Congenital Gi Disorders

Front 1

5 types of congenital GI disorders:

Back 1

1. Cleft lip/palate
2. Tracheoesophageal Fistula/Esophageal Atresia
3. Abdominal Wall Defects
4. Hirschsprung's Disease
5. Obstructive Disorders

Front 2

Definition of Cleft Lip

Back 2

Congenital fissure in the upper lip

Front 3

Definition of Cleft Palate

Back 3

Congenital fissure in either the soft palate alone or in both hard and soft palates

Front 4

Incidence of Cleft Lip

Back 4

Cleft lip can be with OR without cleft palate. It is also more common in boys than girls.

Front 5

Incidence of Cleft Palate

Back 5

More common in girls than boys. Also, more common in Native Americans. Occurs in 1 in 800 live births.

Front 6

Cleft Lip pathophysiology

Back 6

Occurs when maxillary process fails to merge with the medial nasal elevation on one or both sides.

Front 7

Cleft Palate pathophysiology

Back 7

Occurs when the lateral palatine processes fail to meet and fuse with each other, the primary palate, or the nasal septum. In the first trimester, it may be only the soft palate or extend to the hard palate.

Front 8

Cleft Lip/Palate Etiology

Back 8

1. Multifactoral mode of inheritance
2. Maternal nutrition - Folic Acid deficiency
3. Maternal medication in first trimester
4. Occurrence of fever and influenza in first trimester.
5. Maternal prenatal cigarette smoking (associated with increased risk of cleft palate)

Front 9

How is Cleft Lip/Palate manifested clinically?

Back 9

Cleft lip is generally evident at birth. Cleft palate is not so evident.

If not evident, infant may exhibit feeding difficulties, chronic upper airway congestion/infection.

Front 10

How do we therapeutically manage Cleft Lip/Palate?

Back 10

Priority is SURGICAL REPAIR!! You want early closure BEFORE speech development.

Goal - You also want "normal":
1. speech
2. dental occlusion
3. cranial facial development

This requires team approach - speech therapists, social workers, eating disorder counselors, dietitians.

Front 11

What is a HUGE issue for cleft lip/palate babies before operation?

Back 11

Feeding!!! They have difficulty grasping the nipple and creating a suction. This requires patience and special techniques and equipment.

*Note* - an unrepaired baby can breastfeed but it is with great difficulty. Have the mom pump breast milk for special equipment.

Front 12

What are interventions for cleft lip/palate babies AFTER surgery?

Back 12

1. Maintain a patent airway (elevate HOB - at risk for aspiration)

2. Protect suture line (use "no-no's")

3. Keep infant calm (if not, it will put tension on the suture line).

4. Use restraints (know policies and procedures)

5. Support parents by accepting and treating infant as normal; point out great qualities.

Front 13

Feeding instructions for cleft lip/palate babies:

Back 13

1. Clear liquids are preferred to other liquids. Consult physician/surgeon.

2. Put gentle, steady pressure on base of bottle.

3. Burp infant frequently.

4. Get daily weights.

Front 14

Cleft lip/palate prognosis:

Back 14

1. Language and speech - see residual speech impairment.

2. Hearing impairment - will often put PE tubes in the ears.

3. Dental problems - extensive mal-position of teeth.

4. Social adjustment/self image - obtain psych consult.

Front 15

Cleft lip/palate psychosocial implications:

Back 15

1. Parental guilt/grief - let them express their grief in a non-judgmental way.

2. Bonding

3. Cultural beliefs

4. Acceptance/reaction of others - it is very important to make sure we love the baby and present the baby in such a way that we are truly saying that they have a beautiful baby.

Front 16

What is a tracheoesophageal fistula (TEF)?

Back 16

Failure of trachea to differentiate and separate from esophagus

Front 17

What is esophageal atresia (EA)?

Back 17

Failure of esophagus to develop as a passage (becomes a blind pouch)

Front 18

What is the incidence of TEF/EA?

Back 18

*1 in 4,000 live births

*30-40% affected individuals have associated anomalies (cardiac is most frequent anomaly)

*No definitive cause identified

*male is equal in risk to female

*increased in LBW babies

*increased in premature babies

*increased in infants of women with maternal polyhydramnios

Front 19

How is TEF/EA manifested in patients?

