collateral benefit : dr n sewgoolam · pathophysiology cavernous transformation of the portal vein...
TRANSCRIPT
COLLATERAL BENEFIT
BYDR N SEWGOOLAM
CASE PRESENTATION
29 year old maleProblems• Ascites• Hypersplenism
BMAT = hypercellular marrowno malignancy
• Jaundice
Abdominal pain and distensionHaematemesisYellow discoloration of his eyesNo melaenaNo change in bowel habitsNo constitutional symptoms
PAST MEDICAL HISTORY
Not a known hypertensive or diabetic
No cardiac pathology
No previous tuberculosis
SOCIAL HISTORY
Moderate alcohol consumptionNon-smoker
No herbal medicationWater source: river
ON EXAMINATION
Patient was comfortableJaundiced and pallorNo lymphadenopathyTemporal wastingNormotensiveApyrexial
Abdominal examination
No prominent abdominal veinsSoft and distendedAscites 15cm firm splenomegalynon-tender and smoothDiastolic bruit to the left of the epigastriumNo hepatomegaly
CVSCNS non-contributoryRESP
SUMMARY
29 year old male patient problems of ascites
splenomegalyno palpable/ballotable
liverSingle episode of haematemesis
DIFFERENTIALSPORTAL HYPERTENSION secondary
cirrhosis ? Viral ? Autoimmune Hepatitis? Alcohol related? Metabolic - Wilson’s disease
Haemochromatosis Alpha1 antitrypsin Def
Fibrosis ? Idiopathic ? schistosomiasis
Portal vein thrombosis ? 2 cirrhosis ? other
INVESTIGATIONS
FBC: hb 8.8 wcc 1.81 plt 31PANCYTOPENIA
INR: 1.4
U&E: 138/3.9/109/21.8/4.7/73
LFT: TP 72 ALB 35 T.BIL 78ALP 85 GGT 34 ALT 21
HEPATITIS A B negativeC
HIV negative
Cu & 24hr urine Cu NAD
Fe studies = normal
AFP = normal range
ANF = negative
CXR Normal
Urine MCS = no evidence of parasites
Ascitic fluid = transudateno organisms isolated
UPPER ENDOSCOPY
Small distal varices
No signs of a recent bleed
Portal hypertensive gastropathy
ULTRASOUND
Gross ascitesLiver = 12cm. The portal vein is thrombosed with multiple channels in the porta hepatis – cavernous transformationHepatic veins and IVC are patentSpleen – massively enlarged.
CT abdomen
Portal vein thrombosis with portal hypertensionMassive splenomegaly with porto-systemic shunting into the left renal vein
LIVER BIOPSYIrregular fibrosis suggesting underlying cirrhosis
No evidence of granulomatousinflammation
No schistosoma ova
No malignancy
ASSESSMENT
29 year old male with:
1. Cirrhosis with portal hypertension2. Portal vein thrombosis3. Spontaneous spleno-renal shunt
Portal vein thrombosis
Recognised with increasing frequency:
Due to increasing use of ultrasonography in the evaluation of abdominal symptoms.
Exact frequency = unknown
Approximately 5%
Occurs at any age
definition
Restricted to thrombosis developing in the trunk of the portal veinCan extend downstream to involve the left or right branchUpstream to involve the splenicvein, superior or inferior mesenteric veins
UPSTREAM:Little effect on the intestine as long as mesenteric venous arches remain patentIf ischemia results, infarction of the intestine may follow
DOWNSTREAMClinical signs of liver disease are absent/transientBiochemically: albumin prothrombin ratio normalserum bilirubin
Uncommon manifestation of portal hypertensionIn a proportion of patients etiology is unknownPathogenesis is unclear !!Pathophysiology and clinical aspectsdiffer according to the site of the thrombosis in the portal system
PATHOPHYSIOLOGYCavernous transformation of the portal vein ie periportal collaterals occurs Development of multiple small vessels in and around the recanalising main veinLeash of fine / enlarged vessels seen in place of the PVColour/pulsed doppler: shows blood flow in these periportalcollaterals
cavernous transformation of the PV: Appearance of a sub-hepatic sponge-like mass
Bile duct varices-pseudocholangiocarcinoma signseen on ERCP
In portal hypertension, spontaneous spleno-renal shunting, due to the development of collaterals, may lead to clinical benefit
(ie the reduction of oesophageal varicealbleeding.)
thus representing“ COLLATERAL BENEFIT “
15.9% of a total of 151 patients with portal hypertension,showed evidence of spontaneous spleno-renal shuntsCONCLUSION: DEVELOPMENT OF THESE SHUNTS PROVIDES PROTECTION FROM VARICEAL BLEEDING
Journal of Gastroenterology and hepatology (2002)
causes
General thrombogenic factors (60%)
Local factors (40%)
Inherited thrombopilias
Factor V Leiden mutation (5%)Factor II mutataion (3%)Protein C deficiency (5%)Protein S deficiency (5%)Antithrombin (1%)
Local factors
Focal inflammatory lesionsdiverticulitisduodenal ulcerpancreatitisinflammatory bowel disease
Injury to the portal venous systemsplenectomy colectomy
Clinical manifestations
Differ according to the stage that PVT is diagnosedEarly: abdominal pain
inflammatory statefever
Can be completely asymptomatic
Late : thrombocytopaeniaenlarged spleens/s of portal hypertensionGIT bleeding
Compensatory mechanisms have usually developed by this time
COLLATERAL FORMATIONGROUP 1
At the cardia of the stomach where the left gastric vein anastomoses with the intercostal , oesophageal and azygosminor veins
At the anus, involving the superior haemorrhoidal veinCan lead to rectal varices
GROUP 2In the falciform ligament through the
para-umbilical veins
GROUP 3Where abdominal organs are in
contact with retroperitoneal tissuesThey include Lumbar veins
GROUP 4 Portal venous blood is carried to the left renal vein usually via the splenicvein
therapy
No controlled study of any form of RxPrompt anticoagulation is recommended as it allows recanalisation (4-6 months)Later, insertion of TIPS may be necessary to maintain portal circulation
Recommendation is to assess patients on an individual basis and decide on optimal management accordingly
Back to our patient….
Started on low dose duiretics, to which he responded very wellHe was also kept on PPI and a beta blocker to manage the portal gastropathyHe is currently seen in GI clinic every 6/12 with no further complications
The end
The end