Deshmukh P*, Tiwari N**, Chauhan AR***.
(* Third Year Resident, ** Assistant Professor, *** Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India).
We present a case of 24 year old woman with chronic portal vein thrombosis with splenomegaly with extrahepatic portal venous hypertension with bicytopenia, with a successful maternal and neonatal outcome.
Pregnancy is a hypercoagulable state. This may predispose to high chances of portal vein thrombosis (PVT), where a clot may partially or completely occlude the lumen of the portal vein. Management of pregnancy in portal cavernoma is difficult due to various co-morbidities. Mother and fetus have increased morbidity and mortality. Patient should be managed in higher center with multidisciplinary approach.
A 24-year-old primigravida with spontaneous conception presented to us at full term in early labor. She was a case of primary infertility under evaluation and on routine investigations, she had low platelet and WBC counts. Patient had no history of hematemesis, petechiae or any major illness. Her coagulation profile, fasting and postprandial sugar levels, thrombophilia profile, liver and renal function tests were normal. Bone marrow aspiration was suggestive of iron deficient reactive marrow and trephine bone biopsy was suggestive of hypercellular reactive marrow. Her abdominal ultrasonography was suggestive of gross splenomegaly with dilated portal vein with multiple collaterals, most probably due to extrahepatic portal vein hypertension. There was chronic portal vein thrombosis with large portal vein formation. Upper gastroscopy done was suggestive of small varix in esophagus with normal duodenum and pylorus. During pregnancy, she was started on tablet propranolol with iron and folic acid tablets.
During labor, platelet count was 35,000 /cu mm and WBC count 2100/cu mm. Hepato-portal Doppler was suggestive of portal cavernoma with splenomegaly and epigastric collaterals. Obstetric doppler was suggestive of early fetoplacental insufficiency. Her coagulation profile was deranged -her initial INR was 1.3, which further deteriorated to 2.2, hence 4 units of fresh frozen plasma (FFP) were given in early labor. During active stage of labor 8 units platelet and 4 units FFP were given and patient was monitored. Gastroenterologist and hematologist opinion was taken. Her labor progress was uneventful. She delivered a female child of 2.12 kg. Her postpartum period was uneventful. Post-delivery, platelet count was 61,000/ cu mm and TLC was 8000/cu mm. Propranolol was continued. Patient was discharged on day 6 post-delivery and was advised endoscopy and regular follow up with gastroenterologist.
Portal vein thrombosis (PVT) is classified into acute and chronic types. Acute portal vein thrombosis presents within 60 days with symptoms like abdominal pain or distention, diarrhea, nausea, vomiting, anorexia and fever with absence of portal carvernoma and portal hypertension. Chronic PVT is mostly asymptomatic and associated with portal cavernoma and/or complications of portal hypertension. PVT is also classified into complete and partial types, and each is subclassified based on the presence or absence of portal cavernoma. Patients with complete PVT have higher frequency of cavernous transformation than those with partial PVT. The common causes of PVT include cirrhosis, neoplasm, myeloproliferative disorders, inflammatory disorders like pancreatitis and thrombophilias. PVT is a relatively common complication of liver cirrhosis. With the stoppage of the portal venous blood flow, there is a compensatory mechanism of venous rescue due to rapid development of collaterals to bypass the obstruction which is called “cavernomatous transformation”. Because of cirrhosis and advanced liver disease pregnancy occurs rarely in these patients, but in non-cirrhotic portal hypertension, pregnancy can occur as liver function is preserved and patients are not infertile. Complications associated with these pregnancies are jaundice, jaundice with ascites, and gastrointestinal bleeding. In a normal pregnancy many physiological and hormonal changes lead to spider naevi due to hyperdynamic circulation which is similar to decompensated chronic liver disease.
Fetal and maternal outcomes are favorable for most pregnancies reaching gestation week 20. High platelet counts appear to increase the risk for unfavorable outcome. Pregnancy should not be contraindicated in stable PVT patients. Maternal outcome is excellent on anticoagulation due to limited thrombosis recurrence. However, in patients treated with anticoagulation, the rate of miscarriage and preterm birth appears to be increased. Also, post-cesarean section bleeding can be severe. Vaginal delivery and brief interruption of anticoagulation around delivery may reduce bleeding in these patients.
Prognosis of pregnancy with portal hypertension depends on the underlying cause and the degree of derangement of liver function. Termination of pregnancy should be considered in patients with recurrent hematemesis, deranged liver functions and decompensated cirrhosis, especially with abnormal coagulation profile. Maternal mortality in these patients ranges between 2 and 18% and is attributed to hematemesis, hepatic coma, postpartum hemorrhage; it is maximum with cirrhosis.
In pregnant PVT patients, maternal and fetal outcome is favorable with proper management, and pregnancy outcome is good in patients whose disease is well controlled prior to pregnancy. A multidisciplinary approach seems to be key to management. Elective cesarean section may be necessary in cases with digestive varices. However, vaginal delivery, with a passive second stage, seems to be relatively safe and less morbid when maternal and fetal tolerance permits.
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Deshmukh P, Tiwari N, Chauhan AR. Successful Management Of Pregnancy With Portal Cavernoma With Splenomegaly With Bicytopenia. JPGO 2017. Volume 4 No.7. Available from: http://www.jpgo.org/2017/07/successful-management-of-pregnancy-with.html