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  2. 2. GASTROINTESTINAL EMERGENCIES<br />Gastrointestinal emergencies are serious conditions that often develop suddenly and requires prompt and immediate treatment.<br />Gastrointestinal emergencies can develop throughout the digestive system<br />
  3. 3. GI TRACT EMERGENCIES<br />Esophagus<br />Stomach<br />Small Intestines<br />Large intestines<br />Biliary system<br /> ( liver, pancreas, gallbladder, bile duct )<br />
  4. 4. GASTROINTESTINAL EMERGENCIES IN Adults and Infants/Children<br /> Acute Abdominal Pain<br /> Gastrointestinal Hemorrhage<br /> Biliary Tract Obstruction<br /> Hepatic Failure<br />Hepatic Encephalopathy <br />
  5. 5. GI TRACT EMERGENCIES UNIQUE TO THE PEDIATRIC PATIENT<br />Two major categories<br />Intestinal obstruction<br />Neonatal cholestasis<br />
  6. 6. INTESTINAL OBSTRUCTION<br />Antral and Duodenal stenosis and webs<br />Infantile hypertrophic pyloric stenosis<br />Extrinsic Duodenal obstruction<br />Duplications<br />Malrotation with and without volvulus<br />Hirschsprung’s disease<br />Anorectal malformations or Imperforate anus<br />Abdominal Wall Defect<br />Hernias<br />Thick Meconium syndromes<br />Intussception<br />Volvulus<br />Other<br />-Appendicitis<br />- Meckel’s Diverticulum<br />
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  8. 8. GI EMERGENCIES<br />1. 15 year old male presents with severe abdominal pain, and vomiting. <br />There is no history of fever, weight loss , diarrhea , jaundice or joint pain. <br /> Based on above information, what additional history , <br />physical finding ,labs and/or radio imaging are needed to <br />make a diagnosis of :<br /><ul><li>Pancreatitis
  9. 9. Appendicitis
  10. 10. Malrotationwith volvulus
  11. 11. Incarcerated hernia
  12. 12. Intussception </li></li></ul><li>APPENDICITIS<br />Most common condition leading to emergency abdominal surgery in children/adults<br />Incidence : 140 in 100,000 people of all ages<br />Most frequent between 15 and 24 years of age ( rare in preschool children and rarer in < 1 year of age<br />Presentation : periumbilical abdominal pain, vomiting, fever<br />1/3 of all presentation is with perforation of the appendix or lower abdominal mass<br />Note : 11 – 32 % false positive rate pre operatively<br />> 50 % have nonsurgical abdominal <br />
  13. 13. GI EMERGENCIES<br />Pancreatitis<br />Diagnosis:<br /> Key Points<br />Symptoms :( age dependant )<br /> + pancreatic isoenzymes <br />( amylase and / or lipase) 2 to 3 times <br />elevated over normal reference level.<br />Hydration very important especially in the early stages.<br />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<br />Rx : NPO, IV FLUIDS, PAIN MEDS <br />
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  15. 15. GI EMERGENCIES<br />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).<br />Based on your working diagnosis, what labs or test would you request?<br />
  16. 16. INTUSSCEPTION<br />COMMON CAUSE OF INTESTINAL OBSTRUCTION IN CHILDREN<br />Peak incidence is infancy<br /><ul><li>50% < 1year of age
  17. 17. Male to female ratio is 3:2
  18. 18. No lead point/ usually idiopathatic
  19. 19. 90 % proximal to the ileocecal valve –ileocolonic, ileoileal,colocolonic
  20. 20. > 2 years of age associated with a lead point</li></ul>Dx/Rx : KUB features the target sign ( a mass in the RUQ ), cresent sign<br /><ul><li>( 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)
  21. 21. air or barium contrast enema</li></ul>Surgery if lead point or > 48 hours<br /><ul><li>10% recurrence</li></ul>Complication – perforation ( greater in infants, < 6 months of age, and /or symptoms >3 days <br /><ul><li>9</li></li></ul><li>INTUSSCEPTION<br />
