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Management conference MIDDLE AGE WOMAN WITH RECURRENT HEMATEMESIS

Management conference MIDDLE AGE WOMAN WITH RECURRENT HEMATEMESIS. Raika Jamali MD Digestive Disease Research Center Tehran University of Medical Sciences. Middle age woman with massive hemathemesis admitted in emergency department.

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Management conference MIDDLE AGE WOMAN WITH RECURRENT HEMATEMESIS

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  1. Management conferenceMIDDLE AGE WOMAN WITH RECURRENT HEMATEMESIS Raika Jamali MD Digestive Disease Research Center Tehran University of Medical Sciences

  2. Middle age woman with massive hemathemesis admitted in emergency department. • The patient was hypotensive and resusitated with 4 liters of ringer, 3 units of packed cell and FFP. • After resusitation, endoscopy showed 2 rows of G2 varices in distal part of esophagus with red sign that was treated with sclerotheraphy. Fundal varices were seen.

  3. There was history of splenectomy ( huge splenomegaly) due to LUQ pain 10 years ago. • The recurrent hematemesis during admission was treated by several episodes of sclerotherapy. • There was transiant mild right pleural effusion after sclerotheraphy. • No history of OCP use.

  4. EXAM No sign of encephalopathy. • No icterus, • paleness in conjunctiva. • Midline scar of laparotomy, • No abdominal collateral formation(caput medusa) or ascitis. • No organomegaly. • No sign of chronic hepatic insufficiency. • Pedal edema(+).

  5. LAB DATA • WBC=6100 (NL Diff) • Hb=6.6 Ferritin=20 • MCV=98 • MCH=26 • MCHC=33 • Plt=249000

  6. BUN=9 AST=39 • Cr=0.7 ALT=41 • FBS=108 TG=62 ALP=118 Bili direct =2 • Na=142 Bili direct=0.3 • K=3.8 Total protein=6.2 Albumin=2.6 • ESR=16 Pt=16 PTT=36

  7. HBs Ag=neg • HBs Ab=neg • HBc Ab=neg • HCV Ab=neg • ANA=neg

  8. LAB DATA IN THE 3RD DAY OF ADMISSION • Pleural fluid analysis: • LDH=60 • WBC=70

  9. CXR • MILD RIGHT P.E. • NO SIGNIFICANT PARANCHYMAL INFILTRATION.

  10. SONOGRAPHY • Liver size and echogenesity was normal. • Spleen was not seen due to previous splenectomy. • PV diameter = 9 mm. • Biliary ducts were NL.

  11. Color Doppler sonography • IVC and suprahepatic veins were patent. • Portal & splenic veins were occluded. • Cavernous transformation in hilum was seen. • There was hepatopedal flow.

  12. The variceal bleeding was treated by several scleroterapies and octerotide infusion. • Protein C, S, Antitrombin 3, Anticardiolipin Ab and factor 5 Laden were normal. • She was discharged with Inderal. • She did not accepted liver biopsy .

  13. Risk factors for variceal bleeding Portal pressure • HVPG >12 mm Hg Varix size and location • Large esophageal varices • Isolated varices in fundus of stomach Variceal appearance on endoscopy ("red signs") • Red wale marks (longitudinal red streaks on varices) • Cherry-red spots (red, discrete, flat spots on varices) • Hematocystic spots (red, discrete, raised spots) • Diffuse erythema Degree of liver failure • Child-Pugh class C cirrhosis Presence of ascites • Tense ascites

  14. Consensus on extra-hepatic portal vein obstruction Review Article Sarin SK, Sollano JD, Chawla YK, Amarapurkar D, Hamid S, Hashizume M, Jafri W, Kumar A, Kudo M, Lesmana LA, Sharma BC, Shiha G, de Silva HJ and members of the APASL Working Party on Portal Hypertension. Liver International 2006: 26: 512–519.

  15. Abstract: • The Asian Pacific Association for the Study of the Liver (APASL) had set up a working party on portal hypertension in 2002 with a mandate to develop consensus on various aspects of portal hypertension. It was discussed and prepared by the experts in this field from the Asian region and was presented at the annual meeting of the APASL, at Bali in August 2005.

