Syphilis: "The Great Imitator" Presenting as Acute Hepatitis

Introduction: Syphilis is a multi-systemic disease caused by spirochete Treponema pallidum. Very rarely, it can affect the liver and cause hepatitis. Since most cases of hepatitis are caused by viral illnesses, syphilitic hepatitis can be missed. Here, we present a case of syphilitic hepatitis in a 35-year-old male. Case Description/Methods: Patient was a 35-year-old male who presented to the hospital for jaundice and mild intermittent right upper quadrant abdominal pain. His medical history was only significant for alcohol abuse. His last drink was 4 weeks ago. He was sexually active with men. On exam, hepatomegaly, mild tenderness in the right upper quadrant, jaundice, and fine macular rash on both hands and feet were noted. Lab tests revealed an ALT of 965 U/L, AST of 404 U/L, ALP of 1056 U/L, total bilirubin of 9.5 mg/dL, direct bilirubin of 6.5 mg/dL, INR of 0.96, and albumin of 2.0 g/dL. Right upper quadrant ultrasound showed an enlarged liver but was negative for gallstones and hepatic vein thrombosis. MRI of the abdomen showed periportal edema consistent with hepatitis without any gallstones, masses, or common bile duct dilation. HIV viral load and Hepatitis C viral RNA were undetectable. Hepatitis A & B serologies were indicative of prior immunization. Hepatitis E serology and SARS-CoV-2 PCR were negative. Ferritin level was 177 ng/mL. Alpha-1-antitrypsin levels and ceruloplasmin levels were normal. Anti-Smooth muscle antibody titers were slightly elevated at 1:80 (Normal < 1:20). Anti-Mitochondrial antibody levels were also slightly elevated at 47.9 units (Normal < 25 units). RPR titer was 1:32 and fluorescent treponemal antibody test was reactive which confirmed the diagnosis of syphilis. Liver biopsy was then performed which showed presence of mixed inflammatory cells without any granulomas which is consistent with other cases of syphilitic hepatitis. Immunohistochemical stain was negative for treponemes. Patient was treated with penicillin and did have Jarisch-Herxheimer reaction. ALT, AST, ALP, and total bilirubin down trended after treatment. Repeat tests drawn exactly 1 month post treatment showed normal levels of ALT, AST, ALP, and total bilirubin (Figure). Discussion(s): Liver damage can occur in syphilis and can easily be missed because of the non-specific nature of presenting symptoms. In our patient, the fine macular rash on both hands and feet along with history of sexual activity with men prompted us to test for syphilis which ultimately led to diagnosis and treatment in a timely manner. (Figure Presented).

Acute Massive Thrombus Following Fontan Procedure

Background Patients with hypoplastic left heart syndrome (HLHS) undergo a Fontan procedure as part of single ventricle surgical palliation. Post-Fontan, sluggish blood flow and an imbalance in coagulant factor proteins may predispose to thrombus formation. Other risk factors may include chylothorax as well as acute and chronic inflammation. Currently, there is no standardized surveillance strategy to detect thrombus in Fontan patients. Case A 34-month old male with HLHS underwent an extracardiac non-fenestrated Fontan complicated by chylothorax treated with 5 days of IV steroids and diuretics. He was on therapeutic aspirin. After progressive worsening of right pleural effusion, a chest tube was placed three weeks post-Fontan with continued chylous output. Stool alpha 1 antitrypsin was negative. Decision-making Given persistent chylothorax, a repeat echocardiogram was performed revealing a large mass in the Fontan circuit less than one month post-op. Cardiac CT showed occlusive thrombus filling the entirety of the Fontan conduit extending into hepatic veins and bilateral pulmonary arteries. He underwent extensive surgical thrombectomy and Fontan conduit revision. Hypercoagulable work-up revealed elevated factor 8 and von Willebrand factor activity which persisted more than one month post-op. Patient's history was also significant for COVID-19 infection 6 months prior. He was initially anticoagulated with bivalirudin with tirofiban initiated for antiplatelet therapy. He was ultimately transitioned to rivaroxaban, pentoxifylline and aspirin with chylothorax resolution over one month without thrombus recurrence. Conclusion Development of risk stratification tools to identify patients at higher risk for thrombi formation post-Fontan may facilitate patient selection for more aggressive anticoagulation. Consideration of elevated factor 8 as well as persistent or recurrent chylothorax may be beneficial, as increased thrombosis risk has been reported for both conditions in Fontan patients.Copyright © 2023 American College of Cardiology Foundation

Splanchnic vein thrombosis and Budd-Chiari syndrome after SARS-CoV- 2 vaccination-Incidence of VITT

