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Flashcards in Congenital Disorders Deck (29):
1

Cleft Lip

S/S:
-7-8th week of embryonic development
-Hereditary predisposition
-more common in males
-characterized by fissure or opening of the upper lip
-visual,problems feeding, cosmetic
-indication for other underlying problems

Treatment:
-closure within the first few weeks of life
-difficulty swallowing because a seal is not created
-use syringe w/ rubber tip medicine dropper
-feed in upright position to prevent aspiration
-chieloplasty: repair of upper lip
-repair lip as soon as baby is born

Nursing Care:
Post op:
-at risk for aspiration position side lying or on back once conditions get better
-Logan's bar-hold lip in place
-Elbow restraints
-water after feedings
-cleanse incision
-monitor for infection
-pain management
-no sucking, reduce crying
-feed in upright position w/ rubber tip syringe

2

Cleft Palate

S/S:
-Failure of hard palate to fuse during 7th-12th week of fetal development
-may be seen alone or with cleft lip
-forms passage way btw nasopharynx/nose
-unable to suck
-frequent ear and respiratory infections
-difficulty speaking

Treatment:
-palatoplasty before 18months(when speech starts to appear)

Nursing Care:
Pre Op:
-Sucking is important for speech
-Hold child upright to feed
-burp frequently
-LIMIT FEEDING TIME TO 20-30MINS
-feed slowly dont tire the child
-small amounts of strained foods at a time
-food may seep through nose
-provide good mouth care
-speech will be affected
-pre op may be fitted for obdurator feeding
Post Op:
-clear liquids-full liquid-soft diet
-no suckin
-feed by large spoon
-avoid objects that can disrupt the suture line
-water after feedings
-suction prn
-elbow restraints
-pain management
-speech therapy

3

Tracheo-Esophageal Fistula(opening)

Definition
-congenital defects
-abnormal opening
-mother polydramios

s/s:
-3 C's
-Coughing, Choking, Cyanosis

Treatment:
-NPO
-G-Tube
-Surgical Repair

Nursing Care:
Pre-op
-observe s/s of resp distress
-prevent aspiration
-NPO
-Supine with HOB 30 degrees
Post-op
-Gi feeding
-pacifier
-elevated HOB
-emotional support
-gradual increase in feeding

4

Esophageal Atresia

-esophagus ends in blind pouch not connected to stomach
-s/s: vomitting, coughing, choking, cyanosis
-treatment: surgical repair

5

Pyloric Stenosis

Definition:
-sphincter spasm or overgrowth
-no symptoms until third week of life
-more common in males

S/S:
-projectile vomiting(non bilius vommiting)
-hunger
-dehydration
-small round mass in RUQ(olive like mass)
-visible peristalsis
-food has difficulty exiting the stomach

Treatment:
-surgical intervention
-pyloroplasty

Nursing Care:
Pre Op:
-NPO, NGT, IV, i/, Thickened Formula
Post Op:
-Position on right side with HOB elevated
-small amounts of H20 feedings 3-4X
-then diluted formula then progress
-avoid pressure on suture line
-observe for s/s of infection
-monitor for hydration
-some vomiting 24-48hrs after
-report vomiting beyond 48hrs

6

Celiac Disease

Definition:
leading malabsorption problem
-inability to metabolize and absorb glutens(wheat, barley,oats,rye)
-presents at 6months to 2 years as new foods are introduced into the diet

s/s:
-frequent large bulky frothy stool
-FTT(Failure to Thrive)
-weight loss(not gaining any weight)
-abdominal distention
-anorexia

Diagnostic Test:
-stool analysis
-serum IgA
-small bowel biopsy

Treatment:
-dietary restriction-gluten free
-supplemental vitamins
-infection prevention
-patient education(peanut butter has hidden gluten)
-good to eat fresh fruit and veggies

7

Hirschsprings Disease
(Aganglionic Megacolon)

Definition:
-aganglionic megacolon
-absence of ganglion cells in bowel
-can cause partial or complete bowel obstruction
-lack of nerve endings no peristalsis

Diagnosis:
-rectal biopsy(absence of nerves)
-history of meconium ileus

S/S:
-meconium ileus-not passing stool in first 24hrs
ribbon like stools
constipation/alternating with diarrhea
-failure to thrive
-potentional for entercolitis

