Malani K*, Samant P**, Thakur H S***, Bharthi S*.
(* Junior Resident, ** Academic Professor , *** Assistant Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)
Portal vein cavernoma is a rare disease which results from extrahepatic portal vein thrombosis and development of collateral venous circulation. An antenatal patient with this disease poses a challenge to the obstetrician because physiological hemodynamic changes associated with pregnancy that are needed to meet demands of the growing fetus may worsen the condition of the patient and put her at risk of life threatening complications. Here we present a case of successful vaginal delivery of a patient with portal vein cavernoma and chronic thrombocytopenia.
Portal cavernoma described first as phenomenon by Balfour and Stewart is thrombosis and varicose dilatation of portal vein leading to splenomegaly and ascites. The causes of which can be divided into two categories; local (70%) and systemic(30%). The most common presenting symptom is variceal bleeding followed by thrombosis, abdominal pain, jaundice, and splenomegaly. Pregnancy being a hypervolumeic state causes increase in portal flow, which in turn increases portal pressure, which gets transmitted to collateral circulation and leads to variceal bleeding. We are reporting this case for its rarity and successful outcome of pregnancy.
A 37 year old G5P2L2MTP1SA2 previous FTND was admitted at 24 weeks of gestation with chief complaints of giddiness, anorexia, generalized weakness and easy fatigability. She was investigated for the same, platelet count of 14,000/mm3 was detected on her routine investigations. She was transfused two units of random donor platelets. She was advised complete hemogram, bone marrow aspiration and bone marrow biopsy if platelet count was persistently below 30,000/mm3 by the hematologist. Bone marrow aspiration was done. It reported a normocellular marrow with increased megakaryocytes. Bone Marrow Biopsy detected a normocellular marrow with hematopoietic cells of all three series, consistent with megakaryocytic thrombocytopenia.
She was started on Injection methylprednisolone 1 g O.D. for three days followed by oral prednisolone and tapering of the dose was done every week by 5mg.
On obstetric ultrasonagraphy (USG) incidental finding of portal cavernoma of 3.4x4.5 cm was noted. Gastroenterologist was consulted and an upper G.I. scopy was performed. It diagnosed severe portal hypertension with gastropathy. Liver function tests were within normal limits. She had complaint of small joint pain for which β2 glycoprotein, ACLA, APLA IgG and IgM, ANA, anti–dsDNA were tested. Results were negative for each one of them.
She was transfused 4 units platelets in addition to previous two and was discharged at platelet count of more than 50,000/mm3. She was advised to follow up regularly with the hematologist, gastroenterologist and obstetrician.
USG for uteroplacental Doppler was done at 35 weeks of gestation. It was suggestive of fetoplacental insufficiency with oligohydrominos and increased S/D ratio. USG Abdomen was repeated. It showed an increased size of cavernoma of 7x6 cm, altered liver echotexture and mild splenomegaly, but there was no evidence of mesenteric vein thrombosis.
On examination she was clinically asymptomatic, hemodynamically stable with pulse of 88/min, B.P. Of 110/80 mm of Hg, and respiratory rate of 18/min. Obstetric examination indicated intrauterine growth retardation. FHS were within normal limits, and regular.
Her hemoglobin was 9.4 g/dl, platelet count was 40,000/mm3, and liver function tests were within normal limits.
Hematologist reviewed her and advised to continue oral prednisolone. They opined that there was no need of anticoagulation as she was already thrombocytopenic and did not have any history of prior anticoagulation. Gastroenterologist advised to monitor LFT, RFT and INR.
The possibility that large size of cavernoma (7x6 cm) at the time of Valsalva manuever in active labour may lead to rupture was informed by the fact that increase in intra-cavitatory pressure of caveroma will be redistributed to collaterals and hence the necessity of mode of termination of pregnancy only via caesarean section was ruled out.
