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: Boerhaave's Syndrome During Colonoscopy

Figure 1. Chest X ray showing bilateral subcutaneous emphysema extending from the supraclavicular area and above to the neck.

Figure 2. Video of representative coronal views of the thoracic CT scan showing subcutaneous emphysema in the supraclavicular area and neck.

Figure 3. Fluoroscopic esophagram revealing a focus of oral contrast actively extravasating (white arrow) approximately at 2.5 cm above the gastro-esophageal junction consistent with a small perforation.

A 76-year-old woman with no significant past medical history underwent outpatient screening colonoscopy. The procedure was difficult due to a tortuous colon and only multiple diverticula were visualized. She vomited once during the procedure. In the immediate postoperative period, she complained of neck swelling. Her vital signs were stable. On examination, right sided neck and facial swelling with palpable crepitations were noticed as well as coarse breath sounds heard on auscultation of both lung fields. Immediate chest X-ray (Figure 1) was obtained which showed bilateral subcutaneous emphysema extending from the supraclavicular area and above to the neck. Subsequent thoracic CT scan showed extensive subcutaneous air within the soft tissues of the neck bilaterally, extending into the mediastinum and along the anterior chest wall (Figure 2). An esophagram (Figure 3) revealed a focus of oral contrast actively extravasating approximately at 2.5 cm above the gastro-esophageal junction consistent with a small perforation. She underwent left thoracotomy with esophageal repair. Further hospital course was uncomplicated and she was discharged to a sub-acute rehabilitation facility. 

Boerhaave's syndrome is a spontaneous perforation of the esophagus due to sudden increase in intra-esophageal pressure combined with negative intrathoracic pressure caused by straining or vomiting (1). The tear usually occurs at the left posterolateral wall of the lower third of the esophagus. Usually patients have severe retching and vomiting which is followed by excruciating retrosternal chest and upper abdominal pain after perforation. Other manifestations are odynophagia, tachypnea, dyspnea, fever, and shock. On physical examination subcutaneous emphysema (crepitation) is an important diagnostic feature. Chest radiograph usually reveals mediastinal or free peritoneal air as the initial manifestation, and hours to days later pleural effusion with or without pneumothorax, widened mediastinum, and subcutaneous emphysema are typically seen. The diagnosis of esophageal perforation can also be confirmed by water-soluble contrast esophagram using Gastrograffin, which reveals the location and extent of extravasation of contrast. Treatment depends upon the size and location of the perforation. Surgery is generally required for thoracic perforations while cervical perforations can often be managed conservatively with continuous nasogastric suction, intravenous broad-spectrum antibiotics, and parenteral nutrition.

Chandramohan Meenakshisundaram MD, Nanditha Malakkla MD and Venu Ganipisetti MD

Department of Internal Medicine

Presence Saint Francis Hospital

Evanston, IL USA

Reference

1. Nirula R. Esophageal perforation. Surg Clin North Am. 2014;94(1):35-41. [CrossRef] [PubMed]

Reference as: Meenakshisundaram C, Malakkla N, Ganipisetti V. Medical image of the week: Boerhaave's syndrome during colonoscopy. Southwest J Pulm Crit Care. 2015;11(1):42-44. doi: http://dx.doi.org/10.13175/swjpcc058-15 PDF