Curry Bordelon, PNP-AC, MBA

Pediatrix Medical Group

Objectives
Overview of basic GI anatomy
Overview of GI function
Discussion of open abdominal wall defects and their
treatment
Discussion of closed abdominal wall defects and their
treatment

Overview of the GI tract
Stomach
hollow muscular organ.
second phase of digestion, following mastication.
highly acidic environment due to gastric acid production and secretion
pH range usually between 1 and 4, depending onfood intake, time of the day, drug use, and
other factors.
Combined with digestive enzymes, able to break down large molecules to smaller ones so
that they can eventually be absorbed from the small intestine.
Overview of the GI tract
Stomach
produces and secretes about 2 to 3 liters of gastric acid per day
(highest in the evening).
Absorption of vitamin B12 from the small intestine is dependent
on conjugation to a glycoprotein called intrinsic factor which is
produced by parietal cells of the stomach.
Overview of the GI tract
Deuodenal
hollow jointed tube (25-30 cm) connecting the stomach to the jejunum.
first and shortest part of the small intestine and it is where most chemical
digestion takes place.
begins with the duodenal bulb and ends at the ligament of Treitz.
largely responsible for the breakdown of food in the small intestine.
Brunner's glands, which secrete mucus, are found in the duodenum.
Overview of the GI tract
Deuodenal
Regulates the rate of emptying of the stomach via hormonal pathways.
Secretin and cholecystokinin are released from cells in the duodenal epithelium
in response to acidic and fatty stimuli present there when the pyloris opens and
releases gastric chyme into the duodenum for further digestion. These cause the
liver and gall bladder to release bile, and the pancreas to release bicarbonate
and digestive enzymes such as trypsin, lipase and amylase into the duodenum as
they are needed.
Overview of the GI tract
Jejunal
central of the three divisions of the small intestine (between duodenum and ileum).
small intestine is usually between 5.5-6m long in adults (2.5m is the jejunum).
pH in the jejunum is usually between 7 and 8 (neutral or slightly alkaline).
specialized lining for the absorption of carbohydrates and amino acids (proteins broken
down in the stomach by pepsin and acid).

Overview of the GI tract
Jejunal
carbohydrates are broken down in the duodenum by enzymes from the pancreas and liver
into sugars.
Fats are broken down in the duodenum by "lipase" from the pancreas into fatty acids.
Amino acid, sugar, fatty acid particles, vitamins, minerals, electrolytes and water are small
enough to soak into the villi of the jejunum and drop into the blood stream.
Overview of the GI tract
Ileum
last portion of the small intestine (responsible for absorption of fats, and bile salts).
pores in the ileum are slightly bigger than those in the jejunum and allow vitamin B12,
vitamins dissolved in fatty liquids, electrolytes, bile salts and water to be reabsorbed.
ileum joins the colon at the ileocecal valve (prevents the back flow of materials into the small
intestine).
Overview of the GI tract
Colon.
Its function is to move the waste from the small intestine on to the rectum.
material first passes through the ascending colon and then through the transverse
colon absorbing more water and potassium.
The sigmoid colon is designed to slow down this movement of the waste until it is
ready to be eliminated.
Types of defects
open abd wall (all or some of the abdominal contents noted outside the
infant)
closed abd wall (normal appearance outside the infant)

Open abdominal wall defects

gastroschisis

omphalocele
Gastroschisis
Full-thickness defect of the abd wall exposing intestinal contents.
Generally a small defect (3-6 cm).
Right lateral to the umbilicus.
1 in 40,000 births.
Male> female
Infants are generally preterm or SGA
Malrotation affects nearly all infants
Survival rates are higher than omphalocele, 95%
Gastroschisis
Treatment involves
Gastric decompression
Gut rest
Antibiotics
Silo suspension (sealed plastic device surgically attached to infant
and suspended above infant.
allows the bowel to return to normal size.
commonly have underdeveloped abd capacity thus not allowing for
primary closure.
Daily decompression allow for stretching of the abd tissue and minimizes
intestinal damage.
Primary closure- generally for small defects or those term infants
with adequate abd tissue.
Omphalocele
failure of the intestines to return from the umbilical cord into the abd cavity
resulting in a transparent membrane that encapsulates intestinal tissue.
1 in 5,500 births
Male > female
Frequently associated (50% - 77%) with other syndromes such as trisomies, CHD,
CDH
Ranges from 2-15 cm on average. Smaller defects may be overlooked
Larger defects may include spleen and liver also
Omphalocele
Survival rates are high (75% - 95%).
Due to associative chromosomal issues, surgery is the second choice of treatment. First
line treatment involves abdominal wrapping or painting the defect with silver nitrate or
silvadine to promote eschar formation and epitheal tissue growth.
Abd decompression and delayed feedings are important to allow the inflamed intestinal
lumen to return to normal size. Antibiotics may also be used.
Surgical repair is generally reserved for the most severe cases and involves using gortex
flaps to cover the transparent sack.
An unfortunate result of non-surgical closure is malrotation.
Closed abdominal wall defects
Atresias
Stenosis
TE fistula and/or EA
NEC
Perforation
Diaphragmatic hernia
Hirshsprungs
Imperforate anus
Meconium plug syndrome
Meconium ileus syndrome
Atresias/stenosis

