Brandon Nokes

Inhaled nitric oxide mitigates need for extracorporeal membrane oxygenation in a patient with refractory acute hypoxemic respiratory failure due to cardiac and pulmonary shunts

We present a case of refractory acute hypoxemic respiratory failure due to influenza B pneumonia with concomitant large intra-atrial shunt (IAS) and severe pulmonary regurgitation in a patient with Saethre-Chotzen syndrome with prior pulmonary homograft placement. Our patients hypoxemia improved with inhaled nitric oxide as an adjunct to mechanical ventilation without requiring extracorporeal membrane oxygenation, and eventually a percutaneous closure with a 30 mm CardioSeal patent foramen ovale closure device was accomplished. However, his peri-procedural hospital course was complicated by occluder device migration, which was retrieved with eventual surgical closure of the PFO. Nitric oxide has not demonstrated any statistically significant effect on mortality and only reported to transiently improved oxygenation in patients with hypoxemic respiratory failure. Our case demonstrates that inhaled nitric oxide may have a role in acute hypoxemic respiratory failure in a case with significant cardiac and pulmonary shunts.

Pneumocytic adenomyoepithelioma: A case providing support for the benignity of this extremely rare pulmonary neoplasm

Pneumocytic adenomyoepithelioma is an extremely rare and poorly understood pulmonary neoplasm, so experience with this tumor is limited. Since the initial case series where the lesion was first proposed as a distinctive entity, only one additional report has been described. We present a case of pneumocytic adenomyoepithelioma with clinical and radiologic data that provide the first long-term evidence of the benignity of this extremely rare pulmonary neoplasm. We also review the available literature surrounding pneumocytic adenomyoepitheliomas. Our case provides important new data on the behavior of this lesion, as imaging studies showed essentially stable or very slowly progressive disease over the course of approximately 9 years. Collectively, this rare and poorly described lesion appears to behave in an indolent or benign fashion, a notion that our case further supports.

Unicentric castleman disease complicated by paraneoplastic bronchiolitis obliterans and pemphigus

Bronchiolitis obliterans (BO) and paraneoplastic pemphigus are rare and ominous complications of Castleman disease. Collectively, these processes have been reported as part of paraneoplastic autoimmune multiorgan syndrome (PAMS), and they can occur in the setting of various hematologic malignant tumors, carcinoid tumors, and melanoma. Irrespective of the underlying malignancy driving PAMS, the clinical outcomes are uniformly poor, and there are no standard treatment regimens, given the clinical rarity of the syndrome. We describe 2 patients with unicentric Castleman disease complicated by paraneoplastic pemphigus and bronchiolitis obliterans. In addition to primary surgical resection for Castleman disease, we also used therapy from a treatment protocol used for bronchiolitis obliterans resulting from hematopoietic stem cell transplant (HSCT). We were able to treat the patients using intravenous immunoglobulin; rituximab; fluticasone, azithromycin, and montelukast (FAM); and rosuvastatin therapy. One patient demonstrated a favorable response, while the other demonstrated minimal response to this therapy.

Anticoagulation in Behçet related intrathoracic vasculitis

Behçet disease is a rare multisystem condition associated with HLA-B51 positivity that commonly afflicts individuals of Turkish or Middle Eastern descent, less than 10% of whom have pulmonary involvement. Behçet-related pulmonary vasculitis is an uncommon and heterogeneous group of conditions, often with associated pulmonary artery thrombus formation. These microthrombi can result in a misdiagnosis of acute pulmonary embolism. Anticoagulation therapy can be difficult, as blood thinners increase the risk of pulmonary hemorrhage without affording the same benefits as in pulmonary embolism management. We present two cases of pulmonary vasculitis in the context of Behçets syndrome, one in a Native American man with associated superior vena cava syndrome and pericarditis, with an increased risk of hemorrhagic pericardial effusion, and the other in an African American man with acute hypoxic respiratory failure with an increased risk of alveolar hemorrhage. We describe their management and the balancing act surrounding anticoagulation therapy in Behcet-related pulmonary vasculitis.

Monster Lung Cavity in a Heart Transplant Recipient

Invasive mucormycosis infections occur in less than 1% of recipients of orthotopic heart transplants. Given the angioinvasive nature of these infections, the mortality rate is high. Little literature exists regarding the presentation and management of these infections. We present a case of a patient who developed an infection after orthotopic heart transplant, describe the successful multidisciplinary management surrounding his care, and review the available literature regarding mucormycosis infections in heart transplant recipients.

Antiphospholipid Syndrome With a Distinctive Constellation of Neurological Manifestations: Blue Toes, Red Valves, White Retinal Spots

Antiphospholipid syndrome (APS) encompasses a hypercoagulable state with a markedly increased risk for cerebrovascular complications. In addition to the classic stroke features of APS, however, there are numerous recently described non-criteria neurological conditions such as headaches, seizures, and cognitive impairment. We present a case of APS with uncommon neurological manifestations.

Intrathoracic ganglioneuroma presenting as an endobronchial mass

Peripheral nerve sheath tumors (PNST) are exceedingly rare, especially outside of the posterior mediastinum. These tumors represent less than 1% of pulmonary tumors. Very few pulmonary PNSTs are ganglioneuromas. We present a case of a ganglioneuroma presenting as an endobronchial mass. Case presentation An 80 year old male was seen in pulmonary clinic for routine cancer screening. He had a 60-pack year smoking history. CT evaluation noted a 1cm right lower lobe endobronchial lesion. This lesion was present since 2012 and had slightly increased in size since that time from 8mm (Figure 1). The lesion was further assessed using virtual bronchoscopy (Figure 2). Bronchoscopy revealed an obstructing lesion, which was completely excised with the snare (Figure 3). Pathology revealed well-circumscribed tumor consisting of nests and trabeculae of round/polygonal cells with granular eosinophilic and basophilic cytoplasm. The tumor was chromogranin, synaptophysin, S-100, pancytokeratin, SOX10, and TTF-1 positive, consistent with a ganglioneuroma. Discussion Aside from a solitary article regarding 75 patient samples (which included only one ganglioneuroma) only a small number of intrathoracic PNSTs have been reported. Only a single case report of an endobronchial ganglioneuroma has been reported. Each of these lesions were benign, and detected on routine imaging evaluations. Conclusions An intrapulmonary endobronchial location for a PNST is an exceedingly rare presentation of an already uncommon pathology.