GI Malformations/Jaundice Flashcards
A Tracheoesophageal Fistula is associated with what anomalies?
VACTERL
- Vertebral
- Anal
- Cardiac
- Tracheal
- Esophageal
- Renal
- Limb
What are the signs of a Tracheoesophageal Fistula?
Polyhydramnios in utero
Increased oral secretions
Inability to feed/gagging
Aspiration
If Esophageal Atresia is present, what will be a diagnostic on a CXR?
If an NG tube is coiled in the esophagus
With a Tracheoesophageal Fistula, what will be seen on XR?
Air in the GI tract
What occurs with a Diaphragmatic Hernia?
Bowel segments protrude through the left side of the diaphragm into the thorax
What are the signs of a Diaphragmatic Hernia?
Sunken abdomen with bowel sounds present over the left chest
– Pulmonary hypoplasia/HTN causing respiratory distress!
What is Gastroschisis?
Herniation of red, matted bowel through the abdominal wall NOT midline and NOT covered in peritoneum
What are 2 treatment options for Gastroschisis?
Surgery
Silo bag can gradually reduce bowel into abdomen
What is an Omphalocele?
Herniation of intestine through the abdominal wall at the level of the umbilicus and IS covered by peritoneum/amniotic membrane
Omphaloceles are associated with what syndrome?
Beckwith-Wiedemann
Duodenal Atresia involves failure of the duodenum to recanalize during gestation. What 2 conditions are often present?
Down Syndrome
Annular pancreas
What is the main symptom of Duodenal Atresia?
Bilious emesis after birth
What will be seen on XR with Duodenal Atresia?
Double bubble
What can cause Jejunal Atresia?
Cocaine or other vasoconstrictive substances that alter vasculature in utero
What will be seen on XR with Jejunal Atresia?
Triple bubble
GI malformations will often have what presenting sign in utero?
Polyhydramnios due to defective swallowing
What type of bilirubin is always pathologic?
Conjugated (direct)
What is Kernicterus and at what level of UNconjugated bilirubin can it occur?
Deposition of unconjugated bilirubin in the basal ganglia, pons, cerebellum
– > 25
What are the signs of Kernicterus? (4)
Lethargy
Hypertonia
High pitched screaming cry
Seizures
Unconjugated bilirubin may be physiologic in nature. What are the features of physiologic jaundice?
- Not present at birth and resolves in 1-2 weeks
- Levels increase by < 5/day
- Levels peak at < 14
- LOW Conjugated bilirubin
What are the features of pathologic jaundice?
- Present at birth and does not resolve
- Levels increase by > 5/day
- Levels peak at > 14
- HIGH conjugated bilirubin
What are 3 causes of high Unconjugated Bilirubin?
Hemolysis of RBCs
Impaired conjugation
Increased enterohepatic circulation
What are examples of RBC hemolysis that will cause elevated Unconjugated bilirubin?
ABO/Rh incompatibility
G6PD deficiency
RBC structure
What are 3 causes of impaired conjugation that will cause elevated Unconjugated bilirubin?
Physiologic jaundice of the newborn
Gilbert Syndrome
Criggler-Najjar Syndrome
What are 3 examples of increased enterohepatic circulation that will cause elevated Unconjugated bilirubin?
Poor feeding
Breast milk jaundice
Pyloric stenosis
What are the 2 treatment options for Unconjugated hyperbilirubinemia?
Phototherapy
Exchange transfusion (> 20)
What workup should be done for elevated Unconjugated bilirubin?
CBC with peripheral blood smear
Coombs test
Blood typing
What workup should be done for elevated Conjugated bilirubin?
LFTs and bile acids
Blood cultures
Metabolic disorders
US and HIDA scan
What are 2 causes of high Conjugated Bilirubin?
Impaired secretion from the liver
Obstruction of biliary tree
What are 2 syndromes that cause elevated Conjugated bilirubin by impaired secretion from the liver?
Dubin Johnson
Rotor
