Surgery Of Esophageal And Mediastinal Diseases PDF

SURGERY OF ESOPHAGEAL AND MEDIASTINAL DISEASES Walid Abu Arab Professor of Cardiothoracic Surgery University of Alexandria Learning Objectives 1. Describe a systematic method for evalu...

SURGERY OF ESOPHAGEAL AND MEDIASTINAL DISEASES Walid Abu Arab Professor of Cardiothoracic Surgery University of Alexandria Learning Objectives 1. Describe a systematic method for evaluating patients with esophageal or mediastinal disease. 2. How to reach a diagnosis in different esophageal diseases and mediastinal tumors. 3. Discuss differential diagnosis in a patient with dysphagia. 4. Discuss differential diagnosis of mediastinal masses. 5. Describe the outlines of surgical treatment in different esophageal diseases. 6. Describe the outlines of surgical treatment in different mediastinal diseases. 1|Page Walid Abu Arab Cardiothoracic Trauma  SURGERY OF ESOPHAGUS  DYSPHAGIA:  Dysphagia is difficulty in swallowing.  Odynophagia is painful swallowing.  Grades: 1. Grade I: dysphagia for solids 2. Grade II: dysphagia for semisolids 3. Grade III: dysphagia for liquids 4. Grade IV: dysphagia for own saliva  Etiology: o Oropharyngeal dysphagia:  Disorders in the mouth, tongue and pharynx.  Functional (Neuromuscular) - Poliomyelitis, myasthenia gravis - Scleroderma, amyloidosis, hyperthyroidism. - Vascular brain stem disease, pseudo-bulbar syndrome.  Organic - Oral carcinoma, postcricoid carcinoma - Retropharyngeal abscess, Zenker’s diverticulum, inflammatory stricture. o Esophageal dysphagia:  Functional (esophageal motility disorders) - Achalasia. - Scleroderma. - Diffuse esophageal spasm. - Diabetic neuropathy. - Amyloidosis. - Parasitic infection (Chagas’ disease). - Emotional (globus hystericus). - Plummer-Vinson's syndrome. - Contractile lower esophageal ring (Schatzki ring). - Lower esophageal sphincter spasm (hypertensive sphincter).  Organic: - Congenital  Atresia with or without tracheo-oesphageal fistula  Stenosis  Short oesophagus 2|Page Walid Abu Arab Cardiothoracic Trauma   Dysphagia lusoria (by abnormal artery, vascular ring)  Foregut duplication - Acquired Intraluminal obstruction:  Foreign body. Lesions in the wall:  Esophageal webs.  Esophageal tumors.  Inflammatory stricture (peptic stricture).  Anastomotic stricture, post-injection sclerotherapy, post-Sangestaken tube, post traumatic strictures.  Caustic stricture.  Esophageal diverticulae. Extraluminal obstruction.  Compression by tumors, enlarged lymph nodes, retrosternal goiter.  Vascular abnormalities (aortic aneurysm) and enlarged left atrium.  Spurs from anterior cervical spine.  Rolling hiatal hernia, tight hiatal hernia repair, perioesphagitis after vagotomy.  Esophageal function studies:  Esophageal manometry: is used to evaluate motility of esophageal body (propulsive force) and function of upper (UES) and lower esophageal sphincter (LES).  Examples: 1. Achalasia: Failure of LES to relax with swallowing and loss of progressive peristalsis in the body of the esophagus. 2. Diffuse esophageal spasm: Frequent simultaneous and repetitive esophageal contractions of high amplitude or long duration. Esophageal manometry 3|Page Walid Abu Arab Cardiothoracic Trauma   Ambulatory 24-hour pH monitoring o Indications: - To detect and quantitate exposure of lower esophagus to gastric acid. - To test the relationship between symptoms and esophageal exposure to acid refluxate.  Ambulatory 24-hour bile reflux monitoring - The reflux of bile &/or acid predisposes to esophageal injury (esophagitis) and Barrett’s esophagus and malignancy. A fiberoptic sensor probe is used to detect bilirubin in bile refluxate into the esophagus.  Provocative test: Acid perfusion (Berstein test) - Determines whether symptoms are reproduced by the infusion of acid into the esophagus. The distal esophagus is perfused with 0.1 N HCL alternating with saline. The patient reports any symptom that develops during infusion. A positive test reproduces the patient’s symptoms only during acid perfusion.  Eosophagoscopy o Types: 1. Rigid: done under general anesthesia 2. Flexible, fiberoptic: done under conscious sedation Rigid esophagoscopy o Indications 1. Diagnostic a) Cancer esophagus: locate site, taking biopsy (bite , brush biopsy) b) Hiatal hernia: to access degree of reflux c) Achalasia: d) Identify cause of dysphagia and haematemesis 2. Therapeutic a) Removal of foreign body 4|Page Walid Abu Arab Cardiothoracic Trauma  b) Insertion of Stent in cancer esophagus c) Sclerotherapy in hematemesis d) Dilatation of esophageal stricture e) Laser therapy & cryotherapy. ACHALASIA  Definition: - Failure of relaxation of the lower esophageal sphincter in response to swallowing.  