A.

) Structural Defects
1.) Cleft Lip/ Cleft Palate
Single or combo of both
Maxillary process fails to fuse



Causes (both)
Maxillary process fails to fuse
Environment
Genetics
Folate (vit B)
Feeding Difficulties:

Cleft Lip = Cleft Palate =
Depends on severity CONCERN=ASPIRATION
Sucking problems Sucking problems
Breastfeeding is ok Breastfeeding is more difficult
BOTH=
Positing UPRIGHT for feeding
Frequent burping r/t swallowing air while feeding
Bulb syringe PRN
WT gain- closely monitor wt gain to make sure infant is getting nutrition needed

Envi/genetic:
CL
Diagnosis:
More common(1:700) CL
genetic- siblings/parent Visible at birth
CP Early detection with US
Less common (1:2000) CP
chance there are other congenital defects During newborn assessment-
(VACTERL Syndrome) nurse assesses with gloved finger
-vertebral, anorectal, cardiovascular, renal, & limb abnormalities

Treatment: Surgical Repair
Pre-op teaching
Arm/elbow restraints after surgery, so teach parents to apply them occasionally before (so child gets used to
them)
CL done FIRST (2-3 mon)- Cheiloplasty
Post-op
SUPINE position sitting up in infant seat (protects surg site)
Elbow restraints
Clear liquids then resume formula/ breast milk, using breck feeder

CP done prior to speech development (18 mon)
Pre-op need to be drinking from cup, weaned from pacifier, & get used to blenderized food (for postop)
Post-op
pain mgmt.; analgesics and sedatives routinely (NOT PRN)
Lie
o n A BDOMEN (promotes drainage)
Elbow
restaints
Need
t o drink from cup postop
Pureed, blended food, NPO (no syringes for meds, no fingers, no straws)

 Long-term concerns:
CL
Surgical revisions
Accepting the scar- Body image Disturbance
CP
Several disciplines involved:
- plastic surgeons, social work, speech/language therapists orthodontist
- ENT Frequent OM r/t improper drainage of middle ear

Nursing Diagnoses
Imbalanced Nutrition: less than body requirements r/t infants inability to ingest nutrients
Ineffective Breathing Pattern r/t surgical correction of defect
Impaired Tissue Integrity r/t mechanical factors
Ineffective family coping r/t birth of a child with a defect
Risk for Aspiration r/t anatomic defect


2.) Esophageal Atresia (EA) & Tracheoesophageal Fistula (TEF)
Congenital malformation
Associated with maternal polyhydramnios & VACTERL syndrome

Clinical Manifestations:
Frothy Saliva in mouth and nose
Drooling
3 Cs (coughing/choking/cyanosis)
1st drink of glucose water in nursery will come right back out
Stomach distension d/t air taken in when infant breathes
intra-abdominal & thoracic compression can cause gastric contents to regurge
into trachea= chemical pneumonia

Surgical Emergency!!
PRE-OP:
Stop oral feeding
Supine or prone to facilitate drainage
Continuous suction via double lumen catheter into upper esophageal pouch (HOB 30 degrees)
IV fluids
G-tube placement to relieve abdominal distention- left open for air to escape (not for feeding)
TPN is surgery is delayed

SURGERY: division & ligation of the TEF/ end-to-end anastomosis of esophagus

POST-OP:
Radiant warmer
TPN continued then feed through g-tube until anastomosis is healed
Oral feedings begin with STERILE WATER




 3.) Abdominal Wall Defects

Omphalocele: Gastroschisis:
Congenital malformation Abdominal organs herniate through abdominal wall
Intra-abdominal contents herniate through Herniated contents not covered with peritoneal
umbilical cord membrane
Contents covered with peritoneal membrane
(translucent sac) -Surgery more urgent b/c of lack of protective sac
Often associated with other anomalies

-Surgical closure

Initial Management:
Immediately after delivery:
Loosely cover with saline- soaked pads and plastic drape (prevents water loss, drying, and temp instability)
Thermoregulation is critical
- heat & fluid loss from exposed viscera
Fluid replacement
NG for GI decompression of abd

POST-OP:
Monitor for complications
Assess bowel fx
Family support





















 B.) Obstructive Disorders

2.) Meconium Ileus

Normal meconium (first stool) begins to pass within 1st 24-36 hrs after birth
Luminal Obstruction of the distal small intestine by abnormal meconium= stool doesnt pass

Initial Manifestation of Cystic fibrosis (CF causes abnormal meconium)

