GASTROINTESTINAL EMERGENCIES

1. GASTROINTESTINAL EMERGENCIES PEDIATRIC GASTROINTESTNAL EMERGENCIES

2. GASTROINTESTINAL EMERGENCIES • Gastrointestinal emergencies are serious conditions that often develop suddenly and requires prompt and immediate treatment. • Gastrointestinal emergencies can develop throughout the digestive system

3. GI TRACT EMERGENCIES Esophagus Stomach Small Intestines Large intestines Biliary system ( liver, pancreas, gallbladder, bile duct )

4. GASTROINTESTINAL EMERGENCIES IN Adults and Infants/Children • Acute Abdominal Pain • Gastrointestinal Hemorrhage • Biliary Tract Obstruction • Hepatic Failure • Hepatic Encephalopathy

5. GI TRACT EMERGENCIES UNIQUE TO THE PEDIATRIC PATIENT Two major categories Intestinal obstruction Neonatal cholestasis

6. INTESTINAL OBSTRUCTION Antral and Duodenal stenosis and webs Infantile hypertrophic pyloric stenosis Extrinsic Duodenal obstruction Duplications Malrotation with and without volvulus Hirschsprung’s disease Anorectal malformations or Imperforate anus Abdominal Wall Defect Hernias Thick Meconium syndromes Intussception Volvulus Other -Appendicitis - Meckel’s Diverticulum

8. GI EMERGENCIES • 1. 15 year old male presents with severe abdominal pain, and vomiting. There is no history of fever, weight loss , diarrhea , jaundice or joint pain. Based on above information, what additional history , physical finding ,labs and/or radio imaging are needed to make a diagnosis of : • Pancreatitis • Appendicitis • Malrotationwith volvulus • Incarcerated hernia • Intussception

9. APPENDICITIS • Most common condition leading to emergency abdominal surgery in children/adults • Incidence : 140 in 100,000 people of all ages Most frequent between 15 and 24 years of age ( rare in preschool children and rarer in < 1 year of age • Presentation : periumbilical abdominal pain, vomiting, fever 1/3 of all presentation is with perforation of the appendix or lower abdominal mass Note : 11 – 32 % false positive rate pre operatively > 50 % have nonsurgical abdominal

10. GI EMERGENCIES • Pancreatitis Diagnosis: Key Points Symptoms :( age dependant ) + pancreatic isoenzymes ( amylase and / or lipase) 2 to 3 times elevated over normal reference level. Hydration very important especially in the early stages. Studies in animals indicate the CT contrast given early in the course of acute pancreatitis may further diminish blood flow to ischemic areas of the pancreas and increase the likelihood of necrosis------CT contrast should avoided in earlystages Rx : NPO, IV FLUIDS, PAIN MEDS

12. GI EMERGENCIES • A 12 month old infant presents with the history of increased irritability and inconsolable. Growth and development are normal . The physical examination was remarkable for abdominal distension, tenderness and bloody stools ( guiac positive for blood). • Based on your working diagnosis, what labs or test would you request?

13. INTUSSCEPTION COMMON CAUSE OF INTESTINAL OBSTRUCTION IN CHILDREN Peak incidence is infancy • 50% < 1year of age • Male to female ratio is 3:2 • No lead point/ usually idiopathatic • 90 % proximal to the ileocecal valve –ileocolonic, ileoileal,colocolonic • > 2 years of age associated with a lead point Dx/Rx : KUB features the target sign ( a mass in the RUQ ), cresent sign • ( intussceptum, intusscepting lead point, protruding into air filled pocket ), absent liver edge sign/absence of the subhepatic angle, and signsof obstruction ( smooth intestinal wall/dilation- hose or sausage- like due to loss of plication/haustration, air –fluid level –candy cane or upside down J, gas distribution, and orderliness) • air or barium contrast enema Surgery if lead point or > 48 hours • 10% recurrence Complication – perforation ( greater in infants, < 6 months of age, and /or symptoms >3 days • 9

14. INTUSSCEPTION

15. MALROTATION WITH OR WITHOUT VOLVULUS Malrotation occurs during normal formation of the bowel Its presence predisposes to volvulus Its incidence is 1 in 6,000 Male to female 3:1 Presentation : bilious vomiting, abdominal pain, blood in stool etc., but older children may have vague symptoms ( chronic abdominal pain and/or intermittent vomiting) Diff Dx : neonates – duodenal atresia and stenosis, annular pancreas, antral or duodenal web, small bowel atresia Older infant/children – pyloric stenosis, GER, formula intolerance, colic, lactose intolerance Associated anomalies : congenital diaphragmatic hernia, abdominal wall defect (30-60%), duodenal atresia (50%), CHD, Hirschsprung’s disease, mesenteric cysts Dx: Barium enema – cecum localized to the right or left quadrant UGI series – duodenum does not cross midline and the remainder of small bowel lies to the right of the midline U/S – clockwise rotation of the superior mesenteric vein around the mesenteric artery ( “ whirlpool” sign ) Rx : surgery