  22. 22. MALROTATION WITH OR WITHOUT VOLVULUS<br />Malrotation occurs during normal <br />formation of the bowel<br />Its presence predisposes to volvulus<br />Its incidence is 1 in 6,000<br />Male to female 3:1<br />Presentation : bilious vomiting, abdominal pain,<br />blood in stool etc., but older children may have vague<br /> symptoms ( chronic abdominal pain and/or intermittent vomiting)<br />Diff Dx : <br />neonates – duodenal atresia and stenosis, annular pancreas, antral or duodenal web, small bowel atresia<br />Older infant/children – pyloric stenosis, GER, formula intolerance, colic, lactose intolerance<br />Associated anomalies : congenital diaphragmatic hernia, abdominal wall defect (30-60%), duodenal atresia (50%), CHD, Hirschsprung’s disease, mesenteric cysts<br />Dx: Barium enema – cecum localized to the right or left quadrant<br />UGI series – duodenum does not cross midline and the remainder of small bowel lies to the right of the midline<br /> U/S – clockwise rotation of the superior mesenteric vein around the mesenteric artery <br />( “ whirlpool” sign )<br />Rx : surgery<br />
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  24. 24. INFANTILE HYPERTROPHIC PYLORIC STENOSIS<br />Hyper trophy and hyperplasia of the smooth muscle in the antral and pyloric regions<br />Proposed etiology - ? Hypergastrinemia, ?increased prostaglandins E2 and F2alpha, ? Decreased NADPH diaphorase activity ( a lack of nitric oxide synthase in pyloric tissue<br />Sx : nonbilious vomiting < 5 months ( usually by 3 weeks )<br />PE : visible gastric peristalic wave<br />Hypochloremic alkalosis <br />( water loss and hydrochloric acid )<br />Elevated unconjugated bilirubin<br />Dx : pathognomomic “ pyloric olive” <br />+ clinical history + labs<br />No imaging is required<br />Plain film “ caterpillar “shape ( hyperperistalsis and paucity of small and large bowel gas )<br />Radio imaging with contrast – elongated , double channel ( railroad tract )-enlarged distal antrum muscle and duodenal bulb<br />U/S : hypertrophied pyloric muscle ( anechoic mass )<br />Rx : medical/surgery<br />
  25. 25. DUPLICATIONS<br />Esophageal – rare<br />Gastric – incidence 10%<br />-greater curvature<br />-palpable abdominal mass <br />+/- GI bleeding<br />+/- chronic abdominal pain<br />+/- pancreatitis<br />Duodenal – incidence 10-15 %<br />-located in the mesenteric border<br />Presentation – abdominal mass , abdominal pain, jaundice ,GI bleeding , pancreatitis<br />Associated anomalies : Duplication cysts, vertebral anomalies, ectopic pancreas<br />Dx : UGI with contrast –beaklike projection from the duodenal<br /><ul><li>CT scan or U/S – mass next to the duodenum
  26. 26. Endoscopy – mass or extrinsic compression
  27. 27. ERCP – duplication seen if the biliary or pancreatic duct is involved
  28. 28. Rx : surgery</li></li></ul><li>EXTRINSIC DUODENAL OBSTRUCTION<br />Two most common causes<br />Annular pancreas<br />Congenital bands<br />Associated anomalies : duodenal atresia or stenosis, cardiovascular defect, malrotation, Down’s , TEF<br />Dx : plain film – double bubble<br />
  29. 29. GI EMERGENCIES<br />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.<br />What two things performed that would aid in making a diagnosis?<br />What was your differential diagnosis ?<br />Give at least 3 associated anomalies .<br />
  30. 30. INTESTINAL OBSTRUCTION<br /> Atresia – congenital absence or obstruction of the intestinal lumen<br />Types<br />Esophageal - incidence 1 in 3000/4500<br />Duodenal - incidence 1 in 2000/40,00<br />Jejunal and ileal -incidence 1 in 330/1500<br /> Colonic - incidence 1 in 20,000<br />
  31. 31. JEJUNAL AND ILEAL ATRESIA<br />It is 30% of all neonatal intestinal obstruction<br />Male = female <br />30 % proximal jejunum<br />70 % distal jejunum<br />10 – 15 % proximal ileum<br />Presentation – less than 24 hours of age – bilious vomiting, progressive abdominal distension ( ileal > jejunum ), failure to pass meconium<br />Associated anomalies common – gastroschisis, meconium ileus, malrotation with and without volvulus, Hirschsprung’ disease<br />*Microcolon is seen with all ileal atresia<br />Diff Dx : colonic atresia, Hirschsprung’s disease, meconium plug, meconium ileus, malrotation with volvulus, and bowel duplication<br />Dx : Plain film –multiple dilated loops of bowel with air – fluid levels<br />Rx : medical / surgery<br />