The possibility of rupture of the large sized cavernoma (7x 6 cm) at the time of Valsalva maneuver in active labor was considered but the fact that increase in intra-cavitatory pressure of the caveroma would be redistributed to its collaterals thus preventing its rupture was concluded and hence the necessity of mode of termination of pregnancy only via cesarean section was ruled out. Alternate day NST done for fetal well being was reactive. In view of platelets being in the range of 40,000-45,000 /mm3, she was started on Injection Methylprednisolone 1 gm intravenous daily for 3 days. Complete hemogram showed an increase in the platelet count to 80,000 /mm3. She went into spontaneous labor and delivered vaginally a male child of 2.32 kg with an APGAR score of 9/10. She was transfused 4 units of random donor platelets intrapartum.
From the day of safe confinement till discharge as LFT’s remained within normal limits the gastroenterologist did not advise any additional intervention. Platelets at the time of discharge was 1.17 lacs/mm3. She was discharged on oral prednisolone on day 5 of normal delivery.
Portal vein cavernoma is a rare disease resulting from extra hepatic portal vein thrombosis. Due to cessation of portal venous blood flow, liver doesn’t get two thirds of its blood supply which is compensated by development of collateral blood vessels.
Cavernous transformation from portal vein thrombosis requires first 6 to 20 days after acute thrombosis of portal vein and completes within 3 to 5 weeks. Amusingly this occurs much more frequently in patients without an underlying liver disease and leads to portal hypertension because collaterals are not able to handle splenic and mesenteric blood flow.
Conversely, liver cirrhosis has rare chance of cavernoma formation because stasis of portal venous flow prevents formation of collaterals around the portal venous thrombus.
Common factors leading to cavernoma formation are liver cirrhosis, portal hypertension, prothrombotic tendencies like myeloproliferative disorders, antiphospholipid antibodies, anticardiolipin antibodies, thrombophilias, pregnancy, post partum period, and use of oral contraceptive pills.
Pregnancy being a prothrombotic condition predisposes to development of portal vein thrombosis and eventually to cavernoma formation.
Acute portal vein thrombosis presents with abdominal pain, nausea, fever, mostly reflecting the consequences of mesenteric venous thrombosis and resulting bowel ischemia. Chronic portal venous thrombosis presents with esophageal or gastric varices. Variceal bleeding may not be as common as believed because the increased blood volume flows within uteroplacental circulation without affecting portal system and cavernoma significantly. Hyperspleenism and consequent pancytopenia, severe anemia, hepatic decompensation leading to progressive hepatic and renal failure, hepatic encephalopathy, splenic artery aneurysm rupture, ascites and post partum hemorrhage can be seen in chronic portal venous thrombosis.
Treatment is either surgical or medical depending on symptoms. American association for liver disease (AASLD) recommends screening endoscopy in second trimester as that is the time of maximal increase in portal pressure in these patients.
For esophageal varices shunts and band ligation can be done. Medical treatment includes use of beta blockers to decrease flow in the portal circulation. Anticoagulation should be started if diagnosis is made early in pregnancy as it causes recanalization in 80% of cases. Maternal as well as fetal outcome is better with anticoagulation, but should be stopped before term to reduce the chances of excessive bleeding.
Pregnancy with portal cavernoma is a rare condition with very few reported cases. Regarding pregnancy outcome, if diagnosed and treated at a tertiary care hospital, both the fetal and maternal outcomes are good. There is no role of elective termination of pregnancy nor the need to terminate the pregnancy via cesarean section. Cesarean section is indicated only for obstetric or fetal indication. Second stage of labor does not increase chances of gastrointestinal bleeding. In patients with high chances of variceal bleeding, second stage can be cut short with operative vaginal delivery. Pregnancy is not a contraindication, however, periconceptional counseling should be done for such patients and maternal and fetal risks, consequences and complications should be explained.
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Malani K, Samant PY, Thakur HS, Bharthi S. A Rare Case Of Portal Vein Cavernoma In An Antenatal Patient. JPGO 2019. Volume 6 No.1. Available from: https://www.jpgo.org/2019/01/a-rare-case-of-portal-vein-cavernoma-in.html