Case Conference Present: R1 林浚仁 Instructor: Dr. 吳孟書

Case 1 ► Name: 謝 XX ► Age: 4 y/o ► Gender: male ► Date: 95/07/11 ► Vital signs: BT: 36.9 HR: 101, RR: 18 BP: 101/54 BP: 101/54

Chief complaint ► Severe abdominal pain since this early morning

Present illness ► Intermittent abdominal pain for one week ► Severe abdominal pain this early morning ► Knee to chest position with irritable crying ► No fever, cough, diarrhea and rhinorrhea ► No stool passage for one day. ► Poor activity and appetite

Past history Admission hx:(-) OP hx:(-) Travel hx:(-) Drug hx:(-) Allergy hx:(-) BBW:3000+gm BBW:3000+gm GA: fullterm NSD Vaccination : as scheduled

Physical examination ► APPEARANCE:Consciousness: clear, Edematous:(-), Cyanosis:(-), DEHYDRATION: (+) HEENT: Sclera: anicteric Conjunctiva : injected (-), not pale Throat: injection (+); Tonsils: exudate(-) Eardrum: not injected NECK:supple, Lymphadenopathy:(-) CHEST:Shape: symmetric expansion, breathing sound: clear HEART: Regular heart beat ABDOMEN: Tenderness over RUQ Bowel sound: normoactive Liver palpable:(-) EXTREMITIES: Pitting edema:(-) Free movement:(+) SKIN: rash over buttock(+) SKIN: rash over buttock(+)

LAB RBC: 4.70 milon/cmm RBC: 4.70 milon/cmm HGB: 12.9 g/dl HGB: 12.9 g/dl HCT 38.9 % HCT 38.9 % MCV: 82.8 umm MCV: 82.8 umm PLATELET:353000/cmm PLATELET:353000/cmm WBC: 19600/cmm WBC: 19600/cmm SEGMENT: 67% SEGMENT: 67% LYMPHOCYTE: 24% LYMPHOCYTE: 24% MONOCYTE:% 8% MONOCYTE:% 8% EOSINOPHIL:% 1% EOSINOPHIL:% 1% Cr Cr 0.4 mg/dl AMYLASE 59 U/L ALT 16 U/L CRP 8.94 mg/dl

KUB

ER course ► Stool impaction  Fleet enema(normal appearance stool) ► Still abdominal pain ► Bedside echo  intussusception was highly suspected ► Successful air reduction

Air reduction

Case 2 ► Name: 謝 XX ► Age: 3 y/o ► Gender: female ► Date: 95/09/12 ► Vital signs: BT: 36.5 HR: 112 RR: 18 BP: 110/60 BP: 110/60

Chief complaint ► intermittent abdominal pain for one day

Present illness ► Abdominal pain this morning after drinking some milk. ► An episode of non-bilious vomiting ► Intermittent abdominal pain with frequency of 20mins ► No radiating pain, no constipation, no bloody stool, no fever ► Normal appetite and activity

Past history ► Admission hx:(-) OP hx:(-) Travel hx:(-) Drug hx:(-) Allergy hx:(-) Vaccination : as scheduled

Physical examination ► Appearance ： normal ► HEENT ： supple neck, no pale conjunctiva, no injected eardrum, no injected throat, no LAP ► Chest: clear breath sounds, regular heart beat, no murmur ► Abdomen: soft, no tenderness, no Macburney tender, no rebounding pain, hyperactive bowel sounds ► Extremities: freely movable, no edema ► No skin rash

Present illness RBC: 4.44milon/cmm RBC: 4.44milon/cmm HGB: 11.7 g/dl HGB: 11.7 g/dl HCT 35.6 % HCT 35.6 % MCV: 80.2 umm MCV: 80.2 umm PLATELET:206000/cmm PLATELET:206000/cmm WBC: 8100/cmm WBC: 8100/cmm SEGMENT: 54.4% SEGMENT: 54.4% LYMPHOCYTE: 36.5% LYMPHOCYTE: 36.5% MONOCYTE:% 8% MONOCYTE:% 8% EOSINOPHIL:% 0.9% EOSINOPHIL:% 0.9% Cr Cr 0.4 mg/dl BUN 11 mg/dl CRP 5.01 mg/dl Na meq/l K 3.4 meq/l ALT 16 U/L GLU 130 mg/dl

KUB

ER course ► KUB: ileus with soft tissue mass over RUQ ► Suspected intussusception ► Air reduction  ileocolic intussusception and successful reduction She is his sister!