Back 19

1. Frothy saliva and drooling are commonly seen; pt.s have inability to swallow it which leades to chocking or cyanosis with feedings.

2. Gastric tube/suction catheter cannot be passed because of blind pouch in esophagus or failed separation.

3. Recurrent pneumonia as they get older.

4. Triad of: "excessive secretions," reflux, and respiratory.

Front 20

How do you diagnose TEF/EA?

Back 20

It's ideal to diagnose before the first feeding. The main indicator of this condition is maternal polyhydramnios.

It is confirmed by an X-ray after passing radiopaque catheter into the esophagus.

Front 21

How do you therapeutically manage TEF/EA?

Back 21

1. Surgical correction; sometimes a staged repair is required because they might not be able to do it all at once.

2. Prognosis - 100% in otherwise healthy children.

3. Some post-op complications can occur:
- strictures
- reoccurrence of fistulas
- GE reflux

Front 22

What are the main preoperative interventions for TEF/EA?

Back 22

*assessment

*upright position - HOB elevated to reduce risk of aspiration of gastric secretions

*frequent suctioning to keep blind pouch empty

*NPO - IVF for hydration

*I&O's are KEY, as well as getting daily weights.

*G-tube - put it in for gastric decompression

KEY: Keep BLIND POUCH and STOMACH empty!!!

Front 23

What are the main postoperative interventions for TEF/EA?

(KNOW THESE FOR THE TEST!!)

Back 23

1. Airway management

2. Thermoregulation

3. Gastric decompression after surgery

4. Suction with extreme caution PRN; talk to surgeon first to find out what they did and how it went.

5. Insert chest tube

6. Pain control - BIG issue.

7. Comfort - also BIG issue. Pacifiers are NOT contraindicated.

8. Feeds - insert G-tube first, then oral feeds slowly. infant could have oral aversion.

Front 24

What are some psychosocial implications for patients with TEF/EA?

Back 24

1. Encourage attachment

2. Stress normalcy

3. Identify family support

Front 25

Abdominal wall defects: Hernias

(4 types)

Back 25

1. Diaphragmatic

2. Umbilical

3. Gastroschisis

4. Omphalocele

Front 26

Hernia incidence:

Back 26

Occurs during first trimester, usually by the 3rd week gestation as a failure in embryonic development.

Affects approx. 2 in 10,000 live births.

Front 27

What is Omphalacele?

Back 27

Herniation of abdominal contents THROUGH the umbilical ring. It has an INTACT peritoneal sac. It is often associated with other anomalies. It can vary in size and looks like a huge umbilical cord.

Front 28

What is Gastroschisis?

Back 28

Herniation of abdominal contents LATERAL to umbilical ring. It does NOT have a peritoneal sac. It is rarely associated with other anomalies. It is an isolated finding.

Front 29

How do we therapeutically manage patients omphalacele or gastroschisis?

Back 29

1. Cover sac with sterile wrap so it doesn't dry out.

2. IV fluids and antibiotics to prevent fluid loss.

3. Surgery - Start repair within 24 hours of birth. The length of repair depends on the child. It's often a staged repair since the bowel doesn't develop in the abdomen, it can't immediately hold it all inside. They have to gradually increase abdominal width to hold it.

3. Silo - put more abdomen in every few days.

4. Thermoregulation

Front 30

What are preoperative interventions for O or G hernias?

Back 30

1. Infection - open to air until repaired.

2. Fluid management

3. NG-tube - gastric decompression

4. Family support - shocking defect.

Front 31

What are postoperative interventions for O and G hernia patients?

Back 31

1. Assess frequently

2. TPN - prolonged ileus

Front 32

What is an umbilical hernia?

Back 32

Incomplete fusion of umbilical ring where umbilical vessels leave abdominal wall.

Front 33

Umbilical hernia incidence

Back 33

*LBW/VLBW

*African-American

*Isolated defect

Front 34

Umbilical hernia complications

Back 34

There aren't many complications. Incarceration of the hernia isn't an issue with this type of hernia.

