in division of gametes
mother’s alcohol intake per se but to the amount consumed  Incidence increases dramatically with increasing maternal
in excess of the liver’s ability to detoxify it age
 Liver’s capacity to detoxify it is limited and inflexible
 When the liver receives more alcohol than it is able to Trisomy 21
handle,the excess is continually recirculated until the  The most common cause of mental retardation and
organ is able to reduce it to carbon dioxide and water malformation in a newborn.
 The circulating alcohol has a special affinity for brain
tissue Nondisjunction
o The failure of paired chromosomes or sister chromatids to
 Toxic acetyl aldehyde ( a degradation by-product of
ethanol)-may also be teratogenic separate and go to different cells during meiosis.
 Factors that may compound the problem of alcohol abuse
Assessment
o Poor nutritional state
 Facial characteristics
o Smoking
o Almond-shaped eyes
o Polydrug intake
o Round face
o Infrequent or lack of prenatal care
o Protruding tongue
Major Features of FAS
oFlattened posterior and anterior surfaces of the  Engaging in physically dangerous activities without
skull, epicanthus considering the possible consequences
o Flat nose
 Musculoskeletal system Implementation
o Flaccid and joints are loose  Provide safe environment with minimal stimulation
 Extremities o Decrease number of stimuli; reduce extraneous
o Broad hands stimuli
o Abnormal palmar crease o Set limits of behavior
o In-curved fifth finger, first and 2nd toes widely o Structure activities
spaced o Provide for energy outlet, allow large muscle
 Mental capacity movements
o Ranges from slightly incapacitated (educable) to o Provide for quiet area and time
severely retarded  Establish primary relationships if possible with short
contact times
Potential Problems  May establish behavior modification program, encourage
 Congenital Heart Malformation- PDA positive behavior. Assist to build self-esteem which is
 Congenital GI malformation –Pyloric stenosis, Duodenal usually low
atresia, tracheoesophageal fistula  Assist client to establish own controls and behavior
 Hypothyroidism  Coordinate diet: limit sugar, additives and artificial colors
 Visual defect – strabismus, nystagmus, cataracts  Administer medication as prescribed
 Hearing defects-enlarged adenoids, sleep apnea o Methylphenidate hydrochloride (Ritaline) – a mild
nervous system stimulant
Implementation o Permoline (Cylert)
 Refer parents to genetic counseling o Dextroamphetamine suflate (Dexedrine)
 Following discharge from hospital, provide follow-up for o Atomoxetine(Strattera) – new drug to treat ADHD
the family for counseling and child guidance nonstimulant medication that is licensed to
 Alert the parents to child’s increased susceptibility to treat ADHD in children, teenagers, and
infections and the need for extra precautions to prevent adults.
illness  Instruct parents and child regarding medication
administration
ATTENTION DEFICIT-HYPERACTIVITY DISORDER  Inform the child and parents that positive effects of the
(ADHD) medication may be seen within 1-2 weeks if taken as
 A developmental disorder characterized by inappropriate prescribed
degrees of inattention, over activity and impulsivity.  Strattera seems to increase the amount of norepinephrine,
 Incidence: 1-20% in school-age children an important brain chemical. This appears to help ADHD
by increasing attention span and reducing impulsive
Etiology behavior and hyperactivity.
 Unclear  Intuniv has an effect on certain receptors in the brain.
 Recent studies: mother’s intake of essential fatty acids Studies show that it reduces distractibility and improves
during pregnancy is below normal attention, working memory, and impulse control.
 Intuniv is an extended release medicine that contains the
Assessment same active ingredient as Tenex, a blood pressure drug
 Assess presence of diagnostic criteria( modified from that’s been used as an off-label ADHD treatment for years.