May require irrigations of NS ememas
Rarely are surgical interventions needed

1.) Pyloric Stenosis

Hypertrophic obstruction of the pyloric sphincter
Boys more than girls

Hypertrophy of pyloric sphincter Clinical Manifestations:
results in: *PROJECTILE VOMITING (up to 3 ft- may be blood-tinged)
- Obstruction of spincter Constant hunger
- Inability to move food from Iritability
stomach to duodenum Failure to gain weight
- Vomiting & dehydration Fewer/smaller stools

Abdominal assessment:
Inspect abdomen for visible peristaltic waves
Auscultate BS (hyperactive)
Palpate- olive shaped mass RUQ
Fluid status skin turgor, fontanels, urinary output,
mucous membranes
Measure and describe vomitus
Surgical Correction: Pyloromyotomy (open or laparoscopic)
PRE-OP:
NPO
NG for decompression
IV electrolyte replacement
Baseline weight
Monitor I&O

POST-OP
Clear liquids (sm amount) 4-6 hrs
AAT formula, breast
Pain meds (NOT PRN)
Frequent burping
Assess wound
RIGHT SIDE positioning to promote digestion

 3.) Intussusception
Frequent cause of intestinal obstruction
Mostly boys ages 3 months - 6 yrs
Potentially life- threatening

One portion of the intestine prolapses & then invaginates (telescopes) into itself
Most common site is ileocecal valve
ileum invaginates into the cecum and then further into the colon
This results in:
- Obstructed blood flow
- Ischemia and necrosis
- Hemorrhage, perforation (if untreated)



Clinical Manifestations:
Sudden & abrupt onset
Acute colicky abdominal pain in a previously healthy infant/child
Episodic:
- Early= vomiting, may pass normal stool
o Sausage-shaped mass RUQ
- Later= classic sign current jelly-like stool
o r/t blood & mucous in stool
Classic signs may not be present
- wt loss, vomiting, anorexia


Diagnostic Evaluation:
X-rays
Abdominal US
Barium enema

Treatment:
Found on radiograph= may use air, saline, or water to push back out
Barium emena= sometimes barium going through corrects the intussusception
Surgery if above TX unsuccessful




Nursing Management:
NPO until corrected

IV fluids & electrolyte monitoring
Assess for abdominal distention

Assess bowel sounds
- present= begin with clear liquids
- advance diet as tolerated


*If child with intussusception passes a normal brown stool= be sure to NOTIFY

PHYSICIAN because it may have resolved on its own


 C.) Disorders of Motility
1.) Gastroesophageal Reflux (GER)
Affects half of all children (Peak onset= 1-4 months)

Lower esophageal sphincter relaxes allowing passive regurgitation of stomach contents into esophagus
May also enter airway (Aspiration)
Clinical Manifestations: Treatment:
Postprandial regurgitation Mild symptoms:
Hunger - Modify feeding habits
Irritable - Thicken formula (rice cereal)
Eat but loose weight - Sm frequent feedings & burping
HX of vomiting & URIs - Infant- position with upper body raised 30 degrees
Aspiration Pneumonias (big risk) Medications-
- Proton pump inhibitors
Serious Manifestations characterized by: - Antacids
Poor weight gain - Histamine Antagonists
Esophagitis
Persistent respiratory symptoms Severe Symptoms:
- Surgery= Nissen Fundoplication
Nursing management:
Monitor infants weight
Observe for respiratory distress
Elevate HOB
PRONE while awake
- Tracy harness or reflux board
Avoid infant seats- cause pressure on abdomen

difference between GER & pyloric stenosis= projectile vomiting (possessed pyloric pop)

2.) Hirschsprung Disease (acute or chronic)

Congenital aganglionic megacolon/ Congenital anomaly
Results in inadequate motility & obstruction of the intestine
More common in men & in Downs

Patho:
Lack of peristalsis r/t aganglionic in one or more segments of colon
Possible failure of inner anal sphincter to relax
Segment almost always in the rectum & proximal portion of the intestine
Rarely are there skip segments OR total involvement
Accumulation of fecal contents cause distention of the area proximal to the defect

ENTEROCOLITIS:
Can result from Hirschsprungs
Inflammation of small bowel & colon can cause perforation
*Leading cause of death in children with Hirschsprungs




Clinical Manifestations:
Different depending on when DX

Newborn:
*No meconium
*Bilious vomiting
Poor feed
Abdominal distention

Infant:
Failure to thrive
Constipation
Abdominal distention
Vomiting/diarrhea
*OMINOUS SIGN= explosive, watery diarrhea, fever, severe prostration

Child:
Constipation
*RIBBON-like, foul smelling stool
*Visual Peristalsis
Palpable fecal masses
Poorly nourished/ anemia

