1. Snap, crackle, pop!
Lester Mercuur
09 Nov 2006

2. Objectives Case presentation Presentation of X-rays
Discussion of the differential diagnosis
Back to the case

3. Case
71yo lady referred from Claresholm where she had been admitted the previous day with:
Feeling unwell for 3 days with chest discomfort;
Vomiting coffee-ground material x 1 day
PMH: Depression; CHF; hiatus hernia; no previous surgeries
Meds: Maxeran; Prevacid; Trazadone; Lasix
Hospitalised overnight with IV hydration and analgesics.

4. Case
Sudden onset of dyspnoea at 0500 on day of referral to FMC with chest discomfort.
T 37.7ºC; P 97 RR 40; BP 129/78
Physician noticed facial swelling and a pulmonary infiltrate on CXR and referred patient as a SVC syndrome.
No improvement with Ventolin and Lasix.
WBC 10.8; Hb 129; MCV 89; Plt 285
T/F to FMC

5. Case On arrival at 0830, 71 yo female with resp distress.
T 36.6ºC P BP127/60 RR O2 sat on RA 91%
Periorbital swelling bilaterally.

7. 3 months earlier
(PA and lat)

9. Differential diagnosis
Pneumomediastinum 2º to oesophageal perforation: FB
Boerhaave Syndrome
Complication of hiatus hernia

10. Course in the ED
Repeat CXR

12. Course in the ED Repeat CXR
CT chest – water-soluble contrast (Gastrografin)
Blood cultures
Antibiotics
Thoracic Surgery consult OR
Imaging Studies:
Chest radiographs can be very helpful in diagnosing esophageal perforation, with suggestive abnormalities reported in about 90% of cases.
Pneumomediastinum and subcutaneous emphysema are often present an hour after the injury and are highly suggestive of perforation.
Mediastinal air-fluid levels, pleural effusions (often left sided), free air under diaphragm, pneumothorax, and hydropneumothorax encompass later potential findings.
Lateral neck radiographs may demonstrate air in the fascial planes early in cases of cervical perforations.
A contrast esophagogram should be performed in any patient with suspected perforation.
Despite a modest sensitivity (60-75%), a water-soluble contrast agent (Gastrografin) should be the initial study of choice.
A barium study should be undertaken immediately afterward should the initial study show no evidence of perforation.
Barium has a higher sensitivity (90%) for detecting small perforations but may cause a severe inflammatory response in tissues, most notably a mediastinitis.
Studies should be performed with the patient in the right lateral decubitus position.
A contrast-enhanced CT scan of the chest should be obtained if it is not possible to obtain a contrast esophagogram, if the esophagogram was negative despite a high clinical suspicion, or if seeking to evaluate for a more likely alternative diagnosis. Perforation may be suggested by mediastinal air, extravasated luminal contrast, periesophageal fluid collections, pleural effusions, or actual communication of an air-filed esophagus with an adjacent mediastinal air-fluid collection; definitive esophageal communication with outside structures is often difficult to visualize.
Medical/Legal Pitfalls:
With a high index of suspicion, a negative water-soluble contrast study is not sufficient to rule out the diagnosis of esophageal tear.
While evidence has begun to accumulate regarding specific treatment approaches, this remains a rare condition and, as such, many suggestions are based on case series or personal experience.
Special Concerns:
Early diagnosis is crucial in Boerhaave syndrome. It is important to consider the possibility in any patient presenting with atypical chest or abdominal pain.

13. Differential diagnosis
Oesophageal perforation with pneumomediastinum:
Boerhaave Syndrome
Complication of hiatus hernia

