Clinical Pathological Conference 2004-12-29

1. Clinical Pathological Conference2004-12-29 三軍總醫院 小兒科部 劉家宏/華一鳴

2. Case PresentationPresent Illness • This one-year-six-month-old boy came to our pediatric emergency department with the chief compliant of bilious vomiting and intermittent irritable crying since 4 hours ago.

3. Case PresentationPersonal and Family History • Past history: Before this presentation,the child was in good condition without any compliant of abdominal symptoms.

4. Case PresentationPhysical Examination • Vital sign: PR: ↑ 136 /min (70-110), RR: 20 /min(20-30), BT: 35.7 ºC BP: ↑ 112/70(90-105,50-70), decreased urine output. • HEENT: dehydration with dry lip. • Abdomen : soft, neither sign of peritonitis nor hepatosplenomegaly .

5. Case PresentationRadiologic & Lab Findings • Plain film of abdomen : showed a nonspecific local ileus pattern over right upper quadrant. • Laboratory data: white blood count:↑ 25300/nl (6000-17500/nl) with 75% segment(54-62%) and 18% lymphocyte(25-33%).

6. Case PresentationRadiologic & Lab Findings • Serum biochemistry: Sodium :145 (139-146)mEq/L Potassium :4.5 (3.5-5.0)mEq/L Chloride : ↑130 (98-106)mEq/L BUN :19 mg/dl, Creatinene :0.4(0.2-0.4)mg/dl C reative protein level :0.1mg/dl.

7. Case PresentationRadiologic & Lab Findings • Abdomen sonography :revealed a soft tissue mass over right lower quadrant, but no evidence of sign of target appearance; besides, the relationship of superior mesentery artery and superior mesentery vein was in right position.

8. Case PresentationHospital Course-I • Under the impression of intestinal obstruction, he was admitted to our pediatric department for further evaluation and management. • After admission, this patient had persistent bilious vomiting.

9. Case PresentationHospital Course-II • Twelve hours later, heart rate increased to 150-180 per minutes. • Meanwhile, decreased urine output and downhilled blood pressure happened to him despite of intravenous fluid supplement. • Under the impression of intestinal obstruction complicated with shock, our pediatric surgeon arranged emergency laparotomy.

10. Case PresentationHospital Course-II • Post-operative course was relative smooth,and started feeding on the4th day of lapatotomy smoothly. • His condition was stable during the follow-up period at our out patient clinics.

11. Bilious vomiting A soft tissue mass over right lower quadrant Downhilled blood pressure(shock) Intermittent irritable crying Decreased urine output Leukocytosis Hyperchloremia Local ileus pattern over right upper quadrant Tachycardia Hypertension Major Problems Minor Problems

12. Questions –about past, personal, family history • Birth history? • History of trauma? Child abuse? • Operation history? foreign body aspiration? • Drug history? Food history? • Family history about tumor? About cystic fibrosis?

13. Questions- about Physical Examination and laboratory data • Anemic conjunctiva? Lymphoadenopathy? • RLQ soft tissue mass movable or non-movable? • Bowel sound? Hyperactive to be replaced with hypoactive bowel sounds? • Stool rontine examination? occult blood? WBC? pattern? • Blood smear? blast cell?

14. Questions-about radiologic finding • Plain film of abdomen :further finding? Foreign body/Bezoars? • Abdomen Sonography: further finding? Appendix? Ascites?Kidney? Any finding about tumor?

15. Bilious vomiting • Vomitus or nasogastric aspirate containing bile which in children almost always indicates bowel obstruction distal to the sphincter of Oddi. • By contrast, infants with pyloric stenosis have non-bile-stained vomiting.

16. Intestinal obstruction • Intraluminal :polyp, mass, parasites, and tumor. • Intramural :stricture, tumor, hematoma. • Extrinsic:postoperative adhesion, adhesion from peritonitis, hernia, volvulus,and tumor.

17. Clinical Classification of Shock • Septic shock :bacterial,Viral,Fugal.. • Cardiogenic shock : ischemia, cardiomyopathy ,congestive heart failure • Distributive shock:toxins,anaphylaxis… • Hypovolemic shock :enteritis,hemorrhage, • Obstructive shock :tension pneumothroax

18. Hyperchloremia • Pathophysiology Metabolic Acidosis with a normal Anion Gap • Causes • Artifact (low Anion Gap) • Metabolic and Endocrine • Hyperparathyroidism, Renal Tubular Acidosis, Hypernatremia. • Bromide intoxication • Nervine, Sominex • Acetazolamide • Carbonic anhydrase inhibition • Boric acid , Triamterene ,Ammonium Chloride • Excess IV Normal Saline

19. Hyperchloremia • Gastrointestinal • Dehydration • Prolonged Diarrhea • Loss of pancreatic secretion • Ileal loops • Ureteral colonic anastomosis

