Cleft Lip/Palate Incidence

most common craniofacial deformity

Cleft Lip/Palate Etiology

increased risk with history of defect in relatives

15% with CL/P have other defects-feature in other syndromes

genetic syndromes, other facial defects

Cleft Lip/Palate

Pathophysiology

lip: failure of maxillary/median nasal process to fuse

6-8 weeks gestation

palate: failure of 2 sides of palate to fuse

7-12 weeks gestation

lip more in females

palate more in males

less common in af. americans, more common in asians

Cleft Lip/Palate

Presentation

lip, palate, soft palate, hard palate (both)

unilateral, bilateral

dental deformities possible

teeth not coming in right or at all

need follow up with dentist especially with cleft palate

Cleft Lip/Palate

Collaborative Management

diagnosis:

lip - usually apparent

palate - thorough exam of month

sometimes skin may be over to opening and may feel soft like air under skin

management:

orofacial team: plastic surgery

goals:

closure of cleft

prevent complications from surgeries

facilitate normal growth and development

surgery correction

lip: 1st surgery at 6-12 weeks of age

staggered suture line (not straight line) which helps hide or decrease scarring

palate: timing individualized based on degree of deformity and size of child

usually between 6-24 months

Cleft Lip/Palate

Nursing Care: Pre-Op

assessment:

describe and extent of defect

reaction of family

nursing diagnosis:

altered nutrition: difficulty feeding

parenting

ultrasound diagnosis during last weeks of pregnancy mainly to help see for signs of cleft palate (infant at high risk for resp. difficulty during the birth)

interventions

child

feedings-monitor weight: cup feedings, syringe feeding, habermin bottle has longer nipple

parent education

pre-op training

parents

education

support-nursing and parent-parent

attitude of acceptance

Cleft Lip/Palate

Nursing Care: Post-Op

nursing diagnosis

cleft lip: protect surgical site

feeding: no sucking on bottle, pacifier; will do cup or syringe feeding

positioning

comfort

cleft palate: protect surgical site

feeding: no pacifier or bottles

airway: make sure no swelling or airway

infant often in elbow restraints to keep from touching face and sucking on anything

those with hx of cleft lip/palate are at higher risk for improper tooth alignment when teeth come in, ear infections

Esophageal Atresia

Tracheoesophageal fistual

incidence

1: 3000-3500 live births

no gender difference

increased with premature birth

pathophysiology: failure to foregut (esophagus) to differentiate into esophagus/trachea that creates passage to stomach

4-5 weeks gestation

s/s: cough, choking, cyanosis (at birth)

often may have apnea, increased resp distress after eating, abdominal distention, really frothy secretions (white, cloudy) from the mouth

TF Fistula

Types

esophageal atresia with distal TEF: 85%

esophageal atresia without fistula: 6-8%

proximal esophageal fistula with trachea: distal segment blind: 1%

proximal and distal fistulas with trachea: 1%

TEF without atresia (H-type): 4%

TF Fistula

Diagnosis

s/s: excessive drooling/salivation (with blind pouch) - d/t no where to go, apnea, cannot pass NG tube, increased resp distress after feeding, abdominal distention or flat abdomen

x-ray diagnosis

if mother has hx of polyhydramnios, will have higher risk for TEF in infant

TF Fistula

Collaborative Management

prevent pneumonia

surgery

ligation of fistulas, end-end anastomosis of esophagus to stomach; staged procedure

may require trach r/t trachealmalacia

motility disorders common after repair

if suspect TEF: NPO frequent suctioning, need to be on pulse ox and heart monitor, position child 30 degrees HOB on back

TF Fistula

Prognosis

prognosis r/t birth weight, other anomalies, time of diagnosis

100% prognosis in healthy full-term infants without respiratory distress

increased complications with low birth weight and other anomalies

overall mortality 10-15%

TEF often seen with certain other genetic disorders

TF Fistula

Nursing Care

assessment:

respiratory distress

manage excessive oral secretions

choking - NPO

cyanosis

difficulty swallowing

emesis

look for other defects

hardest post-delivery is the emptying into lungs resulting in pneumonia and causes a drowning of secretions

interventions: child

first feed

prevent aspiration

fluids

positioning (HOB 30 degrees)

NG - try to see if it can pass

interventions: parents

plan of care

do not let feed, suck on pacifier, etc. - d/t swallowing secretions and aspirating