Etiology: - Achalasia is a motility disorder of the esophagus. It represents a degenerative nerve process with loss of ganglion cells in the myenteric plexus and a reduction in vagal nerve fibers within the wall of the esophagus. This results in failure of relaxation of the lower esophageal sphincter (LES) and ineffective peristalsis in the body of the esophagus. There is hypertrophy of the circular muscle layer of the esophagus, while the longitudinal layer retains its normal thickness.  Clinical Picture: - Progressive dysphagia to liquids and solids over several years (first for fluids). - Regurgitation of undigested food especially in recumbent position. - Weight loss. (not common as food passes by its weight) - Coughing or wheezing secondary to aspiration.  Investigations: 1. Radiology: a. Plain chest X-ray (CXR) is normal, or shows widened mediastinum with an air-fluid level within the dilated esophagus. b. Absence of gastric air bubble. c. Pulmonary infiltrates of aspiration CXR in achalasia (note the broad mediastinum ‘arrow’ 5|Page Walid Abu Arab Cardiothoracic Trauma  2. Barium swallow demonstrates smooth tapering of the distal esophagus (bird’s beak, or pencil end) and a dilated or sigmoid esophagus and absence of gastric air bubbles. Barium swallow in achalasia 3. Endoscopy: a) Dilated esophageal body containing food residue (despite fasting). b) Easy passage of the rigid esophagoscope through the esophagogastric junction but failure of esophagogastric junction to open spontaneously with repeated air insufflation using the fiberoptic esophagoscope. 6|Page Walid Abu Arab Cardiothoracic Trauma  Endoscopy in achalasia 4. Esophageal function studies: Esophageal manometry demonstrates incomplete relaxation of the LES with swallowing and ineffective peristalsis of the body of the esophagus.  Complications a) Mucosal ulcerations by irritation of retained food. b) Aspiration which leads to pneumonitis. c) Malignancy in 2-3% (squamous cell carcinoma usually at dilated site).  Management 1- Medical treatment: - Oral medications that help to relax the lower esophageal sphincter include groups of drugs called nitrates, for example, isosorbide dinitrate and nitroglycerin, and calcium channel blockers for example, nifedipine. 2- Pneumonetic dilatation: Datation with balloons; here pneumatic dilatation produces a forceful disruption of the lower esophageal sphincter. Pneumatic dilatation of the lower esophagus 3- Botulinum toxin: It is endoscopically injected into the lower sphincter to weaken it. 4- Surgical Treatment: a) Esophagocardiomyotomy: Surgical longitudinal section of the outer longitudinal and inner circular smooth muscle without damaging the submucosa and mucosa. Originally described by Heller as an anterior and posterior myotomy, the procedure has been modified to a single anterior or lateral myotomy (modified 7|Page Walid Abu Arab Cardiothoracic Trauma  Heller), achieved using an abdominal or thoracic approach. Incision extends for about 7-10 cm including lower end of the esophagus and esophago-gastric junction. b) Recently, minimally invasive surgical approaches (laparoscopic or thoracoscopic myotomy) are used. c) Esophageal resection and reconstruction are considered in patients who have failed esophageal myotomy (or re-myotomy), or who have an end-stage megaesophagus. Modified Heller’s myotomy  CORROSIVE ESOPHAGEAL INJURY - Corrosive injury to the esophagus may result from accidental (children) or deliberate (adults) ingestion of strong alkali or acid. The resulting injury (liquefaction necrosis from alkali, coagulation necrosis from acid) to the esophageal mucosa results in superficial or deep burns, stricture or perforation. In Egypt, the chemical agent most often responsible for the injury is commercial caustic potash. This is a mixture of potassium hydroxide, potassium carbonate and sodium carbonate. It is used primarily for washing clothes, cleaning floors and in some homes for making soap.  Classification of corrosive esophageal burns: 1. First degree (superficial) burn: Superficial mucosal hyperemia, mucosal edema with or without superficial sloughing. 2. Second degree (medium, transmucosal) burn: The submucosal and muscular layers are involved, being left exposed after desquamation of the mucosa. 3. Third degree (deep, full-thickness burn): Necrosis through the entire wall of the esophagus, causing periesophagitis and possibly perforation with mediastinitis, pericarditis or broncho-esophageal fistula.  