Pregnancy in patients with liver cirrhosis is uncommon but poses risks to mother and fetus. Common causes of hepatic cirrhosis in childbearing women include congenital, metabolic, autoimmune, and infectious etiologies. Perinatal outcomes in women with cirrhosis are limited to case reports and small studies . Portal hypertension and esophageal varices occur more frequently than in the non-pregnant state and carry a 78%  risk of clinically significant bleeding. In the event of bleeding, mortality approaches 50% . Infants of cirrhotic mothers have an increased risk of death, prematurity, and growth restriction .
Little has been mentioned regarding thrombocytopenia or placental abruption in pregnant women with cirrhosis. We present a patient with Child-Turcotte-Pugh class A cirrhosis complicated by large esophageal varices, severe thrombocytopenia, and placental abruption.
An 18-year-old primigravida was transferred to our institution at 20 weeks’ gestation due to severe thrombocytopenia. Prenatal care, established in the first trimester, was without previous complications. She denied a history of easy bruising, bleeding, or menorrhagia, and she had no pertinent family history.
Her vital signs were normal. A large abdominal mass in the left upper quadrant extended into the left lower quadrant. She had no petechiae or peripheral edema. Laboratory studies revealed leukopenia (2200/mm3) and thrombocytopenia (37,000/mm3, which reached a nadir at 22,000/mm3). Her aspartate aminotransferase and alanine aminotransferase were 49 and 120 U/L, respectively, and her international normalized ratio was 1.4. Partial thromboplastin time was normal (27.2 s). Abdominal ultrasound demonstrated hepatic cirrhosis with mild-to-moderate ascites, splenomegaly (23.2 cm in largest diameter), and portal hypertension, with recanalization of the periumbilical veins, enlargement of the portal vein, and porto-systemic collaterals, but no flow reversal in portal veins. Screening esophagogastroduodenoscopy confirmed large esophageal varices. She was diagnosed with Child-Turcotte-Pugh class A cirrhosis. Obstetric ultrasound noted an appropriately grown fetus without anomalies.
A comprehensive work-up was negative for viral and autoimmune hepatitis, Wilson’s disease, hemochromatosis, and α-1 anti-trypsin deficiency. Liver biopsy was deferred until after delivery. β-Blocker therapy (propanolol 10 mg PO TID) was initiated for primary prophylaxis of esophageal bleeding and was tolerated well. Surgical banding was deemed too high risk in the setting of severe thrombocytopenia. The patient was hospitalized for the duration of her pregnancy. She received 12 mg of betamethasone intramuscularly in two doses, 24 h apart, at 24 weeks’ gestation.
At 24 3/7 weeks’ gestation, the patient noted moderate vaginal bleeding. Her abdomen was nontender and the speculum exam revealed a visually closed cervical os, small clot, and no active bleeding. The following day, when she reported significant vaginal bleeding, speculum exam revealed approximately 40 mL of blood in the vagina, and transvaginal ultrasound noted an anterior placenta, without a previa, and suggested placental separation at the placental edge. Continuous fetal monitoring was initiated. At 24 6/7 weeks’ gestation, the patient underwent an emergency classical cesarean delivery due to a prolonged fetal deceleration. A viable female infant was delivered weighing 760 g, with Apgar scores of 2 and 6 at 1 and 5 min, respectively. The estimated blood loss was 850 mL. Amniotic fluid at delivery was blood-tinged, and the placenta was loosely adherent to the uterine wall. A retroplacental clot was seen. Final placental pathology showed a 160-g placenta with increased neovascularization, atherosis, and fibrinoid necrosis, but without infarcts.
Postoperatively, the patient was transfused with 2 U of packed red blood cells, 2 U of fresh frozen plasma, and 3 U of platelets. A liver biopsy on postpartum day 8 confirmed the diagnosis of cirrhosis without eliciting an etiology. At 5 months postpartum, the patient was doing well without further hepatic decompensation. The neonate did not develop any complications in the days after delivery. Her platelet count was normal. Long-term, the baby has moderate persistent asthma, retinopathy of prematurity, and history of atrial septal defect with pulmonary stenosis.
Cirrhosis in pregnancy is rare, with an incidence of <0.0045%. Our patient presented with Child-Turcotte-Pugh class A cirrhosis without known risk factors, prior medical history, or symptoms of liver disease. Her presenting finding was severe thrombocytopenia. This key finding influenced her pregnancy management and, we believe, likely contributed to the outcome of her pregnancy.
Band ligation and β blockade are the two most effective options for primary prophylaxis of esophageal bleeding in patients with portal hypertension . Although studies suggest similar bleeding and mortality rates for the two methods, the optimal treatment, especially in the setting of pregnancy, is debated . β-Blocker therapy is presumed to decrease the hepatic venous pressure gradient by decreasing portal venous inflow through mesenteric arteriolar vasoconstriction and, at higher doses, by decreasing heart rate and cardiac output. Benefits of this therapy include widespread availability, relatively low cost, and minimal invasiveness . Variceal band ligation acts locally by obliterating the esophageal vessels through a process of inflammation and scar formation. The risks of this procedure include bleeding, bacteremia, esophageal perforation or stricture, and the near-universal need for a repeat ligation procedure to allow for sufficient fibrosis to achieve a clinical effect . Additionally, the fetus is necessarily exposed to anesthetic agents administered during the procedure, with concern for neonatal resuscitation in the event of imminent delivery.
Our patient ultimately delivered prematurely due to a symptomatic placental abruption. Shaheen and Myers  describe a significantly increased risk of placental abruption in women with cirrhosis (7.1 vs. 1.7%, p<0.0001) . This may relate to gestational hypertension . Our patient was normotensive throughout her pregnancy, making this theory an unlikely explanation for her pregnancy outcome. Similarly, β-blocker therapy initiated 3 weeks prior is unlikely to have caused this event. We suspect that her profound thrombocytopenia may have played a large role. Parnas et al.  noted that pregnant patients with thrombocytopenia had a significantly higher rate of placental abruption (8.5% vs. 1.5%, P=0.001) compared to women with normocytopenia. Of patients with severe thrombocytopenia (<50,000 platelets), 23% experienced an adverse outcome compared to 16.2% of patients with platelet counts of 50,000 to 100,000. Etiologies of thrombocytopenia unrelated to pregnancy (e.g., familial thrombotic thrombocytopenic purpura, myeloproliferative disease, etc.) were correlated with worse pregnancy outcomes .
In conclusion, in addition to the high risk of esophageal variceal hemorrhage associated with cirrhosis in pregnancy, physicians should be aware of the increased risk for placental abruption. Limited studies seem to suggest that the more severe the cirrhotic disease, the worse the pregnancy outcomes and potential maternal complications. Future studies may assist in risk stratification based on severity of cirrhotic disease, which may lead to more specific management recommendations regarding timing of inpatient management, parameters for β blockade, or counseling regarding pregnancy termination. In all scenarios, a multi-disciplinary approach is recommended.
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About the article
Published Online: 2013-10-11
Published in Print: 2014-06-01
The authors stated that there are no conflicts of interest regarding the publication of this article.