James Benjamin Gleason

Pulmonary Sequestration: A 29 Patient Case Series and Review.

INTRODUCTION Pulmonary sequestration also known as bronchopulmonary sequestration is a rare disease, with very few case series reviewed in literature. In this study, we review the demographics, presentation, imaging and treatment of pulmonary sequestration in 29 patients from our institution, and provide comparison data from previously published series with an overview of the disease history. MATERIALS AND METHODS Records reviewed for all patients evaluated and treated in our institution with a pathological proven diagnosis of pulmonary sequestration from January 2004 through December 2013. Collected data included demographics, clinical presentation, diagnostic imaging, location of the lesion, type of sequestration, and subsequent treatment. RESULTS Of the 29 patients reviewed 8 (28%) were children 0-2 years, 1 adolescent age 17, and 20 (69%) adults 21-70 years with a mean age of 42 among adults. Systemic arterial supply to the sequestered segment was demonstrated with computed tomographic angiography (CTA) in 25 patients (86%). In 19 patients (66%), the sequestered segment was located in the left lower lobe, and 16 (55%) were intralobar. CONCLUSION Diagnostic delays of pulmonary sequestration were common among the adult population as the presenting symptoms often mimicked other common pulmonary diseases, such as pneumonia and asthma. These findings were consistent among previously published series. CTA was the preferred imaging modality for preoperative planning with high sensitivity and specificity in identifying the lesion.

The Rapid Initiation, Titration, and Transition from Intravenous to Oral Treprostinil in a Patient with Severe Pulmonary Arterial Hypertension

In patients who require urgent initiation of pulmonary arterial hypertension medications due to disease progression, it is customary to start intravenous prostacyclin therapy, typically during a hospital admission. If there are complicating factors or relative contraindications to intravenous and subcutaneous prostanoids, oral treprostinil provides another pathway to prostanoid therapy, but this usually requires a prolonged titration. We describe the case of a thirty-six-year-old male with severe pulmonary arterial hypertension and contraindication to intravenous and subcutaneous prostanoid therapy due to congenital deafness and the risk of not hearing the intravenous pump alarms. Intravenous treprostinil was initiated, titrated to high dose, and then rapidly transitioned to oral treprostinil. A rapid initiation, titration, and transition from intravenous to oral treprostinil can be safely performed under watchful supervision in order to achieve higher and more efficacious doses of oral treprostinil in a timely manner.

A Rare Case of Streptococcus alactolyticus Infective Endocarditis Complicated by Septic Emboli and Mycotic Left Middle Cerebral Artery Aneurysm

To date, S. alactolyticus endocarditis complicated by middle cerebral artery aneurysm has not been reported. We describe the case of a 65-year-old female with a history of hypertrophic cardiomyopathy with left ventricular outflow tract obstruction presenting with confusion and a apical holosystolic murmur. Angiography of the brain identified new bilobed left middle cerebral artery aneurysm. Serial blood cultures grew S. alactolyticus, and aortic and mitral valve vegetation were discovered on transesophageal echocardiography. The patient was treated with antimicrobial therapy, mitral and aortic valve replacements, and microsurgical clipping of cerebral aneurysm. This case serves to highlight the pathogenicity of a sparsely described bacterium belonging to the heterogenous S. bovis complex.

Combined Intrathoracic and Subcutaneous Splenosis Discovered 51 Years after Abdominal Trauma

Splenosis is a rare condition that results from the autotransplantation of splenic parenchyma into unexpected locations such as the abdomen or subcutaneous tissue. In the presence of coexisting injury to the diaphragm intrathoracic transplantation can occur emerging as single or multiple pleural-based masses. This occurs after traumatic rupture of the spleen and is usually asymptomatic, only to be discovered incidentally on routine thoracic or abdominal imaging. To our knowledge this is the third documented case of combined intrathoracic and subcutaneous splenosis found in English literature. This occurred in a 71-year-old male involved in a motor vehicle accident at age 19 requiring urgent splenectomy. He has a significant cigarette smoking history and was referred to our hospital for further evaluation of an abnormality seen on shoulder X-ray.

