A Path Difficult to Tread: Pure Autonomic Failure, A Case Report

Introduction - Pure autonomic failure is a rare disorder characterized by orthostatic hypotension, absence of a compensatory rise in heart rate, and abnormal autonomic functions. In most cases, supine hypertension is seen coupled with orthostatic hypotension, making the management of these patients a big challenge. We present the case of a 74-year-old gentleman, who presented to the ED with altered mental status for a day; weakness, and falls for 3 weeks. The patient had a past medical history of Hypertension, alcoholism, and REM sleep disorder. He was being treated for erectile dysfunction for the last 10 years and had a family history of Parkinson\u27s disease in his mother and sister. The patient was compliant with Lisinopril 40 mg, Amlodipine, and Rosuvastatin, Tamsulosin 0.4 mg. His blood pressure(BP) on presentation was ranging between 109/74-194/76 mm of Hg. Systolic BP dropped by 30mmHg after tilting the angle of the bed to 45 degrees for 1 minute with no change in HR and the patient became symptomatic in this position. Orthostatic vitals showed a dramatic drop in Systolic BP of \u3e80mmHg with no change in heart rate. MRA and MRI showed chronic microvascular changes. The Echocardiogram, Cortisol, and TSH levels were all normal. All anti-hypertensives were discontinued and supportive treatment was started with Midodrine, Droxidopa, and Pyridostigmine, thigh-high TED hose and abdominal binders at bedtime, and Nitroglycerin patch at night for hypertension. The patient was started on fludrocortisone as he continued to drop his BP by 80 mmHg on standing. The use of TED stockings and bed tilting improved the issue of uncontrolled supine hypertension at night. Conclusion- Treatment of autonomic dysfunction continues to be challenging. There are no definitive guidelines and management is largely individualized. Both pharmacological and non-pharmacological measures are used

rare case of incidentally diagnosed pulmonary inflammatory myofibroblastic tumour with dramatic response to crizotinib in a postpartum woman

Introduction: Inflammatory myofibroblastic tumours are rare neoplasms which most commonly affect children and young adults. With an intermediate malignant potential, they are typically detected in the abdomen, lung, mediastinum, head and neck, gastrointestinal tract, and genitourinary tract. Case description: We describe the case of a 33-year-old postpartum woman incidentally diagnosed with a pulmonary inflammatory myofibroblastic tumour following complaints of poorly controlled hypertension a week after caesarean section. She was ALK-negative and received an ALK inhibitor with complete resolution of the lesion. A ROS1–TFG fusion confirmed the diagnosis of an inflammatory myofibroblastic tumour after CT-guided fine needle aspiration. Discussion: This case highlights an uncommon presentation posing a diagnostic and therapeutic challenge and the potential treatment option of crizotinib

A Case Report of Asymptomatic Presentation of Spontaneous Intrahepatic Biloma

Introduction A biloma is a loculated accumulation of bile outside of the biliary tree; it can either be intrahepatic or extrahepatic. They are usually caused by trauma, iatrogenic procedures like endoscopic retrograde cholangiopancreatography (ERCP), laparoscopic cholecystectomy, percutaneous procedures like transcatheter arterial chemoembolization, microwave ablation, or percutaneous biliary drainage. We are presenting a case of a 78-year-old male with an incidental diagnosis of large biloma noted on CT scan which was obtained for routine lung cancer screening. Case presentation A 78-year-old male with a past medical history of prostate cancer and diabetes was admitted to the hospital after a routine Low dose CT lung screening for lung cancer showed an incidental finding of a liver lesion suggestive of neoplasm. Abdominal examination was unremarkable. CT abdomen and pelvis showed a 9 cm cystic and solid liver lesion in segment 5 associated with gallbladder fossa. The gallbladder was large, and hydropic, with an irregular wall, consistent with intrahepatic rupture of the gallbladder. Blood cultures were negative with no organisms seen on the gram stain, and no growth on the anaerobic culture as well. CT was concerning for cholangiocarcinoma associated with the gallbladder or could be intrahepatic rupture of the gallbladder. Ultrasound-guided Biopsy of the lesion showed needle core fragments of liver parenchyma with granulation tissue with cholestatic pigment associated with foreign body giant cell reaction. Rare foci of necrosis were present. The adjacent hepatocytes showed reactive changes. The overall findings were consistent with intrahepatic biloma. A Cholecystostomy tube was placed to drain the biloma. Repeat CT showed persistent intrahepatic biloma with slightly reduced size. A pigtail catheter was placed to drain the biloma. Discussion Spontaneous bilomas are a rare entity caused by bile leaks that are encapsulated either inside or outside of the liver without cause. Most often, they are secondary to iatragenic or traumatic causes. The common manifestations of biloma are abdominal pain, jaundice, fever, and leukocytosis, none were found in our patient. Appropriate diagnostic imaging includes Ultrasound, HIDA, CT, or even MR imaging. Differential diagnoses include bilhemia, angioma, abscess, cystic lesions, lymphocele, seroma, or hematomas, which can be differentiated with fluid studies. Complications such as infection, perforation, and impingement on surrounding structures may arise. Radiological image-guided aspiration gives us a definitive diagnosis, fluid studies help rule out the infection. Smaller bilomas less than 4 cm may resolve. Treatment of choice may include minimally invasive techniques such as percutaneous drainage or endoscopic retrograde cholangiopancreatography with appropriate stent placement and surgical removal for those that are refractory to treatment