Gastrointestinal Emergencies of the Term and Preterm Infant

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    Curry Bordelon, PNP-AC, MBA

    Pediatrix Medical Group

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    Objectives Overview of basic GI anatomy

    Overview of GI function

    Discussion of open abdominal wall defects and theirtreatment

    Discussion of closed abdominal wall defects and their

    treatment

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    Overview of the GI tractStomach

    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.

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    Overview of the GI tractStomach

    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.

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    Overview of the GI tractJejunal

    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).

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    Overview of the GI tractJejunal

    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.

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    Overview of the GI tractIleum

    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).

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    Overview of the GI tractColon.

    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.

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    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)

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    Open abdominal wall defects gastroschisis

    omphalocele

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    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%

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    Gastroschisis Treatment involves

    Gastric decompression

    Gut rest

    Antibiotics

    Silo suspension (sealed plastic device surgically attached to infantand 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 infantswith adequate abd tissue.

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    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.

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    Closed abdominal wall defects Atresias

    Stenosis

    TE fistula and/or EA NEC

    Perforation

    Diaphragmatic hernia

    Hirshsprungs

    Imperforate anus

    Meconium plug syndrome Meconium ileus syndrome

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    Atresias/stenosis Bilary

    Duodenal

    Jeunoileal

    Stenosis

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    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)

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    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 isthe dissection and resection of the extrahepatic duct.

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    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

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    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.

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    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 presentinginfants).

    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 toside or end to end anastomosis

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    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

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    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.

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    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.

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    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.

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    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).

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    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.

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    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.

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    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.

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    http://bms.brown.edu/pedisurg/images/ImageBank/CDH/XRCDHLeftNewborn.jpg
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    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

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    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.

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    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

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    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.

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    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.

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    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.

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    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.

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    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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    Questions?