Author
Information
Sarkar P*,
Mayadeo NM **, Mali
(* Fourth Year Resident, ** Professor, *** Assistant Professor. Department
of Obstetrics and Gynecology, Seth GS Medical
College & KEM Hospital , Mumbai ,
India
Abstract
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.
Introduction
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.
Case
Report
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.
Discussion
Extra hepatic portal
vein obstruction (EHPVO) is a common cause of portal hypertension in the
developing countries and second to cirrhosis in developed countries.[1]
Common presentations of this condition are variceal bleed, recurrent thrombosis
and hypersplenism.[2] 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.[2]
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.[3] 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.[7]
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.
References
- Aggarwal N, Chopra S, Raveendran A, et al: Extra hepatic portal vein obstruction and pregnancy outcome: largest reported experience. J Obstet Gynaecol Res 2011;37:575-80.
- Hoekstra J, Seijo S, Rautou PE, et al: Pregnancy in women with portal vein thrombosis: results of a multicentric European study on maternal and fetal management and outcome. J Hepatol 2012;57:1214-9.
- Cheng YS: Pregnancy in liver cirrhosis and/or portal hypertension. Am J Obstet Gynecol 1977;128:812-22.
- Griesshammer M, Grunewald M, Michiels JJ: Acquired thrombophilia in pregnancy: essential thrombocythemia. Semin Thromb Hemost 2003;29:205-12.
- James AH, Jamison MG, Brancazio LR, et al: Venous thromboembolism during pregnancy and the postpartum period: incidence, risk factors, and mortality. Am J Obstet Gynecol 2006;194:1311-5.
- Kochhar R, Kumar S, Goel RC, et al: Pregnancy and its outcome in patients with noncirrhotic portal hypertension. Dig Dis Sci 1999;44:1356-61.
- Tsochatzis EA, Senzolo M, Germani G, et al: Systematic review: portal vein thrombosis in cirrhosis. Aliment Pharmacol Ther 2010;31:366-74.
Citation
Sarkar P, Mayadeo NM ,
Mali