14. Oesophageal perforations
Oesophagus most vulnerable to perforation
>50% due to iatrogenic causes
Rates increased with procedures – balloon dilatation of strictures; variceal ligation; sclerotherapy
15% due to FB, caustic ingestions
15% due to Boerhaave Syndrome
10% - trauma
Risk factors – Hiatus hernia; carcinoma; strictures; radiation; Barrett’s; oesophageal varices; achalasia
Boerhaave syndrome is rare, accounting for 15% of all cases of esophageal perforation. Iatrogenic causes account for 50-75% of esophageal perforations. The actual incidence depends on the procedure; rigid endoscopy carries a perforation rate %, while that of flexible endoscopy varies from %. Rates increase quickly when interventions are undertaken, such as pneumatic balloon dilatation in achalasia (3-6%) or any procedure involving strictures secondary to radiation or tumor (10%). Furthermore, the rate of perforation is increased in the presence of a large hiatal hernia or esophageal diverticula.
Penetrating trauma to the neck (2-9%), thinning of esophageal membrane secondary to variceal sclerotherapy (1-3%), and foreign body or toxic ingestions (5-15%) make up the bulk of the remaining causes. Even with prompt therapy and advances in surgical technique, the mortality rate can be very high, varying from 5-75%; higher rates correlate with delays in both presentation and diagnosis.
The ingestion of a caustic toxin or foreign body preceding any of the above symptoms may indicate perforation.
A history of preexisting upper gastrointestinal pathology (gastroesophageal reflux disease, hiatal hernia, carcinoma, strictures, radiation therapy, Barrett esophagus, varicies, achalasia, infection) raises a patient's risk of perforation.

15. Oesophageal perforation
Presentation may vary according to:
Location of tear
Upper - neck/upper chest
Mid or lower - interscapular or epigastric pain
Cause of the tear (Iatrogenic/ Boerhaave)
Time from perforation to presentation
Early post-gastroscopy
Late presentation may be overshadowed by sepsis/shock.
Differential: (in absence of pneumomediastinum)
ACS; Aortic dissection; Pancreatitis; GERD; Pleural effusion
Presentation may vary depending on the following:
The location of the tear
The cause of the injury
The amount of time that has passed from the perforation to the intervention
Patients with cervical esophagus perforation may present with neck or upper chest pain.
Patients with middle or lower esophagus perforation may present with interscapular or epigastric discomfort.
When the chest radiogram of a patient with an esophageal perforation shows air or an effusion in the pleural space, the condition is often misdiagnosed as a pneumothorax or pancreatitis. An elevated serum amylase caused by the extrusion of saliva through the perforation may fix the diagnosis of pancreatitis in the mind of an unwary physician. If the chest radiogram is normal, a mistaken diagnosis of myocardial infarction or dissecting aneurysm is often made.

16. Oesophageal perforation
Pathophysiology:
50% have concomitant reflux – GE junction in the chest
During vomiting, abdominal pressures are transmitted to the oesophagus.
> 200 mmHg
Exacerbated by negative intrathoracic pressure
Usually ruptures into the L pleural cavity, above GE junction
Boerhaave – 90%
Spontaneous rupture usually occurs into the left pleural cavity or just above the gastroesophageal junction. Fifty percent of patients have concomitant gastroesophageal reflux disease, suggesting that minimal resistance to the transmission of abdominal pressure into the thoracic esophagus is a factor in the pathophysiology of the lesion. During vomiting, high peaks of intragastric pressure can be recorded, frequently exceeding 200 mm Hg, but since extragastric pressure remains almost equal to intragastric pressure, stretching of the gastric wall is minimal. The amount of pressure transmitted to the esophagus varies considerably, depending on the position of the gastroesophageal junction. When it is in the abdomen and exposed to intra-abdominal pressure, the pressure transmitted to the esophagus is much less than when it is exposed to the negative thoracic pressure. In the latter situation, the pressure in the lower esophagus will frequently equal intragastric pressure if the glottis remains closed. Cadaver studies have shown that when this pressure exceeds 150 mm Hg, rupture of the esophagus is apt to occur. When a hiatal hernia is present and the sphincter remains exposed to abdominal pressure, the lesion produced is usually a Mallory-Weiss mucosal tear, and bleeding rather than perforation is the problem. This is due to the stretching of the supradiaphragmatic portion of the gastric wall. In this situation, the hernia sac represents an extension of the abdominal cavity, and the gastroesophageal junction remains exposed to abdominal pressure. The esophagus is more vulnerable than the rest of the alimentary tract due to the lack of a serosal layer, which provides stability through elastin and collagen fibers. Perforation may be due to several mechanisms, including direct piercing, shearing along the longitudinal axis, bursting from radial forces, and thinning from necrosis of the esophageal wall.
Iatrogenic injury through esophageal instrumentation is the leading cause of perforation by either piercing or shearing and may be due to any number of procedures, especially endoscopy and dilatation of strictures. Such tears often occur near the pharyngoesophageal junction where the wall is weakest. Because the esophagus is surrounded by loose stromal connective tissue, the infectious and inflammatory response can disseminate easily to nearby vital organs, thereby making the esophageal perforation a medical emergency and increasing the likelihood of serious sequelae. Underlying esophageal disease (tumor, stricture) predisposes toward perforation with instrumentation, which often occurs distal to the affected area. Perforation during surgery most often occurs in the abdominal esophagus.
Spontaneous esophageal rupture (Boerhaave syndrome) occurs secondary to a sudden increase in intraluminal pressures, usually due to violent vomiting or retching, and often follows heavy food and alcohol intake. In more than 90% of cases, perforation occurs in the lower third of the esophagus; most frequently, the tear is in the left posterolateral region (90%) and may extend superiorly. The predilection for left-side perforation is due to the lack of adjacent supporting structures, thinning of the musculature in the lower esophagus, and anterior angulation of the esophagus at the left diaphragmatic crus. Fifty percent of ruptures occur in patients with gastroesophageal reflux disease, suggesting that ease of pressure transfer from the abdominal to thoracic esophagus may facilitate rupture.
Shearing forces due to rapid increases in intragastric pressure against a closed pylorus result in a Mallory-Weiss tear (MWT). These longitudinal mucosal lacerations occur most commonly at the gastroesophageal junction or gastric cardia, especially if a hiatal hernia is present, and often present with hematemesis. Ultimately, these tears can perforate if the pressure increases are unrelieved. Further discussion of MWTs is reserved for another section.