Phases of corrosive lesions of the esophagus It is divided into three phases: 8|Page Walid Abu Arab Cardiothoracic Trauma 1. Acute necrotic phase. 2. Ulceration granulation phase. 3. Chronic phase (cicatrisation and stricture formation).  Clinical Picture: I. Acute Phase: 1. Pain and inability to swallow. 2. Excessive salivation. 3. Respiratory distress. 4. Dehydration. 5. In severe cases, there might be laryngitis and stridor.. Chronic Phase: This started 4-6 weeks from the incidence of potash ingestion 1. Dysphagia 2. Dehydration 3. Weight loss  Treatment: 1- Management of acute corrosive esophagitis: a) Give oral and parenteral broad-spectrum antibiotics. b) The patient is maintained on parenteral fluids to keep the burnt tissues clean. c) Oral antibiotics act as a local dressing. d) Corticosteroids are required only to relieve laryngeal edema. e) Tracheostomy is life-saving in distressed patients when stridor is progressive. f) Neither instrumentation (gastric lavage) nor dilatation should be performed at that stage to avid esophageal perforation. g) Esophagoscopy and radiography: Done at any time after corrosive ingestion provided the condition of the patient allows. I. If no burn is found in the esophagus, antibiotics are discontinued. Oral feedings are started as soon as the patient comes out of anaesthesia following esophagoscopy. II. In the presence of first degree burn, antibiotics are continued for ten days. Oral feedings are started progressing from clear liquid diet to a full diet over a period of three days. III. In the presence of second and/or third degree burn, a siliconized catheter splint is applied through transcervical pharyngostomy or esophagostomy. The splinting tube, being tunnelled, serves for enteral feeding as well. It is left in place until the esophagus is resurfaced with epithelium. This requires a period of one to four months. If the patient refuses splinting, as is usually the case, he is followed and managed as in the chronic stage. 9|Page Walid Abu Arab Cardiothoracic Trauma 2- Patients who present in the chronic stage of corrosive esophagitis are examined by esophagoscopy and esophagography to get more information about the stricture, and managed as follow:  Esophageal dilatation a) If there is Short smooth segment (less than two-vertebrae), patients are dilated antegradely, using Savary dilators. b) If there is long segment or irregular strictures (more than two-vertebrae); patients are dilated retrogradely, using Tucker’s dilators. Gastrostomy feeding is required. N.B. Patient suspicious of having esophageal perforation following dilatation is treated conservatively: Stop oral intake, give massive antibiotics, drain any collection (mediastinal abscess, empyema), and institute gastrostomy feeding.  Retrosternal colon interposition is indicated if dilatation failed. A segment of transverse colon interposed between the proximal esophagus and the stomach. ESOPHAGEAL CANCER  Premalignant lesions: Achalasia, Barrett’s esophagus, reflux stricture, post-corrosive stricture and Plummer- Vinson syndrome.  Clinical Picture: Symptoms and signs arise as a result of local invasion of the primary tumour or from distant metastasis. 1. Primary tumour: Dysphagia, regurgitation, vomiting, odynophagia, pain (epigastric, back, neck) arising from local tumour infiltration, esophagitis, and metastasis to intra-abdominal lymph nodes or bone, respiratory symptoms (cough after swallowing) suggesting aspiration or an esophagotracheal or bronchial fistula. General symptoms of weight loss and cachexia are present in 50% of patients. 2. Distant metastases: lung, liver, bone, adrenal, kidney, abdominal lymph nodes, ….  Physical examination: Enlarged cervical or supraclavicular lymph nodes, signs of aspiration (rales, rhonchi, consolidation), upper abdominal mass, hepatomegaly, generalized wasting.  Investigations: 1- Laboratory: Anemia, hypoproteinemia, abnormal liver function tests, hypercalcemia. 2- CXR: Pulmonary metastases, infiltrates or pneumonia, soft-tissue mass or air- fluid level in the mediastinum. 10 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  3- Barium esophagogram: Defines level and extent of esophageal obstruction (malignancy is manifested by fixed irregular filling defect (rate tail appearance and shouldering). 4- Esophagogastroscopy: Done under local anesthesia using flexible fiberoscope or under general anesthesia using rigid esophagoscope. It defines the site and extent of the tumor. Multiple biopsies are taken to obtain a histologic diagnosis. Obstructing tumors may be dilated or stented during esophagoscopy. 