American Psychiatric Association DSM-III)  Nonstimulants have some advantages over many
 Behavior must be inconsistent with developmental level, stimulants used for ADHD. For instance, nonstimulants
persist for at least 6 months and demonstrate at least 6 of o Don’t cause agitation or sleeplessness
the following o Are not controlled substances and don’t pose the
1. Inattention same risk of abuse or addiction
2. Impulsivity o Have a longer-lasting and smoother effect than
3. Hyperactivity many stimulants, which can take effect and wear
4. onset before 7 years of age off abruptly
5. duration of at least 6 months  Strattera Side Effects. The most common side effects of
6. Cause is not identified as schizophrenia, Strattera are:
affective disorder, or mental retardation o Upset stomach
o Decreased appetite, which may cause weight loss
Observe for additional traits o Nausea
 Negativistic o Dizziness
 Emotional lability
o Fatigue
 Easily frustrated
o Mood swings
 Nonlocalizing neurological signs, learning disabilities,
 Intuniv Side Effects include:
and abnormal EEG
o Headache
 Talking excessively
o Fatigue
 Interrupting or intruding on others
o Abdominal pain
o Sleepiness and sedation
SPINA BIFIDA
AUTISM  A CNS defect which results from failure of the neural tube
 Is a disorder of neurological development to close during embryonic development
characterized by impaired social interaction and  Associated deficits may include sensorimotor disturbance,
communication, and by restricted and repetitive dislocated hips, talipes equinovarus (clubfoot) and
behavior. hydrocephalus
 All signs begin before a child is three years old or  Defect closure is done immediately after birth
during the first 2 years of life
 A lifelong disorder defined by the individual’s Types
interactive difficulties 1. Spina bifida occulta
 More common in males than females 2. Spina bifida cystica
3. Meningocele
Etiology 4. Myelomeningocele
 Genetic predisposition

Assessment
 characteristic triad of symptoms:
o impairments in social interaction;
o impairments in communication;
o Repetitive behavior.
Other aspects,
o Atypical eating, are also common but are not essential for
diagnosis.]
Communication impairment
 first year of life
Spina Bifida Cystica
May include delayed onset of babbling, unusual
 Protrusion of the spinal cord and/or its meninges occurs
gestures, diminished responsiveness, and vocal patterns
that are not synchronized with the caregiver.  Defect results in incomplete closure of the vertebral and
neural tubes, resulting in a saclike protrusion in the lumbar
 Second and third years,
or sacral area with varying degrees of nervous tissue
Autistic children have less frequent and less diverse
involvement
babbling, consonants, words, and word combinations; their
gestures are less often integrated with words.
Spina Bifida Occulta
Repetitive behavior  Occulta is Latin for "hidden.“ ; mildest forms of spina
bifida.
 Stereotypy is repetitive movement, such as hand flapping,
making sounds, head rolling, or body rocking.  Spinal cord remains intact and usually is not visible
 Compulsive behavior is intended and appears to follow  Meninges are not exposed on the skin surface
rules, such as arranging objects in stacks or lines.  Neurological deficits are not usually present
 Sameness is resistance to change; for example, insisting  The skin at the site of the lesion may be normal, or it may
that the furniture not be moved or refusing to be have some hair growing from it; there may be a dimple in
interrupted. the skin, a lipoma, a dermal sinus or a birthmark
 Ritualistic behavior involves an unvarying pattern of
daily activities, such as an unchanging menu or a dressing
ritual.
