Gastrointestinal Embryologic Development and Defects

  • After folding, endoderm is still connected to yolk sac via yolk stalk..

yolk stalk = vitelline duct = omphalomesenteric duct

Yolk stalk-marks the place known as MIDGUT

NOTE: pharynx is the most rostral portion of foregut

Foregut-supplied by the celiac artery

Midgut-supplied by the superior mesenteric artery

Hindgut-supplied by the inferior mesenteric artery

  •          We like to think that the rotation of the gut induces some of the rotation of the caudal part of the gut
  •          The foregut goes from the orapharyngeal membrane to a portion of the duodenum-there’s a specialization in the area-area of the stomach

Mesentery-the same tissue that lines that the body-like peritoneum-at two places on the foregut, the posterior midline and anterior midline, peritoneum leaves body and goes out the viscera, folding it, then it goes to the other side body wall

  •          there’s anterior & posterior mesentery
  •          Gut rotates so that its posterior midline faces left and it’s anterior midline faces right-puts the left vagus anterior and the right vagus posterior

SMA-directed towards the stump of the yolk stalk-as the yolk stalk regress, this artery gets shorter and shorter

 

MIDGUT ROTATION-Occurs in two phases:

1) Gut herniates into the umbilical cord-around 6th week, start of the 7th.

  •          As this happens, it is arranged in two cords, one is above the SMA, and the other is below the SMA
  •          ABOVE SMA-PROXIMAL LOOP
  •          BELOW SMA-DISTAL LOOP
  •          obviously, the SMA is the axis around which the gut is rotating 270 degrees
  •          As the gut goes out, the proximal rotates right 90 degrees and the distal loop rotates left 90 degrees-describes twisting

2) Gut Retracts-At the end of the period of herniation, when gut comes back into the body, the distal loop starts coming in first on the right side of body, causing a 180 degree rotation-total rotation is 270 degrees

  •          Looking at person from front, rotation is counter-clockwise

Omphalocoele-persistent herniation of the gut-the gut fails to retract into the abdomen

  •          The proximal loop of the gut ends up posterior to the transverse colon
  •          SMA will pass anterior to a portion of the duodenum
  •          future duodenum is posterior to the superior mesenteric artery

Ø       At the same time as this rotation, other foregut structures are being formed

 

FOREGUT STRUCTURES = digestive tract and abdominal portions of the digestive tract

  •          foregut includes the esophagus, stomach, half of duodenum, liver, pancreas, and the biliary apparatus
  •          these structures are attached to the 2nd part of the duodenum-their site of “emptying” into the duodenum is in the foregut
  •          Their opening into the duodenum is via the major duodenal papilla-yeah, it’s the same thing as Vater
  •          LAST thing in the foregut is the MAJOR DUODENAL PAPILLA

MIDGUT-the 3rd and 4th portions of the duodenum + jejunum, ileum, ascending colon, and part of the transverse

HINDGUT-begins roughly at splenic flexure of the colon

  •          That portion which DID NOT HERNIATE during the midgut herniation

Spleen is in the abdomen but it’s MESODERMAL derived-it’s out on mesentery but it’s NOT a derivative of the abdomen

Pancreas-it can begin to encircle the duodenum

  •          there’s a dorsal and ventral pancreatic bud-as the gut rotates, the two buds are brought together so that they fuse-usually there is one dominant duct remaining
  •          after the stomach rotates 90 degrees, the two buds fuse-the ventral bud becomes the uncinate process and part of the head while the dorsal bud becomes the rest of the pancreas

CLEFT PALATES

VENTRAL PANCREATIC BUD-uncinate process and part of head

POSTERIOR PANCREATIC BUD-body of pancreas

  •          Main duct is Wirsung-opens into the 2nd part of the duodenum in the major duodenal papilla, along with the common bile duct
  •          The dorsal duct is the duct of Santorini-this is the accessory duct

VENTRAL DUCT-Wirsung                          

DORSAL DUCT-Santorini

  •          The entire major pancreatic duct is called “Wirsung”
  •          Santorini is proximal to Wirsung

