scholarly journals Case Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period

2021 ◽  
Vol 9 ◽  
Author(s):  
Suhua Xu ◽  
Peng Zhang ◽  
Liyuan Hu ◽  
Wenhao Zhou ◽  
Guoqiang Cheng
Keyword(s):  
Liver Transplantation ◽  
Conservative Treatment ◽  
Liver Enzyme ◽  
Neonatal Period ◽  
Elevated Liver Enzyme ◽  
Clinical Symptoms ◽  
Treatment Options ◽  
Type Ii ◽  
Neonatal Cholestasis ◽  
Portosystemic Shunts

Objective: The aim of this single-center retrospective study was to analyze the clinical characteristics, treatment options, and course of neonatal-onset congenital portosystemic shunts (CPSS).Methods: We included all patients with CPSS who presented with clinical symptoms within the neonatal period in our institution between 2015 and 2020.Results: Sixteen patients were identified, including 13 patients with intrahepatic portosystemic shunts (IPSS) and three patients with extrahepatic portosystemic shunts (EPSS). The median age of diagnosis was 16 days (range prenatal 24 weeks−12 months). Hyperammonemia (60%), neonatal cholestasis (44%), elevated liver enzyme (40%), hypoglycemia (40%), thrombocytopenia (38%), and coagulation abnormalities (23%) appeared in neonatal CPSS. Twelve patients (75%) presented with congenital anomalies, of which congenital heart disease (CHD) (44%) was the most common. Thirteen patients with IPSS initially underwent conservative treatment, but two of them were recommended for the catheter interventional therapy and liver transplantation, respectively, due to progressive deterioration of liver function. Spontaneous closure occurred in nine patients with IPSS. The shunt was closed using transcatheter embolization in one patient with EPSS type II. Another patient with EPSS type II underwent surgical treatment of CHD firstly. The remaining patient with EPSS type Ib received medical therapy and refused liver transplantation.Conclusion: Hyperammonemia, neonatal cholestasis, elevated liver enzyme, hypoglycemia, and thrombocytopenia are the main complications of neonatal CPSS. Moreover, CPSS is associated with multiple congenital abnormalities, especially CHD. Intrahepatic portosystemic shunts may close spontaneously, and conservative treatment can be taken first. Extrahepatic portosystemic shunts should be closed to prevent complications.

scholarly journals Neonatal Seizures: An Overview of Genetic Causes and Treatment Options

2021 ◽  
Vol 11 (10) ◽  
pp. 1295
Author(s):  
Giulia Spoto ◽  
Maria Concetta Saia ◽  
Greta Amore ◽  
Eloisa Gitto ◽  
Giuseppe Loddo ◽  
...  
Keyword(s):  
Neonatal Period ◽  
Clinical Symptoms ◽  
Current Knowledge ◽  
Treatment Options ◽  
Neonatal Seizures ◽  
Pathogenic Variants ◽  
Ohtahara Syndrome ◽  
The Central Nervous System ◽  
Myoclonic Encephalopathy

Seizures are the most frequent neurological clinical symptoms of the central nervous system (CNS) during the neonatal period. Neonatal seizures may be ascribed to an acute event or symptomatic conditions determined by genetic, metabolic or structural causes, outlining the so-called ‘Neonatal Epilepsies’. To date, three main groups of neonatal epilepsies are recognised during the neonatal period: benign familial neonatal epilepsy (BFNE), early myoclonic encephalopathy (EME) and ‘Ohtahara syndrome’ (OS). Recent advances showed the role of several genes in the pathogenesis of these conditions, such as KCNQ2, KCNQ3, ARX, STXBP1, SLC25A22, CDKL5, KCNT1, SCN2A and SCN8A. Herein, we reviewed the current knowledge regarding the pathogenic variants most frequently associated with neonatal seizures, which should be considered when approaching newborns affected by these disorders. In addition, we considered the new possible therapeutic strategies reported in these conditions.

scholarly journals Indications and outcomes of enterovesical and colovesical fistulas: systematic review of the literature and meta-analysis of prevalence

