Controversies in the delivery suite: obstetrical anesthesia for the parturient with cavernous transformation of the portal vein

Introduction Cavernous transformation of the portal vein (CTPV) is a rare disorder resulting from extrahepatic portal vein thrombosis and development of collateral venous circulation.Case report I herein present the first reported case of a pregnant patient with CTPV associated with persistent consumptive coagulopathy and chronic thrombocytopenia.Outcome Single-dose spinal anesthesia was safely conducted for an uneventful cesarean delivery.     

The Sugiura procedure for patients with hemorrhagic portal hypertension secondary to extrahepatic portal vein thrombosis

The Sugiura procedure (SP) was performed upon 27 patients with hemorrhagic portal hypertension secondary to extrahepatic portal vein thrombosis (EPVT) without associated hepatic disease. There were 14 female and 13 male patients. The mean age was 28 +/- 14 years. The causes of EPVT were protein C deficiency in two; antithrombin III deficiency in one patient, a history of omphalitis in two patients, a history of pancreatitis in one patient and idiopathy in 21 patients. The SP was completed in two surgical stages in 14 patients and in one stage in nine. There was one operative death. One patient had mild postoperative encephalopathy, and two patients rebled at long term follow-up study. Actuarial survival rate was 82 per cent at five and ten years. It is concluded that the SP is a good alternative for the management of hemorrhagic portal hypertension secondary to EPVT.     

Hepatic vein and portal vein Doppler ultrasound of maternal liver in normal pregnancy

Objective: The purpose of our study was to evaluate the changes occurring in maximum flow velocity and waveform patterns of the portal and hepatic veins during pregnancy in second and third trimesters.

Methods: A total of 204 patients were randomly divided into the study and control groups. The control group was classified as group I; the study group was divided into two groups: group II, second trimester pregnancy (14–26 weeks, n?=?91) and group III, third trimester pregnancy (26–40 weeks, n?=?63). Vein waveforms were classified as triphasic, biphasic and monophasic.

Results: Maximum flow velocities of both right hepatic vein and main portal veins were lower in group III compared with group I and group II (p?<?0.05). On the basis of hepatic vein and portal vein waveforms, the incidence of biphasic pattern was prominent in group II (p?<?0.05) whereas the monophasic pattern was prominent in group III (p?<?0.05).

Conclusion: The results demonstrate that as gestational age progresses, maximum flow velocities of both right hepatic vein and main portal veins intend to be decreased with an increased incidence of monophasic wave form pattern, and may be accepted as sensitive parameters of indicators of physiological adaptations related to pregnancy.     

Outcomes of pregnancies complicated by liver cirrhosis,portal hypertension,or esophageal varices

Objective: To evaluate pregnancy outcomes in women with liver cirrhosis, portal hypertension, or esophageal varices.

Study design: We analyzed a retrospective cohort of 2?284?218 pregnancies in 2005–2009 recorded in the California Birth Registry database. Utilizing ICD-9 codes we analyzed the following outcomes for liver cirrhosis, portal hypertension, or esophageal varices in pregnancy: preeclampsia (PET), preterm delivery (PTD; <37 weeks), cesarean section, low birth weight (LBW; <2500?g), small for gestational age (SGA; <10th percentile), neonatal death (NND), and postpartum hemorrhage (PPH).

Results: Cirrhosis in pregnancy conferred an increased risk of PET, PTD, CS in multiparous women, LBW, and NND. Portal hypertension in pregnancy was associated with PTD, LBW, NND, and PPH. Non-bleeding esophageal varices in pregnancy were not associated with the outcomes assessed in a statistically significant manner. One case of bleeding esophageal varices was observed, resulting in PTD with a LBW infant. There were three cases of concomitant portal hypertension or concomitant esophageal varices with cirrhosis in pregnancy.

Conclusion: Pregnancy in women with concomitant liver cirrhosis, portal hypertension, or esophageal varices can be successful. However, pregnancy outcomes are worse and may warrant closer antenatal monitoring and patient counseling. Cirrhosis in pregnancy with concomitant portal hypertension or esophageal varices is rare.     

Thalassemia intermedia and cavernous transformation of portal vein thrombosis in pregnancy

We report a rare case of a cavernous transformation of portal vein (CTPV) thrombosis accompanied by Thalassemia and thrombophilia during pregnancy that was successfully treated by low molecular weight heparin. The clinical presentation, diagnosis and the treatment are discussed.     