Bilary

Duodenal

Jeunoileal

Stenosis

Bilary Atresias
complete obstruction of the bile flow of the extrahepatic ducts.
Most common form of ductal cholestasis
1 in 10,000 births
Prevents bile from entering the duodenum
Cause unknown (malformed or inflammatory process)
Digestion and absorption of fat soluble vitamins (A,D,E,K) is impaired.
Leads to cirrhosis symptoms and death if untreated.
Associated congenital defects found in 15% (CHD, Splenic issues, small
bowel atresias, trisomies)
Bilary Atresias
Normal appearing infant with normal stools
Symptoms usually begin with persistent jaundice after the first week of life
Direct bili levels begin to climb
Green bronze color to skin
Gradually stools become clay-colored
Differential diagnosis (hepatitis, HIV, CMV, HSV errors of metabolism)
Survival untreated is less than 2 yrs.
Nearly all pts have long term liver issues (elevated enzymes [ALT, AST, GGT,
CRP], chronic fatigue, poor weight gain, ascites)
Treatment involves hepatic portoenterostomy (Kasai procedure) which is
the dissection and resection of the extrahepatic duct.
Duodenal Atresia
Incomplete recanalization of the lumen.
Transverse diaphragm of the tissue completely obstructing the
lumen
1 in 7,500 births.
25% of all duodenal atresias are related to Downs
Polyhydramnios is the #1 first sign on prenatal US
60-70% of DA pts also have trisomies, Imperf A, TE anomilies,
CHD, VATER, VACTERL, renal issues, annular pancreas
Bilious Emesis is the #1 presenting symptom after delivery
Duodenal Atresia
Proximal atresias/obstruction generally results in vomiting
within the first few hours of life.
Distal atresias/obstruction results in emesis longer after
delivery.
Classic double bubble on xray. Gasless pattern after the
atresias
65%-84% survival rate with early intervention.
Treatment involves decompression of the stomach by
continuous suctioning followed by surgical removal of the
atresia area with a side to side anastomosis.

Jejunoileal Atresia
obstruction of the distal segment of the small intestine.
Most commonly near the ileo-cecal valve
1 in 20,000 births
Polyhydramnios is not as common of a risk factor (only of the presenting
infants).
Classic triple bubble and microcolon on xray
84%-95% survival rate.
Treatment involves decompression of the stomach by continuous
suctioning followed by surgical removal of the atresia area with a side to
side or end to end anastomosis

