Medical Image of the Week: Boerhaave Syndrome

Figure 1. Panel A: Coronal CT image with IV contrast showing a massively dilated esophagus with retained food particles.  Panel B: Coronal CT image depicting distal esophageal perforation (red arrow) rupturing into the lung parenchyma with resultant abscess formation (yellow arrow). Panel C: Axial image showing the dilated esophagus, ruptured into the lung (arrow). There is also mass effect on the mediastinum and heart. Panel D. After insertion of a nasogastric tube and chest tube in the lung abscess, computed tomography was performed after administration of oral contrast. There is extravasation of contrast into the lung cavity which now contains a drainage catheter. Arrow shows the rupture site.

A 41-year-old woman with a history of gastroesophageal reflux disease (GERD), asthma and iron deficiency anemia presented with complaints of right sided chest pain, nausea and emesis for several days prior to hospital presentation. She had also been experiencing progressive dysphagia to solids for a month preceding admission. CT chest imaging revealed mega-esophagus (Figure 1A) with rupture into the right lung parenchyma and resultant abscess formation (Figure 1B and 1C). A subsequent echocardiogram also confirmed mitral valve endocarditis. An image-guided chest tube was placed in the abscess for drainage. Endoscopy was attempted but visualization was difficult due to the presence of retained food. Given her low albumin and poor nutritional state, a jejunostomy tube was placed. Follow up CT imaging with contrast through a nasogastric tube confirmed extravasation of esophageal contrast into the right lung parenchyma (Figure 1D).  

Blood and sputum cultures grew Candida glabrata. She was initially started on broad spectrum antibiotics which were later tapered to Liposomal Amphotericin B and ampicillin-sulbactam. Following resolution of her fungemia and optimization of her nutritional status 2 months later, she underwent Ivor Lewis esophagectomy, pyloroplasty and serratus anterior muscle flap buttress to the remnant esophageal staple line. Pathology of the excised esophageal tissue revealed muscular hypertrophy and marked reduction of ganglion cells consistent with achalasia. There was also a segment of esophageal mucosal ulceration, acute inflammation and an area of perforation. Post-operative esophagram revealed no obstructions and contrast flowed without issue through the proximal esophagus into the gastroesophageal anastomosis and into the stomach. The patient did well and on discharge from the hospital was tolerating oral intake.

This case illustrates the multi-faceted approach sometimes required for successful treatment of Boerhaave syndrome, or rupture of the esophagus usually after emesis. Initial management included treating the patient’s sepsis with appropriate antifungal therapy in addition to placing a jejunostomy tube for nutrition—a conservative approach which has proven successful in other reported cases (1). Following resolution of the fungemia, she underwent surgical repair for permanent treatment of her esophageal disease.

While the patient had underlying achalasia predisposing her to spontaneous esophageal rupture, Candida glabrata has also been reported to compromise the esophageal lining through angio-invasive mechanisms (2). Given the pathology findings of mucosal ulceration and inflammation of excised esophageal tissue, it is likely that the patient’s Boerhaave syndrome was due to both a combination of achalasia and Candida glabrata esophageal infection.

Nour Parsa MD¹, Bhupesh Pokhrel MD², Arash Meshksar MD³, Mark Meyer MD⁴, and Samuel Kim MD⁴

Departments of ¹Medicine, ²Gastroenterology, ³Radiology, and ⁴Cardiothoracic Surgery, University of Arizona

Tucson, AZ USA

References

1. Shen G, Chai Y, Zhang GF. Successful surgical strategy in a late case of Boerhaave's syndrome. World J Gastroenterol. 2014 Sep 21;20(35):12696-700. [CrossRef] [PubMed]
2. Tran HA, Vincent JM, Slavin MA, Grigg A. Esophageal perforation secondary to angio-invasive Candida glabrata following hemopoietic stem cell transplantation. Clin Microbiol Infect. 2003 Dec;9(12):1215-8. [CrossRef] [PubMed] 

Cite as: Parsa N, Pokhrel B, Meshksar A, Meyer M, Kim S. Medical image of the week: Boerhaave syndrome. Southwest J Pulm Crit Care. 2016;12(6):233-5. doi: http://dx.doi.org/10.13175/swjpcc039-16 PDF

Medical Image of the Week: Left Ventricular Non-compaction

Figure 1. Cardiac MRI showing severely enlarged and remodeled left ventricle (LV) and moderately enlarged right ventricle (RV) with severe global hypokinesis and akinesis of the interventricular septum. Significant trabeculation was noted in the apical, antero-lateral and anterior segments of the LV (red arrows), consistent with LV non-compaction.

