Gastrointestinal Emergencies of the term and preterm infant

1. Gastrointestinal Emergencies of the term and preterm infant Curry Bordelon, PNP-AC, MBA Pediatrix Medical Group

2. 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

4. 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.

5. 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.

7. 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.

8. 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.

10. 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).

11. 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.

12. 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).

13. 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.

14. 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)

15. Open abdominal wall defects • gastroschisis • omphalocele

16. 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%

17. 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.

19. 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

20. 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.

22. Closed abdominal wall defects • Atresias • Stenosis • TE fistula and/or EA • NEC • Perforation • Diaphragmatic hernia • Hirshsprungs • Imperforate anus • Meconium plug syndrome • Meconium ileus syndrome

23. Atresias/stenosis • Bilary • Duodenal • Jeunoileal • Stenosis

24. 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)

25. 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.

27. 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

28. 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.

31. 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 ¼ 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

34. 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

35. 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

36. 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.

39. 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.

40. 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 rest—NPO for 10-14 days • Prevention of progressive injury—NPO, Fluid management, antibiotics • Placement of abd drains.

42. 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).

43. 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 rest—NPO for 10-14 days • Gastric decompression • Fluid management, antibiotics • Placement of abd drains may not be needed.

46. 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 scaphoidabd. • Usually seen during routine prenatal US.

47. 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.

49. 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

50. 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.