MTB 4 (GI) Flashcards
What is choanal atresia?
Membrane b/t nostrils and pharyngeal space
Prevents breathing during feeding
How does choanal atresia present?
Child turns
- Blue when feeding
- Pink when crying
How do we dx choanal atresia?
CT
Tx for choanal atresia?
Surgical intervention
- perforate membrane
- reconnect pharynx to nostrils
What is Hirschsprung dz?
Lack of innervation in distal bowel by Auerbach plexus
What dz is ass’d with Hirschsprung dz?
Down syndrome
MEN type II
What is the presentation of Hirschsprung dz?
Failure to pass meconium in first 48 hours
Or
At all.
Bilious vomiting, FTT
When do normal infants pass meconium?
90% within first 24 hours
How do we Dx Hirschsprung dz?
Barium enema reveals narrowed distal colon w/proximal dilation
What do we seen on plain xrays in Hirschsprung dz?
Distended bowel loops with lack of air in rectum
What is seen on anorectal manometry in Hirschsprung dz?
High pressure in anal sphincter
Definitive Dx is made with what for Hirschsprung dz?
Full thickness Bx
- lack of ganglionic cells in submucosa
Tx for Hirschsprung dz?
3-stage surgery
What is imperforate anus?
Opening of anus is missing
Rectum ends as blind pouch
What dz is ass’d with imperforate anus?
Down syndrome
Presentation of imperforate anus?
Failure to pass meconium
PE: no anus
Tx: surgery
What is CHARGE syndrome
Coloboma of eye, CNS abnormalities Heart defects Atresia of choanae Retardation of growth/development Genital/urinary defect - hypogonadism Ear anomalies/deafness
What is VACTERL syndrome?
Vertebral anomalies Anal atresia Cardiovascular anomalies Tracheoesophageal fistula Esophageal fistula Renal abnormalities Limb anomalies
What is seen in a quad screen (2nd trimester) for Down syndrome?
Increased Inhibin-A, b-HCG
Decreased MSAFP, Estriol
Bilious vomiting on first day of life
Duodenal atresia
What is the cause of Duodenal atresia
Lack/absence of apoptosis -> improper canalization of lumen of duodenum
Presentation of Duodenal atresia?
In utero: polyhydramnios
Bilious emesis w/in hours after first feeding - first 12 hours of life
CXR finding of Duodenal atresia
Double bubble sign - air bubbles in stomach and duodenum proximal to atresia