5- Computed tomography (CT): Scanning of the primary tumor to assess extent of local disease and enlarged nodes (mediastinal, upper abdominal). Scanning of the brain, lung, and liver for the possibility of metastases. 6- Endoscopic ultrasound: To assess the depth of tumor penetration and peri- esophageal lymph node metastasis. 7- Bronchoscopy: Always performed in upper esophageal tumors in close proximity to the airways or if a malignant fistula is suspected. 8- MRI: Has little advantage over CT scanning for staging of esophageal cancer. Barium swallow in cancer esophagus 11 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  Endoscopic image of patient with esophageal carcinoma  Staging of esophageal cancer and its TNM subsets o T descriptor - Tis : Carcinoma in situ. - T1 : Tumor invades lamina propria, muscularis mucosa or submucosa. - T2 : Tumor invades muscularis propria. - T3 : Tumor extends into periesophageal tissue. - T4 : Tumor invades adjacent structures. o N descriptor - N0 : No regional lymph node metastasis. - N1 : Regional lymph node metastasis. o M descriptor - M0 : No distant metastases - M1 : Distant metastases.  TNM subsets Stage TNM subsets 0 Tis N0 M0 I T1 N0 M0 IIA T2 N0 M0 T3 N0 M0 IIB T1 N1 M0 T2 N1 M0 III T3 N1 M0 T4 Any N M0 IV Any T Any N M1  Pathology of esophageal cancer 1- Squamous cell carcinoma: 12 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  - 80% of all primary esophageal cancers. - Mid-third (50%), lower-third (25%), upper-third (15%). - Macroscopic types: Fungating, ulcerating, infiltrating, polypoid. - Histologically: Well-differentiated have prominent keratinization, poorly- differentiated have minimal keratinization. - Submucosal proximal tumor spread is frequent. - Metastasis to regional lymph nodes > 50%. - Metastasis to lung 50% and liver 50%. 2- Adenocarcinoma: - 20 - 40% of primary esophageal cancer. - Should be distinguished from adenocarcinoma of gastric origin. Criteria in favor of esophageal origin include: I. Associated Barrett’s epithelium. II. Greater than 75% of tumour mass involving the body of esophagus. III. Direct invasion of periesophageal tissues. IV. Minimal gastric involvement. V. Clinical symptoms of esophageal obstruction.  Treatment: 1. Early stage tumors (Tis, Stage I): Surgical resection. 2. Locally advanced (T4) & metastatic (M1) tumors : Are excluded from surgery. o Surgery: Procedure: Esophagectomy with gastric pull up and Esophagogastric anastomosis. Idea of procedure : Resection of the esophageal tumour with proximal extension of resected eosophagus of 10 cm to encompass submucosal tumor extension & the distal extension of resection at least 5 cm from tumor. To obtain clear lateral margins, a wide en bloc resection is preferred. Esophageal reconstruction: this achieved by gastric pull up with the stomach positioned in the posterior mediastinum. The healthy proximal portion of the eosophagus is then anastomosed to the stomach (eosophagogastric anastomosis). A pyloromyotomy or pyloroplasty is done to improve gastric drainage. Approach: 1. Right thoracotomy & laparotomy: Laparotomy to exclude abdominal metastasis and mobilize the stomach. Right thoracotomy to resect the esophagus. The stomach is delivered through the hiatus and esophago-gastric anastomosis made intrathoracic, or through neck incision, the stomach is anastomosed to cervical esophagus. 2. Left thoracotomy or Thoracoabdominal: For tumors in the distal esophagus or esophagogastric junction. Getting into the abdomen is either by incising the diaphragm or extending the incision across the costal margin. 13 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma 3. Transhiatal (non-thoracotomy) i.e Blind Esophagectomy: For tumors of the lower or upper esophagus. The lower esophagus is mobilized transhiatal through laparotomy and the upper esophagus through the neck. The stomach is delivered into the neck by way of posterior mediastinum and anastomosed to cervical esophagus without the need for thoracotomy. 4. Minimally invasive video assisted thoracoscopic surgery (VATS): Right-sided VATS is utilized to mobilize the entire intrathoracic esophagus avoiding formal thoracotomy. Esophageal reconstruction is done in the usual manner through laparotomy and left neck incisions. o Radiotherapy: a) Preoperative: Improves resection and reduces local recurrence. b) Postoperative: Not recommended as a routine after surgical resection. o Chemotherapy: a) Single agent (cisplatin, 5-fluro-uracil, mytomycin, bleomycin). b) Combination (cisplatin + 5-fluro-uracil). c) Preoperative: Eliminates micro-metastases and improve respectability. d) Postoperative: Cannot be recommended for routine clinical use. o Palliation: Causes of irresectability (Inoperable Cancer esophagus) 1. General: Weak patients unfit for surgery due to deteriorated cardiac and pulmonary function. 