  32. 32. DUODENAL ATRESIA<br />Presentation : symptoms within hours of birth, <br />abdominal distension, > 30 ml bilious gastric fluid<br />Nonbilious if obstruction is proximal to <br />the ampulla of vater<br />Diff Dx : webs, stenosis, duplication, <br /><ul><li>annular pancreas, congenital bands</li></ul>Associated anomalies are common : <br /><ul><li>esophageal atresia, malrotation,
  33. 33. annular pancreas, imperforate anus,
  34. 34. CHD, IUGR</li></ul>Dx : plain film – “double bubble” sign<br />UGI with contrast ---confirms diagnosis<br />BE with contrast –determine the presence<br /> of malrotation and/or midgut volvulus<br />Rx : medical/surgery<br />** 30 % Down’s syndrome<br />
  35. 35. ESOPHAGEAL ATRESIA<br />Five types<br />1. Proximal atresia with distal fistula ( 85 % )<br />2. Proximal and distal blind pouch and no fistula (8 to 10 %) <br />3. H type , T E fistula and no atresia ( 3 to 4 % )<br />4 and 5. variation of proximal fistula, etc.<br />Presentation depends on the type<br />Dx : Inability to pass NG tube into the stomach<br />Plain film ----coiled NG tube<br />Contrast study only to diagnose H type<br />Diff Dx : choanal atresia, diaphragmatic hernia, <br />CHD<br />Associated anomalies : esophageal atresia (40-70%),<br /> cardiovascular(40%) VSD, TOF, COA,PDA,vascular rings<br />10% VACTERL ( vertebral defects, anal atresia, TEF,<br /> renal defect, radial limb dysplasia )<br />10 – 15 % imperforated anus<br />GI anomalies – malrotation, duodenal atresia <br />
  36. 36. HERNIAS<br />Location is along intestines <br /> diaphragm<br /> umbilical<br /> inguinal<br />internal – rare/ seen at autopsy ( most common type mesocolon /paraduodenum )<br />
  37. 37. INGUINAL HERNIA<br />Its incidence is 0.88 to 4.44 in children ( it is higher in premature infants )<br />Male to female ratio is 6:1<br />Right sided occurrence is 60 %<br />Left sided occurrence is 30 %<br />Bilateral occurrence is 10 %<br />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<br />Presentation : bulge in the groin, scrotum, testicles<br />Dx : clinical<br />Transillumination,<br />Ultrasound<br />Diff Dx : Testicle torsion, epididymis-orchitis, torsion of the appendix testis, hydrocele, inguinal or femoral lymph adenitis<br />Rx : surgery<br />
  38. 38. INGUINAL HERNIA<br />It’s a sac containing abdominal contents and protrude into a patent processes virginals testis through the inguinal canal<br />IT IS THE LEADING CAUSE OF INTESTINAL OBSTRUCTION IN INFANTS<br />*Herniorraphies are the most <br />common surgical procedures <br />performed in children<br />Two types<br /><ul><li>Indirect ( sac passes through </li></ul>the internal ring lateral to <br />the inferior epigastric vessels )<br /><ul><li>Direct ( sac enters the canal</li></ul> through the posterior wall <br />medial to the inferior epigastric vessels )<br />
  39. 39. GI EMERGENCIES<br />KEY POINTS :<br />Abdominal pain<br />Clinical history <br />Chronology – time of onset<br />Location – epigastric, peri-umbilical, or hypogastric<br />Intensity and characterization – type of pain : sharp dull, crampy<br />PE<br />LABS/RADIOLOGY<br />
  40. 40. MECKEL’S DIVERTICULUM<br />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<br />It is located in the antimesenteric aspect of the ileum<br />It is 40 to 100 cm from the ileocecal valve<br />THE MOST COMMON congenital GI ANOMALY<br />THE MOST COMMON CLINICAL PRESENTATION IS LOWER GASTROINTESTINAL HEMORRHAGE <br />– “ currant jelly like “(painless )<br />Dx : Meckel’s (technetium 99m pertechnetate) scan <br />Sensitivity can be increased by administration of cimetidine, glucagon or pentagastrin <br />+/- angiography<br />+/- Tagged RBC <br />
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  42. 42.