 

HEPATIC PLATE-thickening that grows anteriorly from the duodenal wall towards the septum transversum

Ø       Liver is simply an outgrowth from the distal part of the foregut-wall of the endothelium of the tube

Ø       Biliary apparatus-it grows into the mesentery & invades the septum transversum

Ø       Livers grows into the mesentery and grows between two leves,

  •          goes out toward inferior surface of the diaphragm
  •          then pushes through the septum transversum, creating the bare area of the liver

 

Bare Area-is such that endodermal tissue is touching mesodermal tissue without any intervening border-this is where the coronary ligament forms-this ligament is the combination of all the peritoneums that surround the bare area

 

CLINICAL CORRELATION: Annular Pancreas-rotation of pancreas doesn’t occur as it should and the pancreas surrounds duodenum

  •          annular pancreas can cause problems if it compresses the duodenal lumen

Philo jumps to UG for a minute, then leaves

Descent of the testis

  •          testis develop posterior to peritoneal membrane & need to get out into scrotum b/c sperm don’t like it hot, hot, hot
  •          as testis grows out it pulls a little bit of peritoneal cavity, forming the parietal and visceral layers of the tunica vaginalis

 

PERITONEUM: As gut grOWS into the peritoneal cavity, it is covered by peritoneum and is separated out into ventral & dorsal mesentery.

Peritoneal Tissue-there’s a parietal and a visceral peritoneum-visceral covers gut….please say you knew that

Mesentery-twin-walled leaf of peritoneum, containing within it fat

  •          there is a dorsal/posterior mesentery and ventral/anterior mesentery

Dorsal Mesentery-CONTAINS THE ARTERIES going to the gut from the aorta

Ventral Mesentery-is lost except for that of the foregut-liver & biliary apparatus, part of duodenum, stomach, and the most terminal portion of the esophagus retain it

  •          jejunum & ileum have none

Peritoneal-organ suspended by mesentery-it’s covered by peritoneum and suspended by mesentery

  •          stomach, cecum, jejunum, ileum, transverse colon, and sigmoid colon

 

RETROPERITONEAL-not peritoneal-abdominal organ NOT suspended by mesentery

  •          Retroperitoneal = Preperitoneal = Extraperitoneal……….terminology can be mixed but it’s really all the same
  •          ascending and descending colon, rectum-retroperitoneal

Primary Retroperitoneal-those organs that NEVER had a mesentery suspending it, i.e., kidney

Secondarily Retroperitoneal-organs that once had a mesentery and then lost it, i.e., duodenum or pancreas

 

Ø       Stomach begins to rotate as gut is herniating and rotating

Ø       Gut rotates to left such that LT VAGUS GOES ANTERIOR and RT VAGUS GOES POSTERIOR to the esophagus

Ø       So, the old midline of the stomach ends up to the left side-mesentery has grown out to the side

 

OMENTA-specialization of mesentery at the level of the stomach and the liver(foregut)

 

GREATER OMENTUM-a derivative of dorsal mesentery seen as the mesentery grows as a loop over the stomach

  •          greater omentum forms a small sac w/in a sac and binds the lesser sac/lesser peritoneal cavity-remainder of the abdominal cavity is the greater sac-again, greater omentum forms the lesser sac
  •          Lesser sac is subdivision of the peritoneal cavity and it’s only entrance is the epiploic foramen

 

LESSER OMENTUM-a derivative of the ventral mesentery-lesser omenta is the mesentery b/t stomach and liver

Dorsal MesenteryàGreater Omentum-forms lesser sac  //   Ventral MesenteryàLesser Omentum

GUT ROTATION-about 270 degrees clockwise w/ the SMA as the axis-SMA is artery of midgut

  •          Liver, biliary apparatus, and pancreas develop at the very caudal end of the foregut
  •          CELIAC ARTERY-artery of the foregut
  •          SMA-artery of the midgut
  •          IMA-artery of the hindgut

 

