TIPS and splenorenal shunt for complications of portal hypertension in chronic hepatosplenic schistosomiasis–A case series and review of the literature

Background Transjugular intrahepatic portosystemic shunt (TIPS) and shunt surgery are established treatment options for portal hypertension, but have not been systematically evaluated in patients with portal hypertension due to hepatosplenic schistosomiasis (HSS), one of the neglected tropical diseases with major impact on morbidity and mortality in endemic areas. Methods In this retrospective case study, patients with chronic portal hypertension due to schistosomiasis treated with those therapeutic approaches in four tertiary referral hospitals in Germany and Italy between 2012 and 2020 were included. We have summarized pre-interventional clinical data, indication, technical aspects of the interventions and clinical outcome. Findings Overall, 13 patients with confirmed HSS were included. 11 patients received TIPS for primary or secondary prophylaxis of variceal bleeding due to advanced portal hypertension and failure of conservative management. In two patients with contraindications for TIPS or technically unsuccessful TIPS procedure, proximal splenorenal shunt surgery in combination with splenectomy was conducted. During follow-up (mean follow-up 23 months, cumulative follow-up time 31 patient years) no bleeding events were documented. In five patients, moderate and transient episodes of overt hepatic encephalopathy were observed. In one patient each, liver failure, portal vein thrombosis and catheter associated sepsis occurred after TIPS insertion. All complications were well manageable and had favorable outcomes. Conclusions TIPS implantation and shunt surgery are safe and effective treatment options for patients with advanced HSS and sequelae of portal hypertension in experienced centers, but require careful patient selection.

Quality of Life Assessment Among Patients Living With Hepatosplenic Schistosomiasis and Schistosomal Myeloradiculopathy

Schistosomiasis is a major public health problem in tropical areas of the world. Health-related quality of life (HRQOL) measurement is being widely used to evaluate the impact of a disease or treatment in several aspects of daily life. However, few studies evaluated the impact of severe forms of schistosomiasis on HRQOL of affected individuals and compared them to healthy controls with a similar socio-demographic background. Our aims were to evaluate the HRQOL in patients with hepatosplenic schistosomiasis (HS) and schistosomal myeloradiculopathy (SMR) and healthy volunteers (HV) and determine if clinical complications of the disease are associated with HRQOL scores. We interviewed and evaluated the HRQOL in 49 patients with HS, 22 patients with SMR, and 26 HV from an outpatient clinic of the Federal University of Minas Gerais University Hospital using the WHOQOL-BREF questionnaire. SMR and HS patients had a significantly lower overall quality of life score when comparing with the HV control group (p = 0.003 and p = 0.005, respectively). Multivariate ordinal regression model adjusted for sex, age, and educational level indicated that HS and SMR patients have three and five times more chances of having a lower quality of life than healthy volunteers (Odds Ratio 3.13 and 5.04, respectively). There was no association between complications of HS disease and quality of life scores. In contrast, worse quality of life was observed in SMR patients that presented back or leg pain, leg paresthesia, and bladder dysfunction. In conclusion, HS and SMR significantly impact the overall quality of life of the affected individuals, reinforcing the importance of efforts to control and eradicate this debilitating disease and suggesting that multidisciplinary clinical management of schistosomiasis patients would be more appropriate and could potentially improve patient's quality of life.

Diagnosis and clinical management of hepatosplenic schistosomiasis: A scoping review of the literature

Background Hepatosplenic schistosomiasis (HSS) is a disease caused by chronic infection with Schistosma spp. parasites residing in the mesenteric plexus; portal hypertension causing gastrointestinal bleeding is the most dangerous complication of this condition. HSS requires complex clinical management, but no specific guidelines exist. We aimed to provide a comprehensive picture of consolidated findings and knowledge gaps on the diagnosis and treatment of HSS. Methodology/principal findings We reviewed relevant original publications including patients with HSS with no coinfections, published in the past 40 years, identified through MEDLINE and EMBASE databases. Treatment with praziquantel and HSS-associated pulmonary hypertension were not investigated. Of the included 60 publications, 13 focused on diagnostic aspects, 45 on therapeutic aspects, and 2 on both aspects. Results were summarized using effect direction plots. The most common diagnostic approaches to stratify patients based on the risk of variceal bleeding included the use of ultrasonography and platelet counts; on the contrary, evaluation and use of noninvasive tools to guide the choice of therapeutic interventions are lacking. Publications on therapeutic aspects included treatment with beta-blockers, local management of esophageal varices, surgical procedures, and transjugular intrahepatic portosystemic shunt. Overall, treatment approaches and measured outcomes were heterogeneous, and data on interventions for primary prevention of gastrointestinal bleeding and on the long-term follow-up after interventions were lacking. Conclusions Most interventions have been developed on the basis of individual groups’ experiences and almost never rigorously compared; furthermore, there is a lack of data regarding which parameters can guide the choice of intervention. These results highlight a dramatic need for the implementation of rigorous prospective studies with long-term follow-up in different settings to fill such fundamental gaps, still present for a disease affecting millions of patients worldwide.