2. Local: - Tumor more than 10 cm. - Spread to tracheobronchial tree, Aorta. - Peritoneal seedlings. - Celiac and hepatic lymph node affection. 3. Distant metastasis e.g. liver, lung. Types of Palliation 1. Esophageal dilatation. 2. Esophageal stents: Covered self-expandable metallic stents, inserted through endoscopy. 3. Radiochemotherapy. 4. Endoluminal brachytherapy. 5. Laser therapy. 6. Surgery: a) Feeding gastrostomy b) Retrosternal Colon interposition or eosophagogastrectomy. Those 2 aggressive surgeries are rarely used as palliation. 14 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  Esophageal stenting  Barrett’s Esophagus  Definition: Lining of the lower esophagus with columnar epithelium instead of squamous epithelium.  Diagnosis: 1. Endoscopy: It shows columnar epithelium (pink color) instead of squamous epithelium (pale) greater than 3 cm above the anatomic esophagogastric junction (EGJ). 2. Pathology: Finding of specialized columnar epithelium, characterized by intestinal metaplasia, in biopsies taken at any level of the esophagus.  Etiology Barrett’s esophagus is believed to result from chronic GERD. Chronic reflux esophagitis lead to loss of esophageal mucosa and replacement of columnar epithelium with squamous epithelium. a) creeping of gastric mucosa (columnar) to replace eroded squamous cells. b) Presence of totipotent cells at submucosa of esophagus capable of replacing denuded esophageal mucosa.  Clinical importance of Barrett’s esophagus: Barrett’s esophagus predisposes to esophageal cancer i.e. precancerous, particularly adenocarcinoma. High-grade dysplasia in Barrett’s esophagus identifies patients at increased risk of developing esophageal cancer.  Clinical Picture: Symptoms related to gastroesophageal reflux disease (GERD).  Investigations: 1. Radiology: Reflux-related findings (mucosal ulceration, strictures). 2. Endoscopy and biopsy: Barrett’s columnar mucosa is deeper pink compared with the pale squamous mucosa and > 3 cm from the EGJ. Multiple biopsies at 2-cm intervals 15 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  are taken. Endoscopic application of vital stains (Lugol’s iodine, toluidine blue) improves recognition of columnar mucosa. Endoscopic appearance in Barrett’s esophagus  Treatment: 1. Control of associated symptoms of GERD and prevention of reflux complications by medical and surgical anti-reflux therapy. 2. Endoscopic surveillance to detect early stage adenocarcinoma & surgical resection in high-grade dysplasia.  HIATUS HERNIA  Definition: Protrusion of any part of the stomach through the esophageal hiatus into the thorax.  Classification: 1. Type I (sliding): The esophagogastric junction lies above the diaphragm, associated with GERD. 2. Type II (paraesophageal, rolling): The stomach herniates through an enlarged hiatus, adjacent to the esophagus into the mediastinum. The esophagogastric junction is not displaced and there is no reflux, its sequela is strangulation. 3. Type III (combination): A combination of sliding and paraesophageal hernia. 4. Type IV (complex): Herniation of other abdominal viscera with the stomach (colon, omentum, spleen). 16 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  Sliding hiatal hernia Paraesophageal hiatal hernia  Predisposing factors for Hiatal Hernia 1- degeneration of esophageal hiatus: a) fatty infiltration with obesity b) aging c) wasting diseases 2- Increased intra-abdominal pressure a) Chronic cough b) Space occupying lesions c) Straining 2- Increased intra-gastric pressure a) Pyloric stenosis  Complications of hiatus hernia: 1. Functional: GERD and its complications (esophagitis, bleeding, stricture, aspiration, Barrett’s mucosa). 2. Mechanical: a) Incarceration: Results in obstruction of lower esophagus (dysphagia), gastric outlet (postprandial fullness). b) Volvulus: Results from rotation of herniated stomach along a longitudinal (organoaxial) or transverse (mesentericoaxial) axis. This results in bleeding 17 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  (congestion of gastric mucosa, peptic ulceration), perforation (strangulation and gangrene), pulmonary impairment (space occupation, aspiration).  Clinical Picture: 1. Asymptomatic 2. Nonspecific upper abdominal or respiratory symptoms. 