17. Oesophageal perforation
Diagnosis:
CXR – variable findings
Pneumomediastinum – 40%
Time frame
Location of perforation
Integrity of mediastinal pleura (pneumothorax/effusion)
CT chest with Gastrografin
Negative study does not r/o perforation if clinical suspicion is high (10% false-negatives)
Diagnosis Abnormalities on the chest radiogram can be variable and should not be depended upon to make the diagnosis. This is because the abnormalities are dependent on three factors: (1) the time interval between the perforation and the radiographic examination, (2) the site of perforation, and (3) the integrity of the mediastinal pleura. Mediastinal emphysema, a strong indicator of perforation, takes at least 1 hour to be demonstrated, and is present in only 40% of patients. Mediastinal widening secondary to edema may not occur for several hours. The site of perforation also can influence the radiographic findings. In cervical perforation, cervical emphysema is common and mediastinal emphysema rare; the converse is true for thoracic perforations. Frequently, air will be visible in the erector spinae muscles on a neck radiogram before it can be palpated or seen on a chest radiogram (Fig ). The integrity of the mediastinal pleura influences the radiographic abnormality in that rupture of the pleura results in a pneumothorax, a finding that is seen in 77% of patients. In two thirds of patients the perforation is on the left side, in one fifth it is on the right side, and in one tenth it is bilateral. If pleural integrity is maintained, mediastinal emphysema (rather than a pneumothorax) appears rapidly. A pleural effusion secondary to inflammation of the mediastinum occurs late. In 9% of patients the chest radiogram is normal. The diagnosis is confirmed with a contrast esophagogram, which will demonstrate extravasation in 90% of patients. The use of a water-soluble medium such as Gastrografin is preferred. Of concern is that there is a 10% false-negative rate. This may be due to obtaining the radiographic study with the patient in the upright position. When the patient is upright, the passage of water-soluble contrast material can be too rapid to demonstrate a small perforation. The studies should be done with the patient in the right lateral decubitus position (Fig ). In this position the contrast material fills the entire length of the esophagus, allowing the actual site of perforation and its interconnecting cavities to be visualized in almost all patients. Management The key to optimum management is early diagnosis. The most favorable outcome is obtained following primary closure of the perforation within 24 hours, resulting in 80 to 90% survival.