BMC Surgery ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Stefano Granieri ◽  
Francesco Sessa ◽  
Alessandro Bonomi ◽  
Sissi Paleino ◽  
Federica Bruno ◽  
...  
Keyword(s):  
Systematic Review ◽  
Clinical Symptoms ◽  
Rare Complication ◽  
Meta Analysis ◽  
Treatment Options ◽  
Palliative Surgery ◽  
Review Of The Literature ◽  
Colovesical Fistula ◽  
Level Of Evidence ◽  
Meta Analyses

Abstract Background Entero-colovesical fistula is a rare complication of various benign and malignant diseases. The diagnosis is prominently based on clinical symptoms; imaging studies are necessary not only to confirm the presence of the fistula, but more importantly to demonstrate the extent and the nature of the fistula. There is still a lack of consensus regarding the if, when and how to repair the fistula. The aim of the study is to review the different surgical treatment options, focus on surgical indications, and explore cumulative recurrence, morbidity, and mortality rates of entero-vesical and colo-vesical fistula patients. Methods A systematic review of the literature was conducted according to PRISMA guidelines. Random effects meta-analyses of proportions were developed to assess primary and secondary endpoints. I2 statistic and Cochran’s Q test were computed to assess inter-studies’ heterogeneity. Results Twenty-two studies were included in the analysis with a total of 861 patients. Meta-analyses of proportions pointed out 5, 22.2, and 4.9% rates for recurrence, complications, and mortality respectively. A single-stage procedure was performed in 75.5% of the cases, whereas a multi-stage operation in 15.5% of patients. Palliative surgery was performed in 6.2% of the cases. In 2.3% of the cases, the surgical procedure was not specified. Simple and advanced repair of the bladder was performed in 84.3% and 15.6% of the cases respectively. Conclusions Although burdened by a non-negligible rate of complications, surgical repair of entero-colovesical fistula leads to excellent results in terms of primary healing. Our review offers opportunities for significant further research in this field. Level of Evidence Level III according to ELIS (SR/MA with up to two negative criteria).

scholarly journals Transsplenic portal vein recanalization and direct intrahepatic portosystemic shunt placement to optimize liver transplantation

2020 ◽  
Vol 3 (1) ◽  
Author(s):  
Osman Ahmed ◽  
Abhijit L. Salaskar ◽  
Steven Zangan ◽  
Anjana Pillai ◽  
Talia Baker
Keyword(s):  
Liver Transplantation ◽  
Portal Vein ◽  
Splenic Vein ◽  
Portosystemic Shunt ◽  
Refractory Ascites ◽  
Hepatic Veins ◽  
Sonographic Guidance ◽  
Portosystemic Shunts ◽  
Intrahepatic Portosystemic Shunt ◽  
End To End

Abstract Background Percutaneous trans-splenic portal vein recanalization (PVR) has been reported for facilitation of transjugular intrahepatic portosystemic shunts (TIPS), however has not been applied to patients undergoing direct intrahepatic portosystemic shunt (DIPS). We report the utilization of trans-splenic-PVR with DIPS creation in a patient with chronic portal and hepatic vein occlusions undergoing liver transplantation evaluation. Case presentation A 48-year-old male with decompensated alcoholic cirrhosis complicated by refractory ascites, hepatic encephalopathy, and variceal bleeding underwent CT that demonstrated chronic occlusion of the hepatic veins (HV), extrahepatic portal vein (PV), and superior mesenteric vein (SMV). Due to failed attempts at TIPS at outside institutions, interventional radiology was consulted for portal vein recanalization (PVR) with TIPS to treat the portal hypertension and ascites and also facilitate an end-to-end PV anastomosis at transplantation. After an initial hepatic venogram confirmed chronic HV occlusion, a DIPS with trans-splenic PVR was planned. The splenic vein was accessed under sonographic guidance using a micropuncture set and subsequently upsized to a 6 French sheath over a stiff guidewire. A splenic venogram via this access confirmed occlusion of the PV with drainage of the splenic vein (SV) through gastric varices. The thrombosed PV was then recanalized and angioplastied to restore PV flow via the transsplenic approach. A transjugular liver access kit with a modified 21-gauge needle was advanced into the IVC through the internal jugular vein (IJV) sheath and directed towards the target snare in PV. The needle was used to subsequently puncture the PV through the caudate lobe and facilitate placement of a wire into the SV. The initial portosystemic gradient (PSG) was 20 mmHg. The IJV sheath was advanced through the hepatic parenchymal tract into the main-PV and a stent-graft was placed across the main PV and into the IVC. A portal venogram demonstrated brisk blood flow through the DIPS, resolution of varices and a PSG of 8 mmHg. One month after the procedure, the patient had a significant reduction in ascites and MELD-NA score. Patient is currently listed and awaiting transplantation. Conclusions In the setting of chronically occluded portal and hepatic veins, trans-splenic PVR DIPS may serve as an effective bridge to liver transplantation by facilitating an end to end portal vein anastomosis.