959: INVESTIGATING THE INFLUENCE OF CAFFEINE INTAKE AND THE INCIDENCE OF ICU DELIRIUM

Critical Care Medicine • Volume 46 • Number 1 (Supplement) www.ccmjournal.org Learning Objectives: ICU delirium is associated with prolonged intubation, increased length of stay, and higher mortality. Among the many factors which can influence ICU delirium we investigate caffeine. Caffeine is one of the most frequently used substances in modern society and is often overlooked as potential source of significant withdrawal symptoms when abruptly discontinued upon ICU admission. The relationship of caffeine cessation and ICU delirium rates has not been widely studied. The aim of this study was to examine the relationship between the two. Methods: A retrospectively evaluated cohort of 54 patients admitted to Cleveland Clinic Florida Medical ICU were assessed by recording CAM-ICU status (a validated and commonly used score for monitoring the ICU delirium) and the amount of caffeine they consumed daily prior to admission. Collected variables also included age, sex, primary ICU diagnosis, past medical history, ventilator days, ICU length of stay, as well as use of sedatives or pain medications. Statistical tests used in our analysis included one-way ANOVA. Results: 54 patients were enrolled in the study. Mean caffeine intake for the twenty CAM-ICU positive patients was 364.60mg per day and 187.34mg per day in the thirty-two CAMICU negative patients. 35 % of the CAM-ICU positive patients were male and 65% were female. Average age in the CAM-ICU positive group was 59 years. In the CAM-ICU negative group, 63% were male and 36% were female. Average age was in the CAM-ICU negative group was 63 years. ANOVA identified CAM-ICU positive patients to have a significantly higher daily caffeine intake. Conclusions: ICU delirium is a common and well recognized complication of critical illness. Its development can increase patient morbidity and mortality. While numerous factors likely contribute to the development of ICU delirium our data identifies that those with higher chronic daily caffeine use screen positive for delirium during their ICU stay using the CAM-ICU score. Caffeine’s withdrawal symptoms are well documented and its abrupt cessation may contribute to the development of delirium. While it is unknown whether supplementing caffeine has an impact on the incidence of ICU delirium our results do identify the need for ongoing study of this relationship. We speculate that there may be benefit in the prospective investigation of caffeine supplementation in ICU populations with elevated chronic caffeine intake to reduce withdrawal and determine if there is a reduction in rates of ICU delirium.

Diffuse pulmonary meningotheliomatosis: A literature review of a rare diffuse parenchymal lung disease with unclear clinical significance

Introduction: Diffuse pulmonary meningotheliomatosis is a rare disease, with unclear clinical significance and very few reported cases in the literature. In this study, we review the demographics, presentation, imaging, diagnostic workup, and histologic findings of the 25 patients previously published in the literature with an outline of the disease history. Materials and Methods: We conducted a review of the literature through July 2016 for studies reporting cases of diffuse pulmonary meningotheliomatosis by searching multiple scholarly databases. Results: Of the 25 cases identified 2 were male (8%), and 23 were female (92%). Ages ranged from 37 to 73 with a median age of 59.5 years at diagnosis. 15 (60%) were asymptomatic and imaging abnormalities were discovered incidentally. 8 (32%) had unexplained respiratory complaints. 11 (44%) had history of or active malignancy. 3 (12%) were diagnosed by transbronchial biopsy while the remainder had surgical lung biopsies. Conclusion: Diffuse pulmonary meningotheliomatosis should be considered in all patients with diffuse bilateral pulmonary nodules on HRCT. The condition is more prevalent in females and its clinical significance is unclear, although nearly half of those diagnosed had a history of malignancy. CT imaging and surgical lung biopsy are the modalities of choice for diagnosis but transbronchial biopsies have recently been used obtain the diagnosis. Additional research needs to be done to further characterize the nature of this condition and the clinical scenarios in which is presents.