20. Oesophageal perforation
Morbidity and mortality:
Related to:
location of the tear,
cause of the tear, and
time to make the diagnosis.
Highest mortality with Boerhaave Syndrome
Related to inflammatory response of gastric contents in the mediastinum.
Exacerbated by negative intra-thoracic pressure
Morbidity – mediastinitis; empyema; polymicrobial sepsis; multi-organ failure.
Even with prompt therapy and advances in surgical technique, the mortality rate can be very high, varying from 5-75%; higher rates correlate with delays in both presentation and diagnosis.
Mortality also varies by etiology and location of the perforation. The highest rates are attributed to Boerhaave syndrome (up to 72%), partly because of the difficulty in making the diagnosis, followed by iatrogenic (19%) and traumatic perforations (7%). Cervical perforations portend a lower mortality compared with abdominal and thoracic perforations due to containment of potential contamination by tight fascial attachments and mechanisms, which may make injury more obvious.
The morbidity and mortality in esophageal perforation is most often due to an overwhelming inflammatory response to gastric contents in the mediastinum, pleural spaces, and adjoining tissues, as well as swift spread of infection to paraesophageal structures. Negative intrathoracic pressure may draw gastric material out of the esophagus, exacerbating injury.
Morbidity may be due to pneumonia, mediastinitis, empyema, polymicrobial sepsis, and multiorgan failure

21. Discussion of the differential
Boerhaave Syndrome
Complication of hiatus hernia

22. Boerhaave Syndrome
In 1724, Dr Hermann Boerhaave described the first, and likely most well known, case of esophageal perforation. Baron von Wassenaer, the Grand Admiral of Holland, followed a large meal with his customary bout of emetic-induced vomiting. However, on this occasion, the Admiral experienced a sudden and severe pain in his upper abdomen after violent but minimally productive retching. Dead less than 24 hours later, his autopsy revealed a tear of his distal esophagus and gastric contents (duck flesh and olive oil) in the pleural spaces.
The esophagus is more vulnerable than the rest of the alimentary tract due to the lack of a serosal layer, which provides stability through elastin and collagen fibers. Perforation may be due to several mechanisms, including direct piercing, shearing along the longitudinal axis, bursting from radial forces, and thinning from necrosis of the esophageal wall.
Iatrogenic injury through esophageal instrumentation is the leading cause of perforation by either piercing or shearing and may be due to any number of procedures, especially endoscopy and dilatation of strictures. Such tears often occur near the pharyngoesophageal junction where the wall is weakest. Because the esophagus is surrounded by loose stromal connective tissue, the infectious and inflammatory response can disseminate easily to nearby vital organs, thereby making the esophageal perforation a medical emergency and increasing the likelihood of serious sequelae. Underlying esophageal disease (tumor, stricture) predisposes toward perforation with instrumentation, which often occurs distal to the affected area. Perforation during surgery most often occurs in the abdominal esophagus.
Spontaneous esophageal rupture (Boerhaave syndrome) occurs secondary to a sudden increase in intraluminal pressures, usually due to violent vomiting or retching, and often follows heavy food and alcohol intake. In more than 90% of cases, perforation occurs in the lower third of the esophagus; most frequently, the tear is in the left posterolateral region (90%) and may extend superiorly. The predilection for left-side perforation is due to the lack of adjacent supporting structures, thinning of the musculature in the lower esophagus, and anterior angulation of the esophagus at the left diaphragmatic crus. Fifty percent of ruptures occur in patients with gastroesophageal reflux disease, suggesting that ease of pressure transfer from the abdominal to thoracic esophagus may facilitate rupture.
Shearing forces due to rapid increases in intragastric pressure against a closed pylorus result in a Mallory-Weiss tear (MWT). These longitudinal mucosal lacerations occur most commonly at the gastroesophageal junction or gastric cardia, especially if a hiatal hernia is present, and often present with hematemesis. Ultimately, these tears can perforate if the pressure increases are unrelieved. Further discussion of MWTs is reserved for another section.

