Introduction

Acute pancreatitis is an acute inflammatory process of the pancreas associated with local tissue damage which may lead to a systemic inflammatory response [1]. The most common etiologies are alcohol and gallstones but idiopathic cases may also be seen [2]. Vascular thrombosis is one of the complications that can occur in pancreatitis and involves the splenic vein, portal vein, and superior mesenteric vein more frequently [3]. Extra-splanchnic thrombosis is uncommon, particularly in the internal jugular vein and inferior vena cava [4]. We report a rare case of acute pancreatitis complicated by isolated internal jugular vein thrombosis.

Case Presentation

A 26-year-old Indian woman presented with complaints of pain in the abdomen for 4 days, abdominal distension, and vomiting for 3 days. The pain was severe, more localized to the epigastric region, and radiated toward her back. It subsided on taking analgesics. Initially, the pain was associated with 3–4 episodes of vomiting. The vomiting was non-bilious and non-projectile and was relieved on taking medications. There were no complaints of fever or jaundice. There was no history suggestive of peptic ulcer disease. The patient was a non-smoker and non-alcoholic. She was married and had her last menstrual period 2 weeks ago. She was not using oral contraceptive pills. Examination of the patient revealed a blood pressure of 120/70 mm Hg, pulse rate of 110/min, and respiratory rate of 20/min. Her body mass index (BMI) was 23 kg/m2. Icterus, cyanosis, clubbing, lymphadenopathy, ascites, and pedal edema were absent. Pallor, tachycardia, and marked epigastric tenderness were present.

Investigations showed hemoglobin 6.6 g/dL, total red blood cell (RBC) count 3.46 million/mm3, packed cell volume (PCV) 23.4%, white blood cell (WBC) count 11,900/mm3, and platelet count 223,000/mm3. The peripheral blood film showed a microcytic hypochromic picture. Random blood sugar was 124 mg/dL, blood urea nitrogen 21 mg/dL, serum creatinine 1.1 mg/dL, amylase 801 IU/L, lipase 703 IU/L, corrected calcium 8.32 mg/dL, and prothrombin time (PT) 12.1 s. Liver function tests, serum electrolytes, and lipid profile were normal. Hepatitis and HIV markers were negative. Serum ferritin was 115.6 ng/mL, iron 55 mcg/dL, total iron binding capacity (TIBC) 401 mcg/dL, and vitamin B12 285 pg/mL. Urine and stool examination reports were normal. Contrast-enhanced computerized tomography (CECT) of the abdomen showed a diffusely bulky pancreas with heterogeneous attenuation and peripancreatic fat stranding representing acute pancreatitis. Few non-enhancing areas in the head, body, and tail of the pancreas were seen. There was no evidence of calcification or dilatation of the pancreatic duct. Bilateral pleural effusion, mild ascites, and edema-induced subcutaneous fat stranding in the entire abdominal wall were appreciated. The modified CT severity index was 8. Chest X-ray showed bilateral pleural effusion predominantly on the right side (Fig. 1). A diagnosis of acute pancreatitis with severe anemia with hypocalcemia with bilateral pleural effusion and ascites was made. Anemia was attributed to iron deficiency.

Fig. 1
figure 1

X-ray chest showing bilateral pleural effusion mainly on the right side

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The patient was treated conservatively with intravenous fluids, antibiotics (intravenous meropenem) to prevent secondary pancreatic infection, analgesics, pantoprazole, and other supportive treatment. The patient was kept nil by mouth and Ryle’s tube was inserted. Right-sided thoracocentesis was done and about 1 L of fluid was removed. Pleural fluid was straw-colored, exudative in nature, and characterized by high amylase. Pleural fluid adenosine deaminase (ADA) was 18 U/L. Polymerase chain reaction (PCR) assay was negative for Mycobacterium tuberculosis/non-tuberculous mycobacteria.