PANCREAS develops from ANTERIOR to POSTERIOR bud

  •          buds sprout from duodenal foregut into ventral and dorsal mesenteries-the ventral migrates posteriorly to fuse w/ dorsal
  •          Dorsal Bud-bud and duct of Santorini
  •          Ventral Bud-bud and duct of Wirsung
  •          These two buds rotate with the rotation and come together in much harmony
  •          Major duct is that of Wirsung and it opens into Major Duodenal Papilla-of Vater
  •          Stomach rotates so that it’s old left is facing forward-rotating so that its posterior midline points directly to the left
  •          Lesser curvature-see the ventral mesentery-forms the lesser omentum
  •          Greater curvature-see the dorsal mesentery-forms the greater omentum
  •          Liver grows into ventral mesentery, pushing peritoneal membrane apart, and then grows to the septum transversum-this pushing away at the peritoneal membrane forms the bare area of the liver
  •          Coronary ligament-portion of peritoneal membrane pushed aside
  •          Falciform Ligament-ventral mesentery between the liver and the anterior body wall
  •          There’s no ventral mesentery inferior to the falciform ligament, or inferior to the portion of the lesser omentum betweem the liver and the stomach
  •          gut starts out as hollow tube, then becomes filled w/ epithelium growing from the side walls
  •          as gut grows in diameter, there’s not sufficient delivery of nutrients to the central portion of the gut-thus, it recanalizes to form the definitive hollow gut present at birth

 

OMPHALOCOELE–DUE TO FAILURE OF COMPLETE ENFOLDING OF THE ABDOMINAL WALL

  •          these are also seen in Prune Belly Syndrome
  •          GIANT omphaloceole-case where liver is present sac-affected babies have relatively small abdominal wall

CLINICAL CORRELATION: At first these were simply cured with mercuritone(local antiseptic)-problematic b/c not sufficient w/ large omphaloceoles; it left the children with ventral hernia; and it could cause mercury poisoning in some

  •          Using silon mesh, the “stuff” can pretty much be gradually forced back into place
  •          Child can suffer pulmonary hypoplasia or develop infection
  •          Thus, a new silver-based antiseptic, silvadene, is used and kid is closed up gradually after abdomen enlarges

 

Pentalogy of Cantrell-epigastric omphaloceole, ectopia cordis, and other problems combined-mostly incompatible w/ life

  •          problem w/ this is its difficult to fix w/o shutting down blood supply-also, the heart’s exposed-ectopia cordis

 

GASTROSCHISIS-defect in abdominal wall resulting from amniotic membrane rupture during physiologic gut-loop herniation or delayed umbilical ring closure-ONLY INTESTINES ARE EXTRUDED

  •          gastroschisis as a defect is smaller than omphaloceoles-there’s no liver involved, it’s the bowel that’s exposed
  •          may be caused by the fact that as the umbilical vein regresses, there is a weak spot in the right abdominal wall-this is convincing b/c most of reported cases are on the RIGHT

Two problems:            1) Like omphaloceoles, you have to get the intestines back in the abdominal cavity

2) The intestines are shortened, thickened, and matted-they have been exposed to amniotic fluid during the entirety of gestation-an irritant effect that leads to the bowels’ thickening

  •          GI tract has blood flow, but lots of SA that was not covered w/ skin-this means fluid and heat loss
  •          This is a surgical emergency-children are closed w/ silicon patch gradually-like in 6-7 days post-delivery

 

ESOPHAGEAL ATRESIA-name’s indicative-there’s a gradual separation of the esophagus from air tract over time from lateral enfolding-can be associated w/ polyhydramnios; and is very often associated w/ tracheoesophageal fistula

  •          most common type-distal fistula-i.e., proximal esophageal atresia w/ a fistula distal from trachea to stomach
  •          variant-long gap esophageal atresia-there’s blind ending of upper esophagus w/ no connections
  •          w/ the long gap situation, you hope for an associated tracheoesophageal fistula-w/ treatment, closure of the gap, baby can suffer small leaks in the make-shift esophagus and gastroesophageal reflux

 

