1. KSP-1677 - 제출 기관 : 서울 아산 병원 - 토의자 : 서울대병원 전공의 서자희

2. Clinical history  Age : 32 Para 1-0- 1 -1 Hx of miscarriage GA 35+6 wk Near full term Vaginal spotting Suspicious coagulopathy? 1554gm, Male Spontaneous delivery Apgar score : 1 min - 8, 5 min – 9 Placenta 530gm Cord : 3 vessels connected in center of placenta Hard cutting plate

3. Maternal plate Fetal plate

5. Maternal plate

7. Fetal plate

9. Extensive fibrin deposit

10. Villi entrapment

11. Syncytial knot : maternal plate

12. Syncytial knot : fetal plate

14. Mantle formation of cytotrophoblast cells

15. Cytotrophoblastic proliferation

16. Cytotrophoblast syncytiotrophoblast http://www.dartmouth.edu/~anatomy/Histo/lab_6 /female/DMS182/popup.html Normal placenta in the 2 nd trimester of pregnancy

17. Trophoblastic basement membrane thickening

18. Pathologic finding Extensive eosinophilic material deposit –Perivillous area –Fibrin deposit Hypermaturated villi –Excessive syncytial knot formation Proliferation of villous cytotrophoblasts –Mantle formation and spreading out into fibrin –No mitotic figures ; No cytologic atypia or pleomorphism Mild trophoblastic basement membrane thickening No inflammation (Ao CDo Uo Co)

19. Weight estimation of fetus & placenta Mean & SD of body weight of Live-Born infant Normal placental weight range MeanSD 34wk1905g± 625 35wk2093g± 309 MeanSD 34wk542g±125 35wk571g±133 Reference : Handbook of Pedatric autopsy pathology E Gilbert-Barness & ED Debich-Spicer, Humana Press p.24 : Modified data from J Pediatr 1978 May;92(5):787 Reference : BJOG: An International Journal of Obstetrics & Gynaecology Volume 114, Issue 6, 2007, Thompson et al.

20. Differential diagnosis Neoplastic –Choriocarcinoma –Hydatidiform mole Non-neoplastic –Lesions due to disturbance of maternal blood flow Perivillous fibrin deposition Maternal floor infarction –Lesions due to disturbance of fetal blood flow Fetal artery thrombosis

21. Differential diagnosis Neoplastic –Choriocarcinoma –Hydatidiform mole Non-neoplastic –Lesions due to disturbance of maternal blood flow Perivillous fibrin deposition Maternal floor infarction –Lesions due to disturbance of fetal blood flow Fetal artery thrombosis

22. Differential diagnosis Lesions due to disturbance of maternal blood flow –Perivillous fibrin deposition –Maternal floor infarction Lesions due to disturbance of fetal blood flow –Fetal artery thrombosis, etc

23. Differential diagnosis Lesions due to disturbance of maternal blood flow –Perivillous fibrin deposition –Maternal floor infarction Lesions due to disturbance of fetal blood flow –Fetal artery thrombosis, etc

24. Maternal floor infarction Misnomer : massive basal plate fibrin deposition

25. Pathology of placenta : Major problems in pathology 3rd edition H Fox & NJ Sebire, Saunders/Elsevier pp.96-100

26. Massive perivillous fibrin deposition Maternal floor infarction : region of basal villi Maternal floor infarction and perivillous fibrin deposition  Criteria by Katzman & Genest, 2002 Villi of entire maternal floor are embeded in fibrin Fibrin thickness : least 3mm in one slide  Arch Path Lab Med, Vol 126 2002 (Sun et al) Massive perivillous fibrin deposition : perivillous fibrin deposition involving 30% or more of chorionic villi in central (nonbasal) region of the placenta Maternal floor infarction : perivillous fibrin deposition involving more than 50% of basal villi associated with hypovascular or fibrotic villi  Criteria by Katzman & Genest, 2002 Villi of entire maternal floor are embeded in fibrin Fibrin thickness : least 3mm in one slide  Arch Path Lab Med, Vol 126 2002 (Sun et al) Massive perivillous fibrin deposition : perivillous fibrin deposition involving 30% or more of chorionic villi in central (nonbasal) region of the placenta Maternal floor infarction : perivillous fibrin deposition involving more than 50% of basal villi associated with hypovascular or fibrotic villi

27. Fetal Outcome Maternal floor infarctionPerivillous fibrin deposition High rate of fetal mortality (17-40%) Intrauterine growth retardation (51%) Frequently recurs in successive pregnancy Intrauterine growth retardation (31%) : >20% of terminal villi in central basal portion (Redline & Patterson) Low placental weight CNS development abnormality Kurman Blaustein’s Pathology of the Female Genital Tract 5 th edition pp.1135-1137 Pediatr Dev Pathol. 2002 Mar-Apr;5(2):159-64.

28. Maternal problem associated with perivillous fibrin deposition Preeclampsia –H–HELLP syndrome Essential hypertension Diabetes mellitus Coagulopathy Autoimmune disease (ex :Anti-phospholipid syndrome) The clinical important entity of massive preivillous fibrin deposition occurs very rarely (<1% of pregnancy)

29. Diagnosis 1HE > Diagnosis) Placenta ; 1. Massive perivillous fibirin deposition with 1) exccesive syncytial knot formation 2) villous cytotrophoblastic proliferation 2. No inflammation (Ao CDo Uo Co)

30. I could only say what was the balance of probability. I did not at all expect to be so accurate I could only say what was the balance of probability. I did not at all expect to be so accurate OK… What’s your diagnosis, Sherlock? 2010 BBC One, “Sherlock” S01E01 Study in Pink Quote from “The Sign of Four” by Sir Arthur Conan Doyle