scholarly journals Predictors of mortality in patients with hereditary hemorrhagic telangiectasia

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
K. P. Thompson ◽  
◽  
J. Nelson ◽  
H. Kim ◽  
L. Pawlikowska ◽  
...  
Keyword(s):  
Hereditary Hemorrhagic Telangiectasia ◽  
Cox Regression ◽  
Clinical Symptoms ◽  
Vascular Malformations ◽  
Smoking Status ◽  
Treatment Options ◽  
Gi Bleeding ◽  
Cox Regression Analysis ◽  
Predictors Of Mortality

Abstract Background Retrospective questionnaire and healthcare administrative data suggest reduced life expectancy in untreated hereditary hemorrhagic telangiectasia (HHT). Prospective data suggests similar mortality, to the general population, in Denmark’s centre-treated HHT patients. However, clinical phenotypes vary widely in HHT, likely affecting mortality. We aimed to measure predictors of mortality among centre-treated HHT patients. HHT patients were recruited at 14 HHT centres of the Brain Vascular Malformation Consortium (BVMC) since 2010 and followed annually. Vital status, organ vascular malformations (VMs) and clinical symptoms data were collected at baseline and during follow-up (N = 1286). We tested whether organ VMs, HHT symptoms and HHT genes were associated with increased mortality using Cox regression analysis, adjusting for patient age, sex, and smoking status. Results 59 deaths occurred over average follow-up time of 3.4 years (max 8.6 years). A history of anemia was associated with increased mortality (HR = 2.93, 95% CI 1.37–6.26, p = 0.006), as were gastro-intestinal (GI) bleeding (HR = 2.63, 95% CI 1.46–4.74, p = 0.001), and symptomatic liver VMs (HR = 2.10, 95% CI 1.15–3.84, p = 0.015). Brain VMs and pulmonary arteriovenous malformations (AVMs) were not associated with mortality (p > 0.05). Patients with SMAD4 mutation had significantly higher mortality (HR = 18.36, 95% CI 5.60–60.20, p < 0.001) compared to patients with ACVRL1 or ENG mutation, but this estimate is imprecise given the rarity of SMAD4 patients (n = 33, 4 deaths). Conclusions Chronic GI bleeding, anemia and symptomatic liver VMs are associated with increased mortality in HHT patients, independent of age, and in keeping with the limited treatment options for these aspects of HHT. Conversely, mortality does not appear to be associated with pulmonary AVMs or brain VMs, for which patients are routinely screened and treated preventatively at HHT Centres. This demonstrates the need for development of new therapies to treat chronic anemia, GI bleeding, and symptomatic liver VMs in order to reduce mortality among HHT patients.

scholarly journals A systematic review on conservative treatment options for OSGOOD-Schlatter disease

2021 ◽  
Vol 49 ◽  
pp. 178-187
Author(s):  
Cornelia Neuhaus ◽  
Christian Appenzeller-Herzog ◽  
Oliver Faude
Keyword(s):  
Systematic Review ◽  
Conservative Treatment ◽  
Treatment Options

Conservative treatment for Landells and Van Peteghem type II atlas fracture accompanied with atlantoaxial rotatory subluxation

2015 ◽  
Vol 15 (11) ◽  
pp. e31-e33
Author(s):  
Da-Geng Huang ◽  
Ding-Jun Hao ◽  
Zhen Chang ◽  
Bao-Rong He ◽  
Tuan-Jiang Liu
Keyword(s):  
Conservative Treatment ◽  
Type Ii ◽  
Atlas Fracture ◽  
Rotatory Subluxation

scholarly journals Successful Management of Acute Liver Failure Patients Waiting for Liver Transplantation by On-Line Hemodiafiltration with an Arteriovenous Fistula