Liver stiffness is able to differentiate hepatosplenic Schistosomiasis mansoni from liver cirrhosis and spleen stiffness may be a predictor of variceal bleeding in hepatosplenic schistosomiasis

Background Ultrasonography is limited for differentiating portal hypertension due to liver cirrhosis from that secondary to hepatosplenic schistosomiasis (HSS). We aimed to investigate the role of transient elastography (TE) in differentiating HSS mansoni from cirrhosis and the factors associated with liver and spleen stiffness (LS and SS) in HSS. Method A cross-sectional study was conducted including patients with HSS mansoni (n=29) and liver cirrhosis due to non-alcoholic steatohepatitis (n=23). All patients underwent TE and those with HSS were assessed by the Niamey protocol. Results HSS subjects presented lower median LS (9.6 vs 21.3 Kpa, p<0.001) and liver controlled attenuation parameter (229 vs 274 dB/m, p=0.010) than cirrhosis subjects, in addition to higher SS (73.5 vs 42.2 Kpa, p=0.002). The area under the receiver operating characteristic curve for detecting cirrhosis by LS was 0.947 (95% CI 0.89 to 1.00, p<0.001), with an optimal cut-off of 11.75 Kpa. In HSS subjects, higher SS was associated with the presence of the following: diabetes mellitus (p=0.036), metabolic syndrome (p=0.043), esophageal varices (p=0.001), portal vein thrombosis (p=0.047) and previous variceal bleeding (p=0.011). In HSS patients without portal vein thrombosis, variceal bleeding was associated with higher SS (p=0.018). Niamey categories were not associated with LS (p=0.676) or SS (p=0.504). Conclusion TE can play a role in differentiating HSS from cirrhosis, especially by LS. SS may be further investigated for predicting complications in HSS.

Portal vein thrombosis in patients with hepatosplenic schistosomiasis who underwent oesophagogastric devascularization combined with splenectomy

Background Portal vein thrombosis (PVT) has been described in nearly 50% of patients who underwent oesophagogastric devascularization combined with splenectomy (EGDS), but no previous study has compared its occurrence in surgical and non-surgical groups. This study aimed to investigate PVT in hepatosplenic schistosomiasis (HSS) and its association with EGDS and upper variceal bleeding (UVB). Methods Retrospectively, 104 HSS individuals were enrolled. Following EGDS, the occurrence of PVT, mesenteric vein thrombosis (MVT), hospital admissions and UVB were recorded. Results EGDS was performed in 27 (26%) patients. PVT and MVT were detected in 30 (33%) and 8 (9.8%) patients, respectively. Patients who underwent EGDS were at greater risk of PVT (63% vs 19.7%; odds ratio [OR] 6.12 [95% confidence interval {CI} 2.3 to 16.1], p<0.001) when compared with a non-surgical approach. There was no significant difference in UVB occurrence and β-blocker usage. PVT was associated with more hospital admissions (p=0.030) and higher alkaline phosphatase levels (p=0.008). UVB occurrence in patients with and without thrombosis was similar. In multivariate analysis, after adjustment, PVT was associated with the surgical approach (OR 4.56 [95% CI 1.55 to 13.38], p=0.006) and age at HSS diagnosis (OR 0.94 [95% CI 0.90 to 0.99], p=0.021). Conclusions EGDS was not associated with a decreased frequency of UVB when compared with the non-surgical approach but was an independent risk factor for PVT.

Hepatosplenic schistosomiasis, the ignored morbidity: experience from a referral hospital in Ethiopia

Background Hepatosplenic schistosomiasis (HSS) is an important chronic complication of Schistosoma infection. However, its diagnosis and management are complicated due to lack of evidence and uniform guidance. In this study, we described the clinical profile and management of HSS in one of Ethiopia's referral hospitals. Methods A hospital-based, cross-sectional study was conducted using the medical records of patients diagnosed with HSS based on clinical symptoms and typical ultrasound findings observed at the gastroenterology clinic of Dessie referral hospital from September 2018 to December 2019. Results A total of 55 patient files were evaluated. Most patients (n=39; 70%) presented with upper gastrointestinal bleeding and severe anaemia. Nearly three-quarters of patients were admitted and 31 (56%) received a blood transfusion. Hepatitis B coinfection was documented in 11 patients (20%). Upper gastrointestinal endoscopy was performed in 31 patients (56%), in whom large oesophageal varices with red signs were the most common finding. Among patients who had endoscopy, band ligation was carried out in 21 cases (68%). Praziquantel was administered to 11 patients (20%). Conclusion HSS causes severe complications of portal hypertension, like variceal bleeding, which are currently not adequately treated. In addition to already existing preventive efforts, we advise control programmes to also address acute management and rehabilitation of these patients.