Front 35

What are some interventions for Umbilical hernias?

Back 35

It usually repairs on its own, or if not, the patient may have surgery at age 2 years old.

Front 36

What is a Congenital Diaphragmatic Hernia?

Back 36

The diaphragm is not completely formed so it compresses the lungs. This is an EMERGENCY situation!!!

Front 37

How is CDH manifested in patients?

Back 37

1. Acute respiratory distress

2. Scaphoid (shaped like a boat) abdomen due to development in diaphragm

3. Pulmonary htn

4. Hyperplasia

Front 38

When can you diagnose CDH?

Back 38

Pre-natal: 20-25 week gestation. You will see media stinum shift and loops of bowel and polyhydramnios.

Post-natal: Want them to deliver at tertiary care facility to where everyone can immediately take care of it.

Front 39

How do you manage CDH post-birth?

Back 39

1. Respiratory support - VERY IMPORTANT!!! No bag mask ventilation because you could get air in the stomach. Need to intubate the newborn on delivery.

2. Surgical repair - early. Survival rate is improving.

There are LOTS of post-op issues to deal with long-term.

Front 40

What are some interventions for CDH patients?

Back 40

*newborn assessment

*post-operative:
1. close observation because they are very sick patients to care for
2. pain management
3. thermoregulation

Front 41

Definition of Inguinal Hernia:

Back 41

Protrusion of small intestines/fluid into groin through a weakness or tear in the abdominal wall.

Front 42

How is an inguinal hernia manifested?

Back 42

1. Mass in groin. The SAC is present at birth but it may not manifest at birth. It usually presents when baby exerts enough pressure to make it come out (ex. - lusty cry).

2. Boys and preemies have this more.

Front 43

Definition of Hirschsprungs (congenital aganglionic megacolon) disease:

*first defined by Harold Hirschsprung in 1887*

Back 43

Congenital anomaly that results in mechanical obstruction from inadequate motility in part of the intestine.

Front 44

Pathophysiology of Hirschsprung's Disease:

(bolded in notes so know this for test)

Back 44

ABSENCE of autonomic parasympathetic ganglion cells in one or more segments of the colon.

Front 45

Cause of Hirschsprungs Disease:

Back 45

1. Lack of innervation results in absence of propulsive movements (peristalsis)

2. Lack of innervation also leads to decreased relaxation of the internal rectal sphincter.

Front 46

What are the clinical presentations the lead to a diagnosis of Hirschsprungs Disease?

Back 46

1. Chronic constipation

2. Encapresis (stool in underpants because it can't be controlled)

3. Tight sphinctor tone

4. Hx. of chronic constipation

Front 47

What is the incidence of Hirschsprungs Disease?

Back 47

Accounts for about 1/4 of all cases of neonatal obstruction

*1 in 5,000 live births
*Males over females - 4:1

Front 48

What are some clinical manifestations in children of all ages?

(Childhood manifestations is on the test!!)

Back 48

Vary according to the age when symptoms present.

1. Newborn - (41-64%) - abdominal distension, vomiting, no meconium in 48 hours. The majority of cases are seen during this period.

2. Infancy (21-35%)

3. Childhood - (15-26%) -
*chronic constipation
*failure to thrive
*stools - FOUL SMELLING and "RIBBON LIKE" (this is the classic sign in older kids)
*abdominal distension

Front 49

What is therapeutic management for patients with Hirschsprungs disease?

Back 49

1. Primarily involves surgical removal of the aganglionic bowel.

2. Stage I

3. Stage II - 8 months to 1 year or approx. 20 lbs - involves "pulling" the end of the intact bowel down to a part of the rectum for reanastomosis.

3. Stage III - closure of colostomy (if performed) usually 3 months after stage II.

Front 50

There is a very small population who have mild symptoms of Hirschsprungs disease. What kind of conservative therapy do we do for them?

Back 50

1. Occasional enemas

2. Develop regular pattern of defecation

3. AT CONTINUED RISK for development of enterocolitis

Front 51

What kind of interventions do you do for a neonate who might have Hirschsprungs?