Pulmonary Varix: An Uncommon Pulmonary Vascular Anomaly

Pulmonary varices are rare lesions reflecting localized dilation of one or more pulmonary veins. These anomalies are rare, with <100 cases reported in the literature. Pulmonary varices are usually discovered incidentally and may be characterized as congenital or acquired; the latter are often related to occlusion of the distal pulmonary venous vasculature, hepatic cirrhosis, or mitral valve disorders, such as mitral stenosis or regurgitation. Pulmonary varices may not be visible on chest radiography, or may present as solitary pulmonary nodules, typically adjacent to the left atrium. Thoracic computed tomography may show tortuous, serpiginous vessels without a vascular nidus and normal-appearing pulmonary arteries, allowing varices to be distinguished from pulmonary arteriovenous malformations. Catheter pulmonary angiography may be used to diagnose pulmonary varices and exclude the possibility of arteriovenous malformations; catheter pulmonary angiography will show one or more dilated vessels leading to the left atrium opacifying on the delayed phase of contrast injection similar to other pulmonary veins, with normal-appearing pulmonary arteries. Treatment is usually unnecessary, unless the varix rapidly increases in size. Pulmonary varices are usually asymptomatic and treatment is rarely required, unless complications, such as hemoptysis, thromboembolic disease, or rupture, occur; in this case treatment is typically directed at the underlying cause for elevated left atrial pressure, although surgical resection may be considered for selected patients.

Meningotheliomatosis: A Rare Cause of Diffuse Miliary Pattern Pulmonary Opacities

A 63 year old female presented for incidentally discovered pulmonary nodules. The Computed Tomography (CT) scan of the chest with contrast showed innumerable and diffusely distributed nodules up to 4mm in a military type pattern [Table/Fig-1]. There was no mediastinal or hilar adenopathy and she denied any respiratory complaints including dyspnea, cough or sputum production. She was born in Paraguay and moved to the United States of America in her early 30’s. Medical, social and family history was remarkable for a 10 pack-year smoking history and occupational chemical fume exposure, working in a compounding pharmacy.

Biphasic Malignant Pleural Mesothelioma Masquerading as a Primary Skeletal Tumor

Biphasic malignant pleural mesothelioma is a rare malignant tumor, usually presenting as a pleural-based mass in a patient with history of chronic asbestos exposure. We herein report a case of a 41-year-old man who presented with chest pain and had a chest computed tomography (CT) scan suggestive of a primary skeletal tumor originating from the ribs (chondrosarcoma or osteosarcoma), with no history of asbestos exposure. CT-guided core needle biopsies were diagnosed as malignant sarcomatoid mesothelioma. Surgical resection and chest wall reconstruction were performed, confirming the diagnosis and revealing a secondary histologic component (epithelioid), supporting the diagnosis of biphasic malignant mesothelioma.

Pneumocystis Pneumonia Presenting as an Enlarging Solitary Pulmonary Nodule

Pneumocystis pneumonia is a life threatening infection that usually presents with diffuse bilateral ground-glass infiltrates in immunocompromised patients. We report a case of a single nodular granulomatous Pneumocystis pneumonia in a male with diffuse large B-cell lymphoma after R-CHOP therapy. He presented with symptoms of productive cough, dyspnea, and right-sided pleuritic chest pain that failed to resolve despite treatment with multiple antibiotics. Chest X-ray revealed right lower lobe atelectasis and CT of chest showed development of 2 cm nodular opacity with ground-glass opacities. Patient underwent bronchoscopy and biopsy that revealed granulomatous inflammation in a background of organizing pneumonia pattern with negative cultures. Respiratory symptoms resolved but the solitary nodular opacity increased in size prompting a surgical wedge resection which revealed granulomatous Pneumocystis pneumonia infection. This case is the third documented report of Pneumocystis pneumonia infection within a solitary pulmonary nodule in an individual with hematologic neoplasm. Although Pneumocystis pneumonia most commonly occurs in patients with HIV/acquired immunodeficiency syndrome and with diffuse infiltrates, the diagnosis should not be overlooked when only a solitary nodule is present.