 Restricted behavior is limited in focus, interest, or
activity, such as preoccupation with a single television
program, toy, or game. Self-injury includes movements
that injure or can injure the person, such as eye poking,
skin picking, hand biting, and head banging

Interventions
 Determine the child’s routines, habits, and preferences and
Meningocele
maintain consistency as much as possible
 Protrusion involves meninges and a sac-like cyst that
 Determine the specific ways in which the child
contains CSF in the midline of the back, usually in the
communicates
lumbosacral area
 Facilitate communication through the use of picture boards
 Spinal cord is not involved
 Evaluate the child for safety
 Neurological deficits are usually not present
 Implement safety precautions as necessary for self-
injurious behaviors such as head banging
 Monitor for stress and anxiety
 Avoid placing demands on the child
 Initiate referrals to special programs as required
 Provide support to parents
o The child is at high risk for latex allergy

Myelomeningocele HYDROCEPHALUS
 Protrusion of the meninges, CSF, nerve roots, and a portion  An imbalance of CSF absorption, or production caused by
of the spinal cord occurs malformations, tumors, hemorrhage, infection or trauma
 The sac (defect) is covered by a thin membrane prone to  Characterized by
leakage or rupture An abnormal increase in CSF volume within the
 Neurological deficits are evident intracranial cavity and
enlargement of the head in infancy

Hydrocephalus- Types
 Noncommunicating
 Communicating

Noncommunicating Hydrocephalus
 Obstruction in the system between the source of CSF
production (ventricles) and the area of its reabsorption
(subarachnoid space)
 Caused by
Congenital defects- Arnold-Chiari malformation,
Assessment Dandy-Walker cyst, aqueduct stenosis
 Depends on the spinal cord involvement Acquired conditions – infections, trauma,
 Visible spinal defect spontaneous intracranial bleeding and neoplasms
 Flaccid paralysis of the legs
 Altered bladder and bowel function Communicating Hydrocephalus
 Hip and joint deformities  Failure in the absorption system; cause unknown
 Hydrocephalus  Excessive production of CSF-tumor or unknown
causes
Interventions
 Evaluate the sac and measure the lesion Normal Flow of CSF
 Perform neurological assessment
 Monitor for increased ICP which might indicate
developing hydrocephalus
 Measure head circumference; assess the anterior fontanel
for fullness
 Protect the sac; cover with a sterile, moist (normal saline),
nonadherent dressing to maintain the moisture of the sac
and contents and change dressing every 2 to 4 hours as
prescribed
 Place in a prone position to minimize tension on the sac
CSF  Choroid plexus  lateral ventricles
and the risk of trauma; the head is turned to one side for
foramen of Monro ), third ventricle  aqueduct of
feeding
Sylvius (Cerebral aqueduct) fourth ventricle, 
 Change the dressing covering the sac whenever it becomes
foramina of Magendie (Median aperture) and foramina of
soiled because of the risk of infection; diapering may be
Luschka (Lateral apertures)  subarachnoid space
contraindicated until the defect has been repaired
over brain and spinal cord  reabsorption into
 Use aseptic technique to prevent infection venous sinus blood via arachnoid granulations
 Assess the sac for redness, clear or purulent drainage,
abrasions, irritation and signs of infections The CSF is produced at a rate of 500 ml/day. Since the brain
 Early signs of infection include elevated temperature can contain only 135 to 150 ml, large amounts are drained
(axillary), irritability, lethargy and nuchal rigidity primarily into the blood through arachnoid granulations in
 Assess for physical impairment such as hip and joint the superior sagittal sinus. Thus the CSF turns over about 3.7
deformities times a day. This continuous flow into the venous system
 Prepare the child and family for surgery dilutes the concentration of larger, lipoinsoluble molecules
 Administer antibiotics preoperatively, as prescribed for penetrating the brain and CSF.
