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