20. (From: Pediatric Decision-making Strategies accompanied by Nelson) Malrotation with volvulus Appendicitis Other causes of intestinal obstrution Congenital structural abnormalities Postsurgical adhesions Foreign body/Bezoars Meckels diverticulum Incarcerated inguinal hernia Meconium ileus Intussusception Hirschsprungs disease Superior mesenteric artery syndrome Duodenal hematoma Testicular or ovarian torsion Surgery Consult Yes Signs or symptoms suggestive of an acute abdomen Acute History and physical exam Vomiting Sign or symptoms suggestive of increased ICP? Yes No No Sign or symptoms suggestive of increased ICP Chronic

21. Malrotation with volvulus Appendicitis Congenital structural abnormalities Postsurgical adhesions Foreign body/Bezoars Meckels diverticulum with bleeding Incarcerated inguinal hernia Meconium ileus Intussusception Hirschsprungs disease Superior mesenteric artery syndrome Duodenal hematoma Testicular or ovarian torsion

22. (From: Pediatric Decision-making Strategies accompanied by Nelson) Abdomen US Abnormal result Yes Yes Perform abdomen US Neonate? No Lower abdomen mass in female History and physical exam No Normal result Abdomen mass Hepatomegaly or splenomegaly present No Perform Abdomen CT Yes

23. Not neonate , Not female with lower abdomen mass Splenomegaly Wilms tumor Adrenal cortical neoplasms Pancreatic masses/cysts Neuroblastoma Hydronephrosis Rhabdomyosarcoma Urinary retention Hepatic lesion Teratoma Bezoar Appendiceal abscess Intestinal tumor Mesenteric cyst Omental cyst Lymphangioma Lymphoma Choledochal cyst Constipation Inflammatory bowel disease Retroperitoneal hematoma

24. Foreign body/Bezoars Meckels diverticulum Hirschsprungs disease Appendicitis Intestinal tumor Neuroblastoma Rhabdomyosarcoma Lymphoma Congenital structural abnormalities

25. Foreign body/Bezoars (bezôr) • An accumulation of exogenous matter in the stomach or intestine. • peak incidence between the ages of 6 mo and 3 yr • 90% of foreign bodies are opaque. • vomiting, anorexia, and weight loss. • An abdominal plain film may suggest the presence of a bezoar, which can be confirmed on ultrasound or CT examination.

26. Gastric trichobezoarPediatric Emergency Care. 19(5):343-7, 2003 Oct. • On plain abdominal radiographs, the bezoar will appear as a mottled heterogenous mass that may be mistaken for a food-filled stomach. • The classic sonographic appearance is described as a band of increased echogenicity in the region of the stomach with complete loss of posterior echoes.

27. Small bowel obstruction and covered perforation in childhood caused by bizarre bezoars and foreign bodies. • Small bowel obstruction with perforation is an unusual and rare complication of bezoars. Israel Medical Association Journal: Imaj. 2(2):129-31, 2000 Feb.

28. Hirschsprungs disease • Abnormal innervation of the bowel . • Most common cause of lower intestinal obstruction in neonates . • Usually begin at birth with the delayed passage of meconium. • Some infants pass meconium normally but subsequently present with a history of chronic constipation.

29. Hirschsprungs disease • Failure to thrive, with hypoproteinemia from a protein-losing enteropathy, is a less common presentation. • Rectal examination demonstrates normal anal tone and is usually followed by an explosive discharge of foul-smelling feces and gas.

30. Hirschsprungs disease • Rectal manometry and rectal suction biopsy are the easiest and most reliable indicators of Hirschsprung disease. • Barium enema examination is useful in determining the extent of aganglionosis. • Sonography may also help in determining the dynamic or adynamic state of fluid-filled or solid-filled bowel loops.

31. Meckels diverticulum • Remnant of the embryonic yolk sac Arise within the 1st 2 yr of life . • Intermittentpainless rectal bleeding by ulcerationof the adjacent normal ileal mucosa.

32. Meckels diverticulum • Brick colored or currant jelly colored. • Obstructionoccurs when the diverticulum acts as the lead point of an intussusception. • A Meckel diverticulum may occasionally become inflamed (diverticulitis) and present similarly to acute appendicitis. • The most sensitive study is a Meckel radionuclide scan

33. Meckel's diverticulum.Internal hernia and adhesions without gastrointestinal bleeding--ultrasound and scintigraphic findings. • US study was particularly helpful in this case because it shows a nonperistaltic region, which is consistent with a diverticulum or an internal hernia. Clinical Nuclear Medicine. 21(12):938-40, 1996 Dec.

34. Meckel's diverticulum mimicking infantile colic: sonographic detection. • Abdominal sonography at 6 months of age demonstrated an abdominal mass with an anechoic center and a double-layered wall, surrounded by bowel loops. • Histologic examination of the resected mass revealed a Meckel's diverticulum with a perforation sealed off by the neighboring bowel and mesentery to form an inflammatory mass. Journal of Clinical Ultrasound. 28(6):314-6, 2000 Jul-Aug

35. Gastrointestinal bleeding in infants and children: Meckel's diverticulum and intestinal duplication.Seminars in Pediatric Surgery. 8(4):202-9, 1999 Nov. • Meckel's diverticula and intestinalduplications may cause gastrointestinal bleeding in almost any age group and require a high index of suspicion for diagnosis.

