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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! 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: • 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.
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
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.
3 months earlier (PA and lat)
Differential diagnosis • Pneumomediastinum 2º to oesophageal perforation: • FB • Boerhaave Syndrome • Complication of hiatus hernia
Course in the ED • Repeat CXR
Course in the ED • Repeat CXR • CT chest – water-soluble contrast (Gastrografin) • Blood cultures • Antibiotics • Thoracic Surgery consult • OR
Differential diagnosis • Oesophageal perforation with pneumomediastinum: • Boerhaave Syndrome • Complication of hiatus hernia
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
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
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%
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)
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.
Discussion of the differential • Boerhaave Syndrome • Complication of hiatus hernia
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
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
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.
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.
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
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)
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
Sliding hiatus hernia with Cameron ulcer 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. Hiatus hernias
Type II – Paraesophageal hernia view with a retroflexed gastroscope. Hiatus hernias
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)
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.
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
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.
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.
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
“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.