  43. 43. GI EMERGENCIES<br />Hemorrhage<br />KEY POINTS :<br />Bleeding from the gastrointestinal tract may be determined <br />by : <br />Magnitude ( acute vs. chronic )<br />Location of the bleeding site ( upper vs. lower )<br />Pathogenesis mechanisms ( inflammatory vs. vascular)<br />Mode of presentation (hematemesis vs. hematochezia)<br />2. Management in GI Hemorrhage<br />FOUR SUCCESSIVE STEPS APPROACH<br /><ul><li>Assessment of the severity of the bleed
  44. 44. ( HR, BP, and postural changes )
  45. 45. Resuscitation and Empiric Therapy
  46. 46. Locate the bleed
  48. 48. GI EMERGENCIES<br />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.<br />Based on your working diagnosis, what test and / or treatment would you order ?<br />
  49. 49. GI EMERGENCIES<br />Cholestasis – impairment of bile flow<br />Causes – intrahepatic vs. extrahepatic <br />Biliary atresia<br />Choledocal cyst<br />Alagille ‘s<br />Neonatal hepatitis<br />Metabolic causes<br />
  50. 50. BILIARY ATRESIA<br />INCIDENCE 1 IN 10,000 OR 25,000<br />FEMALE TO MALE RATIO IS 1.4:1<br />PRESENTATION : JAUNDICE<br />( CONJUGATED/DIRECT HYPERBILIRUBINEMIA) ONSET 2 TO 3 MONTHS OF AGE )<br />ASSOCIATED ANOMALIES : ABSENT INFERIOR VENA CAVA, PREDUODENAL PORTAL VEIN, INTESTINAL MALROTATION, POLYSPENIA<br /><ul><li>Surgical intervention success is highly weighted on the time of surgery</li></ul> ( greater success performed < 3months of age )<br />
  51. 51. BILIARY SYSTEM<br />
  52. 52. GI EMERGENCIES<br />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?<br />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.<br />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?<br />
  53. 53. GI EMERGENCIES<br />Hepatic failure is defined as impairment/ loss of the liver to perform vital ( synthetic and biliary ) functions.<br />Failure may be classified as acute, subacute /late – onset failure. It is time frame from the onset of hepatic failure to encephalopathy development<br /><ul><li>Fulminant hepatic failure: < 8 weeks after the beginning of acute hepatitis
  54. 54. Subacute hepatic failure : encephalopathy develops 8 to 24 weeks after the onset of liver disease</li></ul>*Note : 1. The lack of preexisting liver disease is KEY to making this diagnosis.<br />2. This strict definition may not be as applicable to all pediatrics disorders –Reye’s syndrome, several inborn errors and Wilson’s disease.<br />
  55. 55. COAGULATION<br /> The liver has a role in the control of coagulation<br />Almost exclusively all coagulation factors are produced in the liver except von Willbrand factor<br /> The liver produces and breakdown factors integral to fibrinolysis<br />It clears activated clotting factors from the circulation<br />Vitamin K dependant factors : Factors ll, Vll, lX, and X <br />Note : Vitamin K ( fat soluble vitamin) is stored in the liver in limited amounts<br />Factor Vlll is made extensively in nonhepatic sites<br /> ( vascular endothelium)<br />
  56. 56. COAGULATION<br />Clotting study<br />PT – measures time to take factor ll, prothrombin, to be converted to thrombin then fibrinogen into fibrin.<br />Factors activated are V, Vll, and X. This is the extrinsic pathway of coagulation. Normal 11.5 to 12.5<br />Prolongation > 2 seconds is pathologic <br />Prolongation > 3 seconds is associated with risk for bleeding<br />PTT – also measures the generation of thrombin. This is the intrinsic pathway of coagulation.<br />All factors are involved except Factor Vll.<br />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.<br />Give 1mg/year of age slowly IM OR IV with a minimum of 1 mg given to full term infant<br />Can check PT 4 TO 6 HOURS AFTER Vit K administration.<br />Other prognostic tests : Factor Vll levels > 8%<br />Be cautious with interpreting prolonged PT or PTT ----? DIC ? ( Factor Vll level may be helpful, normal in liver disease but decreased in DIC ) <br />
  57. 57. GI EMERGENCIES<br /> HEPATIC ENCEPHALOPATHY <br />(neuropsychiatric/ brain dysfunction resulting from acute hepatic dysfunction ) <br />
  58. 58.
  59. 59. GI EMERGENCIES<br />A term infant fails to pass meconium . If the exam is unremarkable , what is the possible etiology and evaluation to perform ?<br />
  60. 60. THICK MECONIUM SYNDROMES<br />Two types<br />Meconium ileus<br /><ul><li>Simple
  61. 61. Complicated</li></ul>Meconium plug syndrome<br />Presentation : <br />Simple 24 – 48 hours after birth<br />Complicated - less than 24 hours of age<br />Note : initial presentation for CF<br />Dx :<br />Contrast study of the colon<br />Rx : contrast study of the colon<br />Sugery for the complicated<br />Note : polyhydraminious especially complicated<br />“ soap bubbles” ( Neuhauser sign )<br />
  62. 62.