Background: Several studies have been published on a rare side effect of severe venous thrombosis at unusual sites and thrombocytopenia after vaccination against SARS-CoV- 2, referred to as vaccine-induced immune thrombocytopenia and thrombosis (VITT). Aim(s): To identify new cases of acute splanchnic vein thrombosis (SVT) or Budd-Chiari Syndrome (BCS) who presented following SARS-CoV- 2 vaccination in the Vascular Liver Disease Group (VALDIG) network, and to evaluate the incidence of VITT. Method(s): We conducted a prospective international cohort study between May 1st, 2021 and January 10th, 2022, on consecutive patients with acute SVT or BCS who presented within 6 weeks following any type or dose of SARS-CoV- 2 vaccination. Anonymous data were collected including baseline characteristics, risk factors, treatment and survival. Cases were identified as definite VITT, probable VITT or possible VITT or unlikely VITT as defined by Pavord et al (NEJM 2021). Result(s): 25 patients with acute (N = 24) or recurrent (N = 1) SVT or BCS were collected from 14 centers in 4 countries (after ChAdOx1 nCoV-19 N = 11, BNT162b2 N = 9, Ad26.COV2.S N = 1, mRNA-1273 N = 1). Median time after vaccination to symptoms was 10 days (2-40). Median age was 52.5 years (21-66), 52% were female. Three patients (12%) fulfilled criteria for definite VITT, 6 (24%) for probable VITT, 2 (8%) for possible VITT, 14 (56%) for unlikely VITT. Thrombosis was located in the portal vein (N = 20), hepatic vein(s) (N = 9), mesenteric vein (N = 18) or splenic vein (N = 9). Concomitant extra-abdominal thrombosis was seen in 5 patients (20%). Patients were treated with LMWH (60%), DOACs (24%) or VKA (40%). Six (2/3 with definite VITT) received IVIG. Thrombophilia was found in 5 patients and 3 had a myeloproliferative neoplasm. Conclusion(s): 25 cases of acute SVT or BCS following SARS-CoV- 2 vaccination were identified. Although definite VITT was rare (12%), no underlying disorder was identified in the majority of patients, contrary to 'typical' cases of SVT and BCS.

A newly diagnosed Budd-Chiari Syndrome with thrombotic thrombocytopenia after BTN162b2 vaccination

Objectives: Worldwide, Pfizer/BioNTech (BTN162b2) mRNA vaccine is now under massive use to be protected from COVID-19, although it may cause thrombotic thrombocytopenia in rare cases. Budd-Chiari syndrome (BCS) is a rare condition and is defined as the obstruction of hepatic venous outflow. Materials and Methods: A 34-year-old woman who was vaccinated with first dose of BTN162b2 6 weeks ago newly developed ascites, liver dysfunction, and thrombocytopenia. Results: Contrast-enhanced CT scan, doppler ultrasound, and hepatic venography showed complete obstruction of three major hepatic veins without membranous structure and without any collaterals, causing portal hypertension and liver dysfunction. Percutaneous liver biopsy showed diffusion dilation of sinusoids with extensive hepatocyte dropout, although there was no portal inflammation or fibrosis. The patient was treated with anticoagulants and intravenous immunoglobulin. After 6 weeks of anticoagulation, hepatic venous outflow became well detectable by doppler ultrasonography and ascites disappeared. Conclusion: This is a rare case of acute BCS with thrombotic thrombocytopenia after BNT162b2 mRNA vaccination.

Unsuspecting Adult Case of Rhinovirus Pleuropericarditis

Rhinoviridae are the most common cause of upper respiratory tract infections, especially in children, and often referred to as “the common cold”. Symptoms are usually mild, nasopharyngeal in nature;they have, however, been implicated in cases of infantile viral pericarditis. Its role in the presentation of adult viral pericarditis remains unclear. We present the case of a 45-year-old male with a past medical history of pre-diabetes, hyperlipidemia and hypertension with complaints of severe left-sided chest pain that worsened with movement and coughing but improved when lying supine. Two weeks prior to presentation, he had developed an intermittent cough, treated with antibiotics and steroids. On presentation to the ED, the patient was afebrile but hypotensive to 80/52 mmHg, tachycardic to 116 BPM, hypoxic to 88% on room air, improving to 91% with 3L nasal cannula. Physical examination was notable for wheezing and egophony. Laboratory findings were concerning for WBC 19.97x10-3/uL, Hgb 13.4 g/dL, CRP 176 mg/L, Ferritin 772 ug/L, D-dimer 3.70 ug/mL FEU;procalcitonin 0.2 ng/mL and troponin <0.015 ng/mL. Respiratory viral panel revealed negative COVID-19 test but positive for rhinovirus/enterovirus. Electrocardiogram showed sinus tachycardia. Chest computed tomography demonstrated moderate pericardial effusion, ground glass attenuation of the lungs bilaterally with moderate left pleural effusion and reflux of contrast into the hepatic veins, suggestive of right heart failure. Echocardiogram demonstrated small to moderate pericardial effusion. The patient was admitted with the diagnosis of acute rhino/enteroviral-associated pleuropericarditis. Broad-spectrum antibiotics, prednisone, colchicine and indomethacin were commenced. Upon clinical stabilization of his condition, steroids were discontinued and he was discharged home with close follow-up. While rhinovirus has been associated with infantile viral pericarditis, it is implicated in pneumonia and COPD exacerbations in adults but rarely reported as a cause of adult pericarditis. A case-control study of adults diagnosed with acute idiopathic pericarditis had an independent association with an upper respiratory tract infection or gastroenteritis in the month preceding pericarditis diagnosis but did not delineate causative viruses. Therefore in cases of unknown causes of viral pericarditis, thorough history is vital. Steroids as part of the treatment algorithm for pleuropericarditis management has long been debated. Older literature has not favored the use of steroids due to high recurrence rate. However, Perrone et al refuted this point, noting that low-dose steroids with gradual tapers have equal efficacy and recurrence rates as compared with NSAIDs/colchicine. Therefore, steroids may be a reasonable option for patients with contraindications to NSAIDs/colchicine.