17. INFANTILE HYPERTROPHIC PYLORIC STENOSIS • Hyper trophy and hyperplasia of the smooth muscle in the antral and pyloric regions • Proposed etiology - ? Hypergastrinemia, ?increased prostaglandins E2 and F2alpha, ? Decreased NADPH diaphorase activity ( a lack of nitric oxide synthase in pyloric tissue Sx : nonbilious vomiting < 5 months ( usually by 3 weeks ) PE : visible gastric peristalic wave Hypochloremic alkalosis ( water loss and hydrochloric acid ) Elevated unconjugated bilirubin Dx : pathognomomic “ pyloric olive” + clinical history + labs No imaging is required Plain film “ caterpillar “shape ( hyperperistalsis and paucity of small and large bowel gas ) Radio imaging with contrast – elongated , double channel ( railroad tract )-enlarged distal antrum muscle and duodenal bulb U/S : hypertrophied pyloric muscle ( anechoic mass ) Rx : medical/surgery

18. DUPLICATIONS • Esophageal – rare • Gastric – incidence 10% -greater curvature -palpable abdominal mass +/- GI bleeding +/- chronic abdominal pain +/- pancreatitis • Duodenal – incidence 10-15 % -located in the mesenteric border Presentation – abdominal mass , abdominal pain, jaundice ,GI bleeding , pancreatitis Associated anomalies : Duplication cysts, vertebral anomalies, ectopic pancreas Dx : UGI with contrast –beaklike projection from the duodenal • CT scan or U/S – mass next to the duodenum • Endoscopy – mass or extrinsic compression • ERCP – duplication seen if the biliary or pancreatic duct is involved • Rx : surgery

19. EXTRINSIC DUODENAL OBSTRUCTION Two most common causes Annular pancreas Congenital bands Associated anomalies : duodenal atresia or stenosis, cardiovascular defect, malrotation, Down’s , TEF Dx : plain film – double bubble

20. GI EMERGENCIES As you are leaving the newborn nursery, your favorite nurse ask if you would see this 5 hour term infant because of feeding intolerance and mild abdominal distension. What two things performed that would aid in making a diagnosis? What was your differential diagnosis ? Give at least 3 associated anomalies .

21. INTESTINAL OBSTRUCTION • Atresia – congenital absence or obstruction of the intestinal lumen Types • Esophageal - incidence 1 in 3000/4500 • Duodenal - incidence 1 in 2000/40,00 • Jejunal and ileal -incidence 1 in 330/1500 • Colonic - incidence 1 in 20,000

22. JEJUNAL AND ILEAL ATRESIA • It is 30% of all neonatal intestinal obstruction • Male = female • 30 % proximal jejunum • 70 % distal jejunum • 10 – 15 % proximal ileum Presentation – less than 24 hours of age – bilious vomiting, progressive abdominal distension ( ileal > jejunum ), failure to pass meconium Associated anomalies common – gastroschisis, meconium ileus, malrotation with and without volvulus, Hirschsprung’ disease *Microcolon is seen with all ileal atresia Diff Dx : colonic atresia, Hirschsprung’s disease, meconium plug, meconium ileus, malrotation with volvulus, and bowel duplication Dx : Plain film –multiple dilated loops of bowel with air – fluid levels Rx : medical / surgery

23. DUODENAL ATRESIA Presentation : symptoms within hours of birth, abdominal distension, > 30 ml bilious gastric fluid Nonbilious if obstruction is proximal to the ampulla of vater Diff Dx : webs, stenosis, duplication, • annular pancreas, congenital bands Associated anomalies are common : • esophageal atresia, malrotation, • annular pancreas, imperforate anus, • CHD, IUGR Dx : plain film – “double bubble” sign UGI with contrast ---confirms diagnosis BE with contrast –determine the presence of malrotation and/or midgut volvulus Rx : medical/surgery ** 30 % Down’s syndrome

24. ESOPHAGEAL ATRESIA Five types 1. Proximal atresia with distal fistula ( 85 % ) 2. Proximal and distal blind pouch and no fistula (8 to 10 %) 3. H type , T E fistula and no atresia ( 3 to 4 % ) 4 and 5. variation of proximal fistula, etc. Presentation depends on the type Dx : Inability to pass NG tube into the stomach Plain film ----coiled NG tube Contrast study only to diagnose H type Diff Dx : choanal atresia, diaphragmatic hernia, CHD Associated anomalies : esophageal atresia (40-70%), cardiovascular(40%) VSD, TOF, COA,PDA,vascular rings 10% VACTERL ( vertebral defects, anal atresia, TEF, renal defect, radial limb dysplasia ) 10 – 15 % imperforated anus GI anomalies – malrotation, duodenal atresia

