Sarcoidosis Resulting in Exsanguinating Esophageal Variceal Hemorrhage
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CASE REPORT Sarcoidosis Resulting in Exsanguinating Esophageal Variceal Hemorrhage Adam Blumenberg, MD, MA; Sophia Sharifali, MD; Richard Sinert, DO A 47-year-old woman with a history of pulmonary and renal sarcoidosis presented for evaluation of hematemesis and melena. arcoidosis is a systemic disorder of made by a compilation of clinical signs unknown etiology and is character- and symptoms, imaging studies, and biop- ized by the formation of granulo- sies demonstrating noncaseating granulo- S mas throughout various organs in mas. This case report describes a patient the body. The most common form is pul- who presented with portal hypertension monary sarcoidosis, which affects 90% of and esophageal variceal bleeding second- patients; the second most common form is ary to sarcoidosis of the liver without cir- oculocutaneous sarcoidosis;1 and the third rhotic changes. most common form is hepatic sarcoidosis, which affects 63% to 90% of patients.2 Case Although the liver is frequently involved A 47-year-old woman presented to the ED in all forms of sarcoidosis, only a fraction via emergency medical services with a of patients present with clinically evident 1-hour history of hematemesis and mele- liver disease.1 Approximately 20% to 30% na. The patient stated that she felt fatigued, of patients have abnormalities on liver nauseated, and light-headed, but had no function tests, whereas only about 1% of pain or focal weakness. Her medical histo- patients show evidence of portal hyperten- ry was significant for pulmonary and renal sion and cirrhosis.3 In fact, in the English sarcoidosis. She underwent a liver biopsy literature, there were 35 reported cases of 1 week prior to presentation, with a 6-day portal hypertension due to sarcoidosis be- hospitalization period, due to new ascites tween 1949 to 2001, of which 16 of the pa- found on examination. tients had no evidence of cirrhosis.4 The patient’s vital signs at presentation The diagnosis of sarcoidosis is usually were: blood pressure (BP), 72/56 mm Hg; Dr Blumenberg is a resident physician, department of emergency medicine, Kings County Hospital Center, Brooklyn, New York; and depart- ment of emergency medicine, Downstate Medical Center, Brooklyn, New York. Dr Sharifali is a resident physician, department of emergency medicine, Kings County Hospital Center, Brooklyn, New York; and department of emergency medicine, Downstate Medical Center, Brooklyn, New York. Dr Sinert is an attending physician, department of emergency medicine, Kings County Hospital Center, Brooklyn, New York; and department of emergency medicine, Downstate Medical Center, Brooklyn, New York. Authors’ Disclosure Statement: The authors report no actual or potential conflict of interest in relation to this article. DOI: 10.12788/emed.2018.0094 118 EMERGENCY MEDICINE I JUNE 2018 www.emed-journal.com SARCOIDOSIS RESULTING IN EXSANGUINATING ESOPHAGEAL VARICEAL Table. Patient’s Laboratory Values Value 1 Week Prior to Presentation At Presentation Reference Range Sodium 139 131 135-146 mmol/L Potassium 4.6 6.9 3.5-5.0 mmol/L Chloride 106 99 98-106 mmol/L Carbon dioxide 13 7 24-31 mmol/L Blood urea nitrogen 74 138 6-20 mg/dL Creatinine 2.9 5.4 0.5-0.9 mg/dL Glucose 96 160 70-99 mg/dL Calcium 8.2 7.7 8.6-10.0 mg/dL Protein 5.1 5.1 6.0-8.5 g/dL Albumin 2.3 2.2 2.8-5.7 g/dL Aspartate aminotransferase 36 25 10-35 U/L Alanine transaminase 18 14 0-31 U/L Alkaline phosphatase 390 497 25-125 U/L T bilirubin 0.45 0.45 0.0-1.2 mg/dL Platelets 256 400 130-400 /nL Hemoglobin 7.7 4.7 12.0-16.0 g/dL Hematocrit 26.3 17.1 37.0-47.0% White blood cells 9.3 18.4 4.5-10.9 /nL International normalized ratio 1.1 1.1 0.8-1.2 Activated partial thromboplastin time 27.0 27.3 22-29 sec heart rate (HR), 133 beats/min, respirato- angiomata, fetor hepaticus, caput medusa, ry rate, 24 breaths/min; and temperature, cutaneous ecchymoses, or any other stig- 97.0oF. Oxygen saturation was 99% on mata of cirrhosis. room air. Physical examination revealed an Two large-bore peripheral intravenous alert and oriented middle-aged woman in (IV) catheters were placed and a massive extremis who was vomiting dark-colored blood transfusion protocol was initiated. blood. The cardiac and pulmonary exami- Packed red blood cells (PRBCs) from the nation revealed no extraneous sounds; the resuscitation-area refrigerator were in- abdominal examination showed ascites fused immediately via a pressurized fluid with a liver edge palpable 4 cm beneath warmer. the right costal margin. The patient had After consultation with gastroenterology no scleral icterus, palmar erythema, spider and general surgery services, the patient www.emed-journal.com JUNE 2018 I EMERGENCY MEDICINE 119 SARCOIDOSIS RESULTING