23. Boerhaave Syndrome Clinical features: Rare disease entity
80% of cases are in middle-aged men.
Commonly associated with binge eating and alcoholic over-indulgence
Occurs typically after forceful vomiting/retching
Mortality also varies by etiology and location of the perforation. The highest rates are attributed to Boerhaave syndrome (up to 72%), partly because of the difficulty in making the diagnosis, followed by iatrogenic (19%) and traumatic perforations (7%). Cervical perforations portend a lower mortality compared with abdominal and thoracic perforations due to containment of potential contamination by tight fascial attachments and mechanisms, which may make injury more obvious.
The morbidity and mortality in esophageal perforation is most often due to an overwhelming inflammatory response to gastric contents in the mediastinum, pleural spaces, and adjoining tissues, as well as swift spread of infection to paraesophageal structures. Negative intrathoracic pressure may draw gastric material out of the esophagus, exacerbating injury. The syndrome is described more commonly in males than in females, with ratios ranging from 2:1 to 5:1.
Age: (Boerhaave)
It is seen most frequently among patients aged years.
Reports suggest that 80% of all patients are middle-aged men.

24. Boerhaave Syndrome Clinical features:
Other precipitants – childbirth, coughing, seizures, weight-lifting; blunt trauma
Followed by severe chest pain (lower thoracic/upper abdomen)
Swallowing may aggravate pain; and precipitate a coughing spell.
Associated shortness of breath and/or pleuritic pain.
Haematemesis uncommon.
History:
The classic clinical presentation usually consists of repeated episodes of retching and vomiting, typically in a middle-aged man with recent excessive dietary and alcohol intake.
This is followed by a sudden onset of severe chest pain in the lower thorax and upper abdomen.
The pain may radiate to the back or left shoulder.
Swallowing often aggravates it.
Typically, hematemesis is not seen after esophageal rupture, which helps distinguish it from the more common Mallory-Weiss tear.
Swallowing may precipitate coughing because of communication between the esophagus and the pleural cavity.
Atypical clinical features sometimes delay a prompt diagnosis and appropriate intervention. This may result in an increase in morbidity and mortality.
Shortness of breath is a common complaint and is due to pleuritic pain or pleural effusion.
Boerhaave syndrome has also been reported with abdominal or chest pain following straining, childbirth, weight lifting, fits of coughing or laughing, hiccuping, blunt trauma, seizures, and forceful swallowing.
The presence of fever; pain in the neck, upper back, chest, or abdomen; dysphagia; odynophagia; dysphonia; or dyspnea following esophageal instrumentation should raise suspicion for perforation.
Patients with thoracic or abdominal perforations may present with any of the above symptoms, as well as low back pain, shoulder pain referred from diaphragmatic irritation, increased discomfort lying flat, or true acute abdomen.

25. Boerhaave Syndrome Pathophysiology
Barogenic injury to lower oesophagus
Sudden ↑ oesophageal intraluminal pressure against a closed cricopharyngeus muscle.
Hydrostatic pressure overcomes the oesophageal tensile strength
Tear in left posterolateral oesophageal wall, 2-3cm proximal to the G-E junction with leak into the left pleural cavity.
Esophageal rupture in Boerhaave syndrome is postulated to be the result of a sudden rise in intraluminal esophageal pressure produced during vomiting, as a result of neuromuscular incoordination causing failure of the cricopharyngeus muscle to relax. The syndrome commonly is associated with overindulgence in food and/or alcohol. The most common anatomical location of the tear in Boerhaave syndrome is at the left posterolateral wall of the lower third of the esophagus, 2-3 cm proximal to the gastroesophageal junction, along the longitudinal wall of the esophagus. The second most common site of rupture is in the subdiaphragmatic or upper thoracic area

