Congenital Malformations Flashcards
What is the most common tracheoesophageql fistula
Type C: upper esophagus is a blind pouch while the lower one connects to the trachea
What is the more common side of diaphragmatic hernia?
Left side
This occurs when the extra embryonic gut fails to return to the abdominal cavity and closure of the abdominal musculature is incomplete. As a result, abdominal viscera, herniate ventrally into a membrane, covered by amnion and peritoneum, separated by Wharton Jelly. It is associated with other birth defect
Omphalocele
This involves all the years of the abdominal wall that is usually limited to the intestine, and occurs as an isolated defect. Risk factors are smoking and exposure to agricultural chemicals.
Which part of the abdominal wall does it usually occur
Gastroschisis
Right side of belly button
This is the most common site of ectopic gastric mucosa
Upper third of the esophagus where it is referred to as “inlet patch”
This is the most common congenital anomaly in the G.I. tract, and it arises due to field involution of the vitelline duct. It extends from the antimesenteric side. Lining may be intestinal or May have gastric and pancreatic tissue.
Meckel diverticulum
Rule of 2s in meckel
2% of the population
Symptomatic by age 2
2x more common in males
2 inches long
2 feet from ICV
Most common side of pseudo diverticula
Sigmoid colon
A three week old neonate was brought to the clinic due to new onset regurgitation, projectile, non-bilious vomiting after feeding and frequent demands for feeding. Physical examination shows a firm, void, 1 to 2 cm abdominal mass ultrasonography reveals gastric outflow tract obstruction.
Dx?
rF
Pathogenesis
Pyloric stenosis
Monozygotic, twins, turner syndrome, trisomy 18
Erythromycin, and azithromycin in the mothers milk
Hyperplasia of the muscularis propria of the pylorus
In Hirschsprung disease, the mutation is what
Loss of function mutation in receptor tyrosine kinase RET
Pathophysiology of Hirschsprung disease
Migration of neural crest cells from the cecum to the rectum is arrested, prematurely, or when the ganglion cells undergo premature death
What are the two types of Hirschsprung disease according to affected sediment
Short segment disease: rectosigmoid only
Long segment disease : more proximal extension
What is the most common point of rupture in the colon in Hirschsprung disease?
Cecum
What is the most common form of congenital intestinal atresia?
Imperforate anus
What is the most common side of fistulization in the GI tract
The esophagus
