James F. Gruden

Transbronchial Biopsy Interpretation in the Patient With Diffuse Parenchymal Lung Disease

CONTEXT The most common lung tissue samples seen by pathologists worldwide are obtained with the flexible bronchoscope. Specimens taken for examination of diffuse or multifocal parenchymal lung abnormalities pose special challenges for the general surgical pathologist, and these challenges are often compounded by high clinical expectations for accurate and specific diagnosis. OBJECTIVE To present and discuss the most common histopathologic patterns and diagnostic entities seen in transbronchial biopsy specimens in the setting of diffuse or multifocal lung disease. Specifically, acute lung injury, eosinophilic pneumonia, diffuse alveolar hemorrhage, chronic cellular infiltrates, organizing pneumonia, alveolar proteinosis, sarcoidosis, Wegener granulomatosis, intravenous drug abuse-related microangiopathy, Langerhans cell histiocytosis, and lymphangioleiomyomatosis are presented. Clinical and radiologic context is provided for the more specific diagnostic entities. DATA SOURCES The published literature and experience from a consultation practice. CONCLUSIONS The transbronchial biopsy specimen can provide valuable information for clinical management in the setting of diffuse or multifocal lung disease. Computed tomographic scans are useful for selecting appropriate patients to undergo biopsy and in limiting the differential diagnosis. Knowledge of the clinical context, radiologic distribution of abnormalities, and histopathologic patterns is essential. With this information, the surgical pathologist can substantially influence the diagnostic workup and help guide the clinician to an accurate clinical/radiologic/pathologic diagnosis.

UIP diagnosed at surgical lung biopsy, 2000-2009: HRCT patterns and proposed classification system

OBJECTIVE High resolution CT (HRCT) is diagnostic of usual interstitial pneumonia (UIP) if honeycombing is present. However, biopsy-proven UIP also occurs in patients without honeycombing. Identification of specific HRCT patterns may enable specific diagnosis and allow more patients to enter clinical trials. Pattern may also predict prognosis. We sought to identify specific HRCT patterns in patients with biopsy-proven UIP (2000-2009) and to assess outcomes and serial change in pattern. MATERIALS AND METHODS We reviewed the HRCT findings in 44 patients with biopsy-proven UIP and identified four distinct patterns: classic UIP (cUIP) with honeycombing, fibrosis without honeycombing (FnoH), minimal fibrosis (Fmin), and ground-glass present (GGOp). We reviewed electronic medical records for outcome information and serial HRCT examinations when available. RESULTS The extent of fibrosis varied between patterns; findings were always heterogeneous in the cUIP and FnoH patterns. Some Fmin patients had a more homogeneous appearance. The lower lobes were predominantly affected, but upper lobe abnormalities were always present. Mortality from respiratory failure and acute exacerbations occurred regardless of pattern. Serial progression from Fmin to FnoH to cUIP occurred, although in a variable manner. Some individuals had an acute illness (GGOp) as the initial manifestation of UIP. CONCLUSION The FnoH pattern may be diagnostic of UIP in the proper clinical setting; heterogeneity of HRCT appearance is critical and has not been previously emphasized. Grouping of patients on the basis of pattern may allow more accurate assessment of treatment effects. Further validation and study of these HRCT patterns is warranted. Histologic UIP predicts clinical course.

Computed Tomography as an Alternative to Catheter Angiography Prior to Robotic Mitral Valve Repair

BACKGROUND Computed tomography angiography (CTA) of the coronary arteries has been proposed as an alternative screening modality to catheter coronary angiography (CCA) prior to noncoronary cardiac surgery. The safety and utility of preoperative coronary CTA in patients undergoing robotic mitral valve repair is unknown. METHODS One hundred and ninety consecutive patients undergoing robotic repair of degenerative mitral leaflet prolapse were eligible for preoperative CT evaluation; retrospective electrocardiogram-gated CTA of the chest to assess the coronary arteries, followed by contrast-enhanced CT of the abdomen and pelvis to assess the systemic arterial and vascular anatomy. If coronary CTA identified coronary artery stenosis 50% or greater, CCA was performed for further assessment. RESULTS Computed tomography evaluation was performed in 178 patients (94%). Six patients (3%) had coronary artery stenosis 50% or greater identified on coronary CTA and underwent CCA. In each of these cases, CCA revealed no significant obstructive lesion. On a per patient basis, coronary CTA had an accuracy of 91% (95% confidence interval 0.81 to 0.96) for excluding obstructive coronary disease. The CT also demonstrated significant noncoronary vascular findings in 6 patients (3%). These findings included iliac artery dissection and aneurysm (n=4), and pulmonary embolism (n=2). Mitral repair rate was 100% and no patients underwent conversion to sternotomy. Median hospital stay was 3 days and there were no deaths. CONCLUSIONS In patients at low-to-intermediate risk of coronary artery disease, CT is useful as a single screening modality of the coronary arteries and peripheral vasculature to determine candidacy for minimally invasive robotic mitral valve repair.

Chest Pain and Diarrhea: A Case of Campylobacter Jejuni-Associated Myocarditis

BACKGROUND Diarrhea and chest pain are common symptoms in patients presenting to the emergency department (ED). However, rarely is a relationship between these two symptoms established in a single patient. OBJECTIVE Describe a case of Campylobacter-associated myocarditis. CASE REPORT A 43-year-old man with a history of hypertension presented to the ED with angina-like chest pain and a 3-day history of diarrhea. Electrocardiogram revealed ST-segment elevation in the lateral leads. Coronary angiogram revealed no obstructive coronary artery disease. Troponin T rose to 1.75 ng/mL. Cardiac magnetic resonance imaging showed subepicardial and mid-myocardial enhancement, particularly in the anterolateral wall and interventricular septum, consistent with a diagnosis of myocarditis. Stool studies were positive for Campylobacter jejuni. CONCLUSIONS Campylobacter-associated myocarditis is rare, but performing the appropriate initial diagnostic testing, including stool cultures, is critical to making the diagnosis. Identifying the etiology of myocarditis as bacterial will ensure that appropriate treatment with antibiotics occurs in addition to any cardiology medications needed for supportive care.