  16. The process for the development of the consensus is as follows: • Review of all published literature, • Asian survey of the current approaches for diagnosis and management, • Identification of the important issues • Discussion and preparation of the consensus statement by a core group of experts.

  17. The working party adopted the Oxford System for developing an evidence-based medicine approach. • The level of existing evidence was assessed by the group and the recommendations were ranked accordingly: • Level of evidence : from 1= highest to 5 = lowest; • Grade of recommendation : from A = strongest to D = weakest.

  18. Terminology • EHPVO occurs when the site of block is in the portal vein before the blood reaches the liver. • The use of the term portal vein thrombosis has two drawbacks: • first, it does not exclude the intrahepatic portal vein thrombosis due to cirrhosis or invasion by hepatocellular carcinoma; • second,the term does not include formation of portal cavernoma and development of portal hypertension,

  19. The term EHPVO has been accepted, as it is not primarily associated with a primary liver disease.

  20. Consensus statements: definition of EHPVO • EHPVO is a vascular disorder of the liver. • It is defined by obstruction of the extra-hepatic portal vein± involvement of the intra-hepatic portal or splenic or superior mesenteric veins. • Isolated occlusion of the splenic vein or superior mesenteric vein does not constitute EHPVO. • Portal vein obstruction associated with CIRRHOSIS or HCC is a separate entity

  21. Prevalence • EHPVO is a common cause of portal hypertension in the developing countries (up to 30% of all variceal bleeders) • It is secondory to cirrhosis in the West (up to 10%). • EHPVO is the most common cause of upper gastrointestinal bleeding (70%) in children.

  22. Etiology of EHPVO • EHPVO is a heterogenous disease with regard to etiology and pathogenesis, especially with respect to age and geographical location (2a). ___________________Children:__________________ • Umbilical sepsis, umbilical catheterization in a small percentage of patients with EHPVO (2b). • Congenital anomalies rarely account for EHPVO in children (4c). • There is insufficient evidence to suggest an underlying hypercoagulable disorder in hildren(2b). • In the majority of children with EHPVO, the etiology is unknown. (4c) • There is a need for thorough etiological work-up (5, d).

  23. Adults: • Hypercoagulable states are commonly reported from the West (2B). • These have not been adequately studied in Asian patients (4D). • Intra-abdominal inflammatory process and trauma has been reported in a small proportion of patients (3B). • Thrombosis at other sites is present In a small percentage of patients (3B).

  24. Consensus statements: pathology • If the liver functions are deranged ,liver biopsy is necessary in a patient with EHPVO (1A). • It also helps to exclude other liver diseases (1A).

  25. Childhood EHPVO • Variceal bleedingwithout hepatocellular failure. • Ascites: (transient) develops following hemorrhage or surgery, • ascites signifies hepatic dysfunction and circulatory changes. • Growth retardation: Reduced portal blood supply to the liver, resistance to growth hormone function and reduced insulin like growth factor have been alleged for this.

  26. Adult EHPVO Acute EHPVO:Acute abdominal painof varying severity and duration. • Fever • Ascites(rarely).

  27. Chronic: Variceal bleedingand hypersplenism(common) • Ectopic varices • Intestinal ischemiaand protein-losing enteropathy (rare). • Encephalopathy ( very uncommon) when large spontaneous porto-systemic shunts have developed. • Jaundice(rarely) is generally due to portal biliopathy.

  28. Portal biliopathy • Refers to abnormalities of the bile ducts in patients with portal hypertension due to: • paracholedochal collaterals on bile ducts, • localized strictures, • angulation of ducts, • displacement of ducts, • focal narrowing, • dilatations and irregular walls.

  29. Clinical presentation Consensus statements: Acute or chronic:

  30. Acute EHPVO This can present with abdominalpain, ascites or fever.(1c) No evidence of portosystemic collaterals.(1c)

  31. Chronic EHPVO • Presents commonly with repeated bleeding fromesophageal varices (1c). • Gastric varicesare present in 1/3 of the patients with EHPVO. • The IGV1 (isolated fundal varices) bleed severely (3a). • Ectopic varicesare common (duodenum, anorectal, biliary tree and gallbladder, etc.). • These may manifest as obscure GI bleed, hematochessia or biliary obstruction (1c).

