Pediatric GI/GU/Endocrine Practice
Questions and Correct Answers
Embryonic developmental failures r/t multiple genetic and environmental factors. These
developmental failures result in an abnormal opening in the lip, palate, and, sometimes,
nasal cavity. This issue is an abnormal opening in the lip or palate. The defects may
occur unilaterally (on either side) or bilaterally and are the most common congenital
craniofacial deformity. ✅Cleft Lip and Palate
Results when the medial nasal and maxillary processes fail to join at 6 to 8 weeks of
gestation. ✅Cleft lip
Results from failure of the primary palatal shelves, or processes, to fuse at 7 to 12
weeks of gestation. ✅Cleft palate
Seen predominantly in male infants. The prevalence is higher in Asians and Native
Americans and has a lower frequency in African-Americans. ✅Cleft lip
Seen predominantly in female infants. The prevalence is higher in Asians and Native
Americans and has a lower frequency in African-Americans. ✅Cleft palate
The etiology is thought to be multifactorial, including both genetic and environmental
factors. Cause not known. Poses aspiration risk. ✅Cleft Lip and Palate
Folate before pregnancy will decrease incidence; 400 mcg/day for all women of
childbearing age. ✅Cleft Lip and Palate
Manifestations: a notched vermilion border, variably sized clefts that involve the alveolar
ridge, and dental anomalies. ✅Cleft Lip and Palate
Manifestations include nasal distortion, midline or bilateral cleft with variable extension
from the uvula and soft and hard palates, and exposed nasal cavities. ✅Cleft palate
Readily diagnosed through inspection of the lip. ✅Cleft lip
The first sign may be formula coming from the nose. ✅Cleft palate
The first intervention involves modifying feeding as needed to allow adequate growth.
Use of special feeding techniques, obturators, & unique nipples & feeders can usually
accomplish this goal & allow early discharge home. Before surgical repair, removable
orthopedic devices such as a Latham device may be used to expand & realign parts of
the palate/decrease the size of a wide lip cleft. A feeder with compressible plastic sides
allows gentle squeezing of the sides of the bottle to help eject the breastmilk/formula. A
,slightly longer nipple allows milk to be swallowed w/ less chance of milk entering the
nasopharynx and w/o stimulating the gag reflex. Feed in upright position. "Spoon"-
feeding (pouring motion) may be used or feeding tube. Will swallow lots of air so burp
often. Listen to breath sounds before and after feeding. ✅Cleft Lip and Palate
Repair is usually performed by age 3 to 6 months. The surgical technique involves the
use of a staggered suture line to minimize scarring. Some cosmetic modifications may
be needed again at age 4 to 5 years. ✅Cleft lip
Repair is individualized and based on the degree of deformity and size of the child.
Closure is completed between ages 6 and 24 months (6-12 is optimal for speech
development.) High risk for developing chronic otitis media, which can cause long term
hearing loss. ✅Cleft palate
Post-Operative: Continue special feeding for about 1 week to allow healing (lip.) Full
liquid diet for about 2 weeks (palate.) Clean suture line with water. Elbow restraints (no
grabbing.) Control pain. ✅Cleft Lip and Palate
Congenital malformations in which the esophagus terminates before it reaches the
stomach and/or a fistula is present that forms an unnatural connection between the
esophagus and the trachea. Prematurity and low birth weight are frequent concomitant
problems that significantly impact long-term prognosis. ✅Esophageal Atresia with
Tracheoesophageal Fistula
Occurs at 4th - 5th week pregnancy. Medical emergency. The child is prepared for
surgery, remains NPO, and is hydrated with IV fluids. The BF mom may pump and save
the milk. The infant still needs to suck, use pacifier with sucrose (also for pain.) A
pacifier satisfies sucking needs, provides early training in swallowing, makes later
feeding easier, and provides comfort through distraction. Pacifiers should not be offered
until the child can manage oral secretions. ✅Esophageal Atresia with
Tracheoesophageal Fistula
Manifestations include failure to pass suction catheter, NG tube at birth; excessive oral
secretions, coughing, choking, and cyanosis; vomiting; abdominal distension; airless,
scaphoid abdomen (atresia without fistula.) ✅Esophageal Atresia with
Tracheoesophageal Fistula
A h/o maternal polyhydramnios is a significant prenatal clue. Should be suspected if an
NG tube cannot be passed 10 to 11 cm beyond the gum line. This suspicion is
confirmed w/ an abdominal radiograph. Bronchoscopy and endoscopy are also used to
identify and assess fistulas. ✅Esophageal Atresia with Tracheoesophageal Fistula
Keeping the infant supine w/ the HOB raised decreases the chance of gastric secretions
entering the lungs. An NG tube must be in place and aspirated q5-10m to keep the
proximal pouch clear of secretions. IV fluids are essential. Surgical repair is the
, mainstay of treatment. Initial repair includes ligation of the fistula and end-to-side
anastomosis of the atresia to decrease the severity of stricture formation. If a staged
repair is necessary, a gastrostomy tube (G-tube) and cervical esophagostomy are
placed. ✅Esophageal Atresia with Tracheoesophageal Fistula
Assessment for respiratory distress in the immediate period after birth is essential.
Difficulty swallowing, regurgitation, vomiting, and unexplained cyanosis after an initial
feeding in the infant who is not diagnosed at birth are important assessment findings
that must be reported to the physician immediately. In the immediate period after birth,
placing the newborn in a radiant warmer and administering humidified oxygen are
essential to relieve respiratory distress. The child is prepared for surgery, remains on
nothing by mouth status, and is hydrated with IV fluids. A chalasia board that helps keep
the child at a 30-degree angle while supine can be useful to decrease reflux. Placing a
suction catheter in the proximal pouch and mouth will keep secretions to a minimum.
Even after surgical repair, these children are prone to gastroesophageal reflux.
✅Esophageal Atresia with Tracheoesophageal Fistula
In the immediate postop period, monitoring resp status, supporting fluid balance and
nutrition, maintaining thermoregulation, providing pain relief, monitoring for infection,
and promoting bonding with parents take priority. The child will likely have a chest tube
in place and patency must be maintained. Monitor intake and output; and assess for
signs of F&E alterations, including sunken fontanel and increased urine specific gravity
measurements. ✅Esophageal Atresia with Tracheoesophageal Fistula
If a cervical esophagostomy has been performed as the first stage of a surgical repair, it
is kept covered with gauze to absorb saliva and provide skin care. Referral to an
enterostomal therapist can be helpful in teaching parents esophagostomy care.
Immediately postoperative, there will be a G-tube and a chest tube. The G-tube is left
open to drainage in order to allow gastric contents and air to escape; this promotes
comfort and decreases risk of pressure at the anastomosis. ✅Esophageal Atresia with
Tracheoesophageal Fistula
If a cervical esophagostomy has been performed as the first stage of a surgical repair, it
is kept covered with gauze to absorb saliva and provide skin care. Referral to an
enterostomal therapist can be helpful in teaching parents esophagostomy care.
Immediately postoperative, there will be a G-tube and a chest tube. The G-tube is left
open to drainage in order to allow gastric contents and air to escape; this promotes
comfort and decreases risk of pressure at the anastomosis. ✅Esophageal Atresia with
Tracheoesophageal Fistula
Parents should be taught the techniques of G-tube feeding and care. Skin care at the
site may include using half-strength hydrogen peroxide to remove crusty drainage,
rotating the tube, and using a skin barrier product, as well as other ostomy skin care
products. Redness, exudate, pus, heat, or leakage of formula should be reported.
✅Esophageal Atresia with Tracheoesophageal Fistula
