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