25. HERNIAS Location is along intestines • diaphragm • umbilical • inguinal • internal – rare/ seen at autopsy ( most common type mesocolon /paraduodenum )

26. INGUINAL HERNIA • Its incidence is 0.88 to 4.44 in children ( it is higher in premature infants ) • Male to female ratio is 6:1 • Right sided occurrence is 60 % • Left sided occurrence is 30 % • Bilateral occurrence is 10 % • Associated anomalies : bladder exstrophy, Ehlers-Danlo’s syndrome, ascites, VPS, peritoneal dialysis, CF, congenital dislocation of the hip, undescended testicles,ambiguuous genitalia, congenital wall defect • Presentation : bulge in the groin, scrotum, testicles • Dx : clinical Transillumination, Ultrasound Diff Dx : Testicle torsion, epididymis-orchitis, torsion of the appendix testis, hydrocele, inguinal or femoral lymph adenitis Rx : surgery

27. INGUINAL HERNIA • It’s a sac containing abdominal contents and protrude into a patent processes virginals testis through the inguinal canal IT IS THE LEADING CAUSE OF INTESTINAL OBSTRUCTION IN INFANTS *Herniorraphies are the most common surgical procedures performed in children • Two types • Indirect ( sac passes through the internal ring lateral to the inferior epigastric vessels ) • Direct ( sac enters the canal through the posterior wall medial to the inferior epigastric vessels )

28. GI EMERGENCIES • KEY POINTS : • Abdominal pain • Clinical history • Chronology – time of onset • Location – epigastric, peri-umbilical, or hypogastric • Intensity and characterization – type of pain : sharp dull, crampy • PE • LABS/RADIOLOGY

29. MECKEL’S DIVERTICULUM Remnant of the embryonic proximal portion of the yolk stalk ( omphalomesenteric or vitelline duct in utero ) It usually disappears 5 the and 7th week of life It is located in the antimesenteric aspect of the ileum It is 40 to 100 cm from the ileocecal valve THE MOST COMMON congenital GI ANOMALY THE MOST COMMON CLINICAL PRESENTATION IS LOWER GASTROINTESTINAL HEMORRHAGE – “ currant jelly like “(painless ) Dx : Meckel’s (technetium 99m pertechnetate) scan Sensitivity can be increased by administration of cimetidine, glucagon or pentagastrin +/- angiography +/- Tagged RBC

32. GI EMERGENCIES • Hemorrhage KEY POINTS : • Bleeding from the gastrointestinal tract may be determined • by : Magnitude ( acute vs. chronic ) Location of the bleeding site ( upper vs. lower ) Pathogenesis mechanisms ( inflammatory vs. vascular) Mode of presentation (hematemesis vs. hematochezia) 2. Management in GI Hemorrhage FOUR SUCCESSIVE STEPS APPROACH • Assessment of the severity of the bleed • ( HR, BP, and postural changes ) • Resuscitation and Empiric Therapy • Locate the bleed • Diagnostic tests BE ABLE TO DIFFERENTIATE BETWEEN UPPER AND LOWER BLEEDS DETERMINE IF PAINLESS VS. PAINFUL

33. GI EMERGENCIES • A 28 day old infant has a history of jaundice in the first of life that resolved but has icteric sclera of recent onset. Growth and development are normal. On examination, the liver was enlarged ( span by percussion is 8 cm) but the spleen is nonpalpable. On rectal examination, soft pale yellow stool ( quaiac negative ) was obtained. • Based on your working diagnosis, what test and / or treatment would you order ?

34. GI EMERGENCIES Cholestasis – impairment of bile flow Causes – intrahepatic vs. extrahepatic Biliary atresia Choledocal cyst Alagille ‘s Neonatal hepatitis Metabolic causes

35. BILIARY ATRESIA INCIDENCE 1 IN 10,000 OR 25,000 FEMALE TO MALE RATIO IS 1.4:1 PRESENTATION : JAUNDICE ( CONJUGATED/DIRECT HYPERBILIRUBINEMIA) ONSET 2 TO 3 MONTHS OF AGE ) ASSOCIATED ANOMALIES : ABSENT INFERIOR VENA CAVA, PREDUODENAL PORTAL VEIN, INTESTINAL MALROTATION, POLYSPENIA • Surgical intervention success is highly weighted on the time of surgery ( greater success performed < 3months of age )

36. BILIARY SYSTEM

37. GI EMERGENCIES • You are asked to see a patient in the ER for elevated liver enzymes of unclear etiology. What tests/labs should be ordered to determine liver function? • The clotting function tests were prolonged. What should be done next? What is the time above the normal PT level that is suggestive of the risk for bleeding. • If mother describes her child as becoming more restless with wide mood changes, and confusion, what would the above suggest in regard to his present clinical status?