Essential thrombocythemia and pregnancy

Essential thrombocythaemia (ET) is an acquired myeloproliferative neoplasm, characterised by persistent thrombocytosis and a tendency for either thrombosis or haemorrhage. Among myeloproliferative neoplasms, ET is the most prevalent in young women, which constitute a special group due to their childbearing potential. An increased risk of fetal and maternal complications has been demonstrated in patients with ET. The most common pregnancy-related complication is spontaneous abortion during the first trimester. Recurrent abortion, fetal growth restriction, stillbirth and placental abruption are less frequent. Maternal complications are relatively rare and essentially represented by thromboembolic and bleeding events. Here we summarize the literature describing pregnancy and its outcome in patients with ET and discuss some recommendations for the management of pregnancy.     

Pregnancy in cirrhotic and noncirrhotic portal hypertension.

The course of pregnancy in 1 patient with chronic active hepatitis (CAH) and cirrhosis, and another with extrahepatic portal vein obstruction (EHPVO) is described. The management of pregnancy in these diseases associated with portal hypertension is discussed and risks of pregnancy are compared. The patient with CAH presented with anovulatory cycles, and ovulation occurred following immunosuppressive therapy. Both women experienced massive upper gastrointestinal bleeding from esophageal varices. Bleeding was difficult to control and required variceal ligation in 1. Both patients manifested features suggesting cerebral edema indicating the need for caution with fluid and electrolyte therapy. Recovery of the woman with CAH after termination of pregnancy was slow. Review of literature demonstrated that variceal bleeding occurred in 43% of women with EHPVO compared to 23% of those with CAH and cirrhosis. Additional complications including hepatocellular failure (24%) occurred in patients with CAH but not in EHPVO. The management of pregnancy in portal hypertension and advice for contraception or sterilization are discussed.     

Non-cirrhotic portal hypertension in pregnancy.

OBJECTIVE: To study the outcome of pregnancy in women with non-cirrhotic portal hypertension (NCPH). METHOD: A retrospective analysis of 50 pregnancies in 27 women with NCPH was carried out. Pregnancy outcome was compared in extra hepatic portal vein obstruction (EHPVO) and non-cirrhotic portal fibrosis (NCPF). RESULTS: The mean maternal age was 24.60+/-2.857 years, and the disease was diagnosed during pregnancy in 15 (55.6%) patients. Variceal bleeding occurred in 17/50 (34%) pregnancies and the majority (88.2%) of them responded to endoscopic sclerotherapy. Incidence of variceal bleeding during pregnancy was lower in pregnancies where the disease was diagnosed prior to pregnancy (8.6%), and it was 43.5% in EHPVO and 25.9% in NCPF. The mean birth weight of the neonates was 2668.4+/-427.42 g, and the incidence of abortion, prematurity, small for gestational age babies and perinatal death was 20, 17.5, 12.5 and 20%, respectively. Variceal bleeding during pregnancy was associated with a higher incidence of abortion (29.4%) and perinatal death (33.3%). CONCLUSION: Variceal bleeding is the most common complication in pregnancies with NCPH. Pregnancies can be allowed and managed successfully in patients with NCPH.     

The successful management of portal vein thrombosis after hepatic transplantation with a splenorenal shunt

Portal vein thrombosis is an infrequent complication after hepatic transplantation, but is quite dramatic when it occurs. It is usually managed by retransplantation with a significant mortality rate. We present a patient in whom portal vein thrombosis after hepatic transplantation was ultimately managed by a splenorenal shunt. The portal vein thrombosis was manifested by bleeding esophageal varices and, yet, normal hepatic function obviated the need for a new graft (one was not readily available). To the best of our knowledge, this is the first presentation of a patient with a transplant of the liver with acute portal vein occlusion and maintained hepatic function who has been successfully managed by a portosystemic shunt.     

Successful pregnancy in a woman with secondary biliary cirrhosis with portal hypertension from recurrent pyogenic cholangitis. A case report.