36. Appendicitis • The risk ofperforation is greatest in1- to 4-yr-old children (70–75%) and is lowest in the adolescent age group (30–40%) . • The classic triad consists of pain, nausea with vomiting, and fever. • The progression from onset of symptoms to perforation usually occurs over 36–48hr.

37. Appendicitis • History included onset of pain before vomiting or diarrhea, loss of appetite, migration of painfrom periumbilical to right lower quadrant. • Auscultation may reveal normal or hyperactivebowel soundsin early appendicitis, to be replaced withhypoactive bowel sounds as it progresses to perforation.

38. Appendicitis • Findings of appendicitis on abdominal films include calcified appendicolith, small bowel distention or obstruction, and soft tissue mass effect. • Graded compression ultrasonography is a noninvasive study with false-negative and false-positive rates of 8–10% . • CT is more sensitive and specific than ultrasonography and more likely to change patient management.

39. Pediatric appendicitis in "real-time": the value of sonography in diagnosis and treatment.Pediatric Emergency Care. 17(5):334-40, 2001 Oct. • The natural progression in appendicitis from initial symptoms to perforation is about 36 to 48 hours . However, perforation may occur more rapidly in the younger child, sometimes within 6 to 12 hours . • Extensive necrosis of the appendix may render it difficult to visualize .

40. Pediatric appendicitis in "real-time": the value of sonography in diagnosis and treatment.Pediatric Emergency Care. 17(5):334-40, 2001 Oct. • We may have to rely on the other ultrasound features of “peri-appendiceal inflammation.” • Studies have shown that the presence of loculated pericecal fluid, prominent pericecal fat, atonic bowel loops, thickened bowel walls, and the circumferential loss of the appendiceal submucosal layer on ultrasound were the significant predictive factors for perforation .

41. Distal Intestinal Obstruction Syndrome • In the older child or young adult with CF, the distal small bowel may by obstructed by thick stool. This condition was called "meconium ileus equivalent" by Jensen in 1962 . • Palpable mass in the right lower abdominal quadrant. Bilious vomiting as a result of the intestinal obstruction

42. Distal Intestinal Obstruction Syndrome • Radiographs of the abdomen demonstrate dilated small bowel loops and a bubbly ileocecal soft-tissue mass .

43. Carcinoid tumor • About 85% of carcinoid tumors develop in the gastrointestinal tract, usually theappendix. • Carcinoid syndrome:flushing,diarrhea, wheezing. Carcinoid crisis:generalized flush, tachycardia, severe diarrhea with abdominal pain, hypotension converting to hypertension, and central nervous system changes leading to coma and then death.

44. Carcinoid tumor • Approximately 40% of the tumors occurred within 2 feet of the ileocecal valve, with very few in the proximal small intestine. • These tumors frequently elicit a mesenteric fibrosing reaction, in which the bowel becomes shortened and kinked, frequently causing partial small bowel obstruction.

45. Carcinoid tumor • On CT, the mesenteric extension from carcinoid will usually appear as a soft tissue-density mesenteric mass . • Calcification can be seen in up to 70% of cases .

46. Lymphomafrom manual of pediatric hematology and oncology ,3rd edition • Non-Hodgkins lymphoma: peak age 5-15 years ,rick factor including genetic and poettransplantation immunosuppression. • Clinical feature:Head and neck(13%), medicatinum(26%),abdomen(35%).

47. Non-Hodgkins lymphoma • The ileum is mostly involved due to a higher number of lymphocytes in the distal gut, accounting for about 50% of small bowel lymphomas • Present with abdomen pain, vomiting and diarrhea, abdominal distension, palpable mass, intussusception,peritonitis, ascites, GI bleeding, hepatosplenomegaly.

48. Multidetector-row computed tomography and 3-dimensional computed tomography imaging of small bowel neoplasms: current concept in diagnosis. • Lymphoma can appear as a single mass lesion, which varies in size.These can lead to intussusception, but rarely will result in obstruction because the masses are typically pliable and soft. • Again, because the masses are characteristically soft, it is rare that the mesenteric vasculature is compromised. Journal of Computer Assisted Tomography. 28(1):106-16, 2004 Jan-Feb.

49. Neuroblastomafrom manual of pediatric hematology and oncology,3rd edition • Give rise to adrenal medulla and the sympathetic ganglia. • Most common tumor in infancy ,peak incidence is 2 years of age • Clinical finding related to anatomic site of abdomen :anorexia ,vomiting,abdomen pain,massive involvement of theliverwith metastasis (especially in the newborn)

50. Neuroblastoma • Paraneoplastic manifestations : excessive catecholamine secretion (sweating,flushing, paller,palpitation, hypertension) ,VIP secretion (watery diarrhea,abdomen distension, hypokalemia) ,and acute myoclonic encephalopathy.