Sikarwar R*, Panchbudhe S**, Satia M ***.
(*Second Year Resident, **Assistant Professor, *** Professor, Department of Obstetrics and Gynecology, Seth G. S. Medical College and K.E.M. Hospital, Mumbai, India.)
Pregnancy with portal hypertension presents a challenge to the obstetrician due to physiological hemodynamic changes to meet the needs of growing fetus thereby putting mother at risk of variceal hemorrhage. The management revolves around managing portal hypertension and its related complications. We present a case of 34 year old woman who was a diagnosed case of non cirrhotic portal fibrosis with thrombocytopenia, with a successful maternal and neonatal outcome.
Portal hypertension in pregnancy can be due to multiple causes. In western countries cirrhosis is most common cause and pregnancy is rare in these patients due to hepato-cellular damage causing amenorrhea and infertility. In developing countries, non cirrhotic portal hypertension is more common and as liver function is preserved, fertility remains unaffected. The management starts from preconceptional period to delivery and postpartum period, and requires a multidisciplinary team approach involving obstetrician, hepatologist and neonatologist at tertiary care center.
A 34 year old, married since 3 years Gravida 2, Para 1, with previous intrauterine fetal demise, 36 weeks and 6 days by dates, a diagnosed case of non cirrhotic portal fibrosis with portal hypertension, presented to the receiving room in early labor.
Patient first presented four years back in casualty with two episodes of hematemesis. She was admitted and thoroughly investigated at that time to rule out causes of upper gastrointestinal (GI) bleeding, and she was transfused with three units packed cell volume in view of acute blood loss. Endoscopy was done suggestive of esophageal varices for which banding was done. Patient was asymptomatic for two years. She again had similar episode for which upper gastrointestinal scopy was repeated, suggestive of grade II esophageal varices for which patient underwent endoscopic variceal ligation. Patient was again transfused with 2 units packed cell volume and started on non selective beta blocker (tab propranolol 20 mg daily) for primary prophylaxis. Patient underwent trans-jugular liver biopsy one year ago which was suggestive of liver cirrhosis with mild fibrosis and expansion of portal areas (score 2/6). Following variceal ligation patient did not have similar episodes again.
Antenatal registration was done at 16 weeks of gestation at which time she was admitted for evaluation of portal hypertension with thrombocytopenia. Hematology opinion was sought for thrombocytopenia; bone marrow biopsy was performed which was suggestive of mild hypo-cellularity. Gastroenterologist advised hepato- portal Doppler along with second trimester upper GI endoscopy. She was advised to continue tab propranolol 20 mg daily. Hepato- portal Doppler was suggestive of liver parenchymal disease with normal flow in superior and inferior vena cava with massive splenomegaly; patient refused upper GI endoscopy and was subsequently discharged.
She was readmitted at 33- 34 of gestation for evaluation of thrombocytopenia as platelet count was 40,000/ cmm with massive splenomegaly. GI medicine opinion was re taken and she was advised to continue with tablet propranolol 20 mg once daily; platelet transfusion was planned for peripartum period, and she was discharged. During her antenatal period she never complained of hematmesis or melena or bleeding from any other site; liver function tests were within normal range throughout pregnancy.
Patient presented in latent labour with complaint of pain in abdomen. All routine investigations including coagulation profile were in normal range except for platelet count which was 37,000/ cmm for which hematologists' advised to keep platelets available and transfuse 4 units of platelets at the time of delivery. Her labor progress was uneventful and before platelets could be arranged she delivered a male child of 2.39 kg. She did not have postpartum hemorrhage and was transfused with 1 unit single donor platelet. Her postpartum period was uneventful. Post delivery, she was advised endoscopy and regular follow up with gastroenterologist. Post delivery platelet count was 70,000/ cmm and as she was not actively bleeding no active intervention was needed. Patient was discharged on day 7 post delivery.
In developing countries non cirrhotic causes of portal hypertension are more common due to liver cirrhosis. The various causes of non cirrhotic portal hypertension are extra hepatic portal venous obstruction (EHPVO), non cirrhotic portal fibrosis (NCPF), portal vein thrombosis, Budd -Chiari syndrome and infections. Prognosis of portal hypertension during pregnancy depends on the underlying cause and the degree of derangement of liver function. EHPVO and NCPF have good maternal prognosis and pregnancy is not contraindicated in this group of patients. 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, and is maximum with cirrhosis. The causes of perinatal mortality are prematurity or intrauterine growth restriction (IUGR) seen in 11% and 18%. Various physiological changes during pregnancy like increased plasma volume, increased cardiac output along with decreased peripheral vascular resistance contributes to hyper dynamic state and increased blood flow in collaterals thereby increasing risk of variceal hemorrhage. Hematemesis during pregnancy is attributed to increased portal pressures during pregnancy, reflux esophagitis and obstruction to the inferior vena cava by the gravid uterus, the incidence being 7% in patients with EHPVO and NCPF. The timing and severity of variceal rupture and hematemesis is however unpredictable and 75% of patients with varices bleed during pregnancy. Variceal bleeding can occur at all stages of pregnancy, and is more common in second and third trimester with greatest risk in the second stage of labour. Presence of large varices, endoscopic red signs, history of preconceptional variceal bleed, undiagnosed varices are predictors of esophageal bleeding. Management comprises of immediate resuscitation and stabilisation of mother and immediate treatment of varices by endoscopic variceal ligation. The other treatment modalities include endoscopic sclerotherapy and medical management, with use of vasopressors like octreotide (category B drug). Pregnant patients who are at risk of variceal bleeding should receive primary prophylaxis either by endoscopic variceal ligation or beta-blockers like propranolol, nodolol (category C drug). Trans jugular intra hepatic portal shunt i.e. TIPS is reserved for patients when medical and endoscopic procedures fail to control variceal bleeding and is associated with risk of radiation exposure.
Management of portal hypertension during pregnancy is similar to that in non-pregnant state. Patients with EHPVO and NCPF generally tolerate labor well and cesarean section is reserved for obstetric indication. Adequate amount of blood should be cross matched and Sengstaken-Blackmore tube should be readily available. American association for study of liver disease recommends screening endoscopy in second trimester.
Hepatic decompensation can occur at any stage of pregnancy and precipitating factors include variceal bleed, infections, drugs or hypotension. Post partum hemorrhage develops in 7- 11% of patients which can be due to coagulopathy or thrombocytopenia.
Non-cirrhotic portal hypertension (NCPH) is commonly seen as a most important causative factor for portal hypertension in developing countries. Research has shown that reproductive function is nearly normal and outcome of pregnancy is good for NCPH compared with cirrhosis. Though patients with NCPH have normal fertility and no increase in the incidence of hematemesis in pregnancy, they have an increased incidence of SGA babies.
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Sikarwar R, Panchbudhe S, Satia M. Successful Maternal And Fetal Outcome In A Case Of Non Cirrhotic Portal Hypertension In Pregnancy. JPGO 2017. Volume 4 No. 1. Available from: http://www.jpgo.org/2017/01/successful-maternal-and-fetal-outcome.html