Test 2

Question 0

Risk factors for cleft lip/palate

Answer 0

Crouzon syndrome
Previous child has
Family history
Folate deficiency
Teratogens

Question 1

Cleft lip/cleft palate

Answer 1

Maxillary process fails to fuse during 6th week of gestation. Failure of fusion of secondary palate 5-12 weeks gestation. Higher incidence in males and Asians.

Question 2

Cleft lip

Answer 2

Dimple in vermillion border
Complete separation to floor of nose
Unilateral or bilateral
Alone or with cleft palate

Question 3

Cleft palate

Answer 3

Opening between mouth and nasal cavity

Unilateral or bilateral

Question 4

Cleft lip repair

Answer 4

Lip sutured together
Z-plasty usually done 6-12 weeks if healthy
Immobilize elbows to prevent touching sutures
Pain medication
Feeding devices before correction
Do early (before 6 months) to allow sucking and speech development.

Question 5

Feeding devices for cleft lip/palate

Answer 5

Brecht feeder (drips into mouth)
Lambs nipple
Oburator from orthodontist
Flanged nipple

Question 6

Cleft palate repair

Answer 6

Varies: usually done before 18 months for speech
Palatoplasty (palate) 12-18 months, much harder to repair than lip
Associated problems: orthodontics, audiology, speech

Question 7

Post op care for cleft palate repair

Answer 7

Position only on back
Use elbow immobilizers 
Cups for drinking
No pacifiers
Sitting position when feeding
Logan's bar

Question 8

Esophageal atresia and Tracheoesophageal fistula

Answer 8

Failure of esophagus to develop as a continuous tube during 4-5 week of gestation. May end in a blind pouch or connected to the trachea (fistula)

Question 9

Anomalies associated with esophageal atresia

Answer 9

Heart defects
GI or GU tract anomalies
Musculoskeletal anomalies 
Vertabral
Anorectal
Renal
Limbs

Question 10

S/S of esophageal atresia

Answer 10

THREE C's:
Cyanosis
Choking
Coughing
Excessive salivation
Sneezing
Distended abdomen
Aspiration pneumonia

Question 11

Surgery for esophageal atresia

Answer 11

Stage 1: ligation of fistula, insertion of G-tube
Stage 2: reconnect two ends if esophagus or leave G-tube in place
*complications: reflux, aspiration, stricture formation

Question 12

Hypertrophic Pyloric stenosis

Answer 12

Hypertrophic obstruction of the circular muscle of the pyloric canal

Question 13

Risk factors for pyloric stenosis

Answer 13

Male
First born
Caucasian 
Familial predisposition 
Infants that received erythromycin before 1 month old
Prostaglandin E infusion
Hypergastrinemia

Question 14

S/s of pyloric stenosis

Answer 14

Regurgitation immediately after feedings (projectile vomiting begins 2-3 weeks of age)
Hungry but fails to gain weight
Fewer and smaller stools
Dehydration
Metabolic acidosis
Observable reverse peristaltic waves
Mass in RUQ

Question 15

Diagnosing pyloric stenosis

Answer 15

Abdominal ultrasound
Thickened pylorus (>4mm diameter; >14mm length)
Upper GI series (barium swallow, contrast medium will not pass)
Blood test for anemia, hypochloremia, hypokelemia, hyperbilirubinemia

Question 16

Treatment for pyloric stenosis

Answer 16

Restore fluid and electrolyte balance

Open or laparoscopic pyloromyotomy

Question 17

Intussusception

Answer 17

Portion of the intestine prolapses and invignates (telescopes) into another portion. Obstructed passage if stool. Walls of intestines rub together, become inflamed leading to edema which decreases blood flow which results in necrosis. Common with cystic fibrous.

Question 18

S/S of intussusception

Answer 18

Acute abdominal pain
Brown stool which becomes red (current jelly)
Vomiting
Mass in RUQ
Colicky 
Screaming and drawing up of legs
Distended abdomen
*Abrupt onset*