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Unit 3 clinical correlation
Unit 3 clinical correlation - embryology of GI, respiratory and heart
39
Biology
Professional
10/20/2009

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Term
Omphalocele
Definition
Midgut fails to return to abdominal cavity

Protruding intestines are in the umbilical cord, covered by amnion and peritoneum
Term
gastroschisis
Definition
defect in body wall leads to extrusion of abdominal viscera into amnion;

viscera not covered by peritoneum or amnion
Term
umbilical hernia
Definition
imperfect closure of umbilical ring; usually resolves itself
Term
non-rotation
Definition
midgut rotates only first 90 degrees; large intestine and appendix found on left
Term
reverse rotation
Definition
first 90 degrees normal; last 180 degrees rotates counterclockwise;

results in duodenum anterior to transverse colon - may cause compression of colon/vessels to colon
Term
subhepatic cecum
Definition
cecum fails to elongate and descend; appendix in upper right quadrant; absence of ascending colon
Term
incomplete fixation of midgut
Definition
results in mobile cecum, duodenum, etc; volvulus (twisting) may occur, resulting in obstruction of blood supply = necrosis and gangrene;

usu accompanied by bilious vomiting
Term
atresia
Definition
small intestines 'blind end'; failure of recanalization (duodenum) or vascular accident, resulting in failure of on portion to grow
Term
stenosis
Definition
narrowing in small intestines (possily due to error in recanalization)
Term
duplication
Definition
small intestine; pobably due to error in recanalization
Term
Ileal (Meckel's) diverticulum
Definition
persistence of vitelline duct on antimesenteric border of ileum; may contain gastric or pancreatic tissue; ulceration mimics appendicitis

2 in. long, 2 feet from ileocecal jxn, 2% of live births
Term
vitteline cyst
Definition
sac connected to umbilicus and ileum by fibrous cords
Term
vitelline fistula
Definition
passage from ileum to umbilicus; fecal matter may pass through
Term
esophageal atresia or tracheoesophageal fistula
Definition
due to abnormal partitioning of trachea and esophagus by tracheoesophageal septum;

most common: upper part of esophagus ends in blind pouch; lower part opens into trachea
Term
anorectal malformation
Definition
more common in males; often associated anomalies of lumbar and sacral verterae
Term
imperforate anus
Definition
no anal opening; associated with atresia, stenosis, high imperforate anus, low imerforate anus
Term
agenesis
Definition
failure to develop
Term
Hirschsprung disease (congenital aganglionic megacolon)
Definition
abscence of PS ganglia in colon wall; no peristalsis; proximal colon becomes dilated;

failure of neural crest cells to migrate or differentiate
Term
polyhydramnios
Definition
^ amniotic fluid; results in maternal abdominal swelling and fetal ipaired uteroplacenal perfusion
Term
oligohydramnios
Definition
v amniotic fluid (often due to kidney problem); compressoin of abdomen and thorax, resulting in pulmonary hypoplasia
Term
respiratory distress syndrome (RDS)
Definition
due to insufficient surfactant (collapsed alveoli); corticosteroids and artificial surfactant are given; labor delayed until treatment with corticosteroids to help surfactant to develop (ideally)
Term
pulmonary hypoplasia
Definition
most common: cong diaphragmatic hernia = decreased intrathoracic space; thoracic compression (oligohydramnios); decreased fetal breathing (cns damage)
Term
congenital hiatal hernia
Definition
esophagus too short
Term
congenital hypertrophic pyloric stenosis
Definition
hypertrophy of pyloric muscle leads to stenosis; projectile vomiting; more common in males
Term
duodenal obstruction
Definition
usually 2nd part; bilious vomiting soon after birth (delayed of only stenosis); often assoc with down's syndroe; commonly assoc with other GI abnormalities; anular pancreas,stenosis, atresia, midgut volvulus, etc
Term
biliary atresia
Definition
most common: born with ducts, but unknown inflam. process destroys them; develops jaundice shortly after birth; liver transplant required
Term
paucity of intrahepatic bile ducts
Definition
too few ducts; cause unknown - progressive destruction?; some benign, others require liver transplant
Term
probe patency of foramen ovale
Definition
(ASD) imcomplete adhesion of septum primum and secundum; not usu clinically significant
Term
secundum ASD
Definition
defect in fossa ovalis; result from: excessive resorption of primum; defective formation of secundum
Term
endocardial cushion defect with primum ASD
Definition
endocardial cushions don't fuse - foramen primum persists;

often assoc with Down's Syndrome
Term
membranous VSD
Definition
defect in membranous IV septum; VSD accounts for 25% of cardiac defects
Term
mscular VSD
Definition
from excessive cavitation of myocardial tissue during muscular septum formation
Term
persistant truncus arteriosus
Definition
truncoconal swellings fail to grow; single artery arises from both vesicles, mixing blood; divides into aorta and pulmonary trunk
Term
transposition of the great vessels
Definition
aorta from rt ventricle and pulmonary trunk from the left (from failure of spiral); also a ventricular septal defect and patent ductus arteriosis - 2ndry defects make life possible
Term
tetralogy of fallot
Definition
1) pulmonary stenosis 2)ventricular septal deffect (membranous portion) 3) overriding aorta 4) right ventricular hypertrophy (from ^ pressure)

most common cause of 'blue baby'; conus septum develops too far anteriorly, restulting in unequal vessles and septum too far anterior
Term
aortic or pulmonary stenosis
Definition
valve is thickened and narrowed - ^bp in ventricle; wall thickens; restricted blood flow results in murmur; may start mild, but usu. worsens with growth; evidence of 'strain' may require valve treatment
Term
persistent ductus arteriosus
Definition
normal closure within days to weeks; blood flows from aorta to pulmonary artery; treat with prostaglandin synthase inhibitor;

assoc with premature birth and rubella; accounts for 10% of CV malformations in infancy
Term
coarctation of aorta
Definition
narrowing of aorta; often unidentified until childhood; anastomoses make up for loss of flow through descending aorta - large intercostal and epigastric arteries, internal thoracic;

always suspect in young adults with HTN
Term
dextrocardia
Definition
heart is reversed - often occurs when all organs are reversed - situs inversu;
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