Congenital Disorders Flashcards
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
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
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
Esophageal Atresia
- esophagus ends in blind pouch not connected to stomach
- s/s: vomitting, coughing, choking, cyanosis
- treatment: surgical repair
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
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
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
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
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
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
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
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)
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
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
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
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)
.
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
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
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
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
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
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
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
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)
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
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.
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
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
Enuresis
defintion:
bed wetting
