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Snap, crackle, pop!

Snap, crackle, pop!. Lester Mercuur 09 Nov 2006. Objectives. Case presentation Presentation of X-rays Discussion of the differential diagnosis Back to the case. Case. 71yo lady referred from Claresholm where she had been admitted the previous day with:

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Snap, crackle, pop!

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  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 P100 BP127/60 RR36 O2 sat on RA 91% • Periorbital swelling bilaterally.

  6. 3 months earlier (PA and lat)

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

  8. Course in the ED • Repeat CXR

  9. Course in the ED • Repeat CXR • CT chest – water-soluble contrast (Gastrografin) • Blood cultures • Antibiotics • Thoracic Surgery consult • OR

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

  11. 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

  12. 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

  13. 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%

  14. 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)

  15. 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.

  16. Discussion of the differential • Boerhaave Syndrome • Complication of hiatus hernia

  17. 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. Boerhaave Syndrome

  18. 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

  19. 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.

  20. 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.

  21. 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

  22. Boerhaave Syndrome • Morbidity and Mortality: • 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)

  23. 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 Hiatus hernias – 4 types

  24. Sliding hiatus hernia with Cameron ulcer Hiatus hernias

  25. 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. Hiatus hernias

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

  27. 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)

  28. 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.

  29. Clinical features of paraesophageal hernias • Clinical features: • 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

  30. 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.

  31. 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.

  32. Back to the case

  33. Operative findings • Intra-operative gastroscopy @ 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

  34. “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.

  35. Questions/Comments?

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