infection
 Teach the parents and eventually the child about long-term
home care
o Positioning, feeding, skin care and ROM Pathophysiology
o Performing clean intermittent catheterization Tumorobstruction of the flow of CSFincreased ventricular
o Administer propantheline (Pro-Banthine) as pressurethinning of the cerebral cortex and cranial bones (frontal,
prescribed to improve continence parietal and temporal areas)
o Implement a bowel program including a high-fiber Floor of the 3rd ventricle bulges downward
diet, increased fluid, suppositories as needed
Compression of the optic Basal ganglia, brainstem
Surgical Management
nerve  Extracranial Shunt Procedure
& hypophysis cerebri and cerebellum 1. Ventriculoperitoneal (VP) shunt
Dilation of sella turcica choroid plexus atrophied 2. Ventriculoatrial (VA) shunt)
3. Ventriculopleural shunt
Hydrocephalus- Clinical Manifestations 4. Ventriculo-gall bladder shunt
 Infants
o Excessive head growth (maybe seen up to age 3) Ventriculoperitoneal shunts
o Delayed closure of the anterior fontanelle  Diverts CSF from lateral ventricle or the spinal
o Fontanelle tense and elevated above the surface of subarachnoid space to the peritoneal cavity
the skull  A tube is passed from the lateral ventricle through an
o Signs of increased intracranial pressure occipital burr hole subcutaneously through the posterior
o Alteration of muscle tone of the extremities including aspect of neck and paraspinal region to the peritoneal
clonus or spasticity cavity through a small incision in the RLQ
 Later Physical Signs
o Forehead becomes prominent (bossing) Ventriculoatrial shunt
o Scalp appears shiny with prominent scalp veins  A tube is passed from the dilated lateral ventricle through a
o Eyebrows and eyelids may be drawn upward, burr hole in the parietal region of the skull
exposing the sclera above the iris  It then is passed under the skin behind the ear and into a
o Infant cannot gaze upward, causing “sunset eyes” vein down to a point where it discharges into the right
atrium or superior vena cava
o Strabismus, nystagmus, and optic atrophy
 A one-way pressure-sensitive valve will close to prevent
o Difficulty holding head up
reflux of blood into the ventricle and open as ventricular
o Child may experience physical or mental
pressure rises, allowing fluid to pass from the ventricle
developmental lag into the bloodstream
 Older Children
 With closed sutures and present with signs of increased Ventriculoperitoneal shunt
ICP  Diverts CSF to the pleural cavity
o Vomiting
 Indicated when the VP or VA route cannot be used
o Restlessness and irritability
o High-pitched, shrill cry Ventricle-gall bladder shunt
o Rapid increase in head circumference (infants)  Diverts CSF to the common bile duct
o Tens, bulging fontanelle (infants)  Used when all other routes are unavailable

Signs of increased ICP


 Pupillary changes Most shunts have the following components
 Papilledema o Ventricular tubing
 Possible seizures o A one-way or unidirectional pressure-sensitive flow
 Change in vital signs valve
 Increased systolic blood pressure o A pumping chamber
 Decrease pulse o Distal tubing
 Decreased and irregular respirations
 Increased temperature Shunt Complications
 Lethargy, stupor, coma  Need for shunt revision frequently occurs because of
 Older children may also experience occlusion, or malfunction, especially in the first year of life
 Headache, especially on awakening  Shunt revision may be necessary because of growth of the
 Lethargy, fatigue, apathy child
 Personality changes  Shunt dependency
 Separation of cranial sutures  Endocardial contusions and clotting, leading to bacterial
 Visual changes such as double vision endocarditis, bacteremia, and ventriculitis or
thromboembolism and cor pulmonale (VA shunts)
Diagnostic Evaluation Complications of Hydrocephalus
 Infant’s head transilluminates, indicative of abnormal fluid  Seizures
collection
 Herniation of the brain
 Percussion of the infant’s skull may produce a typical
 Spontaneous arrest due to natural compensatory
“cracked pot” sound (Macewen’s sign)
mechanism persistent increased ICP and brain
 Ophthalmoscopy may reveal papilledema herniation
 CT scan is the diagnostic tool of choice  Developmental delays
 With ventriculography, abnormalities are visualized in the  Depression in adolescent
ventricular system or the subarachnoid space
 Skull x-rays show widening of the fontanelle and sutures Nursing Diagnosis
and erosion of intracranial bone
 Ineffective Cerebral Tissue Perfusion related to increased  Usually brief and generalized but may be complex partial
ICP before surgery as well
