Understanding Boerhaave’s Syndrome: A Rare but Life-Threatening Esophageal Perforation

1. CASES Dr Rakhi ,R2 Dept of Radiodiagnosis , Medical college , Vadodara.

2. Case - 1

3. Fifty-nine-year-old male with unknown past medical history complaining of vomiting and severe chest pain. • There was no record of him having had any recent procedures

4. Bilateral pneumothoraces Pneumomediastinum-Bilateral effusions

5. Boerhaave’s Syndrome • Foreign Body Perforation of the Esophagus • Mallory- wiess tear

6. Contrast extravasation from the distal esophagus into both pleural cavities--The exam was subsequently terminated

7. Esophageal perforation is a rare, albeit life-threatening, event . • It has been a reported complication in about one per 1000 patients who undergo endoscopic examination . • 55% - iatrogenic, • 15% - spontaneous, • 14% - foreign body ingestion, and • 10% - traumatic. • Cervical esophagus most commonly perforated by endoscopic procedures & Thoracic esophagus may be perforated during stenting or dilatation procedures.

8. The spontaneous esophageal rupture after a violent vomiting episode (ie, Boerhaave syndrome) is typically located in the left posterolateral esophagus, near the left diaphragmatic crus. • About 10 -15% of perforation are caused by ingested foreign bodies such as chicken and meat bones. • When an intra-abdominal segment of the esophagus is perforated by an endoscope, then the perforation is usually into the lesser sac.

9. Boerhaave’s Syndrome • Spontaneous esophageal perforation is secondary to phenomena that increase intraluminal pressure within the esophagus such that all three layers of wall are disrupted (as opposed to a Mallory-Weiss tear, which only involves the mucosa). • The condition is grave secondary to subsequent development of mediastinitis & retropharyngeal or mediastinal abscess . • The syndrome is most common in adults (particularly males), in whom the most common location of the tear (90%) is the site least protected from herniation by adjacent structures--that is, the left posterolateral wall of the distal esophagus. • In neonates, the syndrome is more common in females and usually involves the right distal esophagus. • Clinically, vomiting, severe chest pain, and subcutaneous air in an adult should alert one to the diagnosis, although patients may present without this “textbook” pattern.

10. On a conventional chest radiograph, pneumothorax, pneumomediastinum, mediastinal widening, and subcutaneous air are common findings. • A delayed radiograph twelve to twenty-four hours after the inciting event may reveal a left pleural effusion or left pneumothorax (reflecting the fact that most ruptures occur on the left). • The Nacleiro sign represents air dissecting from the mediastinum down to the left diaphragmatic pleura and appears as a “V”-shaped lucency. • CT, while it may not be as good at identifying the exact site of perforation, rules out other causes of chest pain and/or nausea and vomiting.

11. The other primary modality for evaluating Boerhaave’s syndrome is the contrast esophagram. • A water-soluble contrast such as gastrografin is the agent of choice initially, because barium is known to cause a severe mediastinitis. However, if the exam is negative and if esophageal rupture is still suspected, barium should be used to confirm the diagnosis as well as to aid the surgeon by delineating the location and length of the perforation. • Management is primarily surgical/interventional (e.g. primary repair, diversion procedures, T-tube drainage, or expanding stents) but also includes antibiotics and supportive measures such as fluid resuscitation.

12. a 58-year-old man with burning substernal pain. (a) Esophagogram shows a massive leak of barium to the mediastinum. (b) CT scan shows a periesophageal mediastinal abscess (arrow) with bilateral pleural effusion. A tube is present in the right side of the chest. A right paracardiac collection is also seen (*). Spontaneous perforation of the esophagus (Boerhaave syndrome) with mediastinal abscess

13. Radiological examination: Chest x-ray Extraluminal gas, which forms a radiolucent triangle behind the heart - earliest chest x-ray feature ,extraluminal gas is easier to recognize when it spreads and lifts the visceral pleura from the margin of heart and aorta. Surgical emphysema in neck and upper thorax Mediasinitis cause sympathetic pleural effusion, or mediasinal pleura may rupture to produce a hydropneumothorax, which is more common on left side where the mediastinal pleura is closely applied to the distal esophagus. However plain radiograph may be completely normal.

14. Contrast examination: It is important to obtain decubitus films with horizontal x-ray beam to detect small leaks. Common practice is to start with a nonionic water soluble agent and, if this shows no evidence of significant leakage, then a little dilute barium can be used to obtain best mucosal detail.