Treatment:
-abdominal resection=anastomosis
-temporary colostomy

Nursing Care:
-monitor vs, bs, and incision
-ngt to low wall suction
-strict I/O, IV FLuids
-colostomy care
-erythromycin/neomycin-medication given for side effect: diarrhea

8

Intussusception

Definition:
-telescoping into distal end of intestines
-more common in males

Diagnosis:
-Barium enema
-abdominal flat plate

S/S:
-severe abdominalpain
-vomiting bile
-currant jelly stools(red/mucous stool)

Treatment:
-surgey
-spontaneous repair
-exploratory laparotomy with resection if needed

Nursing Care:
-increased pulse
-pallor
-diaphoresis
-hypotention
-ridid abdomen

post op care:
-possible NGT
-npo until bowel sounds return
-clear liquids



9

Meckels Diverticulum

Definition:
-out pouching in ileum
-develops in uterol(congenital)
-contains gastric and pancreatic tissue

S/S:
-occur by 2yr
-bleeding
-abdominal pain

Treatment:
-surgery
-good prognosis

10

Hernias

Definition:
-protrusion of orgran through the peritoneum
Location: umbilical, inguinal, diaphramatic
Types:
-reducible wiith gentle pressure can be flattened
-non reducible-surgery needed
-incarcerated-emergent surgery,necrosis may occur

S/s:
-mass at the site
-pain
-irritability
-vomitting

Treatment:
-hernorraphy

Nursing Care:
-pre op
-routine nursing care

post op care
-npo until bowel sounds return
-observe for sign and symptoms of infection

11

Gastroenteritis

Definition:
-acute, chronic or infectious characterized by N,V,D

S/S
-watery stools
-abdominal cramps and pain
-potential for dehyration

s/s of dehyration
-weight loss
-no urine output for 6hrs
-no tears(older than 3yrs)
-sunken eyes
-dark circles under eye
-listless, decrease activity
-poor skin tugor

treatment:
-stool analysis
-serum IGA

Hospitalized care
-isolate child
-iv fluid
-clear liquids
-BRAT DIET: banna rice apple sauce toast
-handwashing

Nursing care
-strict i/o, monitor v/s, daily weight, good skin care

patient teaching for child at home:
-small amount of fluid every 30mins on 1tsp
-progress slowly to solid foods when vomiting stops
-avoid milk products
-no OTC meds(anti-diarreals)

inform md if
-sudden high fever
-serve abdominal pain
-blood in stool

12

Appendicitis

Definition:
-inflammation of small appendage at the cecum

Diagnosis
-CT,X-RAY

S/S:
-elevated wbc(25-2600
-elevated temp.(101-104)
-abdominal pain and rebound tenderness @ Mcburneys point
-RLQ abdominal pain

Treatment:
-surgery appendectomy
-laproscpoic-scope of the abdomen

pre-op:
routine, npo(until bowel sounds return), pain management, no laxative

Nursing Care:
npo, monitor BS, early ambulation(prevent DVT, infection, help with bowel sound movement), monitor incision, vs, no laxative, antibiotics(dirty surgery)

13

Failure to Thrive

Defintion:
-general term for babies who are not growing or are losing weight
-can be physical or environmental

s/s:
-weak, irritable, nausea, vomitting, diarrhea, listless, apathetic(no emotion)

Treatment:
-correction of cause, parent teaching,. nutritional consult. observation of G&D

14

Colic

Definition:
abdominal pain caused by spasm of the intestines

S/S:
-loud cry, pull up of arms and legs, spitting up mucous, undigested formula
-most kids have this up to a year

Treatment:
-feed slowly, reduce amount of air
-frequent burping
-rocking gently

Nursing care
-risk for abuse and shaken baby

15

Gastro-esphageal reflux disease

definition:
-relaxation of cardiac sphincter allows gastric contents to return to esophagus
-common in premature infants
-can lead to aspiration PNA APNEA

diagnose: endoscopy, xray

s/s:
-vomitting, weight loss,irritability,fussy baby

Treatment:
mild: thickend formula, upright postition, meds to reduce acid
severe: surgery(fundopilcation) anchors sphincter below the diaphragm

Nursing care:
-may have GT post op
-teach patient to use GT
-asiration precautions
-oberve for vomiting

16

Lactose intolerance

defintion:
inability to digest/absorb lactose

s/s:abdominal distention,cramps,diarrhea

treatment:
-lactose free dire
-soy formula/diary products(based on individual)

.