BACKGROUND: Pregnancy in women with secondary biliary cirrhosis due to recurrent pyogenic cholangitis is extremely rare. Little information is available on the effect of pregnancy on the disease and vice versa. CASE: A patient who had secondary biliary cirrhosis due to recurrent pyogenic cholangitis complicated by splenomegaly and portal hypertension had a successful pregnancy. Although she had a history of esophageal variceal bleeding before this pregnancy, there was no such bleeding during pregnancy. She had an uneventful antenatal course except that her liver enzyme level fluctuated slightly. The serum bilirubin level increased during the third trimester of pregnancy but returned to the prepregnant level after delivery. CONCLUSION: Termination of pregnancy may not be the only option for management. The management protocol for patients with primary biliary cirrhosis complicating pregnancy, which includes regular fetal surveillance and monitoring of maternal liver function, should be considered for pregnant women with secondary biliary cirrhosis.     

Non-cirrhotic portal hypertension and pregnancy outcome

OBJECTIVE: Non-cirrhotic portal hypertension (NCPH) is a common cause of portal hypertension in developing countries, especially Asia. Recent data have shown near-normal reproductive function and good pregnancy outcome for NCPH compared with cirrhosis. The aim of the present study was to evaluate complications during pregnancy and pregnancy outcome in women with NCPH. METHODS: Twelve pregnancies in five patients with NCPH were evaluated. RESULTS: In the present study, 20% of patients had hematemesis during pregnancy that was managed successfully. Four patients (80%) had severe anemia (hemoglobin 6-7 g%) and three (60%) had thrombocytopenia. There were no preterm births. Of a total of nine live births, six were vaginal deliveries and three were delivered by Cesarean section (performed for obstetric indications). Four babies (44%) were small for gestational age (SGA). CONCLUSIONS: The results show that patients with NCPH have normal fertility, no apparent increase in the incidence of hematemesis during pregnancy, an increased incidence of SGA babies and no indication for elective Cesarean delivery.     

Atypical abdominal pain in the first trimester of pregnancy

We report the case of a 22-year-old woman who presented a violent epigastric pain at eight-weeks gestation. Superior mesenteric vein thrombosis was detected, with an extension to portal vein and remaining blood flow. Screening for thrombophilia revealed a heterozygote prothrombin gene mutation. Portal vein thrombosis is uncommon and difficult to diagnose. Diagnosis is made by Doppler ultrasound, a second intention test to be done in case of unusual upper abdominal pain during pregnancy.     

Management of portal venous thrombosis in hepatic transplant recipients

Portal venous thrombosis in hepatic transplant candidates is considered a relative contraindication to transplantation. In addition to thrombectomy, which is often technically impossible, donor portal venous arterialization or extra-anatomic venous bypass have been described. Two patients who underwent portal venous resection and subsequent anatomic reconstruction are presented herein. In the first patient, a graft with donor common iliac vein was interposed, and in the second, the donor portal vein was long enough to be anastomosed to the mesentericosplenic venous confluens. One patient is well 12 months after transplantation with patent portal vein and the other died of fungal sepsis after rejection treatment (the portal vein being open and unobstructed at autopsy).     

Pregnancy and delivery in women with esophageal varices due to hepatic vein thrombosis

Aim: Retrospective analysis of the course of pregnancy, labor and mode of anesthesia in women with portal hypertension and esophageal varices induced by portal vein thrombosis.

Material: From 2000 to 2012 seven pregnant were admitted. None had liver transplantation (Ltx), the varicose have been in the 1st stage. Each of them has been consulted by the obstetrician, transplant surgeon and anesthetist. The patient condition during pregnancy, labor and postpartum period was analyzed.

Results: Pregnancy in five cases proceeded physiologically. In one threatening miscarriage was diagnosed and treated with gestagens, two patients had tocolytic. One required variceal banding twice. In three thrombocytopenia worsened, with platelet count Conclusion: Patients with portal hypertension can deliver at term. It is a high-risk pregnancy. In this group it is desirable to shorten the second stage of labor or complete it by c-section under general anesthesia with remifentanyl which allows getting desired analgesia without complications in the newborn. Surveillance of pregnant with portal hypertension must include monitoring of liver function and coagulation disorders.     

Thrombosis of the portal vein following distal splenorenal shunt.

Portal vein thrombosis with a patent shunt is a distinct clinical entity which can follow selective distal splenorenal shunt and should be looked for in patients in whom ascites and abdominal pain develop postoperatively. Possible mechanisms include an increase in blood viscosity and a decrease in portal flow which may be aggravated by inadequate devascularization.     

