Embryology Flashcards
Esophageal Atresia
Common, presents as maternal polyhydramnios
usually presents with other stuff
One blind pouch of mouth to esophagus,
and the rest of the esophagus to trachea
Requires surgical repair
physiologic Jaundice in newborns
Why newborns have jaundice, newborns have decreased RBC Half-life compared to adults, the liver has a decreased ability to conjugate bilirubin, there is decreased enterohepatic circulation
Full- term, formula fed, avg. peak total bilirubin about 6 at day 3
Full term breast fed total bilirubin- 15-18 by Day 4
Bilirubin should begin to decrease by day 6-7
bili will start to decrease after a week of life
Abnormal jaundice
Jaundice beyone 2-3 weeks of age requires eval
Direct>2, t>12 or >14 for premies
Metabolic evals, ultrasound, and essentially a workup to see if there is something anatomical going on
neonatal bile duct obstruction- biliary atresia
rare, presents a few weeks later, conjugated hyperbilirubinemia, pale stool and dark urine
Ultra sound, IDA scan , at 2 months can get kernicterus
Progressive, acute then chronic, inflammatory cholangiography with complete obliteration of extrahepatic biliary structures, typically by 12 wks of birth cholestasis, neocholangiogenesis, hepatocellular injury occurs, cirrhosis, portal HTN, END stage liver disease, death under 2 yrs of age w/o portoenterostomy and or transplantations
can be infectious cytokeratin 19 stain
A cirrhotic liver is end stage
Treatment KASAI procedure (1/3rd of caseS)
most common reason for pediatric liver transplant
Choledochal cysts
can be a little older, elevated direct conjugated bilirubinemia–jaundiced
bile plug, infection or mass
Several types, type 4-5 need a new liver
Pathogenesis- disordered organogenesis, pancreaticobiliary malunion, acquired weakness, distal obstruction
Can lead to biliary adenocarcinoma
Duodenal atresia
double bubble
usually due to failure to recanalization
The duodeneum passes thru a sold phase early in development, the lumen reestablishes and opens by week 8-10
Embryological insult during this time results in duodenal stenosis, web, atresia
repair surgically
Malrotations!
had a cyanotic episode immediately postpartum but resuscitated immediately
Bilious emesis after first feeding attempt
Any shade of yellow or green vomit
Normally the bowel is fixated in 2 places- left upper quad (ligament of treitz), lower right near the cecum
The narrowing mesenteric base allows abnormal mobility of the small bowel, allowing the mesentery to twist= midgut volvulus (entire midgut can die)- twists around the SMA- 6 hours
Ladd’s bands- which cross the duodenum–> obstruction
Ladds procedure- fix everything, and appendectomy
meckels diverticulum
2 year old female with bloody diaper, otherwise healthy
Remnant or incomplete involution of the vitaline duct
Tachy cardia
Abnormal lining of the stomach, rules of 2 (2% of pop, 2 types of abnormal lining (stomach and pancreas), 2 feet of the ileocecal valve, 2 inches in length and usually symptomatic by 2ys
Bleeding into intestine, could be blockacge or etc
Just resect
jejunoileal atresia
polyhydramnios, no passage of meconium, large bilious emesis, transport to the NICU
Large dialation
Inutero vasculat disruption leading to ischemic necrosis of the fetal intestine, necrotic intestine is REABed leving behind the proximal and distal ends with a gap in the mesentary
Surgery, usually good prognosis
Hirschspring disease
Congential defect in intestinal innervation, can be associated with 21
RET mutations
a segment of the distal colon results without nerve cells, both meissner submucosal and auerbachs myenteric plexus lacking, A-ganglionosis
Functional peristaltic obstruction
Most common in rectosigmoid – bacterial overgrowth–> sepsis
Fail to pass meconium- ressection and connection
Anorectal malformations
no polyhydramnios, stops feeding after a few days, normal wet diapers
trisomy 21
no true anus
Colostomy with new anus