TF Fistula

Nursing Care: Post-Op

interventions

respiratory status

fluids

nurition

thermoregulation

comfort

infection

skin

parents

Hernias

diaphragmatic - (bad one) congenital where diaphragm is opening and allows contents below diaphragm to go upward and take up space in the upper abdominal area

hiatal - sliding of  stomach up through small opening in diaphragm (tear or weakness)

abdominal

umbilical

omphalocele

gastroschisis

Diaphragmatic Hernia

diaphragm opened up and allows contents of GI to travel up into upper body

less space for heart and lung, can happen during fetal development and results in difficulty in heart and lungs to grow

usually have poor lung development; diagnosed on ultrasound on pregnant mother

usually infants will be delivered in intensive care

mortality very high

Hiatal Hernia

if severe, can cause esophageal stricture, pain, split up after feeding; parents should not lie child down after feeding, keep up about 30=45 degrees for half hour to 45 minutes to prevent reflux

sliding of stomach up through opening in diaphragm (d/t tear or weakness)

large can cause esophageal stricture

can cause spitting up after feedings, pain after feedings

parents; do not lie child down after feeding for 30-45 minutes to prevent reflux

Umbilical Hernia

usually correct by age 5 years d/t stomach muscles getting stronger

if not corrected by 5 years, surgery done

if moving on own, its nothing to worry about

when child may cry, it may make it bigger

Omphalocele

defect in abdominal wall in which it doesn't close, usually has sac covering, abd organs on the outside of body (intestines, liver, maybe kidneys, etc)

at level of umbilicus and organs in protective visceral sac

can see on ultrasound

usually some other congenital defect occuring

born by Csec, surgery close after delivery

protect sac with NS and wet sterile gauze to cover - staged surgical procedures usually (skin stretching because sometimes not all organs can fit back all at the same time)

high risk for infection or losing part of bowel functioning

Gastroschisis

opening of abd wall with  no protective covering

protect organs

requires surgery

belly-band to keep sutures from opening

higher risk for infection or for losing part of bowel function

Pyloric Stenosis

increased Northern European ancestry

first-born male 4 x's

family history esp mother

20% males

10% females

B&O blood groups

other defects

usually diagnosed in the first 6-8 weeks of life; sometimes seen with other defects

Pyloric Stenosis

Patho/Etiology

etiology: unknown

patho: circular muscle of pylorus enlarged resulting in severe narrowing of pyloric canal

dec. stomach emptying and content will build up

s/s extreme vomiting after feeding (exorcist baby); electrolytes screwed up; weight loss; feeding intolerance; gastric reflux

upper GI ultrasound diagnosis first - infant NPO for a few hours and then went ultrasound done, feed baby, and watch

occasionally in URQ can palpate "olive-shaped" mass, but honestly difficult to feel

Pyloric Stenosis

Diagnosis

non-bilious vomiting immediately after feeding, progresses to projectile

usually start after 3 weeks of age and progressively get worse

labs - electrolyte imbalance, weight loss, hx of feeding intolerance

UGI or ultrasound of pyloric sphincter

infant NPO for a few hours

palpation of pyloric mass - "olive shaped" mass in UQ

Pyloric Stenosis

Collaborative Management

surgery

pyloromyotomy: longitudinal incision through circular muscle fibers stopping short of mucosa

correct acid-base disturbance

incision through muscle fibers in abdomen

IV fluids to correct electrolytes

Pyloric Stenosis

Nursing Care: Pre-Op

nursing diagnosis

interventions

hydration

electrolyte balance

NPO/IV fluids

I&O, daily weights

vital signs

skin

Pyloric Stenosis

Nursing Care: Post-Op

nursing diagnosis

interventions

NPO/Fluids at first start of post-op and inc. slowly

NG - not often done post-op; only if having complications

Resume feedings (start on clears and advance)

comfort - pain from incision

incision care

parents

Intestinal Atresia

Duodenal

malformation of duodenum

25-40% of intestinal atresia

20-30% trisomy 21

female

33% with other life-threatening anomalies

most occur just distal to ampulla of Vater

Duodenal Atresia

s/s & tx

s/s

bilious vomiting with abdominal distention

history of polyhydramnios

xray of abdomen

treatment

gastric decompression

thorough check for other anomalies

surgery

j-tube feeds

Jejunal & Ileal Atresia

rarer and harder to diagnose

one third are premature

60-75% do not pass meconium

diagnosis: xrays

treatment: surgical resection

Biliary Atresia

obstruction or absence of extrahepatic ducts

high risk for liver failure

females slightly > males

50% liver transplants

etiology: unknown, no familial link

liver enzymes high values

exploratory lap to diagnose

birth poor weight gain, extreme irritability, difficult to comfort, FTT, jaundice

80-90% will get liver transplant

intially try a Kasai procedure - take part of intestine and make a port to drain liver

Biliary Atresia

Clinical Manifestations

healthy at birth

jaundice, acholic stools, bile-stained urine and hepatomegaly

overtime develops malnutrition, growth failure and fat soluble vitamin deficiencies