From Inferior Vena Cava to Right Atrium: A Rare Case of a Unique Large Burden Thrombus in a Patient with Antiphospholipid Syndrome

Introduction: Antiphospholipid Syndrome is a condition where self-antibodies are directed against phospholipid binding proteins resulting in thrombosis and/or pregnancy loss. Diagnosis is made via history, physical, positive anticardiolipin and anti-beta-2-glycoprotein antibodies. We describe a case of a large thrombus in a previously diagnosed patient with antiphospholipid syndrome and discuss the need for prophylaxis in these patients. Case Report: 34-year-old G7P1161 Hispanic female with past medical history of uncontrolled diabetes mellitus type 2 presents with an acute onset of sharp abdominal pain radiating to the back associated with nausea, non-bloody non-bilious emesis and dysuria. Vital signs on admission are significant for tachycardia and hypertension. Labs are noteworthy for elevated Creatinine at 1.7 mg/dl, thrombocytopenia, transaminitis, elevated Ddimer at 14272 ng/ml. Urine analysis is positive for nitrites, trace leukocytes and bacteria. Her serum pregnancy test and COVID PCR are negative. CT Abdomen/Pelvis with contrast revealed an extensive thrombus in the Inferior Vena Cava (IVC) to the Right Atrium (RA), also extending into the hepatic veins and upper lumbar veins. Moderate perinephric fat stranding is also noted around bilateral kidneys. Ultrasound of the abdomen reveals cholelithiasis without evidence of acute cholecystitis. Venous Doppler of lower extremities reveals patent deep veins. Patient was started on heparin drip immediately and intravenous Cefepime. Interventional Radiology performed mechanical thrombectomy. Hematology was consulted and converted patient to Warfarin with an INR goal of 2.5-3. Patient was discharged and instructed to follow up with hematology. Discussion: There are few case reports of extensive thrombi ranging from IVC to RA with most cases occurring in elderly population. We present a unique case of an extensive thrombus ranging not only from the IVC to RA but also extending into the hepatic veins and the upper lumbar veins. The patient described has a history of multiple spontaneous abortions with her only successful preterm birth required daily therapeutic Lovenox during pregnancy. Her recurrent pregnancy loss and current large burden thrombus can be attributed to her antiphospholipid syndrome. This begs the question whether these patients should be started on prophylaxis anticoagulation. There have been limited studies with aspirin and warfarin which at times demonstrated positive results. Our patient had her thrombus identified incidentally due to an admission for pyelonephritis. If her thrombus was not recognized in time, outcomes could have been devastating. In conclusion, there should be further studies to determine the efficacy of anticoagulation prophylaxis in patients with positive antiphospholipid antibodies. (Figure Presented).

Congenital Portosystemic Shunts in Children: A Case Series From Single Centre

Background: Congenital portosystemic venous shunts (CPSS) are uncommon foetal vascular developmental anomalies where splanchnic venous flow bypasses liver. Four cases of CPSS are reported at our centre. Case Summary (1) Eight years old female child presented with Dengue with no features of chronic liver disease and normal liver function test (LFT). Ultrasonography (UGS) abdomen reported an incidental finding of abnormal vascular shunt in liver. Further imaging revealed an anastomosis between portal vein and intrahepatic part of inferior vena cava (IVC), hypoplastic portal vein and multiple nodules in bilateral liver lobes. Interventional Radiologist closed the anastomosis using vascular plug. Child sustained the procedure well. (2) Two months old female patient presented with high GGTP cholestasis, dysmorphism and deranged LFT. On USG abdomen there was intrahepatic portosystemic shunt. MDCT abdomen revealed 2 vascular shunts between left portal vein to middle hepatic vein and left portal vein. Cholestasis responded with symptomatic treatment, hence being followed-up for observation till 1year of age for complications and possibility of spontaneous closure. (3) Twenty-two days old, full term female child presented with convulsions and high GGTP cholestasis with multiple hematomas in brain. LFTs were deranged. 2D-ECHO showed small PFO. USG abdomen suggested a channel between left portal vein and hepatic vein. Patient tested COVID positive hence quarantined now and further evaluation is awaited. (4) One day old, late preterm male baby presented with respiratory distress and pulmonary hypertension with antenatal scan suggesting ductus venous agenesis with hepatic vascular malformation. Patient developed cholestasis with deranged LFT. 2D-ECHO showed PDA and ASD. MDCT abdomen revealed connection between main portal vein and intrahepatic IVC. Conclusions: CPSS has heterogeneous presentation. It can be diagnosed antenatal or postnatal, may be asymptomatic or may present as neonatal cholestasis and may be associated with anomalies. Management may vary from case to case and mainly depends on complications and age of presentation.