3. Symptoms of GERD. a. Reflux: gastric juice reach mouth with acid taste b. Heart burn: burning pain, retrosternal. c. Blenching (wind from mouth) 4. Symptoms related to complications of reflux esophagitis (haematemesis, dysphagia due to fibrosis, acute chest pain, inability to vomit , postprandial fullness, chronic iron deficiency anemia due to chronic blood loss) 5. Symptoms related to intrathoracic hernia (stomach at chest) a. Dyspepsia, fullness and abdominal distension b. Shorteness of beath c. Tachycardia d. Severe abdominal pain  Investigations: o Chest X-ray: Retrocardiac air-fluid level, mediastinal mass. o Barium study of upper GIT: Defines foregut anatomy, position of the stomach and its ability to empty. o Endoscopy: Used to assess level of EGJ, associated esophagitis, esophageal ulcers, stricture or tumor, taking biopsy if there doubt for metaplesia or malignant transformation. o Esophageal function studies (manometry, 24-hour pH): To assess esophageal function preoperatively. o Motility study - Decrease lower esophageal shincteric pressure - Blood examination: anemia due to blood loss. - US to the abdomen to exclude gall bladder disease (Saint’s triad: Hiatus hernia,Diverticulosis and Gall stone)  Treatment: o Type I: a. Medical treatment of GERD. b. Surgery is done when the indications for anti-reflux surgery are justified. o Type II, III, IV: Surgery. Indications of surgery in H.H: 1. All paraesophageal hernia to avoid complications e.g. strangulation, obstruction 18 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma 2. Failure of medical conservative treatment in sliding H.H for 3-6 month 3. Complicated sliding H.H e.g. hemorrhage, ulcers, short esophagus, recurrent pulmonary infection, and cancer.  Principle of surgery in H.H: - Reduce the hernia and its contents (abdominal or left thoracic approach). - Excise the sac and close the hiatus (posterior to the esophagus). - Correct associated pathology (oversew bleeding ulcer, excise gangrenous viscera). - Fix the stomach in the abdomen (gastropexy): o Suture lesser curve of stomach to median arcuate ligament. o Suture fundus to diaphragmatic hiatus. o Fashion an anterior gastrostomy. - Perform an anti-reflux procedure in patients who have GERD e.g. Nissan Fundoplication, Belsey IV repair, Hill’s repair.  Comparison between sliding & paraesophageal hernia Sliding H.H Rolling H.H Incidence 80% 10% Sac No sac True hernia with sac Esophago-Gastric Junction Above diaphragm Below diaphragm complication Less common More & severe management -First medical Only surgery -Surgery when indicated  GASTROESOPHAGEAL REFLUX (GERD) - GERD usually occur with hiatal hernia, but it may be independent of it. Severe esophagitis might occur; it usually followed in long standing cases with fibrosis, stricture formation and short esophagus.  Preventive mechanism against reflux esophagitis: 1. Presence of intraabdomial part of the esophagus which has higher pressure than thoracic esophagus. 2. The sphincteric action of circular muscle fibers of the lower esophagus (inferior esophageal sphincter). 3. Valve like effect of the angulation of the esophagogastric junction. 19 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma 4. The “pinch cock” action of the right crus of the diaphragm maintaining the forward bent of the esophagoastric junction. 5. The rosette like fold of gastric mucosa at the cardia. 6. Clearing effect of reflux by: a) Swallowing saliva (alkaline) b) Erect position c) Repeated esophageal peristalsis movement d) Gastro-duodenal emptying.  Medical management: 1- Lifestyle modification: a) Elevate the head of the bed on 15 cm blocks: Gravity helps esophageal clearance. b) Weight loss in obese patients: Increased abdominal pressure in obese patients promotes reflux. c) Avoid eating near bedtime: This allows the stomach to be empty at the time of recumbency. Avoid bulky food d) Avoid aggravating factors (chocolate, fatty foods) they decrease lower esophageal sphincter (LES) pressure. e) Avoid cigarettes and alcohol: They decrease lower esophageal sphincter (LES) pressure. f) Avoid medications (nonsteroidal anti-inflammatory drugs, calcium channel blockers): They decrease LES pressure and delay gastric emptying. g) Avoid staining 2- Cytoprotectives: Sucralfate, a basic aluminum salt, exerts a generalized cytoprotective effect in acidic environment by enhancing gastroesophageal mucosal defense mechanisms, acting as a barrier to the diffusion of acid, pepsin and bile salts. 3- Prokinetic agents: Act by increasing LES pressure and by enhancing gastric and esophageal emptying. Examples: Metoclopramide. 4- Antacids: - Reduce gastric acidity. - Examples: Silicates, H2-blockers (cimetidine, ranitidine), Proton-pump inhibitors (omeprazole, lansoprazole).  Indications of anti-reflux surgery for GERD: 1. Peptic stricture. 