Biliary Atresia

Diagnosis

diagnosis

liver function labs

screening for metabolic disorders

hepatitis screen (see extent of liver damage)

biopsy

seen with a lot of metabolic disorders

mitochondrial disorders will not get liver transplant

Biliary Atresia

Collaborative Management

treat malnutrition

wasting (arms and legs skinny)

ascites easily

portal hypertension

esophageal varices

surgery:

hepatic portoenterostomy

liver transplant

Biliary Atresia

Nursing Care

nutritional support

skin care - jaundice, bile salts make itchy (antihistamine such as benadryl)

developmental stimulation - need chances to practice growth and development for first couple years of life

continued assessment

education

emotional support

Hirschsprung Disease

congenital aganglionic megacolon

incidence

most common cause of intestinal obstruction in neonates

males 4x's over females

associated with trisomy 21 and others

familial link with a certain subtype

Hirschsprung Disease

Patho

patho

lack of ganglion cells in bowel wall 75% limited to rectosigmoid

this part of intestine is dead, nothing passes through it

meconium doesn't pass in first 2 days

abd distention

Hirschsprung Disease

Clinical Manifestations

newborn: doesnt pass within first two days, abdominal distention, vomiting, bilius-looking vomit, poor feeding, weight loss, FTT

infancy: FTT signs (constipation, abd distention, periods of contipation and diarrhea)

childhood: abd distention, periods of vomiting, look malnourished (FTT look)

Hirschsprung Disease

Collaborative Management

surgical removal of aganglionic portion of bowel and make it an ostomy

done in 2 stages - temporary colostomy

surgery 2 stages

1st - aganglionic portion of bowel and make ostomy

2nd - get rid of ostomy and do a pull through procedure (pull down procedure and connect to rectum)

Hirschsprung Disease

Nursing Care

depends on age of diagnosis

neonate

parents

abdominal circumference

infant/child

parents

bowel prep

nutrition

post-op: care same as other abdominal surgery

Meconium Ileus

failure to pass meconium within 24-36 hours after birth

often an initial sign of cystic fibrosis

Imperforate Anus

all anorectal malformation classified as imperforate anus

type differs according to site of rectum to puborectalis muscle

diagnosis

exam of perineum and rectum

observe passage of meconium

Imperforate Anus

Collaborative Management

depends on type of defect

anal stenosis: manual dilations (different size of dilators that gradually inc in size)

surgery

lower anomalies: reconstruction

higher anomalies: temporary colostomy

nursing care

depends on surgery

if lower anomalie: do not do rectal temps (cause tearing); stool softeners to prevent straining

if higher anomalie: temp colostomy care

Intussesception

telescoping of one portion of intestine into are next to it

most common cause of intestinal obstruction in children 3 mo - 5 yrs

50-60% occur in child less than 1 year old

80% prior to 2 years of age

increased in males: 4 to 1

increased in spring and fall - may be related to GI/stomach viruses (change in motility, etc)

increased with cystic fibrosis

Intussusception

Clinical Manifestations

sudden acute abdominal pain

vomiting

lethargy

passage of red, currant-jelly like stools

tender, distended abdomen

palpable sausage-shaped mass in ULQ

evential fever, prostration, septic shock s/s peritonitis if not treated

Intussesception

Collaborative Management

non-surgical hydrostatic reduction

works 50% of time if s/s > 48 hrs

75-80% of time within 1st 48 hrs

not done with s/s shock or perforation

surgery: reduction and resection

Intussescpetion

Nursing Care

teaching - explain what it is, how procedures work

prep for surgery - NPO

hydration - correct electrolyte imbalance and monitor/correct dehyrdation status

post-op - NPO for a little while work way up with clear liquids and advance up to solids

Diarrhea

one of leading causes of death throughout the world

acute: 0-7% pediatric deaths

continued: 10-35%

Consequence of Diarrhea

dehydration

electrolyte imbalance

metabolic acidosis

Acute Diarrhea Causes

bacterial: e coli, salmonella, c diff (meds, contaminated food)

viral: rotavirus (spring/fall common times; very severe dehydration seen)

parasites: giardia, shigella (in contaminated drinking water)

dietary: toddler's; normal finding, nothing to worry about as much - going from formula to eating foods; various eating habits as well day to day

medications: antibiotics

Diarrhea Treatment

prevention - infection control (if someone in family has stomach virus)

oral rehydration therapy (usually first step to correct; use Pedialite)