26. Boerhaave Syndrome Physical findings: Mackler’s triad (50%):
vomiting;
lower thoracic pain;
subcutaneous emphysema. (28-66%)
Hamman’s crunch
due to air in the mediastinum(20%)
Pleural effusion
Usually left-sided
Thoracentesis – undigested food and gastric juice – pH <6; sq cells; elevated amylase
SOB; fever; tachycardia; hypotension; abdo pain
Physical:
The Mackler triad defines the classic presentation. It consists of vomiting, lower thoracic pain, and subcutaneous emphysema.
Findings of pleural effusion are common.
If present, subcutaneous emphysema is particularly helpful for confirming diagnosis.
It is seen in 28-66% of patients at initial presentation.
More typically, it is found later.
Other classic findings, as described by Anderson and Barrett, include tachypnea and abdominal rigidity.
Tachycardia, diaphoresis, fever, and hypotension are common, particularly as the illness progresses. However, these findings are nonspecific.
Unusual findings may include the following:
Peripheral cyanosis
Hoarseness of voice due to recurrent laryngeal nerve involvement
Tracheal and mediastinal shift
Cervical vein distention
Proptosis
Pneumomediastinum is a very important finding.
It may cause a crackling sound upon chest auscultation, known as the Hamman crunch.
The crunch typically is heard coincident with each heartbeat and may be mistaken for a pericardial friction rub.
This is present in 20% of patients.
Later stages of illness may manifest as signs of infection and sepsis.
Symptoms may include fever, hemodynamic instability, and progressive obtundation.
Establishing a diagnosis in the later stages can be quite difficult because septic complications begin to dominate the clinical picture.
Early diagnosis is critical.
Many patients present with a pleural effusion.
Thoracentesis with examination of the pleural fluid can aid in diagnosis.
Undigested food particles and gastric juices usually are found.
If no gross particles are found, cytology can confirm or exclude their presence, but time is of the essence.
The pH of the pleural fluid will be less than 6, and the amylase content will be elevated.
Squamous cells from saliva may be found.
Although the physical examination is often nonspecific, certain findings can be helpful.
Subcutaneous emphysema is palpable in the neck or chest in up to 60% of perforations but requires at least an hour to develop after the initial injury.
Tachycardia and tachypnea are common initial physical examination findings, but fever may not be present for hours to days.
The Mackler triad, consisting of vomiting, chest pain, and subcutaneous emphysema, is classically associated with spontaneous esophageal rupture, though it is only fully present in about 50% of cases.
Auscultation of the chest can be of particular value.
The Hamman sign is a raspy, crunching sound heard over the precordium with each heartbeat caused by mediastinal emphysema, often present with thoracic or abdominal perforations.
Breath sounds may be reduced on the side of the perforation due to a contamination of the pleural space, often on the left.
In cases of delayed presentation, patients may be critically ill and present with significant hypotension.

27. Boerhaave Syndrome Morbidity and Mortality: Treatment:
Highest mortality rate of all GI tract perforations – 35%
Best outcome is with surgery within 12 hours
50% mortality if delayed >24 hours;
90% if >48 hours
Mediastinitis; Septic shock; Empyema
Treatment:
Surgical – Barrett (1946)
Diagnosis can be difficult because often no classic symptoms are present and delays in presentation for medical care are common. Approximately one third of all cases are clinically atypical. Prompt recognition of this potentially lethal condition is vital to ensure appropriate treatment. Mediastinitis, sepsis, and shock frequently are seen late in the course of illness, which further confuses the diagnostic picture.
A reported mortality estimate is approximately 35%, making it the most lethal perforation of the GI tract. The best outcomes are associated with early diagnosis and definitive surgical management within 12 hours of rupture. If intervention is delayed longer than 24 hours, the mortality rate (even with surgical intervention) rises to higher than 50% and to nearly 90% after 48 hours. Left untreated, the mortality rate is close to 100%.
Spontaneous esophageal rupture (Boerhaave syndrome) occurs secondary to a sudden increase in intraluminal pressures, usually due to violent vomiting or retching, and often follows heavy food and alcohol intake. In more than 90% of cases, perforation occurs in the lower third of the esophagus; most frequently, the tear is in the left posterolateral region (90%) and may extend superiorly. The predilection for left-side perforation is due to the lack of adjacent supporting structures, thinning of the musculature in the lower esophagus, and anterior angulation of the esophagus at the left diaphragmatic crus. Fifty percent of ruptures occur in patients with gastroesophageal reflux disease, suggesting that ease of pressure transfer from the abdominal to thoracic esophagus may facilitate rupture

28. Hiatus hernias – 4 types Type I - Sliding >95% of hiatus hernias
Weakness/elongation of the phren-oesophageal ligamentous structures, with GE junction in chest
Abnormal LES with reflux
GERD symptoms predominate
Younger patients
Obesity; pregnancy; chronic cough
Driving force to progression of the sliding HH is an increased intra-abdominal pressure combined with negative intra-thoracic pressure– eg obesity, pregnancy; chronic cough

29. Hiatus hernias Sliding hiatus hernia with Cameron ulcer
Cameron ulcer. A large hiatal hernia is seen on an endoscopic retroflex view, with a Cameron ulcer at the level of the diaphragmatic hiatus. (Feldman)

