Mayadeo NM**, K***,
Mirchandani A***. Mali
(* Fourth Year Resident, ** Professor, *** Assistant Professor. Department of Obstetrics and Gynecology, Seth GS
College & KEM Hospital, ) Mumbai,
Portal pressure physiologically increases in pregnancy, leading to exacerbation of preexisting portal hypertension, thereby causing esophageal varices and variceal hemorrhage. Portal vein thrombosis is an uncommon cause of portal hypertension complicating pregnancy. The coexistence of the manifestations of portal hypertension as well as hypersplenism and esophageal varices together with Factor V deficiency during pregnancy is a rare presentation and poses a real challenge for diagnosis and management. We report the clinical follow-up of a 25-year-old pregnant woman who is a known case of Factor V Leiden deficiency and portal vein thrombosis with cavernoma formation, for which she underwent splenectomy and lieno-renal shunt placement 5 years before conception. Antenatal period was uncomplicated and both maternal and fetal outcome was good.
Portal venous cavernoma secondary to portal venous thrombosis is a rare entity and presents with various complications to both mother and fetus. Literature regarding its management protocol presents with various conflicts. Hence we present this unique case and its antenatal management.
A 25 year old primigravida who was a known case of extrahepatic portal venous obstruction was registered at our antenatal clinic from first trimester. From the age of 5, she was diagnosed with portal venous obstruction. At the time of diagnosis a splenoportogram showed portal hypertension with portal vein thrombosis with left gastric and short gastric varices with portal cavernomas. She had an uneventful course till the age of 20 years when she presented with acute abdomen, hematemesis, epistaxis, hematochezia and hematuria. CT angiography showed extrahepatic portal venous obstruction with multiple porta-systemic collaterals and splenomegaly with thrombosis of the main portal vein. On examination she had pallor and massive splenomegaly. Hemogram showed pancytopenia, following which bone marrow examination was done which revealed features of hypersplenism with dimorphic anemia and thrombocytopenia. Subsequently, splenectomy was done with proximal lieno-renal shunt placement. Blood counts improved in the post operative period. One year later, hepatoportal system Doppler showed cavernoma formation with patent lieno-renal shunt. Thrombophilia work up revealed Factor V Leiden deficieny, following which she received oral warfarin for six months. After 5 years, she conceived spontaneously and her antenatal period was uneventful and she was started on oral aspirin prophylactically from second trimester which was continued till 36 weeks. No prophylactic anticoagulant was given. Her hemogram, liver function tests (LFT) and coagulogram were monitored in each trimester and found to be normal. She was normotensive throughout pregnancy and had no evidence of gastrointestinal bleed. Anti-phospholipid antibody (APLA) and rest of thrombophila work up was normal. Regular sonography showed adequate growth of the baby. She was initially planned for trial of vaginal birth, but at 37 weeks she presented in labor and emergency cesarean section was done in view of oblique lie. She delivered a baby girl of 2.5 kg with APGAR scores of 8, 9 and there was no intra-operative complication. Post operative period was uneventful.
Extra hepatic portal vein obstruction (EHPVO) is a common cause of portal hypertension in the developing countries and second to cirrhosis in developed countries. Common presentations of this condition are variceal bleed, recurrent thrombosis and hypersplenism. EHPVO can either present as acute (recent) or chronic. Recent EHPVO usually presents with abdominal pain, ascites, fever, and jaundice whereas chronic EHPVO presents with repeated, well tolerated bleeding from esophageal varices. The outcome of chronic portal venous thrombosis (PVT) has improved over the past years.
Because of intensified prophylaxis for bleeding related to portal hypertension and control of underlying prothrombotic conditions survival among women with PVT has increased remarkably. As a result, desire for pregnancy has become a major issue for young women with well-controlled chronic PVT. Thrombosis is the leading cause of maternal morbidity during pregnancy in a case of portal hypertension as in most of the cases there is underlying thrombogenic condition.[4,5]
The mode of delivery did not appear to influence the risk of bleeding. As a cesarean section is associated with a significantly increased risk of thromboembolic complications, which may be risky in patients with portal hypertension, it seems safe to recommend vaginal delivery whenever possible, restricting cesarean section to obstetrical indications. In PVT patients with prothrombotic conditions, or with thrombosis involving the mesenteric veins, who are at greater risk of intestinal ischemia, anticoagulation therapy can be considered during pregnancy, as recommended elsewhere for patients with other forms of thrombosis. Management strategy is based on a systematic portal hypertension screening before pregnancy in known cases of chronic EHPVO. Prophylaxis of portal hypertension bleeding/ thrombosis and interruption of anticoagulation for a short duration at delivery are required. In acute cases management can be surgical or medical. Surgical treatment includes shunts and band ligation of esophageal varices. Medical treatment includes use of β-blockers to decrease the flow in portal circulation. Index patient was a chronic case with pre-pregnancy USG Doppler showing normal flow and patent shunt. Hence we did not advice prophylactic anticoagulation in this case. Feto-maternal outcome was favorable and post operative course was uneventful.
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A. Portal Venous Thrombosis And Related
Complications In Pregnancy: Management. JPGO 2015. Volume 2 No. 2. Available
from: http://www.jpgo.org/2015/02/portal-venous-thrombosis-and-related.html Mayadeo NM,