 Imbalanced Nutrition: Less than Body Requirements
related to reduced oral intake and vomiting Pathophysiology and Etiology
 Risk for Impaired Skin Integrity related to alterations in  Seizures accompany intercurrent infections- viral illness,
LOC and enlarged head tonsilitis, pharyngitis and otitis
 Anxiety of parents related to child undergoing surgery  Appear to occur in a familial pattern
 Risk for injury related to malfunctioning shunt  Unclear whether the seizure is triggered by a rapid rise in
 Risk for Deficient Fluid Volume related to CSF drainage, temperature or the actual temp is attained
decreased intake postoperatively  Most febrile seizure consist of generalized tonic-clonic
 Risk for infection related to bacterial infiltration of the seizures
shunt
 Ineffective Family Coping related to diagnosis and surgery Clinical Manifestations
 Seizures generally last less than 15 minutes
Nursing Interventions  Fever is usually more than 101.8 F (38.8C) rectally
 Maintaining Cerebral Perfusion  Seizures usually occur near the onset of fever rather than
1. Observe for evidence of increased ICP, and report after prolonged fever
immediately
2. Assist with diagnostic procedures to determine Diagnostic Evaluation
cause of hydrocephalus and indication for surgical  CSF examination to detect CNS infection
interventions  CBC and urinalysis to detect signs of infection
A) Explain the procedure  Cultures of nasopharynx, blood, urine as appropriate to
B) Administer prescribed sedative 30 minutes determine cause of fever
before the procedure  Blood glucose, calcium and electrolyte levels to detect
C) Organize activities so the child is permitted to abnormalities that may cause seizures
rest after administration of the sedative  EEG
D) Observe closely after ventriculography for the following
1. Leaking of CSF Management
2. Reaction to the sedative-respiratory depression  Goals – to control seizures and decrease temperature
3. Changes in vital signs-indicative of shock o Administration of antipyretics and other cooling
4. Signs of increased ICP- if air has been injected measures
into the ventricles o Prompt control of fever is the desired course of
action
Provide Adequate Nutrition o Rectal diazepam gel (Rectal Diastat) for prolonged
 Hold the infant is a semi-sitting position with head well seizures greater than 5 minutes or someone who is at
supported during feeding. Allow ample time for bubbling risk for status epilepticus
 Offer small, frequent feedings o Airway management
 Place the child on side with head elevated after feeding to
prevent aspiration Emergency Treatment for Seizures
 Ensure airway patency
Maintaining Skin Integrity
 Have a suction equipment and oxygen available
 Prevent pressure sores by placing the child on a sponge
 Time the seizure episode
rubber or lamb’s wool pad or an alternating-pressure or
 If the child is standing or sitting, ease the child down to the
egg-crate mattress to keep weight evenly distributed
floor placing the child in a side-lying position
 Keep the scalp clean and dry
 Place a pillow or folded blanket under the child’s head; if
 Turn the child’s head frequently; change position at least
no bedding is available, place your own hands under the
every 2 hours
child’s head or place the child’s in your own lap
 Provide meticulous skin care to all parts of the body, and
 Loosen a restrictive clothing
observe skin for the effects of pressure
 Remove eyeglasses from the child if present
 Give passive ROM exercises to the extremities especially
 Clear the area of any hazards or hard objects
the legs
 Allow the seizure to proceed and end without interference
 Keep the eyes moistened with artificial tears if the child is
unable to close the eyelids normally to prevent corneal  If vomiting occurs turn the child to one side as a unit
ulcerations and infections  Do not restrain the child, place anything in the child’s
 Reduce Anxiety mouth or give any food or liquids to the child
 Improving Cerebral tissue perfusion postoperatively  Prepare to administer medications as prescribed
 Maintaining fluid balance  Remain with the child until the child fully recovers
 Preventing infection  Observe for incontinence, which may have occurred
during the seizures
 Strengthening family coping
 Document the occurrence
FEBRILE SEIZURE
 Seizure that occurs in the context of a febrile illness in a Seizure Precautions
previously normal child  Raise the side rails when the child is sleeping or resting
 Pad the side rails and other hard objects
 Place a waterproof mattress or pad on the bed or crib  Assess for changes in level of consciousness and