38. GI EMERGENCIES • Hepatic failure is defined as impairment/ loss of the liver to perform vital ( synthetic and biliary ) functions. Failure may be classified as acute, subacute /late – onset failure. It is time frame from the onset of hepatic failure to encephalopathy development • Fulminant hepatic failure: < 8 weeks after the beginning of acute hepatitis • Subacute hepatic failure : encephalopathy develops 8 to 24 weeks after the onset of liver disease *Note : 1. The lack of preexisting liver disease is KEY to making this diagnosis. 2. This strict definition may not be as applicable to all pediatrics disorders –Reye’s syndrome, several inborn errors and Wilson’s disease.

39. COAGULATION • The liver has a role in the control of coagulation Almost exclusively all coagulation factors are produced in the liver except von Willbrand factor The liver produces and breakdown factors integral to fibrinolysis It clears activated clotting factors from the circulation • Vitamin K dependant factors : Factors ll, Vll, lX, and X Note : Vitamin K ( fat soluble vitamin) is stored in the liver in limited amounts Factor Vlll is made extensively in nonhepatic sites ( vascular endothelium)

40. COAGULATION • Clotting study PT – measures time to take factor ll, prothrombin, to be converted to thrombin then fibrinogen into fibrin. Factors activated are V, Vll, and X. This is the extrinsic pathway of coagulation. Normal 11.5 to 12.5 Prolongation > 2 seconds is pathologic Prolongation > 3 seconds is associated with risk for bleeding PTT – also measures the generation of thrombin. This is the intrinsic pathway of coagulation. All factors are involved except Factor Vll. Since all factors are exclusively made by the liver except Factor Vll, the clotting study can reasonably assess the live synthetic function. HOWEVER, Vitamin K deficiency must be ruled out as the cause for the prolonged coagulation. Give 1mg/year of age slowly IM OR IV with a minimum of 1 mg given to full term infant Can check PT 4 TO 6 HOURS AFTER Vit K administration. Other prognostic tests : Factor Vll levels > 8% Be cautious with interpreting prolonged PT or PTT ----? DIC ? ( Factor Vll level may be helpful, normal in liver disease but decreased in DIC )

41. GI EMERGENCIES HEPATIC ENCEPHALOPATHY (neuropsychiatric/ brain dysfunction resulting from acute hepatic dysfunction )

43. GI EMERGENCIES A term infant fails to pass meconium . If the exam is unremarkable , what is the possible etiology and evaluation to perform ?

44. THICK MECONIUM SYNDROMES Two types Meconium ileus • Simple • Complicated Meconium plug syndrome Presentation : Simple 24 – 48 hours after birth Complicated - less than 24 hours of age Note : initial presentation for CF Dx : Contrast study of the colon Rx : contrast study of the colon Sugery for the complicated Note : polyhydraminious especially complicated “ soap bubbles” ( Neuhauser sign )

46. HIRSCHSPRUNG’S DISEASE • * Most common cause of lower intestinal obstruction in the neonate • Incidence is 1 in 5,000 • Male to female - 3.8:1 ( 2.2:1 for total aganglionosis) • Rectum and sigmond colon are the most common sites • Positive family history – 3 to 7 % • Etiology ? Ret proto-oncogene mapped to chromosome 10q11,2 ( gene has a critical role in oncgenesis and in the development of mammalian enteric neuron system • MEN 11a and MEN 11b syndrome Associated anomalies : Down’s syndrome, Waardenburg’s syndrome, cerebral palsy, mental retardation or developmental delay, meconium plug syndrome, MEN 11a, MEN11b Presentation : failure to pass meconium < 24-48 hours, or infrequent defecation from birth Diff Dx : meconium plug syndrome, microcolon, hypothyroidism, drugs that decrease intestinal transit, sepsis, neuronal intestinal dysplasia, functional constipation, anterior anus displacement, pseudo-obstruction Dx : • BE without prep- transition zone * negative after daily enemas in infants less than 2 weeks old and presence of total aganglionosis • Rectal suction biopsy (RSB) or full thickness • Anorectal manometry (ARM) RX : medical and surgery

50. CLINICAL STAGES OF HEPATIC ENCEPHALOPATHY Stage 1 ( Prodrome ) altered mental alertness 2 ( Impending coma) drowsiness, altered sleep pattern, confusion, mood swing, inappropriate behavior 3 (Stupor) sleepy but arousable, unresponsive to verbal commands , hyperreflexia 4( Coma) unconscious, decerebrate, decorticate • Response to pain present (4A) or absent (4B) • Electroencephalographic Asterixis /changes 1 slight/minimal 2 easily elicited/usually generalized slowing of rhythm 3 present if patient cooperates / grossly abnormal slowing 4 usually absent/appearance of delta waves, decreased amplitude