Gastric Atresia-unknown to exist-however, there is such thing as microgastrula, or pyloric atresia-both are rare as well

 

DUODENAL ATRESIA-atresia-may arise from annular atresia, or annular pancreas

  •          baby-neonatal-with bubble of gas in the stomach
  •          Using ultrasound-baby in utero seen with amniotic fluid-filled stomach and proximal duodenum
  •          upper GI tract atresia means polyhydramnios
  •          1/3 of these atresias are seen assoc. w/ Downs-note: this is not the case alternately
  •          mom’s w/ this prenatal diagnosis are given an amniocentesis to check for abnormal chromosomes
  •          Theory on duodenal atresia-failure of recanalization of the duodenum

 The lower down the atresia, the lower the incidence of polyhydramnios-b/c amniotic fluid is allowed to circulate more

  •          Theory on jejunal and ileal atresias-has to do w/ vascular accidents
  •          Jejunal & Ileal atresias-problematic b/c baby may have no gut since blood supply has been cut-thus severe short gut syndrome is present

Jejunal Atresia-could be fatal-if large portion of GI tract is missing-or could be a simple web

  •          difficult to deal w/ b/c you are working near Ligament of Treitz and consequently, the pancreatic blood supply

Ø       With GI tract atresias you have the problem of a shortened gut post-operation

Ø       These atresias are seen easily on X-ray-you see dilated bowel-could in stomach, duodenum, etc. but it’s indicative of where the atresia is at-you can see several dilated segments as well

Ø       In lot’s of these cases you see incorrect innervation; w/ lost supply from SMA, you get severe short gut syndrome

Ø       Also, these are all endoderm derived

 

IMPERFORATE ANUS/ cloacal anomalies, etc…

  •          problem is that the uro-rectal septum comes down and meets the cloacal membrane and becomes the anal plate
  •          anomaly occurs at hindgut-there is thus spearation of urinary and rectal structures

MALES: incidence of fistulas to urinary tract is extremely high

Low lesion imperforate anus-occurs b/t coccyx and pubis-above this is a High imperforate anus

  •          Assoc. problems: renal anomalies, spinal cord and sacral anomalies
  •          In treatment, muscles must be preserved-also, though many of the babies are repaired anatomically, many suffer incontinence due to faulty innervation-only 50% end up w/ true continence

 

MECKEL’S DIVERTICULUM-vitelline duct persists as remnant of variable length and disposition

  •          often presents w/ bleeding b/c they have ectopic gastric muscosa that produces acid & this acid leads to ulceration
  •          post-op, you can get persistent tethering & resultant volvulus, or twisting of intestine
  •          the duct may remain open to the umbilicus or form an enterocyst of fibrous omphalomesenteric ligament
  •          symptoms not unlike appendicitis
  •          w/ volvulus, you can lose as much as 2/3 of ileum and still be alright-so long as the ileocecal valve is intact

 

MALROTATION

Remember-we go from a single-endoderm-lined tube to an absorptive surface that has grown and grown and grown

  •          w/ that growth, there’s rotation-and this is rotation around the SMA
  •          w/ bowel elongation, there’s a duodenal jejunal limb and a cecal ileocolic limb
  •          the duodenal-jejunal limb rotates 90, then 180, for 270 total-around the SMA
  •          w/ this rotation, you get cecum in lower right quadrant and duodenal-jejunal junction underneath stomach, giving a broad base for the SMA
  •          w/ malrotation, you get a short base for the SMA-it can rotate on itself and volvulus occurs
  •          patient presents w/ GI tract obstruction distal to Vater ampulla and bilious vomiting
  •          side note-ileal atresia also presents w/ bilious vomiting
  •          Radiograph shows gas in bowel-in stomach-and then volvulus, or twisted bowel
  •          Problem here-infant otherwise normal & if this not all picked up quickly, you got dead bowel due to insufficient blood
  •          In some cases, operation means putting the cecum in lower left quadrant

Duplications-they’re infrequent, can occur at any level, and can tend to be asymptomatic

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