BACKGROUND: Portal hypertension is secondary to either cirrhotic or non-cirrhotic causes, and complicating pregnancy poses a challenge to the treating team. A systematic review was performed to determine maternal and perinatal outcomes in women with portal hypertension. Outcomes were compared among those with cirrhotic (CPH) with non-cirrhotic portal hypertension (NCPH) as well as non-cirrhotic portal fibrosis (NCPF) with extra-hepatic portal vein obstruction (EHPVO).
METHODS: Medline and EMBASE databases were searched for studies reporting outcomes among pregnant women with portal hypertension. Reference lists from relevant papers and reviews were hand-searched for appropriate citations. Data were extracted to describe maternal complications, obstetric and neonatal outcomes. A random-effects model was used to derive pooled estimates of various outcomes, and final estimates were reported as percentages with a 95% confidence interval (CI). Cumulative, sequential and sensitivity analysis was studied to assess the temporal trends of outcomes over the period.
RESULTS: Information on 895 pregnancies among 581 patients with portal hypertension was included from 26 studies. Portal hypertension was diagnosed during pregnancy in 10% (95% CI 4-24%). There were 22 maternal deaths (0%, 95% CI 0-1%), mostly following complications from variceal bleeding or hepatic decompensation. Variceal bleeding complicated in 14% (95% CI 9-20%), and endoscopic interventions were performed in 12% (95% CI 8-17%) during pregnancy. Decompensation of liver function occurred in 7% (95% CI 3-12%). Thrombocytopenia was the most common complication (41%, 95% CI 23-60%). Miscarriages occurred in 14% (95% CI 8-20%), preterm birth in 27% (95% CI 19-37%), and low birth weights in 22% (95% CI 15-30%). Risk of postpartum hemorrhage was higher (RR 5.09, 95% CI 1.84-14.12), and variceal bleeding was lower (RR 0.51, 95% CI 0.30-0.86) among those with CPH compared to NCPH. Risk of various outcomes was comparable between NCPF and EHPVO.
CONCLUSIONS: One in ten pregnancies complicated with portal hypertension is diagnosed during pregnancy, and thrombocytopenia is the most common complication. Hepatic decompensation and variceal bleeding remain the most common cause of maternal deaths, with reduced rates of bleeding and its complications reported following the introduction of endoscopic procedures during pregnancy. CPH increases the risk of postpartum hemorrhage, whereas variceal bleeding is higher among NCPH.

OBJECTIVE: To assess the maternal and perinatal outcomes of pregnant women with non-cirrhotic portal hypertension (NCPH).
METHODS: This was an observational study done by retrieving the records of pregnant women with non-cirrhotic portal hypertension admitted to a tertiary hospital in South India, over a 9-year study period. Data regarding the clinical course, complications during pregnancy, labor, and delivery details were reviewed. We also compared the outcomes among women with non-cirrhotic portal fibrosis (NCPF) with extrahepatic portal vein obstruction (EHPVO).
RESULTS: During the study period, portal hypertension was noted in 0.07%(n = 108) of the pregnancies and 74.1% of them had NCPH. The diagnosis was made for the first time in 54.7% of them when presented with pancytopenia or splenomegaly. Variceal bleeding complicated 25% of the pregnancies in women with NCPH pregnancies, with three among them having a massive bleed. Eighteen among them underwent endoscopy following bleeding; variceal banding procedure was performed in nine of them without any complications. Preterm birth was the most common (20.6%) obstetric complication. There was one maternal death from severe sepsis, acute kidney injury, and disseminated intravascular coagulation, following a massive variceal bleed. Obstetric outcomes and medical complications were similar in women with NCPF and EHPVO. Perinatal loss was comparable in both the groups (14.3% vs. 9.6%, p = 0.417) CONCLUSION: Multidisciplinary team approach, with optimal and timely intervention with intensive monitoring, can reduce the morbidity and help achieve an optimal maternal-perinatal outcome in pregnancies complicated with portal hypertension.

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OBJECTIVE: To evaluate the clinical presentation, possible etiological factors, management and outcome of patients in our hospital with extrahepatic portal vein obstruction (EHPVO).
METHODS: This study included patients with EHPVO followed up in our department during last 10 years. Patients of cirrhosis with EHPVO were excluded. Patients\' clinical presentation, etiology of EHPVO, management and outcome results were analyzed.
RESULTS: Of 30 patients, 19 (67.9%) were males. Median age was 12 years. Of 14 patients who underwent liver biopsy 9 had histological activity index stage of 1/6. History of omphalitis and pulmonary tuberculosis was present in one case each. Of 22 patients with the available thrombophilia profile, nine patients had a deficiency of protein C, five patients had a deficiency of protein S, one each had reduced level S of anti-thrombin III and factor V mutation. The predominant presenting symptom was hematemesis (15 patients, 53.6%). Seven patients (25%) had splenomegaly. Three patients (10.7%) had no esophageal varices on endoscopy. Three patients underwent splenectomy due to severe pancytopenia. Endoscopic retrograde cholangipancreatography was performed in four patients (14.3%) due to portal biliopathy. Common bile duct stenting was performed in all four patients. Of them, one patient underwent splenorenal shunt operation for indication of hemobilia. One patient died at the age of 40 years, due to cholangitis and sepsis.
CONCLUSIONS: Results from this study show that the anticoagulant deficiency is a common cause of EHPVO in our setup. Hematemesis is a common presenting symptom. Some of these patients have symptomatic portal biliopathy.

A laparoscopic cholecystectomy can be technically challenging with co-existing portal hypertension, as commonly seen with cirrhosis of the liver. Extra hepatic portal vein obstruction (EHPVO) although less common, is a significant cause of portal hypertension in India. EHPVO has a unique clinical profile, which differentiates it from portal hypertension associated with cirrhosis of the liver. This impacts therapy in EHPVO algorithmically and operatively. We report two cases of symptomatic gall stones with portal cavernoma. Further evaluation revealed non-obstructive portal biliopathy. Both underwent a successful laparoscopic cholecystectomy. We highlight the importance of careful operative strategy, diligent haemostasis and the feasibility of performing a laparoscopic cholecystectomy in patients with symptomatic gall stones associated with a portal cavernoma.