2016 ◽  
Vol 10 (1) ◽  
pp. 144-150 ◽  
Author(s):  
Yuki Haga ◽  
Shin Yasui ◽  
Tatsuo Kanda ◽  
Noriyuki Hattori ◽  
Toru Wakamatsu ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Prognostic Factors ◽  
Infectious Diseases ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Arteriovenous Fistula ◽  
Vascular Access ◽  
Treatment Options ◽  
Successful Management ◽  
On Line

On-line hemodiafiltration (OLHDF) is one of the treatment options in the management of acute liver failure (ALF) in Japan. It is essential to avoid infection in the management of ALF. In fact, infection is one of the prognostic factors in ALF. In this report, we present a middle-aged Japanese man with ALF associated with benzbromarone use. He was successfully managed without infection until liver transplantation by creating an arteriovenous fistula for OLHDF. Utilizing an arteriovenous fistula for OLHDF, rather than inserting a vascular access catheter, is a beneficial option to avoid infectious diseases in the management of ALF.

Venus occlusion in the upper limbs in patients with CIDS

2018 ◽  
Vol 1 (46) ◽  
pp. 24-27
Author(s):  
Anna Jędrzejczak ◽  
Przemysław Mitkowski
Keyword(s):  
Left Atrial ◽  
Clinical Symptoms ◽  
Treatment Options ◽  
Electronic Devices ◽  
Left Ventricular ◽  
Venous Anatomy ◽  
Device Implantation ◽  
Vein Occlusion ◽  
Venous Stenosis ◽  
Vein Stenosis

Benefits of treatment with cardiological implantable electronic devices have been confirmed in numerous studies. Similarly to other treatment options this therapy is not free from complications. Among them venous stenoses and occlusions are observed. Its presence is related to impaired blood flow in the vessel as an effect of lead presence, endothelial malfunction and procoagulation. Despite vein occlusion is present in 9-11% of patients after device implantation and vein stenosis which is grater then 70% of diameter in 17-38% or even in some groups up to 50%, clinical symptoms are reported only in 1-3% of patients. What is interesting in 13,7% of individuals before implantation venous stenosis over 60% is present and occlusion in 4,4% patients. Significant venous stenosis is more frequently observed on the left side. Among risk factors responsible for stenosis one can find: numer of leads, total diameter of all leads, usage of anticoagulation and antiplatelet drugs, decreased ejection fraction, increased left ventricular end diastolic diameter, left atrial diameter, higher concentration of procoagulation and inflammatory markers. Presence of significant venous stenosis is important mainly before of up-grade procedures or lead replacement and knowledge about venous anatomy allows better planning of the subsequent procedure.

Comparing plaque brachytherapy and proton therapy for choroidal melanoma: a review of the literature

2010 ◽  
Vol 9 (1) ◽  
pp. 53-61
Author(s):  
Jeremy P. Appleton ◽  
Peter Bridge
Keyword(s):  
Conservative Treatment ◽  
Proton Therapy ◽  
Normal Tissue ◽  
Treatment Options ◽  
Choroidal Melanoma ◽  
Review Of The Literature ◽  
Tumour Control ◽  
Local Tumour Control ◽  
Local Tumour ◽  
Plaque Brachytherapy

AbstractThe aims of conservative treatment in patients with ocular melanoma are globe retention, good visual acuity (VA) and local control. Two well-established radiation conservative treatment options are proton beam radiotherapy and episcleral plaque brachytherapy (EPB). Patients who receive treatment with either of these options will experience some degree of radiation-related ocular complications and poor VA. The purpose of this review of the literature is to establish whether there is a significant clinical difference in normal tissue morbidity and local tumour control between proton therapy and EPB, and whether this difference can justify the purchase and implementation of additional proton therapy facilities. Based on this review, evidence suggested that both treatment options are comparable, and that neither proton therapy nor EPB is clinically superior than the other regarding normal tissue morbidity and local tumour control. This review highlighted the need for further research on a larger scale in order to bridge the gap that is apparent within the literature.

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