2. Ulcerative esophagitis. a) Recurrent aspiration and/or pneumonia. b) Barrett’s esophagus (to correct GERD-related symptoms). 20 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  3- Associated lesions e.g. gall bladder stone. Aims of surgery for GERD 1- Reduction of the hernia 2- Creation of an intraabdominal segment of esophagus (5 cm). 3- Narrowing of the hiatus by approximation of 2 halves of right crus of the diaphragm. 4- Creation of acute angle of entry of esophagus to the stomach. 5- Prevention of herniation of esophagogastric junction into the chest. 6- Strengthening of anti-reflux mechanism (fundoplication) that allows normal swallowing, belching and vomiting, but prevents pathologic GER  Operative approaches for GERD (anti-reflux surgery): o Route a. Abdominal: Effective with normal length abdominal esophagus. b. Thoracic: Used for the Belsey Mark IV partial fundoplication & when esophageal lengthening (Collis gastroplasty) is required. c. Thoracoabdominal: For re-operation. d. Laparoscopic: Long-term efficacy is still unproven. 1- Nissen Fundoplication - Done through transabdominal approach - After reduction of the stomach and bringing eosophago-gastric junction below the diaphragm. - The hiatus is narrowed by one or two nonabsorbable sutures approximate the crura posteriorly to prevent migration of the fundoplication. - Wrapping the fundus of the stomach around esophagus (360°) over wide bored stomach tube to avoid narrowing. 21 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  Nissen fundoplication 2- Belsey Mark IV (270 ْ). - A transthoracic approach through bed of 7th rib. - Restoring cardio-esophageal angle by suturing the fundus of the stomach to the lower esophagus utilizing 2 - 3 rows of horizontal mattress sutures. (Fig. 90). - Tightening diaphragmatic hiatus, reduction of the stomach to abdomen. Belsey Mark IV fundoplication 3- Hill gastropexy: - Tarnsabdominal - Suturing fundus to the median arcuate ligament overlying aorta i.e. posterior Gastropexy. - 22 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  4- Collis gastroplasty (Esophageal lengthening): In the presence of esophageal shortening due to reflux esophagitis, Collis creates a neo- esophagus as gastric tube from the greater curve. Then a partial (Collis-Belsey) or total (Collis-Nissen) fundoplication is performed around the neo-esophagus, which is reduced below the diaphragm. Collis gastroplasty  Specific complications of anti-reflux surgery: A. Dysphagia: Related to the extent of fundoplication (too long, too tight) or presence of associated motility disorder. B. Gas bloat syndrome: Inability to belch and postprandial fullness. (vagal nerve injury, aerophagia, delayed gastric emptying, extent of fundoplication). C. Technical failures: “Slipped Nissen”, breakdown of fundoplication, herniation into mediastinum, esophageal or gastric perforation.  ESOPHAGEAL DIVERTICULAE  Definition: Esophageal diverticula are true (traction diverticula containing all layers of the esophageal wall), and false (pulsion diverticula containing mucosa only).  Etiology: 23 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma True (traction) diverticula are secondary to an inflammatory process. False (pulsion) diverticula are the end-result of esophageal dysfunction. Pharyngoesophageal (Zenker’s diverticulum) Zenker’s diverticulum is an acquired pulsion diverticulum, arising from a triangular weakening (Killian’s triangle) in the posterior midline of the lower pharynx, between the oblique and transverse (cricopharyngeus) muscle fibers of the inferior pharyngeal constrictor. It results secondary to dysfunction of cricopharyngeus or upper esophagus. o Clinical picture: Patients present with dysphagia, regurgitation of undigested material and chronic aspiration with alteration of the voice and wheezes. Large diverticulum may present a neck mass. o Complications: - Retained foreign body. - Tracheal fistula. - Hemorrhage. - Complications of aspiration. - Malignant transformation. o Diagnosis: - Barium swallow. - Endoscopy and manometry. Zenker’s diverticulum Endoscopic view o Treatment: - Large diverticula are excised and closed transversely through a left neck approach. If left unexcised, suspension of the apex of the diverticulum (diverticulopexy) to the prevertebral fascia or pharyngeal musculature may be done. - A cricopharyngeal myotomy (dividing cricopharyngeus and extending the myotomy 2-3 cm onto the proximal esophagus) is esential. N.B. Rare diverticulae: 1- Epiphrenic (lower esophageal), pulsion type. 2- Mie-esophageal, traction type due to mediastinal fibrosis. 