Diagnosis:
Rectal Biopsy: reveals absence of ganglionic cells

Nursing Management:
Rectal irrigations with NS to promote elimination & prevent obstruction
Frequent diaper changes to prevent skin breakdown

Surgical Goals:
Remove obstruction
Remove aganglionic portion of the intestine
Restore normal bowel motility
Restore FX of the internal anal-sphincter

Surgical Procedures: done in stages
1.) Temporary colostomy proximal to the defect
2.) Corrective surgery
a. When child weighs 20 lbs
b. Soave pull-through= normal bowel pulled through the muscular sleeve of the rectum
3.) Ostomy closed

Post-Op Nurse mgmt:
Monitor for infection
Pain mgmt. (routinely)
Measure abdominal circumference
Ostomy care





D.) Malabsorption Disorders
1.) Celiac Disease
Auto-immune disorder

Gluten-sensitive enteropathy: Nursing Management:
Instruct parents & child on gluten-free diet=
Intolerance for GLUTEN (proteins):
- Wheat Food to avoid:
- Barley - anything made with wheat, barley, oats, rye:
- Rye o Bakery items, crackers, noodles,
- Oats pizza (unless dough made from corn)
Affects fat & protein absorption Can eat corn/rice
Impaired absorption of fat soluble vitamins Mineral & vitamin supplements (A/D/E/K)
- A/D/E/K Difficult to manage away from home (school)
LIFETIME diet management*
Clinical Manifestations:
Bulky, foul smelling fatty diarrhea stools
(Steatorrhea)

2.) Cystic Fibrosis

Exocrine (mucus-producing) gland dysfuntion
Multisystem involvement
common lethal genetic illness among white children, adolescents & young adults
Most
Primary factor for multiple clinical manifestations= mechanical obstruction caused by increased viscosity
of mucous gland secretions
2 Primary systems affected: respiratory & GI
GI Tract:
Thick secretions block ducts of the pancreas
Prevents essential pancreatic enzymes from reaching duodenum
Results in marked impairment of the digestion & absorption of nutrient (esp fats & proteins)

Clinical Manifestations: Meconium ileus= earliest postnadal manifestation
Disturbed absorption of fats & proteins results in=
Steatorrhea= excessive stool fat

Azotorrhea= excessive stool protein
Good appetite but NO WEIGHT GAIN
- no pancreatic enzymes to aid in digestion, so food not being digested

Management:
Principal TX for pancreatic enzyme insufficiency is replacement of enzymes
Administer with each meal AND snack:
- every time they eat- take enzymes
- 1-15 enteric-coated capsules with each meal & smaller amount with each snack
- swallowed whole or taken apart & sprinkled on (sprinkle on top of food, not mixed in)
- dont chew or crush beads
- high-fat foods= add extra enzymes
- Take within 30 minutes of eating
DIET=
high protein & high calorie
VITAMINS= water-miscible form of fat-soluble vitamins (A/D/E/K)
 E.) Feeding Disorders
1.) Colic
Paroxysmal abdominal pain- intestinal in orgin
Onset= first weeks of life- remission around 3 months

Clinical Manifestations:
Continuous loud crying for several hours
Abdominal distention

Nurse Management:
Teach on relieving colic:
Rhythmic movement car rides, swaddle with knees flexed up to the abdomen

Reduce stimuli:
Quiet, soothing music
No smoking

Tactile stimuli:
Pacifier
Warm bath
Massage abdomen

*Notify Physician if:
- Temp 100
-Cries continuously 3 hrs
-Change in stool characteristics
-Abdomen rigid (will be rigid when they cry- but abdomen should settle when they take breath)

2.) Failure to Thrive (FTT)

(TRUST VS MISTRUST)
Syndrome in which infants & children fail to eat food for adequate nourishment

Organic:
AIDS Nurse Mgmt:
Congenital heart defect Assess developmental age
Neurologic Primary care- same nurse each shift
Nonorganic: Gentle handling (calm persistence in
(called feeding disorder of infancy/childhood) feeding)
Parents may suffer from depression, substance abuse, Hold, rock
mental retardation, psychosis Consistent schedule
Unresponsive to infants hunger cues* Sensory Stimulation
INVOLVE PARENTS (&TEACH)
Clinical Manifestations: Remain non-judgemental
Persistent failure to eat with no weight gain or, Provide supporting environment
Weight loss in child 6 y,o.
Growth failure Eval of interventions:
Developmental retardation (milestones not met) Wt gain

Apathy - min of 1-2 oz./day
hygiene
Poor Responds positively to feeding practices

Wthdrawal (b/c they would push food away thinking
eye contact
Avoid you were teasing them)
-babies normally cry when hungry- these babes get tired of crying &
realize that it doesnt help- so stop crying when they are hungry.