15. Spontaneous esophageal perforation or boerhaave’s syndrome. A a p/a chest x ray shows a right sided pneumomediastinum and a left pleural effusion. This findings should be highly suggestive of spontaneous esophageal perforation in a patient with vomiting. B, water soluble contrast medium confirms the presence of a localized perforation of the left lateral wall of the distal esophagus, with extension of the leak laterally and superiorly in the mediastinum.

16. The initial study using water soluble contrast medium shows an irregular contour below the esophagogastric anastomosis. However, no difinite site o fperforation is seen. B. A second study performed moments later with barium shows a sealed off anastomotic perforation. so barium should be given to all patients with suspected perforation if the initial study using water soluble contrast medium fails to demonstrate a leak.

17. Frontal radiograph in patient with perforated distal esophagus due to chicken bone ingestion. Naclerio's V sign (arrows) is seen as an air lucency outlining the medial portion of the left hemidiaphragm and the lower lateral mediastinal border.

18. EXPLANATION • This V-shaped air collection occurs in the setting of pneumomediastinum. • One limb of the V is produced by mediastinal air outlining the left lower lateral mediastinal border. • The other limb is produced by air between the parietal pleura and medial left hemidiaphragm. • Mediastinal air at this location is frequently seen in the presence of esophageal perforation.

19. On chest radiographs, pneumomediastinum is seen as multiple lucent streaks of air outlining mediastinal structures. • It may be extensive, with air tracking up into the neck or chest wall • Pneumomediastinum can be secondary to alveolar rupture, which leads to pulmonary interstitial emphysema that travels centrally back to the mediastinum. • Other conditions that can produce pneumomediastinum include asthma, chest trauma, and barotrauma. • Tracheobronchial injury and esophageal perforation are less common causes of pneumomediastinum.

20. Naclerio described the V sign in patients with spontaneous esophageal rupture. • Leakage of air from the perforated or ruptured distal esophagus produces pneumomediastinum, which results in outlining of the medial left hemidiaphragm and left lower lateral mediastinal area on radiographs. • Naclerio attributed the finding to air "dissecting along diaphragmatic and mediastinal fascial planes in the region of the lower esophagus". • Iatrogenic and traumatic perforations, usually occurring in the proximal esophagus, may not produce the V sign. • Naclerio's V sign is not entirely specific to that condition . Regardless, the presence of Naclerio's V sign in an appropriate clinical scenario may provide an early radiologic clue to the presence of esophageal rupture

21. Esophageal perforation in a 78-year-old man after laser therapy for a Zenker diverticu!um. CT scan shows pneumomediastinum and air bubbles in the mediastinum, compatible with perforation of the esophagus.

22. Mallory – Weiss tear • The tear result from a sudden increase in intraesophageal pressure, as may occur from vomiting or retching. • This tear may cause severe haematemesis. • Endoscopically the mucosal tear is above the gastro- esophageal junction. • On DC barium study the tear can sometimes be seen as a short vertical white line.

23. Esophageal tear can also result from the ingestion of foreign bodies or from endoscopy. Hemorrhage may strip the mucosa from the underlying circular muscle coat to produce a smooth, broad-based filling defect which bulge into the lumen. Esophageal haematoma that spread circumferentially produce an annular stricture, whereas those that spread longitudinally narrow the esophageal lumen. Barium may enter the tear and dissect under the mucosa.

24. Mallory – weiss tear there is a linear collection of barium in the distal esophagus just above the g-e junction. Although a linear ulcer form reflux esophagitis has similar appearance, the correct diagnosis can be made by the clinical history.

25. Esophageal haematoma there is smooth submucosal mass in the distal esophagus, h/o luminal dilatation of achalasia is present. So esophagus is markedly narrowed below the hematoma due to the underlying achalasia.

26. Multiple esophageal perforation due to chicken bone impaction in a 75-year-old woman with sensation of a foreign body, dysphagia, odynophagia, and drooling. (a) Barium esophagogram reveals a double esophageal tear. (b) Photograph of an autopsy specimen shows chicken bone impaction and perforation to the mediastinum. L left perforation, R right perforation

27. Case - 2

28. 30 yr old female with h/o prior small-bowel resection presented with left upper quadrant abdominal pain. • She was afebrile with a normal WBC. • No signs of peritonitis were noted on clinical examination.

33. The abdominal radiograph obtained at presentation with left abdominal pain shows a large mottled gas and debris collection in left upper quadrant. • CECT scans show a large mass proximal to the jejunostomy site. The mass has multiple air bubbles, fluid, and debris scattered throughout, giving it a mottled appearance. Oral contrast material is noted surrounding the mass. • The abdominal radiograph obtained 1 day after CT shows a large mottled gas collection with contrast material. • Axial CT scans obtained 5 months before presentation show focal dilatation of the jejunum proximal to the jejunostomy.