Stenosis
Far less common than atresias/obstructions.
Generally from excessively narrow areas of the small intestine
(duodenum or the ileo-cecal valve).
Most common cause is by webbed areas of the inner lumen.
Occurs most often following other GI surgeries
TE fistula and/or EA
Failure of the trachea to differentiate from the esophagus
There are different types of disorder
85% have EA and a TE fistula
8% have EA without any connection to the trachea
1% have esophageal fistula and no connection to the stomach
4% are an H type fistula
1 in 4,500 births
TE fistula and/or EA
VATER and VACERL association is common
20%-30% are preterm
Signs are excessive oral secretions (from an inability to swallow), inability to pass
OG/NG tubes, aspiration, chronic pneumonias.
97% survival rates
Mortality is associated with associative disorders
Surgery depends on the type of disorder.
NEC
Necrosis of the mucosal and submucosal layer of intestine.
Any portion of the GI tract can be affected.
Causes are still a debate (most common causes are selective bowel ischemia,
infection or delayed/improper feedings.
Osmolarity of certain formulas/meds increase risk of NEC.
Feeding of EBM decreases the risk of NEC by 65% in the preterm infant.
Most common disease affecting hospitalized infants, 8% of all inpatient infants.
NEC
65%-92% of infants affected with NEC are preterm infants.
Most commonly seen in infants 3-21 days post delivery.
Xray shows tiny lucent bubbles.
Generally infants present with abd distention, increased emesis, bloody stools or feeding
intolerance.
Treatment involves 3 basic principles:
Bowel restNPO for 10-14 days
Prevention of progressive injuryNPO, Fluid management, antibiotics
Placement of abd drains.
Perforation
Spontaneous rupture of the intestine/colon allowing for leakage of air into the abd cavity.
Most commonly associated with NEC and ischemic bowel
Most common risk factors are NEC, sepsis, mechanical ventilation, prematurity, long term
steroid usage and postoperative abd complications.
Survival is directly related to how quickly the staff is able to identify clinical changes.
Pneumoperitoneum (free air in the abd cavity) is the most common radiographic sign of
perforation (football or sail sign in the most lateral abd on a left lateral decub).
Perforation
INTESTINAL PERFORATION IS AN EMERGENCY!!!!
Surgical removal of the damaged and perforated area is almost always required.
Treatment also involves preventing additional bowel injury by:
Bowel restNPO for 10-14 days
Gastric decompression
Fluid management, antibiotics
Placement of abd drains may not be needed.
Diaphragmatic hernia
Herniation of intestinal contents into the thoracic cavity resulting
in underdeveloped lung tissue leading to respiratory distress.
1 in 4,000 births
Usually present with cyanosis, respiratory distress and scaphoid
abd.
Usually seen during routine prenatal US.
Diaphragmatic hernia
Post delivery xray reveals intestinal loops in the chest cavity
Immediate intubation and gastric decompression is essential to
higher survival rates.
Treatment also involves surgical placement of abd tissue back into
abd cavity once the infant has become stable.
Hirshprungs
Also known as megacolon or aganglionic colon
Congenital absence of ganglionic cells in the distal colon and rectum.
Failure of the neural crest cells to migrate properly to caudal region.
Local failure of the colon to relax thus causing a restrictive, narrowed area.
Zone between the ganglionic and the tapered aganglionic area of the distal colon is called
the transitional zone.
The rectum is always involved.
1 in 5,000 births.
Males > females
Hirshprungs
Most infants are chromosomally normal
Primary symptoms are bilious vomiting, failure to pass stools and abdominal distention. All
infant should pass first stool within 48 hrs of birth.
A significantly dilated colon proximal to the transitional zone on xray.
Survival rates are high at 80%-95% with early intervention
Diagnosis is only definitive by performing a rectal biopsy
Treatment involves a standard rectal pull trough which involves a complete removal of
the affected aganglionic zone, allowing for 7-10 days of bowel rest and slow reintroduction
of feeds.
Imperforate anus
The abnormal or incomplete partitioning of the cloacae resulting in anal stenosis
or atresia.
Rectourethral and rectovaginal fistulas are common.
Cause is unknown
1 in 5,000 births
20% - 75% have associated anomalies.
VATER and VACTERAL syndromes are common
Imperforate anus
Atresias are the most obvious types.
Stenosis is less obvious and usually present as stooling from the penis/vagina or meconium
stained urine.
Spinal anomalies occur in 50% of patients.
Overall mortality (20%) depends on the type if defect and the associated anomalies.
Treatment for stenotic anal openings involves daily progressive dilation of the anus.
For more involved complete atresia, surgical opening and pull through is essential.

Meconium plug syndrome
Intestinal obstruction of the lower distal colon and rectum
Most commonly associated with decreased colonic motility and
hyptonicity (CF).
Different from hirschprungs (no aganglionic cell regions).
Risk factors include prematurity, diabetic mothers, neurologically
devastated infants and mothers treated with magnesium sulfate.
Meconium plug syndrome
Primary symptoms are bilious vomiting, failure to pass stools and
abdominal distention.
Mortality is very low once the stool is passed.
Surgical intervention is rare.
Small saline enemas are the preferred treatment.

Meconium ileus
Obstruction of the distal colon due to abnormally thick meconium
Generally a result of meconium plug syndrome.
95% of the infants with meconium ileus have CF.
1 in 2,000 births
Primary symptoms are progressive abdominal distention within the first
12-24 hours of life followed by bilious vomiting and a failure to pass
stools.
Meconium ileus
On xray the infant will have dilation proximal to the obstruction and a soap-bubble
appearance at the point of obstruction (due to the trapped air in the dense meconium).
Non operative therapy is generally successful in 15%-25% of infants and involves regular
hyperosmolar enemas to evacuate the thickened stool. Medication therapy also should be
implemented.
A temporary ostomy to decompress the obstructed area may be necessary until fluid status
is stabilized.
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