A 38-year-old woman with history of type 2diabetes mellitus and hypertension presented to emergency department with worsening exertional dyspnea and orthopnea for the past 2-3 months. She also reported a 14 pound weight gain within the 2 weeks prior to presentation. She denied any prior history of cardiac or pulmonary disease. Also, there was no family history of heart disease. She denies any recent sick contacts, smoking, alcohol drinking, or substance abuse.

Physical exam revealed jugular venous pressure of 10 cm H₂O and significant bilateral lower extremity pitting edema. Chest x-ray showed an enlarged cardiac silhouette. Brain naturetic peptide (BNP) was 2,917 pg/mL. A subsequent echocardiogram revealed a left ventricular (LV) ejection fraction of 23% with severe global LV hypokinesia with moderate mitral regurgitation. Thyroid panel as well as iron panel were within normal range. Other laboratories were unremarkable. For the new onset systolic heart failure, a coronary angiography was performed, which demonstrated normal coronary arteries. The patient was diagnosed with non-ischemic cardiomyopathy and underwent a cardiac MRI, which showed severely enlarged and remodeled LV and moderately enlarged right ventricle (RV) with severe global hypokinesis and akinesis of the intraventricular septum. Moreover, a significant trabeculation was noted in the apical, antero-lateral and anterior segments of the left ventricle (Figure 1), consistent with “LV non-compaction” without any evidence of LV thrombus. The patient was started on diuretics and safely discharged with significant symptoms improvement.  

LV non-compaction is a cardiomyopathy characterized by altered myocardial wall with prominent left ventricular trabeculae and deep intertrabecular recesses (1). Some authors believe that non-compaction of the ventricular myocardium results from abnormal persistence of the trabecular layer  while others believe that altered regulation in cell proliferation, differentiation, and maturation during ventricular wall formation, resulting in hyper-trabeculation (2). Its prevalence in the general population is unknown but among patients undergoing echocardiography is estimated at 0.014 to 1.3 percent. In patients with heart failure, its prevalence has been reported as 3 to 4 percent (3). Patients with LV non-compaction may present with heart failure, arrhythmias, sudden cardiac arrest, syncope, and thromboembolic events. The diagnosis is usually established by transthoracic echocardiography. When echocardiography is indeterminate, cardiac MRI, computed tomography, or left ventriculography could be an alternative diagnostic modality. Data on treatment of LV non-compaction are limited, and there is no standard therapy established for this condition. Medical management depends on the clinical manifestations, LV ejection fraction, presence of arrhythmias, and risk of thromboembolism.

Rostam Khoubyari MD^1,2 and Seongseok Yun MD PhD³

¹Department of Cardiology, ²Sarver Heart Center; and the ³Department of Medicine, University of Arizona

Tucson, AZ USA

References

1. Maron BJ, Towbin JA, Thiene G, et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation. 2006 Apr 11;113(14):1807-16. [CrossRef] [PubMed]
2. Henderson DJ, Anderson RH. The development and structure of the ventricles in the human heart. Pediatr Cardiol. 2009 Jul;30(5):588-96. [CrossRef] [PubMed]
3. Kovacevic-Preradovic T, Jenni R, Oechslin EN, Noll G, Seifert B, Attenhofer Jost CH. Isolated left ventricular noncompaction as a cause for heart failure and heart transplantation: a single center experience. Cardiology. 2009;112(2):158-64. [CrossRef] [PubMed]

Cite as: Khoubyari R, Yun S. Medical Image of the week: left ventricular non-compaction. Southwest J Pulm Crit Care. 2016;12(6):229-30. doi: http://dx.doi.org/10.13175/swjpcc036-16 PDF