34. Oral contrast material was noted to surround the mass, suggesting the mass was intraluminal. • No thick enhancing wall was identified to suggest an abscess. • The gas was noted to be diffusely scattered in the mass and not in the wall of the bowel, excluding pneumatosis. • The most likely diagnosis of this case is small-bowel bezoar. • Exploratory laparotomy showed an intraluminal mass at the site of the previous jejunostomy. Pathology revealed a phytobezoar of the small bowel with vegetable products in the small-bowel lumen.

35. DISCUSSION • Bezoars of the gastrointestinal tract are most often found in the stomach as persistent concretions of substances such as plant and vegetable fibers (phytobezoar), persimmons (disopyrobezoar), or hair (trichobezoar). • Phytobezoars are the most common and are characterized by concretions of poorly digested fibers, fruit seeds, and pulpy fruits, especially oranges and persimmons. • A primary small-bowel bezoar without associated gastric bezoars is uncommon.

36. Usually, bezoar fragments from the stomach or bolus of food small enough to pass through the pylorus will enter the small bowel where they absorb water, increase in size, and become impacted. • Complete mechanical intestinal obstruction is the most frequent clinical presentation of bezoar. Small-bowel obstruction caused by bezoar is rarely diagnosed preoperatively.

37. Small-bowel bezoars can arise in small-bowel diverticula, in a segment of bowel associated with stricture formation, or proximal to small-bowel tumors. • They almost always present with obstruction and rarely with perforation. • If a small-bowel bezoar is found, the stomach should be inspected closely. Concurrent gastric bezoar is found in 17-21% of patients with small-bowel bezoar.

38. CT reveals a well-defined ovoid intraluminal mass with a mottled gas pattern in the dilated small bowel at the site of obstruction and an abruptly collapsed lumen beyond the lesion. • The mottled appearance is a result of air bubbles retained in the interstices of the mass. • The site of impaction is usually the narrowest portion of the small bowel 50-75 cm from the ileocaecal valve or at the valve itself. • Any part can be affected, especially in patients with postoperative adhesions.

39. D/D: • Small-bowel pneumatosis -gas in the wall of the intestine-usually occurs in the setting of bowel ischemia or infarction. • CT findings of pneumatosis are curvilinear, serpiginous, bubbly collections of gas that occur circumferentially or in peripheral or dependent portions of the bowel wall. • Long segments of the bowel are usually involved.

40. Pneumatosis Intestinalis Cystic or linear collections of gas can be located in subserosa or submucosa of small bowel or colon Causes of air in the bowel wall – -Disruption of the bowel mucosa (eg, bowel obstruction, inflammatory bowel disease), -Increased bowel permeability (eg, graft-versus-host disease, acquired immunodeficiency syndrome, steroid therapy), and -Pulmonary disease (eg, chronic obstructive pulmonary disease) Diagnosis on plain x-ray & CT ( mainly) Important to recognise ischaemic, inflammatory, ulcerative & traumatic conditions 40

41. Abscesses usually develop as a complication of intraabdominal inflammatory processes or after laparotomy. • Patients usually present with fever and elevated WBC. • On CT, abscesses appear as circumscribed round or oval soft tissue-density masses with an attenuation of 10-30 H. • If a well-formed capsule is present, it often shows contrast enhancement, whereas the central area of the abscess, which contains necrotic material. • Intracavitary gas is a suggestive finding in 40-50% of abscesses. The gas may be distributed as finely dispersed air bubbles throughout the collection or as an air-fluid level.

43. Case - 3

44. 53-year-old patient presented with recurrent headaches

45. CT and MRI demonstrated a large non-enhancing thin-walled cyst following CSF signal in the lateral and 3rd ventricles, causing obstructive hydrocephalus.

48. Ependymal cysts • These are rare, benign, ependymal-lined cysts of the lateral ventricle or juxtaventricular region of the temporoparietal region and frontal lobe • They have been infrequently identified in the subarachnoid spaces, brainstem, and cerebellum • Most are incidental, but symptomatic cysts may manifest with – • headache, • seizure, and/or • obstructive hydrocephalus.

49. Ependymal cysts are thought to arise from sequestration of developing neuroectoderm during embryogenesis. • They are thin walled and filled with clear serous fluid secreted from ependymal cells. • Columnar cells, with or without cilia, line ependymal cysts.

50. Imaging • The best diagnostic clue is a well-defined , nonenhancing , thin-walled CSF-containing cyst of the lateral ventricle. • On CT they are isodense to CSF. • On MRI they follows CSF signal on all sequences and do not demonstrate diffusion restriction. • Occasionally the cyst may be hyperintense to CSF if there is a high protein content.