  32. Moderate to massivesplenomegaly is universal, and may be a presenting feature (1c). • A proportion of children havegrowth retardation (3b). • Uncommon presentations include : hypersplenism, ascites and jaundice (4d). • Continuous abdominal pain should arouse suspicion of extension of venous thrombosis or splenic infarction(4d).

  33. Portal biliopathy is present in 48% of patients with EHPVO based on ERCP studies(1c). • Majority of patients are asymptomatic (1c). • Abdominal pain, jaundice and fever develop in a minority(5%) on long-term follow-up (3b). • ERCP is the definitive method for diagnosis of biliopathy (1c).

  34. ERCP is recommended only if a therapeutic intervention is contemplated (5d). • MRCPis a good non-invasive alternative (3b). • There is insufficient evidence to suggest whether portal biliopathy is progressive (4b).

  35. Cholangitis, • Secondary biliary cirrhosis, • Gall stones, • Hemobilia, • Hypoalbuminemia • Coagulation disturbances have been reported (3b).

  36. Natural history in children • The bleeding risk (number of bleeds/year) is about 1.3. • The bleeding risk remains unchanged after puberty. • The incidence of re-bleeds from varices after obliteration is low in long-term follow-up (2a). • In a proportion of patients, hepatic dysfunction develops during long follow-up (3a). • Repeated transfusion / hospitalization increases the risk of acquiring HBV and HCV infection, which may lead to chronic liver disease (5d).

  37. Portal biliopathy presenting with jaundice or cholangitis develops in a small proportion of patients (1c). • EHPVO in children has a relatively benign course. • In follow-up studies of up to 10 years, nearly 90% survival is reported (3a).

  38. Natural history in adults • The natural history of EHPVO in adults is related to the prognosis of the underlying disease and the prothrombotic state (3b)

  39. EHPVO and pregnancy • Pregnancy occurs normally in EHPVO (4d). • There are insufficient data to recommend cesarean section in these patients (4 d). • The risk of variceal bleeding in pregnant EHPVO patients is higher than in pregnant cirrhotic women (4d). • Abortion, pre-maturity, small for gestational age babies and perinatal death are high in pregnant patients with EHPVO (4d).

  40. Hemodynamics of EHPVO • Hepatic vein pressure gradient is normal in EHPVO (1c). • Intrasplenic pressure is elevated in EHPVO and suggest portal hypertension(1c). • Currently, portal pressure measurement has a limited role in the clinical management of patients with EHPVO (5 D).

  41. Diagnosis • EHPVO is diagnosed by imaging techniques such as Doppler US, CT or MRI, which demonstrate portal vein obstruction, intraluminal thrombus in the portal vein ± portal vein cavernoma (1 A). • Gray-scale (B-mode) US is helpful in its initial suspicion, if an obstruction, a cord-like structure of EHPV or a bead-like cystic structure (cavernoma)next to the obstructed PV is demonstrated (3 B). • Color Doppler US is efficient in its confirmation if no color flow or Doppler signal within the EHPV, hepatopetal signal within the cavernoma or varices at the GB wall are demonstrated (3 B).

  42. Contrast-enhanced CT and MRI (MRA) can be diagnostic if cavernomatous transformationwith splenomegaly and/or no opacification of the hepatic portal vein are demonstrated (3 B). • The role of liver biopsy in the diagnosis of EHPVO is limited, as mentioned above.

  43. Management

  44. Bleeding • To control acute variceal bleeding, endoscopic therapy is effective (1 A). • For primary prophylaxis of variceal bleeding, there are insufficient data on whether B-blockers or endoscopic therapy should be preferred (5 D).

  45. For secondary prophylaxis, endoscopic therapy is effective (1 A) • Endoscopic band ligation of varices (EVL) is preferred due to its safety and efficacy (1 A). • There are insufficient data to recommend B-blockers (5 D).

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