Plexiform leiomyoma of the esophagus: a complex radiographic, pathologic and endoscopic diagnosis

Radiographic identification of an abnormal lesion in the esophagus routinely occurs during workup of patients with symptomatic dysphagia. Leiomyoma is the most common benign finding; however, plexiform leiomyoma, a distinctive but rare variant, follows an unusual pattern of growth which can be a challenging surgical resection. A review of indexed literature identified a single previous report. We contribute a second case of plexiform leiomyoma with a discussion of the clinical, radiographic, and pathologic characteristics, as well as the differential diagnosis for plexiform lesions.

Coronary Artery Ectasia in an Adult Noonan Syndrome Detected on Coronary CT Angiography

Coronary ectasia is rare in patients with Noonan syndrome. When suspected during echocardiography more common causes including Kawasaki disease in children and atherosclerosis coronary artery disease in adults should be ruled out. Coronary CT angiogram, a non-invasive imaging tool may be preferred over conventional coronary angiogram in the initial diagnosis and monitoring the progression of coronary ectasia in such patients. Aspirin may be considered to prevent coronary thrombosis.

A new intracavitary lesion at echocardiography and MR: A case of mistaken identity

Atrial myxomas often show contrast enhancement following administration of intravenous gadolinium, whereas thrombus appears as a hypointense structure, typically without any contrast enhancement. This case report presents a diagnostic challenge involving a recently developed left atrial mass in which echocardiography and cardiac MRI provided discordant results. While the morphological characteristics of the new left atrial lesion were suggestive of myxoma, the signal characteristics and behavior following intravenous gadolinium at MR, and, in particular, the rapid interval appearance of the lesion, prompted consideration for left atrial thrombus. Subsequent intra-operative and histopathologic evaluation proved the mass to be a left atrial myxoma.

Pulmonary artery sheath haematoma with pulmonary arterial compression: a rare complication of type A aortic dissection mistaken for aortitis

An 89-year-old woman with a history of hypertension and temporal arteritis treated with corticosteroid therapy presented complaining of moderate right-sided chest pain with shortness of breath beginning 1 day earlier. She had no fever, vomiting, syncope, or light headedness. CT aortography showed hyperattenuating aortic wall thickening with unenhanced imaging (arrow, Panel A , and crescentic low attenuation thickening …

Tubular pulmonary opacities detected at chest radiography: An unusual etiology

Pulmonary varices are rare lesions reflecting localized dilation of one or more pulmonary veins. The etiology of pulmonary varices is unknown, but the lesions may be classified as congenital or acquired. Acquired pulmonary varices are most commonly associated with mitral valve disease, typically valvular stenosis. Other associations include pulmonary artery hypertension, coarctation of the aorta, or pulmonary valve stenosis. Pulmonary varices are usually detected asymptomatically; complications, such as thromboembolic disease or rupture, are rare and are generally unassociated with congenital pulmonary varices. Although pulmonary varices may not be visible at chest radiography, these anomalies may appear as a solitary pulmonary nodule, generally adjacent to the left atrium. Tortuous, serpiginous vessels without a vascular nidus and with normal-appearing pulmonary arteries may be seen in thoracic computed tomography scan in patients with pulmonary varices. Catheter pulmonary angiography has traditionally been used to confidently diagnose pulmonary varices and exclude the presence of arteriovenous malformations—the diagnosis is established by identifying a dilated vessel that leads to the left atrium, opacifying on the delayed phase of contrast injection in a manner similar to other pulmonary veins, in the presence of normal pulmonary arteries. Treatment is unnecessary, unless the vein is rapidly increasing in size. Typically, in the case of acquired pulmonary varices, treatment of the underlying cause for elevated left atrial pressure is the preferred approach.

Hyperattenuating Lung Parenchyma: A Rare Diagnostic Consideration

Pulmonary alveolar microlithiasis is a rare idiopathic, autosomal recessive pulmonary parenchymal disorder that may result from mutations in the SLC34A2 gene, which is highly expressed in type II alveolar cells and is involved in phosphate metabolism. Pulmonary alveolar microlithiasis is characterized by the intra-alveolar and interstitial deposition of microliths composed of calcium and phosphate in the absence of calcium and phosphorus metabolic abnormalities. The disorder is often detected asymptomatically at thoracic imaging studies obtained for incidental reasons. When symptoms are present, progressive dyspnea, chest pain, and cough are most commonly encountered. Late in the disease course, cor pulmonale and respiratory failure develop leading to death. The chest radiographic appearance of pulmonary alveolar microlithiasis is frequently suggestive of the disorder, showing multifocal or diffuse, bilateral small circumscribed dense opacities with a mid and lower lung predominance, creating a “sandstorm” appearance. Linear and reticular opacities are often present as well. High-resolution thoracic computed tomography typically shows very dense, small pulmonary parenchymal nodules associated with areas of ground-glass opacity and consolidation. Linear opacities consistent with perilobular accumulation of calcipherites or the accumulation of microliths within the alveolar septae are often observed. The radiographic and clinical presentations are frequently markedly discordant, which is an important clue to the correct diagnosis. Most therapies for pulmonary alveolar microlithiasis have proven ineffective, with lung transplantation reserved for advanced cases.