IV fluids

probiotics (also found in yogurt)

medications

diet - stop intake that increases diarrhea (no fiber, low sugar, no sodas/koolaid/jello/juices or other concentrated sweets)

skin care - leave rash open to air, zinc oxide ointment

Chronic Diarrhea Causes

malabsorption

inflammatory

immunodeficiency

motility disorders

allergies

lactose intolerance

parasites

Malabsorption

digestive defects: enzymes for digestion decreased or absent

cystic fibrosis, biliary disease

absorptive defects: intestinal mucosal transport system impaired

anatomic defects

Malnutrition

Kwashiorkor

infants are lethargic, irritable, decreased growth, enlargement of liver and lost of muscular tissue, wasting of extremities

d/t decrease in protein intake (not enough food supply possible)

get fluids back on track and then slow reintroduction of food

marasmus: protein/calorie deficiency

causes

improper feeding habits

insufficient diet

metabolic/congenital malformation

s/s: FTT, loss of weight, atrophy of muscles, decreased vital signs

treatment: correct cause if you can

Celiac Disease (AKA) Celiac Sprue

disorder of small bowel mucosa

does not present until exposure to gluten products

6 mo - 2 yrs old (depending on when food intact intakes place)

genetic link: 2-5% 1st degree relatives symptomatic, 10% asymptomatic

Celiac Disease

Clinical Manifestations

incidious/chronic process

celiac crisis: acute severe episode of severely watery diarrhea and vomiting

thin look, wasting

diarrhea (steatorrhea), anemic, abd distention, irritability, FTT, weight loos

diagnosis: biopsy of small intestine

takes place when exposed to gluten products (wheat, barley, rye, oats)

restrict intake of lactose food

Celiac Disease

Dx & Tx

diagnosis

jejunal biopsy

dramatic improvement with gluten free diet

treatment: gluten free or very low diet

wheat, barley, rye, oats, white flour need to avoid

rice, corn, potatoes, tapioca fine

Gluten Free Diet

restrict intake of lactose foods to help mucosa heal

restrict intake of gluten containing foods (wheat, barley, etc)

Celiac Disease

Nursing Care

nursing diagnosis

assistance with adherence to diet

early on need diet high calorie and proteins, simple CHO, low fat

avoid high fiber until gut healed

education of parents - reading labels, types of food you can get in restaurants, etc

Short Bowel (Gut) Syndrome

loss of at least 50% of small intestine

common causes

congenital anomalies

ischemia

trauma

vascular injury

small intestine does adapt

increase height of villi

increased number of cells/absorptive area

Short Gut Syndrome

Clinical Manifestations

malabsorption

diarrhea

FTT

dehydration

hyponatremia, hypokalemia

acidosis

anemia

Short Gut Syndrome

Collaborative Management

goals

preservation of as much bowel as able

maintain child's nutritional status

stimulate adaptation of bowel

enternal nutrition - elemental formula (specific, that can be digested however, not uncommon for child to be completely n TPN)

parental nutrition

Short Gut Syndrome

Nursing Care

nursing diagnosis

nutrition

oral stimulation program (if cant take anything by mouth)

prevent complications (bacterial overgrowth, higher risk for rejection, central line infection)

appropriate growth and development

short bowel transplants (rejection rate very high; often need liver transplants too)

Constipation

alteration in the frequency, consistency, or ease of stool passage

causes can be from disorders of the GI tract or systemic disorders and drugs

often begins in infancy

often occurs in childhood

entry to school, painful bowel movements

encopresis - bowel clean out (milk mag to get out everything)

stricture in intestines, hypothyroidism, certain meds (iron supp, vitamins with irn, antihistamines, opioids (morphine)), switching diet (breast to formula, formula to whole, increase intake of table foods), holding it at school, daycare, summer camp, etc

management: increase fiber, intake of water (decrease milk if too much, skim milk), establish bowel routine (evenings after meal best), stool softeners (miralax)

bowel clean-out

Pinworms

most common heminthic

eggs are ingested or inhaled

female worms migrate outside of the rectum and lay eggs, causing intense itching

child scratches, eggs get under fingernails, child reinfects

diagnosis - tape test

management

vermox (one dose then repeated in 2 weeks to make sure)

can live indoor for 2-3 weeks

can also get from untreated water, people growing food where using bathroom (3rd world)

Acute Appendicitis

inflammation of the vermiform appendix

patho

lumen of appendix gets obstructed, pressure builds, compression of vessels, necrotic area (bacterial invasion, appendix perforates, spreads)

management

rehydration, antibiotics, appendectomy

if peritonitis, antibiotics before and after surgery, NG, penrose drain

s/s pain at belly button to RLQ, low grade fever

fever and sudden relief of pain may be perforated appendix; tachy, chills, etc.

signs of shock

post - NG tube and pinrose drain if had peritonitis

Meckel Diverticulum

an opening that persists after fetal closure was to occur

fibrous band holds the small intestine to the umbilicus

complications include bleeding, obstruction, and inflammation

most common CM is painless rectal bleeding (current jelly stools), abdominal pain, intestinal obstructio

surgical removal of the diverticulum

Lead Poisoning