1. Pediatric ED conference 2008/08/27 R4 張繼中

2. 14-year-old female Vital sign : 37.9/105/22 121/70 E4V5M6 Brought to pediatric ED by her mother Chief complaint : Abdominal pain noted for 2 days

3. Personal History A case of Peutz-Jeghers syndrome Was admitted in Jun, 2008 due to persisted abdominal pain (PES and colonfiberscopy at 2008/07/11 : Single polyp in colon and stomach) Her abdominal pain improved after A.M.G use  discharged

4. After discharge, her abdominal pain persisted (upper abdominal intermittent pain, no radiation) The pain became severe since 2 days ago Vomiting >10 times (food and light greenish content) Visited Pediatric GI OPD this morning  suggest OPD admission  visited ED due to no bed available

5. Physical Examination PAT : A ill looking B fair C fair General appearance alert Consciousness clear, E4V5M6 Pupil size 3+/3+ HEENT not anemic, not icteric Chest BS clear, HS : RHB, no murmur Abdomen : soft and flat whole abdominal pain, especially middle lower abdomen No rebounding pain No McBurney point tenderness No CV knocking pain Extremities : free movable, no edema

6. WHAT’S YOUR IMPRESSION? …AND YOUR NEXT STEP? For this 14 y/o female with history of PJS and 2-day-long abdominal pain..

7. Time / mode of onset Progression Location of the pain Character of the pain ; Referral or radiation of the pain Precipitating and relieving factors Prior episodes Associated symptoms : vomiting, diarrhea Further history Trauma history Abuse Sexual experience / pregnancy

8. (Lay on bed) Exposure whole abdomen : hernia, petechiae, mass Inspection and auscultation Abdominal palpation Rectal examination

9. Pediatric abdominal Pain Etiology of pain : over 11 years Appendicitis Cholecystitis Diabetic ketoacidosis Dysmenorrhea Ectopic pregnancy Functional pain Gastroenteritis Incarcerated hernia Inflammatory bowel disease Obstruction Pancreatitis Peptic ulcer disease Pneumonia Pregnancy Renal stone Sickel cell disease Torsion of ovary or testicle Toxins (lead, arsenic) Trauma Urinary tract infection ~Emergency Medicine 6 th edition  Possible  No DM Hx ; check sugar Check ICON, Hx Possible, must be rule out Check ICON

13. NPO CBC/DC, BUN/Cr, electrolyte, liver function test, Alk-P, lipase, Na, K Blood culture IVF Finger sugar ICON test, U/A CXR standing view ; KUB Consider analgesic agent Further image study

14. Initial Treatment NPO CBC/DC, BUN, Na, K, ALT, Sugar, U/A, ICON, KUB (if ICON negative) Admission to Pediatric GI ward  due to persisted abdominal pain, Demerol 1amp IM stat Arrange abdominal echo (for rule out appendicitis)

15. WBC : 21700 RBC : 3.85 Hb : 9.8 Hct : 31.3 MCV : 81.3 MCH : 25.5 MCHC : 31.3 RDW : 21.9 Plt : 364 Segment : 90.5 Lymphoctyte : 4.5 Monocyte : 5.0 Sugar :142 BUN (B) : 12 ALT/GPT : 11 Na : 139 K : 3.7 CRP : 8.29

16. KUB

17. Abdominal echo Dilated bowel loops are demonstrated in pelvic area. A bizarre cystic mass (8*5*5 cm) with internal air and fluid accmulation is demonstrated Imp : Cystic lesions caused by Tubo-ovarian obstruction or uterine origin, or volvulus Local ileus (pelvis) suspect adhersion or volvulus

18. Arrange abdominal CT after discussion family  abdominal pain, R/O PPU, R/O bowel obstruction, R/O GYN disorder Abdominal CT (C±)  visit file

19. Operation finding *100ml turbid ascites with fibrtin coating and intestinal adhesion *intestinal dilatation with bowel ischemia from 150 cm distal to Trietz ligment to 80cm proximal to ileocecal valve, bowel perforation at distal part, resction of the gangrene bowel with end- to-back anastomosis (the discrepancy of proximal to distal bowel was 3:1) *a huge polyp about 3*3 cm as the leading point of intussusception *another two small polyps (1*1 cm) at the distal end of the resected bowel *two Fr.8 silastic penrose at right paracolic gutter and Cul-de-sac *retrocecal appendix was noted and incidental appendectomy was done smoothly

20. OP method 1.Reduction of intussusception with bowel anastomosis 2.Enterolysis with retrograde decompression 3.Silastic penrose drainage (Fr.8*2) 4.Incidental appendectomy

21. After operation the patient was transferred to ward  referred to ICU due to hypotension and low urine output (IVF and A/B use) Transferred to ordinary again on 8/8 Discharge on 8/13

22. DISCUSSION

23. About Peutz-Jeghers syndrome Melanotic macules on the lips and mucous membranes and by gastrointestinal (GI) polyposis. Autosomal dominant trait (STK11 gene) Nose, hands, and feet; around the mouth, eyes, and umbilicus ; and as longitudinal bands or diffuse hyperpigmentation of the nails Polyposis usually involves the jejunum and ileum but may also occur in the stomach, duodenum, colon, and rectum Nelson Textbook of Pediatrics, 18th ed

24. Episodic abdominal pain, diarrhea, melena, and intussusception are frequent complications Increased risk of GI tract and non-GI tract tumors at a young age (2~3%) Relative risk of GI and non-GI malignancy Evidence of intestinal lesions can come from bleeding but more commonly arises from painful intestinal cramps associated with obstruction due to recurrent intussusceptions

26. Key Point Peutz- Jegher syndrome is a rare inherited disease and intussesception is a common complication of PJS Evaluate the patient by yourself, not only by order of other doctor or patient/family !

27. THANKS FOR YOUR ATTENTION! And any question?