2017/04/21 R1 歐宗頴. Case Discussion
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1 2017/04/21 R1 歐宗頴 Case Discussion
2 Case Demography Name: 18143xxx Age: 14y/o Gender: boy Admission: 2017/04/07 Chief complaint: recurrent fever with RUQ pain for 6 weeks
3 Past History G3P3 full term NSD uneventful BW 52kg H 168cm Milestone: WNL Vaccination as scheduled influenza (-) TOCC (-) animal bite (-) bug bite (-) Underlying disease: deny Hospitalization: UTI and circumcise when 1+y/o
4 Present Illness Late Feb. 3/31 4/7 Fever (39 C) for 3 weeks Fever (39 C) for 1week Fever pattern near every day every 4-24hrs RUQ pain sharp breathing exaggerated by deep breath radiation (-)
5 Review Of System Late Feb. 3/31 4/7 CNS CV URI GIGU BW loss 1 kg in 1 m
6 Present Illness Late Feb. 3/31 4/7 Chi-Mei H.
7 04/06 1. portal vein thrombosis 2. partial SMV and splenic vein thrombosis 3. cavernous transformation over hepatic hilum
8 04/06 1. peri-portal linear infiltration 2. splenomegaly
9 Physical Examination flat, soft normative tenderness on RUQ Murphy s sign (+) rebound (-) hepatosplenomegaly (-) shifting dullness (-) knocking pain (-) T: 37.0 P: 70 R: 18 BP: 106/65 no injected eye no injected TM no ulcers, no throat injection clear breathing sounds RHB without murmur spider angioma (-) skin rash (-) wound (-) lower limb swelling no neck, axilla, inguinal lymphadenopathy
10
11 Impression portal vein thrombosis DDx: 1. Infection, suspect cholecystitis or intra-abdominal infection 2. Malignancy 3. Autoimmune disease 4. Thrombophilia
12 Autoimmune ANC, anti-ndna Ab, C3, C4 Cardiolipin IgM, IgG B2 glycoprotein I IgM, IgG IgG4 Malignancy HCG, AFP, CA125, CA199, CA153, CEA Infection HAV, HBV, HCV, ALK-P, r-gt, Bilirubin Thrombophilia Factor V/VIII, Protein C/S, antithrombin-iii
13 04/07
14 04/07
15 04/07 susp. Biliary Tract Infection Ceftriaxone Metronidazole
16 CT-guided biopsy 04/07 04/11 RUQ pain Murphy s sign
17 Autoimmune ANC, anti-ndna Ab, C3, C4 Cardiolipin IgM, IgG B2 glycoprotein I IgM, IgG IgG4 Malignancy HCG, AFP, CA125, CA199, CA153, CEA Infection HAV, HBV, HCV, ALK-P, r-gt, Bilirubin Thrombophilia Factor V, VIII, Protein C, Protein S
18 04/07 Protein C, Protein S acute phase reactant?
19 04/07 ceftriaxone and metronidazole 04/12
20 mild ductular reaction and sinusoid dilatation blood culture tissue culture
21 Diagnosis 1. portal vein thrombosis, suspect caused by Infection (pathogen?), suspect with underlying Thrombophilia (elevated factor VIII), chronic status, accompanied with splenomegaly and cavernous portal veins Ceftriaxone, Metronidazole Enoxaparin, then Rivaroxaban
22 Discussion portal vein thrombosis in children
23 common abdominal venous thrombosis?
24 Cerebral venous and sinus Retinal vein occlusion Upper extremity venous Jugular vein Superior vena cava Inferior vena cava Portal vein thrombosis Hepatic vein Mesenteric vein Splenic vein Renal vein Ovarian vein Penile vein thrombosis at Unusual sites Superficial vein thrombosis of the lower limb
25 Discussion
26 Venous Thrombosis At Unusual Sites systemic local autoimmune inherited thrombophilia acquired hypercoagulability: dehydration, myeloproliferative neoplasms(polycythemia vera, thrombocytosis), active malignancy (mucinous-secrecting), metabolic disorders, drugs (contraceptives, leunase), pregnancy
27 Venous Thrombosis At Unusual Sites systemic local infection, inflammation structure stenosis: thoracic outlet syndrome endothelium injury: surgery, catheter nephrotic syndrome
28 portal vein system!
29 Portal Vein Thrombosis systemic local infection, inflammation: pancreatitis, appendicitis, pylephlebitis structure stenosis: cirrhosis, liver cyst endothelium injury: surgery, catheter (Umbilical Vein)
30 Portal Vein Thrombosis systemic local autoimmune: antiphospholipid Ab, Paroxysmal nocturnal hemoglobinuria (PNH) inherited thrombophilia acquired hypercoagulability: myeloproliferative neoplasms, active malignancy
31 Portal Vein Thrombosis Clinical presentation, depend on the =extent of thrombosis =collateral circulation Acute PVT: intestinal congestion and ischemia pain, distention, N/V, fever, sepsis, lactacidosis, splenomegaly, perforation
32 Portal Vein Thrombosis Clinical presentation, depend on the =extent of thrombosis =collateral circulation Chronic PVT: collateral (+) esophageal varices, GI bleeding ascites hypersplenism (pancytopenia) cavernous transformation of portal vein (CTPV): (pseudocholangiocarcinoma sign) portal bioliopathy
33 Portal Vein Thrombosis - Treatments anticoagulant: no evidence Recommend: failure to spontaneous recanalization thrombophilic cases recurrent thrombosis thrombolytic therapy shunt surgery
34 Summary 14 y/o boy fever with RUQ pain for 3 weeks then subsided but recurred 2 weeks later Murphy s sign CT: portal vein thrombosis with cavernous transformation, suspect chronic status good response to empirical antibiotics, favor infection related extremely high factor VIII, can not rule out acute phase or underlying thrombophilia
35 Take Home Message thrombosis survey: provoked local factors with underlying thrombophilia common sites / unusual sites Learning Issue: Timing To Survey Thrombosis Factors
36 Thank you for your attention
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