Interssusception ► Invagination of a part of the intestine into itself ► The most common abdominal emergency in children <2 y/o ► The most common cause of intestinal obstruction between 6~36 months ► Male:female  3:2.

Pathogenesis ► Most often near the ileocecal junction ► 75% of children with ileo-colic type is idiopathic ► Seasonal variation  viral gastroenteritis ► Rotavirus vaccine ► 30% experience viral illness before onset ► Hypertrophy of Peyer patches

Pathogenesis ► >5 y/o  lead point :  Small bowel lymphoma  Meckel diverticulum  Duplication cysts  Polyps  Vascular malformations  Inverted appendiceal stumps  Parasites (Ascaris lumbricoides)  Henoch-Sch ö nlein purpura( bowel wall hematoma )  Cystic fibrosis ► Post-OP: jejuno-jejunal, 2wks within laparotomy

Clinical manifestation ► Abdominal pain:  Sudden onset  Intermittent  Severe  Colicky  Progressive ► Inconsolable crying ► Drawing up of the legs toward the abdomen ► 15 to 20 minute intervals

Clinical manifestation ► Vomiting (non-bilious  bilious) ► Currant jelly stool (mesenteric veins were compressed) ► Sausage-shaped abdominal mass in the R ’ t of abdomen. ► 70% :stool OB + ► Triad of pain, abdominal mass, and currant- jelly stool  < 15%

Diagnosis ► KUB  Distended loops of bowel with absence of colonic gas  Pneumoperitoneum  bowel perforation  Target sign  2 concentric radiolucent circles superimposed on the right kidney  Crescent sign  soft-tissue density projecting into the gas of the large bowel

Diagnosis ► Ultrasonography  bull's eye or coiled spring lesion  lack of perfusion detected with color duplex imaging  Sensitivity 98%~100% ► CT scan : the other imaging modalities are unrevealing. ► Contrast studies  standard procedure for diagnosis and treatment  Barium, water-soluble contrast or air

Air VS Barium ► Air contrast has advantage of  Less radiation exposure and cost  Relatively harmless  Smaller colonic wall tears  Higher successful rate

Treatment ► Successful rate: 75~90% in ileo-colic type ► Complication  perforation( 3%) ► Higher perforation rate  5 y/o  Small bowel obstruction  long duration of symptoms(>48 hours)  passage of blood via the rectum (hematochezia)  significant dehydration ► Signs of peritoneal irritation or free peritoneal air  surgery

Recurrence ► Nonoperative reduction : 10 % ► Surgery: 1% ► Recurrence is not necessarily an indication for surgery.

Familial intussusception ► 1998, Journal of Pediatric Gastroenterology and Nutrition, NTUH  case4case3case2case1

Familial intussusception ► Case 4 (7.5-month-old): ileocolic type, barium enema reduction ► Case 3 (7-month-old): ileo-ileocolic type, surgery ► Case 2 (28-month-old): ileocolic type, surgery ► Case 1 (5-month-old): ileocolic type, surgery 2.5 years 9 years 15 years

Discussion ► None of the 4 pts had pathologic leading point ► Infection cause is unlikely ► Genetic or hereditary factor ► Incidence of intussusception: per 1000 live birth in Taipei City ► Inherited predisposition (abundant lymphoid tissue in terminal ileum) triggered by an acquired virus agent ► Inform parents about the possibility of familial occurence

Familial intussusception ► 1999, Journal of Pediatric Surgery ► Family 1: 10 children, 6 boys, 4 girls  5 boys had intussusception( 8,2,3,6,4 months respectively), 3 surgery ► Family 2: 2 of 2 girls, ( 3, 6 months respectively), no surgery

Discussion ► MacMahon(1995): 296 cases, risk of intussusception for siblings : 2.5%, 20 times higher than general population ► Need further genetic study

Familial intussusception ► 2002, Journal of Pediatric Surgery

Familial intussusception ► Patient 1: 9 months, ileocolic type, surgery ► Patient 2: 3 months, ileocolic type, surgery ► Patient 3: 3.5 months, hydrostatic reduction ► Patient 4: 4 months, hydrostatic reduction

Discussion ► Review 38 cases reported in literature, 16 (42%) infants were younger than 4 months ► General population: 7 % younger than 4 months ► Knowledge of previous history of familial intussusception help early diagnosis and prompt treatment.

Key points ► Clinical manifestation of intussuseption: intermittent abdominal pain, vomit, bloody stool, abdominal mass ► Diagnosis: ultrasonography ► Inform parents the possibility of familial intussusception  early intervention ► Familial intussusception occurs more frequently in babies younger than 4 months

Thank you for your attention