17

Cystic Fibrosis

Defintion:
-lack of enzyme pancrease
-thick secretions
-decrease motility

s/s:
-bulky stools(sterrohhea)
-meconium ilieus
-prolapse rectium

Nursing Care:
-pancrease before meals and large snacks
-well balanced diet(HIGH CAL/HIGHT FAT/HIGH PROTEIN)
-increase fat soluble vitamins
-family education

18

Parasitic Infection

defintion:
pinworms(enterobiasis)
enter by being ingested or through anus
Diagnosis: tape test

s/s:
-anal itiching
-irriatility
-poor appetite/weight loss

treatment:
antihelminitcs
short fingernails
warm baths
linens in hot h20
soothing ointments

19

Toxic conditions:
lead posioning

defintion:
lead ingested from older housing, paint, drinking water, food grown in contaminated

PICA-children eat lead

s/s:
-lowering cognitive functioning
-hearing impairment
-growth delays

treatment:
-chelation therapy: chemical absorbs the lead and body excretes chemical
-treated for 3-5 days go home for a week then come back to see levels

nursing care:
community teaching
school nurse obervation

non-specific poisoning:
-call poison control
-ask substance amount, time frame
-do not induce vomitting

patient teaching
-household safety
-give large volume of milk and water

20

Diagnostic procedures for urinary system

-U/A: urinalysis(not sterile), color,rbc,wbc,ph, u-bag if cant urine properly

-ultrasound-size,shape,structure and location

-intravenous pyelogram-injection of dye to visualize flow of urine(allergy)

-cat scan-tumors or abnormality of structure

-biopsy-tissue analysis

-cystoscopy-scope to visualize, inside of bladder and urethra

-vcug-injection of dye to fill bladder and observe emptying

21

Urinary problems

Dysuria-difficulty unrinating

Frequency-how often urination occurs

Urgency-inability to wait to unrinate

Nocturia-waking @ night to urinate

Enuresis-Bed wetting

Polyuria-large amounts of urine

Oliguria-lack of or small amounts of urine

22

UTI

-more common in female, infancy, 2-6year
-e. coli
-girls have shorter urethra
-location of urethra is near anus
-improper wiping
-wearing of close fitting nylon underwear
-retention of urine
-vaginintis
-normal urine is acidic=decreases incidence of bacteria
-alakaline urine favors pathogens

s/s:
infants:
fever,frequent urination,foul smelling urine,persistant diaper rash,jaundice,convulsions,vomitting,FTT
older child:
urinary frequency pain during mictuntion
-onset of bedwetting in previously dry child
-abdominal pain

Diagnosis:
-urinalysis (SG decreased, +protein,+wbc,+cast)
-urine culture and sensitivity(+ growth of bacteria)
-cbc
-increased wbc

Treatment and nursing care:
-infants under 1year
-hospitalized for i/v antibiotics
olderchild
-treat at home w/ oral antibiotics
-increase fluids
-comfort measures
-monitor vs(esp temp)
-adminster antibiotics
-parent teaching

nursing care
-stress need for proper amounts of fluid to maintain sterility and flushing of the bladder
-proper perineal cleaning(front to back)
-completion of antibiotics
-emptying the bladder when the urge is felt
-encourage complete bladder emptying
-avoid bubble baths
-cotton underwear
-keep peri area dry
-handwashing b4 and after bathroom use

23

Nephrotic syndrome(Nephrosis)

defintion:
a number of different types of kidney conditions that are distinguished by the presence of marked amounts of protein in the urine, edmea, hypoalbuminemia

s/s:
-large amounts of edmea(anasarca)
-ascities(fluid in the abdomen
-child gains weight on daily weights
-scrotum large in males
-pallor(pale)
-no energy
-anorexia
-bp is usually normal
-albumin levels high
-urine exam reveals massive albumin and few RBCs

Treatment:
-diagnosis: urinalysis, proteinuria, albuminuria, hematuria
-observation of symptoms
-diuretics have not been effective in reducing nephrotic edema
-steroids -prednisone
-immunosuppressants
-fluids are generally not restricted except when massive edema is present