 Instruct the child to wear or carry medical identification irritability
 Instruct the child to swim with a companion  Monitor I and O
 Instruct the child to use a protective helmet and padding  Assess nutritional status
when engaged in bicycle riding, skateboarding and in-line  Determine close contacts of the child with meningitis
skating because the contacts will need prophylactic treatment
 Alert caregivers to the need for any special precautions  Meningococcal vaccine is recommended to protect against
meningitis
MENINGITIS
 An infection process of the CNS OTITIS MEDIA
 Caused by bacteria and viruses  An inflammatory disorder usually caused by an infection
 Viral meningitis is associated with mumps, paramyxovirus, of the middle ear occurring as a result of a blocked
herpesvirus and enterovirus eustachian tube, which prevents normal drainage
 Bacterial meningitis is frequentlly preceded by an upper  A common complication of an acute respiratory infection
respiratory infection which is complicated by bacteremia –  Infants and children are more prone to otitis media because
invade the CSF their eustachian tubes are shorter, wider and straighter
 Bacteria may also gain entry through penetrating wound, spinal
tap, surgery or anatomic abnormality Assessment
 Infective process – inflammation, exudation and tissue damage  Fever
in the brain  Irritability and restlessness
 Loss of appetite
Bacterial Meningitis  Rolling of head from side to side
 An inflammation of the meninges that follows the invasion  Pulling on or rubbing the ear
of the spinal fluid by a bacterial agent  Earache or pain
 Most cases are seen in children younger than age 5  Signs of hearing loss
 Purulent ear drainage
Etiology  Red, opague, bulging or retracting tympanic membrane
 Birth to age 3 months – E. coli, Streptococcus, group B,
Listeria monocytogenes Interventions
 Age 3 months to 6 yrs – Streptococcus pneumoniae,  Encourage fluid intake
Neisseria meningitidis (meningococcal meningitis),  Teach the parents to feed infants in upright position to
Haemophilus influenzae prevent reflux
 Ages 6 to 16 – S. pneumoniae, N.meningitidis,  Instruct the child to avoid chewing as much as possible
Mycobacterium tuberuclosis during the acute period because chewing increases pain
 Provide local heat and have the child lie with the affected
Diagnosis
ear down
 Lumbar puncture to assess CSF
 Instruct the parents in the administration of analgesics or
o Fluid is cloudy with increased pressure
antipyretics to decrease fever and pain
o Increased WBC count
 Instruct the parents in the appropriate procedure to clean
o Elevated protein drainage from the ear with sterile cotton swabs
o Decreased glucose levels  Antibiotic administrations- emphasize that the 10-14 days
period is necessary to eradicate infective organisms
Assessments  Instruct parents that screening for hearing loss may be
 Fever, chills, headache necessary
 Vomiting, diarrhea  Instruct the parents about the procedure for administering
 Poor feeding or anorexia ear medications
 Nuchal rigidity In a child younger than age 3, pull the lobe down
 Poor or high-pitched cry and back
 Altered level of consciousness-lethargy and irritability In a child older than 3 years, pull the pinna up and
 Bulging anterior fontanel in infant back
 Positive Kernig’s sign (inability to extend the leg when the
thigh is flexed atneriorly at the hip) Myringotomy
 Positive Brudzinski sign (neck flexion causes adduction  Insertion of tympanoplasty tubes into the middle ear
and flexion movements of the lower extremities) in to equalize pressure and keep the ear aerated
children and adolescents  Postop interventions
 Muscle or joint pain o Instruct the parent and child to keep the ears dry
 Petechial or purpuric rashed (meningococcal infection) o The child should wear earplugs while bathing,
Interventions shampooing and swimming
 Provide isolation and maintain it for at least 24 hours after o Diving and submerging under water are not allowed
antibiotics are initiated
 Administer antibiotics and antipyretics as prescribed  Instruct the parents that if the tubes fall out, it is not an
 Perform neurological assessment and monitor for seizures; emergency but the physician should be notified
assess for the complication of ADH secretion – fluid
retention (cerebral edema) and dilutional hyponatremia
 Parents can administer an analgesic such as acetaminophen
to relieve discomforts following insertion of
tympanoplasty
 Parents should be taught that the child should not blow his
or her nose for 7-10 days after surgery

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