Syndrome In Setting Research Articles

Rare Case of Secondary Evans Syndrome in Setting of Triple Positive Anti-Phospholipid Antibodies

Rare Case of Secondary Evans Syndrome in Setting of Triple Positive Anti-Phospholipid Antibodies

ID: 218987 Utilization of Neuromodulation for Failed Back Surgery Syndrome in Setting of Recurrent Infections

A 70-year-old female with a past medical history significant for recurrent skin infections, fibromyalgia, Ehlers Danlos Syndrome, and chronic back pain secondary to T12 compression fracture, multilevel spondylosis, and stenosis s/p L2-S1 laminectomy/fusion presented to the ED for significant worsening of her chronic back pain. One-week prior, patient was seen by her primary care physician for a left forearm abscess, underwent incision and drainage in the office, and was discharged on oral antibiotics. One month later, patient began experiencing intractable back pain with associated bilateral lower extremity weakness and subsequently went to the ED. Manual muscle testing of her lower extremities was 3/5 throughout, with no other red flag signs or symptoms. A thoraco-lumbar MRI revealed a T9-T10 epidural collection suggestive of an epidural abscess with cord compression, in addition to a chronic T12 compression fracture. She underwent a T9-T10 laminectomy, with her post-operative course being uneventful. She was discharged to subacute rehab to complete a course of intravenous antibiotics. She was told to follow up for kyphoplasty after her infection clears. One month post-operatively patient continued to have radicular symptoms bilaterally and continued back pain and returned to the ED. An MRI of her thoraco-lumbar spine revealed worsening of T12 compression fracture, T10-T11 vertebral disc herniation with adjacent enhancing fluid collection in the right ventral epidural space suspicious for abscess vs extruded disc material.

S2327 Gurvits Syndrome in Setting of Uncontrolled Diabetes and Acute Gastrointestinal Bleeding Successfully Treated with Extended Duration PPI Despite Continuing DOAC

Introduction: A young male with past medical history (PMH) of severely uncontrolled non-insulin dependent diabetes mellitus (NIDDM) with recurrent admissions for diabetic ketoacidosis (DKA) and hypertriglyceridemia-induced pancreatitis, both secondary to medication non-adherence, presented via emergency medical services (EMS) after being found unresponsive at home and subsequently developed acute esophageal necrosis (AEN) during a prolonged stay in the intensive care unit (ICU) for acute respiratory failure. He was successfully treated with extended duration of proton pump inhibitor (PPI) therapy and control of underlying risk factors despite being continued on anticoagulation. Case Description/Methods: A 39-year-old male with PMH of uncontrolled NIDDM presented was admitted for acute respiratory failure secondary to DKA and pneumonia. He was intubated and admitted to ICU, while continued on DKA protocol including antibiotics. GI was consulted for hematemesis and melena 2 weeks into hospitalization. EGD revealed circumferential black esophageal mucosa in the distal esophagus abruptly ending at the gastroesophageal junction (GEJ) and hiatal hernia. Recommendations included Intravenous (IV) proton pump inhibitor (PPI) and strict avoidance of naso/orgastric (NG/OG) tube. Follow up EGD few months after discharge showed remarkable improvement of mucosa as noted in Figures A and B, despite continuation of DOAC as per patient wishes for DVT developed during the stay. Discussion: AEN is a rare syndrome disproportionately reported in men which is characterized by circumferential black esophageal mucosa in the distal 2 thirds of the esophagus, abruptly ceasing at the GEJ. Ischemia is postulated to be an inciting event. Conditions associated with AEN are antibiotics, sepsis, gastric volvulus, hernia, DKA, malignancy, and prolonged vomiting. Symptoms of upper gastrointestinal bleeding (UGIB) and shock are common presentations. Although biopsy establishes diagnosis and rules out other causes, EGD finding is generally sufficient. Initial management consists of IV fluids and treatment of the underlying cause. IV PPI and nil per os (NPO) is recommended. NG/OG tubes are avoided unless vomiting or obstruction is present. Mortality is largely due to underlying disease rather than directly from AEN, hence supportive care results in resolution in most cases, as seen in our patient. Our case demonstrates the importance of addressing underlying causes, as well as PPI therapy, which can overcome continued anticoagulation.Figure 1.: A. Acute esophageal necrosis discovered on endoscopy while undergoing EGD for acute GI bleeding during ICU admission for DKA and acute respiratory failure requiring mechanical ventilation B. Resolution of acute esophageal necrosis with grossly normal mucosa on follow up endoscopy conducted after extended duration PPI therapy and relatively controlled diabetes.

S2825 Budd-Chiari Syndrome in Setting of Pregnancy and Myeloproliferative Neoplasm

S2825 Budd-Chiari Syndrome in Setting of Pregnancy and Myeloproliferative Neoplasm

1802 A Hard Truth to Swallow

INTRODUCTION: Pseudoachalasia is a condition typically caused by lower esophageal sphincter (LES) obstruction in the setting of invasive neoplasm. In rare cases, a syndrome known as paraneoplastic achalasia can present similarly, but is not associated with tumor invasion into the esophageal mucosa. CASE DESCRIPTION/METHODS: 76-year-old female with history of breast cancer status post mastectomy and radiation presented to her oncologist with one month of progressive dysphagia. She was referred to gastroenterology and scheduled for an outpatient EGD, but presented to the ED with weakness, fatigue and weight loss prior to completion of the EGD. CT scan in the ED showed two right lung masses, mediastinal adenopathy and esophageal wall thickening. Patient was admitted for further workup. Day 1 of admission, patient underwent EGD that was pertinent for severe Candida esophagitis, but was aborted after progressing only 1/3 of the way into the esophagus. Patient was started on fluconazole for esophagitis with improvement in dysphagia. On day 2, patient underwent bronchoscopy with biopsy and was discharged home with oncology follow up. Five days later, she was directly readmitted from the oncology office for worsened dysphagia and continued weight loss. Esophagram showed distal esophageal obstruction presumably secondary to post-inflammatory stricture. EGD the following day showed dilated and poorly contractile esophagus with a spastic LES concerning for achalasia. Manometry revealed absence of esophageal body contractility, but was unable to assess the LES due to intolerance of the procedure by the patient. Further workup including pathology showed that patient had metastatic non-small cell carcinoma (NSCC), lung primary. It was determined that patient had pseudoachalasia due to paraneoplastic syndrome in setting of non-small cell carcinoma of the lung without invasion of the tumor into the LES. Patient ultimately underwent palliative botox injection at the LES with improvement in symptoms. DISCUSSION: Paraneoplastic syndromes are considered rare disorders triggered by an altered immune response in the setting of malignancy. Paraneoplastic achalasia is a type of paraneoplastic syndrome that presents as a rapidly progressive dysphagia. Though it has been reported in the setting different malignancies, this is one of the very few case reports of paraneoplastic achalasia due to NSCC. This case also emphasizes the importance of considering other causes of achalasia when evaluating patients with abnormal presentations.