After 4 days, the patient was started on an oral liquid diet followed by a semi-solid diet. Sequential X-rays showed the resolution of the pleural effusion. After 10 days of illness, the patient complained of swelling and pain on the right side of the neck with fever, difficulty in swallowing, and mild headache. The patient did not have any previous instrumentation to the IJV or any indwelling lines like central venous catheter (CVC) or peripherally inserted central catheter (PICC) line. There was no history of trauma to the neck, prior deep venous thrombosis, or intravenous drug abuse. Local examination revealed tenderness and swelling over the right side of the neck. Fundoscopic examination of the eyes was normal. Investigations showed hemoglobin 8.24 g/dL, WBC count 11,200/mm3, platelet count 254,000/mm3, erythrocyte sedimentation rate (ESR) 54 mm in the first hour, serum ferritin 207.64 ng/mL, procalcitonin 0.07 ng/mL, and thyroid stimulating hormone (TSH) 0.51 mIU/mL. The real-time polymerase chain reaction (RT-PCR) test was negative for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) RNA. Blood and urine cultures were sterile. PT, activated partial thromboplastin time (aPTT), and thrombin time (TT) were normal. D-Dimer test was positive — 4.74 µg/mL (FEU). Ultrasonographic examination of the neck revealed an ill-defined hetero-echoic material in the right internal jugular vein (IJV) for a craniocaudal span of 5–6 cm which occupied the whole lumen of the vein. Right IJV was dilated and non-compressible with no color flow on color Doppler examination, which was suggestive of venous thrombosis. Other great vessels appeared normal (Fig. 2). A diagnosis of right IJV thrombosis was made. In view of IJV thrombosis, the patient was further evaluated for a hypercoagulable panel including homocysteine, protein C and S, antithrombin III, factor V Leiden, and antiphospholipid antibodies which were normal.

Fig. 2
figure 2

Axial (A) and longitudinal (B and C) gray images showing distended right internal jugular vein with echogenic content within. Color Doppler images show no flow within.

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The patient was started on subcutaneous enoxaparin 40 mg subcutaneously twice daily and oral anticoagulants (warfarin 4 mg once daily) with other symptomatic treatment. On achieving the appropriate prolongation of the international normalized ratio (INR) after 5 days of treatment with warfarin, enoxaparin was discontinued. The neck swelling and pain started to subside after 7 days of anticoagulant therapy. The patient improved symptomatically and was discharged after 15 days on antibiotics (faropenem 200 mg twice daily orally) to prevent subsequent sepsis and septic emboli, warfarin 3 mg daily, oral iron, and other supportive treatment. Venous Doppler examination during follow-up after 3 months showed a partial resolution of the clot with few venous collaterals. The patient was maintained on warfarin for 6 months and is presently doing well.

Discussion

Vascular complications like venous thrombosis, arterial erosion, hemorrhage into pseudocyst, and varices formation develop in around 25% of patients with acute pancreatitis. Thrombosis of veins is a relatively uncommon complication with an incidence of 1–2% [3]. Venous thrombosis most commonly occurs in the splanchnic circulation, most commonly the splenic vein (40%) while the portal vein and superior mesenteric vein also are frequently involved [5, 6]. Thrombosis of splanchnic veins supposedly results from direct inflammatory insult caused by the release of proteolytic and lipolytic enzymes from inflamed pancreatic tissue, extrinsic compression by the edematous gland, and fluid collection or hypercoagulable state [7, 8].

The involvement of extra-splanchnic circulation is quite uncommon in patients with pancreatitis [9]. Inflammatory vasculitis and a generalized hypercoagulable state are postulated as causes of extra-splanchnic venous thrombosis [8]. Ma et al. reported a case of inferior vena cava thrombosis along with renal vein thrombosis [11]. Parikh et al. reported a rare case with subclavian vein, superior and inferior vena cava, and internal jugular vein thromboses with normal splanchnic circulation [8]. Similarly, Patel et al. also observed a case with suprahepatic inferior vena cava and internal jugular vein thrombosis [4] while recently Atif and Hamed reported a case with bilateral internal jugular vein and subclavian vein thromboses [12]. Another rare but recognized vascular complication of pancreatitis is pulmonary embolism [13, 14].

Isolated thrombi have also been reported in the IVC [10] and the renal vein [9]. To the best of our knowledge, isolated internal jugular vein thrombosis observed by us has not been reported earlier as a complication of acute pancreatitis. The absence of risk factors predisposing to venous thrombosis like obesity, smoking, pregnancy, oral contraceptive pills, prior deep vein thrombosis, and prolonged immobilization suggests that IJV thrombosis could be attributed to acute pancreatitis. Moreover, the absence of common etiological factors like prolonged central venous catheterization, trauma to the neck, any obvious malignancy, sepsis, and hypercoagulable states like hyperhomocysteinemia, protein C deficiency, protein S deficiency, antithrombin III deficiency, antiphospholipid antibodies, and polycythemia too supports this presumption. Also, the normal ovaries shown by the CECT examination negate the possibility of ovarian hyperstimulation syndrome (OHSS) as a cause of IJV thrombosis. The presence of co-existing anemia, pleural effusion, and unusual complication of isolated IJV thrombosis could delay the diagnosis of IJV thrombosis with an adverse impact on the outcome. However, an early diagnosis of the IJV thrombosis by ultrasound Doppler led to its proper management and a positive outcome in our case.

Conclusions

Isolated internal jugular vein thrombosis may occur rarely in acute pancreatitis. Awareness of such complications aids in early detection and prompt management of the patient and prevents further morbidity and mortality.