Nursing care:
-diet avoid added salts to food
-strict monitoring of I/O
-daily weights
-protection from infetion
-measure abdominal girth-use same measurement
-daily urine testing for protein
-postioning
at least every 2 hrs
-scrotum support
-frequent turning to prevent resp. tract infection
-HOB elevated to prevent eyelid edema
-swelling impairs the circulation of the lacrmal secretions
-responds well to prednisone

patient teaching:
-supportive care to the parents and child throughout the disease
-parents instructed to keep daily record of the child weight, urinary protein levels, and medication
-steroids mask infection,therefore it is important to monitor the child for signs of infection

24

Acute Glomerulonephritis(Brights Disease)

Defintion:
allergic reaction to group A beta hemolytic streptococcal infection
-antibodies prodiced to fight the invading organisims also react against the glomerular tissure
-both kidnetys are affected usually
-mild cases generally recover with a couple of weeks
-occur twice as often in boys
-peak ages 6-7 years
-stage 1 renal failure in young adults

s/s:
-periorbital edema epon wakening in the morning
-urine is smokey brown or bloody
-urine output maybe decreased
-urine specifc gravity is high with albumin, rbc,wbc, and casts
-hyperkalemia
-elevated BUN,creatinine, and ESR
-HTN may occur
-increased temp
headache, malaise, vomitng

treatment:
-urinalysis 3-4+ protein in urine, proteinuria
-BUN increased
-azotemia-excessive amounts of nitrogen due to kidney failure to remove from the blood
-chest-xray - show cardiac enlargement and pulmonar congestion
-H/o of strep infection 1-2 wks prior
-Antistreptolysin titer(ASO) elevated

S/s:
-sudden flank pain
-irritabilty, malaise, fever, hematuria, dysuria, edema

Nursing care:
-supportive treatment, bed rest, fluid restriction, check if i/v above fluids, strict i/o, monitor vital signs, observe for signs of cerebral edema- change in behavior
-encourage rest periods(as child starts to feel better they want to increase their activity but still need rest)
-anti-hypertentive if necessary check if bp and pulse
-antibiotics if blood cultures are positive for strep
-adequate nutritution(low salt)

25

Wilms Tumor(Nephroblastoma)

defintion
-one of the most common malignancies of early life
-embryonal adenosarcoma, thought to have a genetic basis
-most discovered before the age of 3years old
-few or no symptoms during the early stages of growth

s/s:
abdominal mass usually found by parent or during routine health checkup
-IV pyelogram reveals a growth, tumor compresses kidney tissue, usually encapsulated
-may cause hyertension

treatment:
-combination of surgery
-radation/chemotherapy
-affected kidney and tumor are removed as soon as possible afer diagnosis confirmed

nursing care:
-pre-op
-avoid abdominal examination by care-givers
-routine postoperative care
-prognosis
-good with surgery, chemo,radiation

26

Hydrocele

definition:
-excessive amount of fluid in the sac that surrounds the testicle and causes the scrotum to swell
-can be mistaken for hernia
-a chronic hydrocele that persist beyond 1 year is corrected by surgery. small incision made and fluid is drained.

27

cyrptorchidism(undescended testicle)

defintion:
-testes fail to descend in the scrotum
-unilateral form is more common
-testes are warmer in abdomen, sperm cells begin to deteriorate
-if both testes invovled sterilty can result
-inguinal hernia often accompanies this condition
-secondary sex characteristics are not affected because the testes contine to secrete hormones directly to the bloodstream

treatment:
-hormonal management before surgery consist of adminstration of human chronic gonadotropin(hcG)
-may precipitate descent of the testes into the scrotal sac
-orchiopexy improves the condition,fertility rate among these patients may be reduced
-increased risk of testicular turmors s the child reaches aduthood .

nursing care:
-scrotal support
-prevent contamination of suture line
-teach testicular self exam
-psychological and emotional support
-surgery on private parts can be embrassing
-nursees assures the child that his penis will not be affected
-before 18month
-before school age

28

Hypospadias and Epipadias

Hypospadaias: a congenital defect in which the urinary meatus is located on the lower portion of the shaft. more comon, may be accompanied by chordee, a downward curvature of the penis from fibrous band of tissure

Epispadias: urinary meatus is on the upper surface of the penis-less common

-in mild cases surgery not indicated
-surgery may be indicated if child will not be able to stand to void, it may cause psychological issues or difficulties in future sexual relationships
-surgery is usually performed before 18months of age
-routine circumcision is avoided in these children, because foreskin may be useful in the repair

29

Enuresis

defintion:
bed wetting