30. Hiatus hernias Type II - Paraesophageal < 5% of hiatus hernias
Preservation of the phren- oesophageal ligament with the GE junction fixed in abdomen.
GERD less common
Chest pain; SOB; dysphagia
Older patients (70’s)
M:F ratio 1:4
⅓ are anaemic.
Gastric fundus herniates into the anteriorly located hernia sac
Gastric ulceration within the intrathoracic stomach or chronic mucosal venous engorgement can lead to anaemia
GERD symptoms are less prominent. Can present with chest pain syndromes. More commonly preseent with symptoms relating to the space occupying nature of the hernia within the chest – dysphagia, chest pain, dyspnoea. Obstruction of the stomach result in dysphagia, gastric ulcerationpulm aspiration risk abd vascular compromise of the organoaxially rotated stomach within the hernia sac.;
Schwartz - The true incidence of a hiatal hernia in the overall population is difficult to determine because of the absence of symptoms in a large number of patients who are subsequently shown to have a hernia. When radiographic examinations are done in response to gastrointestinal symptoms, the incidence of a sliding hiatal hernia is seven times higher than that of a paraesophageal hernia. The age distribution of patients with paraesophageal hernias is significantly different from that observed in sliding hiatal hernias. The median age of the former is 61; of the latter, 48. Paraesophageal hernias are more likely to occur in women by a ratio of 4:1.
Approximately one third of patients with a paraesophageal hernia are found to be anemic, which is due to recurrent bleeding from ulceration of the gastric mucosa in the herniated portion of the stomach. Respiratory complications are frequently associated with a paraesophageal hernia, and consist of dyspnea from mechanical compression and recurrent pneumonia from aspiration.

31. Hiatus hernias Type II – Paraesophageal hernia
view with a retroflexed gastroscope.

32. Hiatus hernias Type III – Mixed
Sliding component with GE junction migrating into chest combined with a
Paraesophageal component – hernial sac anterior to this with gastric herniation.
Largest group of patients with paraoesophageal hernias.
Type IV – Complex (spleen, colon, liver)

33. Clinical features of paraesophageal hernias
Most are symptomatic – postprandial fullness; dysphagia; chest pain; anaemia; aspiration
AF level behind cardiac silhouette on CXR – “incidental finding”. Importance is not universally appreciated.
The paraesophageal hernia patient is almost universally misdiagnosed for some time as having an “ordinary” but large hiatal hernia, until the patient develops important symptoms, or until a discerning physician investigates in greater detail the patient's abnormal chest roentgenographic findings characteristic of paraesophageal herniation. Most medical physicians and gastrointestinal specialists poorly understand the clinical importance and pathophysiologic nature of paraesophageal hernias. With these thoughts in mind, it is common for the authors to note that the patient who has a paraesophageal hernia comes to us after undergoing a negative cardiac work-up for chest pain, or an inconclusive investigation of recurrent respiratory problems. The occult anemia associated with the above complaints is noted to have confounded the diagnostic puzzle further. The medical team has ultimately been faced with evaluating the “funny-looking” hiatal hernia that has been ignored for some time.

34. Clinical features of paraesophageal hernias
Upper abdo pain with inability to vomit
classic for gastric incarceration.
Borchardt’s triad
Severe chest/upper abdo pain
Inability to vomit
Inability to pass n/g tube
Simple sliding hiatal hernias do not require treatment. Patients with symptomatic giant sliding hiatal hernias, paraesophageal, and mixed hernias should be offered surgery. Many experts suggest that surgery should be offered to patients with asymptomatic paraesophageal hernias, because about 30% of these patients will develop complications if left untreated.[1] [2][20] (Feldman textbook)
1. Oddsdóttir M:  Paraesophageal hernia.   Surg Clin North Am  2000; 80:

35. Clinical features of paraesophageal hernias
Complications of Type II and III:
Space-occupying nature:
Intra-thoracic stomach - gastric incarceration with dysphagia and sub-sternal chest pain
Pulmonary complications with dyspnoea; aspiration
Bleeding:
Venous engorgement; mucosal ulceration; ischaemia; Fe-deficiency anaemia
Mechanical:
Obstruction; incarceration; volvulus
Strangulation, ischaemia with perforation.
Indeed, the primary presenting complaints of patients who have paraesophageal hernias usually relate to the space-occupying nature of the herniation within the chest, causing dyspnea or chest pain. Obstruction of the stomach resulting in dysphagia, gastric ulceration, pulmonary aspiration risk, and possible vascular compromise of the organo-axially rotated stomach within the hernia sac are other important clinical features of paraesophageal hernias. Venous engorgement and ulceration of the herniated gastric mucosa can lead to anaemia and haematemesis. Ischaemia can result in perforation

36. Clinical features of paraesophageal hernias
More than 30% will have a severe complication (perforation; strangulation; life-threatening bleed) if left untreated.
Elective surgical repair recommended in all except the most physiologically-impaired
Complications can be catastrophic
Emergency surgery carries higher morbidity and mortality.
DeMeester TR, Bonavina L:  Paraesophageal hiatal hernia.   In: Nyhus LM, Condon RE, ed. Hernia,   3rd ed. Philadelphia: JB Lippincott; 1989: Patients with type II and III paraesophageal hernia may develop severe complications, such as intrathoracic incarceration of the stomach with obstruction (vomiting, dysphagia, and chest pain), decreased pulmonary function (from compromised lung expansion), bleeding (mechanical or ischemic gastric ulcerations), strangulation, and perforation, [13] [18] [29] [37] so the diagnosis of type II or III paraesophageal hernia has similar implications. Surgical repair is recommended for paraesophageal hernias, regardless of type or symptoms, because, if left untreated, the reported rate of severe complications is approximately 30%, with a high rate of morbidity and mortality. [13] [18] [29] .
Since the sentinel articles by Skinner and Belsey [3] and Hill [2] describing the high morbidity and mortality associated with these complications, an aggressive surgical approach has been advocated. Historically, the incidence of developing a catastrophic complication has been estimated too be as high as 29%. This was reported in 1967, when 21 asymptomatic patients were treated nonoperatively for PEH, and 6 went on to develop strangulation, perforation, or significant bleeding [3]. Recent papers have documented a much lower incidence. Allen et al [6] followed 23 PEH patients for a median of 78 months, with none developing gastric complications.
Treatment - Schwartz The treatment of paraesophageal hiatal hernia is largely surgical. Controversial aspects include (1) indications for repair, (2) surgical approach, and (3) role of fundoplication. Indications The presence of a paraesophageal hiatus hernia has traditionally been considered an indication for surgical repair. This recommendation is largely based upon two clinical observations. First, retrospective studies have shown a significant incidence of catastrophic, life-threatening complications of bleeding, infarction, and perforation in patients being followed with known paraesophageal herniation. Second, emergency repair carries a high mortality. In the classic report of Skinner and Belsey, six of 21 patients with a paraesophageal hernia, treated medically because of minimal symptoms, died from the complications of strangulation, perforation, exsanguinating hemorrhage, or acute dilatation of the herniated intrathoracic stomach. These catastrophes occurred for the most part without warning. Others have reported similar findings. Recent studies suggest that catastrophic complications may be somewhat less common. Allen and colleagues followed 23 patients for a median of 78 months with only four patients progressively worsening. There was a single mortality secondary to aspiration that occurred during a barium swallow examination to investigate progressive symptoms. Although emergency repairs had a median hospital stay of 48 days compared to a stay of 9 days in those having elective repair, there were only three cases of gastric strangulation in 735 patient-years of follow-up.

37. Back to the case

38. Operative findings
Intra-operative 30cm - necrotic stomach and esophagus.
L sided thoracotomy: Strangulated/incarcerated paraeoesophageal hiatus hernia with necrosis and perforation.
Procedure – distal oesophagectomy and proximal gastrectomy; decortication and pleurectomies.
Developed mediastinitis with bilateral empyemas

39. “Take home” message
Paraoesphageal hernias are uncommon. (<5% of all hiatus hernias)
30% suffer catastrophic complications.
Fixed paraoesophageal hernias - “incidental finding” on CXR - should be referred for thoracic consultation.
If oesophageal perforation is suspected, CT chest with Gastrografin is the diagnostic procedure of choice.

40. Questions/Comments?