妊娠合并慢性骨髓增殖性疾病11例临床分析

目的 探对妊娠合并慢性骨髓增殖性疾病(CMPD)的临床特点及处理、妊娠结局及预后.方法 回顾性分析北京大学人民医院妇产科2000-2009年收治的11例妊娠合并CMPD患者[其中原发性血小板增多症(ET)5例,原发性骨髓纤维化(PMF)1例,慢性粒细胞白血病(CML)5例]的临床资料.结果 11例妊娠合并CMPD患者共妊娠12例次.(1)有规律产前检查者5例次,均行定期血常规等检查,适当给予抗凝治疗,预防并发症发生.其中1例PMF患者为孕前诊断,初次妊娠至32周时发生轻度子痫前期及胎死宫内;2年后再次妊娠,孕早期筛查抗β2糖蛋白Ⅰ抗体升高,给予小剂量阿司匹林口服及低分子肝素抗凝治疗,孕期顺利、足月分娩.(2)无规律产前检查者5例次,其中ET患者2例次,CML患者3例次.1例ET患者于妊娠25周发生重度子痫前期,脐动脉舒张期血流反向,经解痉、降压及抗凝治疗效果不佳,因血压进行性升高、胎盘早剥而紧急行剖官产术终止妊娠,胎儿娩出即死亡.从未产前检查者2例次,均因临产入院,诊断为CML.(3)合并羊水过少4例次,子痫前期3例次,其中重度子痫前期2例次,轻度子痫前期1例次,所有患者均无严重出血及血栓形成等并发症发生.(4)妊娠至足月者8例次,其中4例次剖宫产分娩;4例次阴道分娩.早产2例次,均因羊水进行性减少以剖宫产术终止妊娠.分娩的10例新生儿出生体质量1820～3600 g,除1例并发重度子痫前期者出现胎儿生长受限(FGR)外,其余均为适于胎龄儿.(5)11例患者妊娠期间原有疾病均病情稳定,其中3例CML患者妊娠晚期口服羟基脲治疗,4例ET患者及1例CML患者终止妊娠前进行血小板单采术治疗,效果良好.除1例CML患者分娩后5个月因疾病进展死亡外,余患者产后病情均平稳.结论 妊娠合并CMPD患者多数妊娠结局良好,孕期管理需警惕血栓形成、子痫前期、胎死官内、FGR等并发症.合理应用抗凝等治疗,有助改善母儿结局.     

Pregnancy following the surgical treatment of portal hypertension

The course and results of 10 pregnancies in 7 patients who had undergone surgical treatment up to 9 years previously for portal hypertension are described. Four patients had extrahepatic portal hypertension, two had noncirrhotic intrahepatic portal hypertension, and one had frank biliary cirrhosis. Eight live babies were produced with no maternal deaths; the commonest complication was anemia during pregnancy, but only one patient bled from esophageal varices after shunting. Pregnancy after decompression operations and in extrahepatic obstruction is reviewed, and the particular hazards associated with pregnancy are discussed.     

Mesenteric vein thrombosis in a pregnant patient heterozygous for the factor V (1691 G --> A) Leiden mutation

Factor V Leiden mutation is a risk factor for the development of thromboembolic events in pregnancy. Thrombosis of the mesenteric vein is a fairly infrequent condition complicating pregnancy. In this paper, we described a pregnant patient with mesenteric vein thrombosis who was heterozygous for the factor V Leiden mutation.     

Internal jugular venous autograft in extrahepatic portal hypertension.

A 26 year old female with repeated episodes of upper gastrointestinal bleeding, secondary to extensive extrahepatic portal and superior mesenteric venous obstruction, was surgically treated by interposing an internal jugular vein autograft between a collateral channel and the inferior vena cava. The follow-up observation during a seven and one-half year period has shown no recurrence of bleeding, disappearance of esophageal varices and a widely patent graft with normal portal vascular pressures. The internal jugular vein is a readily accessible autogenous graft of adequate caliber that can maintain patency under rigorous conditions. Its use is recommended in constructing portal-systemic venous shunts in those instances of extrahepatic portal hypertension in which conventional shunts cannot be established or, if they can be established, are liable to closure.     

Case of asymptomatic portal vein thrombosis after cesarean delivery

We report a case of acute portal vein thrombosis (PVT) after a cesarean delivery. The patient was admitted for treatment of severe pre-eclampsia. On the second day after cesarean delivery, the elevations of aspartic aminotransferase and alanine aminotransferase were observed. Thereafter, acute PVT was diagnosed with ultrasonography. Although early anticoagulant therapy seems to be effective in the treatment of acute PVT, close observation must be made due to the risk of bleeding.