IN EXSANGUINATING ESOPHAGEAL VARICEAL plasma, and platelets over a 3-hour period. During transfusion, the patient’s vital signs improved to a systolic BP ranging between 110 to 120 mm Hg and an HR ranging be- tween 90 to 110 beats/min; she did not ex- perience any further hypotensive episodes throughout her stay in the ED. Laboratory studies were significant for metabolic acidosis, hyperkalemia, acute on chronic anemia, leukocytosis, and acute on chronic renal failure. Synthetic function of the liver and transaminases ap- peared normal (Table). The patient’s hyperkalemia was treated with 1 g calcium chloride IV, 50 g dextrose IV, and 10 U regular insulin IV. A portable chest radiograph showed an appropriately positioned endotracheal tube, and an elec- Figure 1. Computed tomography scan of the patient’s abdomen and pelvis dem- onstrating ascites and hepatomegaly with scattered hypoattenuating lesions and trocardiogram revealed sinus tachycardia periportal lymphadenopathy. without signs of hyperkalemia. A comput- ed tomography (CT) scan of the abdomen was given 1 g ceftriaxone IV, 1 g tranexam- and pelvis from the patient’s recent hos- ic acid IV, 20 mcg desmopressin IV, 50 mcg pitalization, 1 week prior to presentation, octreotide IV, 40 mg pantoprazole IV, 8 mg showed hepatomegaly, liver granulomas, ondansetron IV, 4 g calcium gluconate IV, ascites, and periportal lymphadenopathy and 100 mg hydrocortisone IV. (Figure 1). Throughout the patient’s first 10 minutes A review of the patient’s recent liver in the ED, she remained persistently hypo- biopsy and ascitic fluid analysis revealed tensive and continued to vomit. Since the noncaseating granulomas compressing the patient’s sensorium was intact, the team hepatic sinusoids, and a serum ascites al- quickly discussed goals of care with her. bumin gradient greater than 1.1 g/dL, im- The patient’s wishes were for maximal life- plying portal hypertension without cirrho- sustaining therapy, including endotracheal sis. The surgical team attempted to place intubation and chest compressions, if nec- a Sengstaken-Blakemore tube, but the de- essary. vice could not be positioned properly due After this discussion, the patient was to the patient’s narrowed esophagus. given IV etomidate and rocuronium and The ED nurses cleaned the patient, pre- was intubated using video-assisted laryn- serving her dignity; thereafter the patient’s goscopy. Following intubation, she was adult children visited with her briefly be- sedated with an infusion of fentanyl and fore she was taken for an upper endoscopy, underwent orogastric tube placement to which was performed in the ED. The en- aspirate stomach contents. A total of 2.5 L doscopy revealed actively hemorrhaging of frank blood were drained from the pa- esophageal varices at the gastroesophageal tient’s stomach. junction (Figure 2). The varices were treat- A size 9 French single lumen left- ed with endoscopic ligation; the gastroen- femoral central venous catheter also was terologist placed a total of 11 bands, result- placed, through which additional blood ing in cessation of bleeding. products were infused. The patient re- After the endoscopy, the patient was ad- ceived a total of 28 U PRBCs, fresh frozen mitted to the medical intensive care unit 120 EMERGENCY MEDICINE I JUNE 2018 www.emed-journal.com SARCOIDOSIS RESULTING IN EXSANGUINATING ESOPHAGEAL VARICEAL (ICU). Approximately 1.5 hours after ar- riving at the ICU, she developed renewed hematemesis. Despite efforts to control bleeding and provide hemodynamic sup- port, the patient died 1 hour later. Discussion Etiology Esophageal variceal hemorrhage is caused by pressure elevation in the portal venous system, leading to engorged esophageal veins that can bleed spontaneously. Ap- proximately 90% of portal hypertension is due to liver cirrhosis.5 The remaining 10% of cases are primarily vascular in eti- ology, with endothelial dysfunction and thrombosis leading to increased portal re- sistance. Noncirrhotic causes of portal hy- pertension include malignancy, congenital diseases, viral hepatitides, vascular throm- Figure 2. Endoscopy demonstrating ligation of the patient’s esophageal varices. boses or fistulae, constrictive pericarditis, fatty liver of pregnancy, drugs, radiation chance for survival. injury, and infiltrative diseases.5 Sarcoidosis may cause noncaseating Medical Therapy granulomas to form in the liver, leading to The first priority in managing and treat- portal hypertension and fatal exsanguina- ing esophageal varices is to secure the pa- tion from esophageal variceal hemorrhage. tient’s airways to prevent aspiration. Two Although the lesions of sarcoidosis classi- large bore IV lines should be placed to cally form in the lungs, any organ system permit rapid infusion of crystalloid fluids may be affected.6,7 Frank cirrhosis of