  63. 63. HIRSCHSPRUNG’S DISEASE<br />* Most common cause of lower intestinal obstruction<br />in the neonate<br />Incidence is 1 in 5,000<br />Male to female - 3.8:1 ( 2.2:1 for total aganglionosis)<br />Rectum and sigmond colon are the most common sites<br />Positive family history – 3 to 7 %<br />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<br /><ul><li>MEN 11a and MEN 11b syndrome</li></ul>Associated anomalies : Down’s syndrome, Waardenburg’s syndrome, cerebral palsy, mental retardation or developmental delay, meconium plug syndrome, MEN 11a, MEN11b<br />Presentation : failure to pass meconium < 24-48 hours, or infrequent defecation from birth<br />Diff Dx : meconium plug syndrome, microcolon, hypothyroidism, drugs that decrease intestinal transit, sepsis, neuronal intestinal dysplasia, functional constipation, anterior anus displacement, pseudo-obstruction<br />Dx :<br /><ul><li> BE without prep- transition zone * negative after daily enemas in infants less than 2 weeks old and presence of total aganglionosis
  64. 64. Rectal suction biopsy (RSB) or full thickness
  65. 65. Anorectal manometry (ARM)</li></ul>RX : medical and surgery <br />
  66. 66.
  67. 67.
  68. 68.
  69. 69. CLINICAL STAGES OF HEPATIC ENCEPHALOPATHY<br />Stage <br />1 ( Prodrome ) altered mental alertness<br />2 ( Impending coma) drowsiness, altered sleep pattern, confusion, mood swing, inappropriate behavior<br />3 (Stupor) sleepy but arousable, unresponsive to verbal commands , hyperreflexia<br />4( Coma) unconscious, decerebrate, decorticate<br />Response to pain present (4A) or absent (4B)<br />Electroencephalographic<br />Asterixis /changes<br />1 slight/minimal<br />2 easily elicited/usually generalized slowing of rhythm<br />3 present if patient cooperates / grossly abnormal slowing<br />4 usually absent/appearance of delta waves, decreased amplitude<br />
  70. 70. ABDOMINAL WALL DEFECTGastroschisis<br />Its incidence is 1 in 12,00<br />Its greater in prematurity or LBW INFANTS<br />It results from mechanical insult ( early tear of the cord before the ring is complete. The abdominal wall is well developed<br />It is associated with lower mortality than omphalocele as well as congenital anomalies<br />NOTE : There is no change in mortality or morbidity with vaginal vs. C section. Also, there is no advantage to early delivery unless volvulus is highly suspected<br />Dx : prenatal ultrasound <br />Increased alpha fetoprotein (100 % )<br />Rx : medical/surgery<br />
  71. 71. OMPHALOCELE<br />Its incidence is 1 in 10,00<br />Associated anomalies are determined by the site of fusion failure ---cephalic, lateral, or cardiac<br />Pentalogy of Cantrell – combination of distal sternal, an omphalocele, an anterior diaphragmatic defect, and pericardial and intracardiac defect<br />Associated anomalies : cardiovascular, chromosomal abnormalities (trisomy D and E ), Beckwith- Wiedman syndrome, gigantism<br />Dx : prenatal ultrasound<br />Increased alpha fetoprotein <br />
  72. 72. UMBILICAL<br />Its an out pouching of the intestine through a defect of the fascia at the umbilical ring<br />Common in newborns<br />Five to ten times more common in Afro-American infants<br />It’s a cosmetic problem<br />
  73. 73. ANORECTAL MALFORMATION OR IMPERFORATION<br />This results from abnormal development of the urorectal septum leading to an absent or abnormal anus<br />Classification based on relationship of the rectal pouch to the pelvic musculature<br />-high : rectal pouch lies above the levator muscles (male > female)<br /><ul><li>Intermediate : rectum partially passes the levator muscles
  74. 74. - Low : rectum tranverses the levator muscles completely ( male = female )</li></ul>Associated anomalies is common (50-70%)<br />-genitourinary tract, skeletal, CNS, CVS, GI tract and esophageal atresia<br />Dx : clinical/PE<br />MRI AND / OR CT SCAN TO DISTINGUISH BETWEEN HIGH AND LOW<br />
  75. 75. GI TRACT EMERGENCIES : Intestinal Obstruction<br />Give an example of intestinal obstruction emergency include clinical presentation, differential diagnosis, diagnostic approach, associated anomalies and treatment approach.<br />What is the most common cause of intestinal obstruction in the infant ? <br />What is the most common cause in children?<br />
  76. 76. GI TRACT EMERGENCIES<br />What are the GI tract emergencies that are seen in both infants/children and adults ?<br />What are the two categories that are seen in infants and children because of its unique presentation and/or more frequently seen in early in life ?<br />