Back 51

1. Observe for passage of meconium

2. Look for abdominal distension

Front 52

What kind of interventions do you do pre-op and post-op for a pt. who might have Hirschsprungs?

Back 52

1. NG placement

2. Strict I&O's

3. Measurement of abdominal girth

4. Vital signs

5. S&S of bowel distension

6. Bowel prep - before surgery if older

Post-op: (same as pre-op plus these below)

7. IV fluid maintenance

8. Dressing changes

9. Foley care

10. Discharge education

Front 53

What are some lifespan considerations for patients with Hirschsprungs Disease?

Back 53

1. Dependent on amount of bowel involved.

2. Most return to normal bowel pattern.

Front 54

What are two types of GI Obstructive disorders?

Back 54

1. Hypertrophic Pyloric Stenosis

2. Intussusception

Front 55

What are some clinical manifestations of Acute Intestinal Obstruction?

Back 55

1. Abdominal pain

2. Distension

3. Nausea and Vomiting

4. Change in stool

Front 56

Definition of Pyloric Stenosis:

Back 56

A narrowing of the pyloric sphincter at the outlet of the stomach, obstructing the flow of food into the small intestines.

Front 57

What is the incidence of Pyloric Stenosis?

Back 57

*Full-term infants

*Primarily found in FIRST BORN, WHITE MALES

Front 58

Pathophysiology of Pyloric Stenosis

Back 58

Muscles become thick and elongated and obstruct pyloric channel.

Front 59

What are some clinical manifestations of Pyloric Stenosis?

Back 59

1. Prolonged projectile emesis (causes metabolic alkalosis)

2. Hyperperistaltic stomach (going to feel like an olive-shaped mass)

Front 60

How do you therapeutically manage a patient with Pyloric Stenosis?

Back 60

Surgical repair - Pyloromyotomy

Front 61

What are some pre-op and post-op interventions for a patient with Pyloric Stenosis?

Back 61

Pre-Op: I&O's

Post-Op: Feed 4-6 hours after with clear liquids; advance as tolerated.

Front 62

Definition of Intussusception:

Back 62

Telescoping of the bowel upon itself.

*Erin gave the example of pulling the sock into itself.

Front 63

What is the incidence/etiology of Intussusception?

Back 63

2.4 in 1,000 live births

3 months - 3 years

Male at greater risk than female

Recurrence (10%) - unknown why it does reoccur

Front 64

Pathophysiology of Intussusception

Back 64

Telescoping bowel - circulatory compromise - ischemia in that part of the bowel.

Front 65

What are some clinical manifestations of Intussusception?

(KNOW THE TRIAD OF ACUTE MANIFESTATIONS FOR TEST)

Back 65

Presents as sudden onset of crampy abdominal pain.

Acute Triad of symptoms:

1. Abdominal pain
2. Sausage shaped abd. mass
3. Currant jelly, red stool.

Front 66

How do you therapeutically manage patients with Intussusception?

Back 66

1. Most often, reduction by barium enema or whatever latest test they're doing.

2. Reduction by surgical manipulation.

3. Surgical resection.

Front 67

What happens if the symptoms are left untreated?

Back 67

Puts patient at increased risk for:

1. Sepsis
2. Peritonitis
3. Perforation

Front 68

What are some interventions for Intussusception?

Back 68

1. H&P
2. Family support

Front 69

What is Celiac Disease?

(NCLEX questions)

Back 69

Permanent intestinal intolerance to dietary wheat and related proteins that produce mucosal lesions in genetically susceptible individuals.

- Malabsorption syndrome

- 2nd to CF in malabsorptive diseases.

- They can't digest gluten so it becomes a toxin.

Front 70

What is the incidence of Celiac Disease?

Back 70

1 in 3,000

Females at greater risk than males

Rarely in Asians or African-Americans

Front 71

What are symptoms of Celiac Disease?

Back 71

1. Diarrhea

2. Failure to thrive

3. Abdominal distension

4. Anorexia

5. Irritability

Front 72

How do you diagnose someone with Celiac Disease?

Back 72

Withdraw gluten from their diet; have full remission of symptoms.

Front 73

What is the treatment for Celiac Disease?

Back 73

Dietary Management