24 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma   Foreign bodies of the esophagus  It may be coins, meat or dental prosthesis.  Coins usually obstruct the upper part of the esophagus at the level of cricopharyngeus muscle being the narrowest part all-over the gastrointestinal tract.  Meat obstruction occurs in: 1- Healthy esophagus. 2- Post-corrosive stricture. 3- Cancer esophagus.  Levels of physiological narrowing of the esophagus 1. Cricopharyngeal narrowing: at 15cm from the upper incisor teeth. 2. Bronchoaortic narrowing: at 25cm from UIT. 3. Cardia: 40cm from UIT.  Clinically: There is sudden onset of dysphagia.  Diagnosis: CXR Foreign body in the upper esophagus  Management: Esophagoscopy and extraction of the foreign body using the rigid esophagoscope. MEDIASTINUM  ANATOMY: The mediastinum is the space between the thoracic inlet and the diaphragm, the right and left pleural surfaces, the sternum and the vertebral column. It is divided into superior and inferior mediastinum by an arbitrary line extending from the manubriosternal angle to T4/T5 intervertebral space 25 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  Subdivisions of the mediastinum  Based on the lateral CXR, the mediastinum can be subdivided into compartments: 1. Anterior or anterosuperior: Contains the thymus, lymph nodes and fat. 2. Middle: Contains the heart and pericardium , superior and inferior venae cavae, ascending aorta, aortic arch and its branches, brachiocephalic vein, pulmonary arteries and veins, phrenic and vagus nerves, trachea and main bronchi, lymph nodes and fat. 3. Posterior: Contains the esophagus, descending aorta, azygous and hemiazygous veins, thoracic duct, sympathetic chain and lymph nodes. Radiological subdivisions of the mediastinum  Mediastinal Masses and Cysts  Differential diagnosis of a mediastinal mass 1. Anterosuperior mediastinum (4Ts) - Thymic tumor or cyst. - Thyroid (Retrosternal thyroid or neoplasm). - Terratoma (Germ-cell tumors). 26 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  - T Lymphoma. 2. Middle mediastinum - Lymphadenopathy. - Vascular: Aortic aneurysm, anomalies of great vessels. - Pericardial cyst or diverticulum. - Bronchogenic cyst. - Tracheal tumors. - Parathyroid tumors or cysts. - Neural tumors of the phrenic, vagus, sympathetic nerves. - Morgagni hernia. 3. Posterior mediastinum o Neurogenic tumors (benign B and malignant M): - Peripheral intercostal nerves (B neurofibroma from nerve fibers, neurilemmoma or schwanoma from nerve sheath and M neurofibrosarcoma) - Sympathetic ganglia (B ganglioneuroma and M neuroblastoma). - Paraganglia (phaeochromocytoma and paraganglioma B or M). o Meningocele. o Esophageal tumors, diverticulae, duplication cysts. o Hiatus hernia. o Thoracic duct cysts, cystic hygroma (lymphangioma). o Extramedullary hematopoesis. o Vertebral chordoma. o Paravertebral abscess (tuberculous).  Differential diagnosis of a mediastinal Cysts o Anterior Mediastinum: 1. Dermoid cyst. 2. Thymic cyst. 3. Cystic degeneration in retrosternal Goitre. 4. Lymphatogenic cyst. o Middle Mediastinum: 1. Bronchogenic cyst. 2. Pericardial cyst. o Posterior Mediastinum: 1. Foregut cyst. 2. Cystic degeneration in neurogenic cyst. 3. Cystic hygroma.  Clinical Picture: Half of patients are asymptomatic with normal physical examination. Symptomatic lesions and lesions in children are more likely to be malignant. 27 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma 1. Symptoms - Local: Pain (chest or back), dyspnea, cough, dysphagia,… - Systemic: Fever, malaise, weight loss, night sweats,… 2. Signs - Local: Cervical lymphadenopathy, facial and/or arm swelling, tracheal deviation. - General: Testicular masses, hepatosplenomegaly, muscle weakness.  Investigations: 1. Laboratory - Complete blood picture: Anemia, leucocytosis, thrombocytopenia. - Lactate dehydrogenase: Elevated in lymphomas, seminomas. - ß-human chorionic gonadotrophin: Elevated in non-seminomatous germ-cell tumors. May be increased in seminomas. - α-fetoprotein: Indicates a non-seminoma germ-cell tumor. It is normal in pure seminoma. - Alkaline phosphatase and calcium: Altered with parathyroid tumors. - Urine metanephrines: Vanilyl mandelic acid increased in phaeochromocytoma. 2. Radiological - CXR. - CT scan with IV contrast. - MRI for neurogenic and vascular lesions. - Angiography for vascular lesions. - Barium swallow for posterior mediastinal masses. - Radionuclide scintigraphy for thyroid or parathyroid masses. - Galliums scan in lymphoma. 3. Biopsy o Needle (image – guided): 1. Fine-needle aspiration biopsy. 2. Core-tissue biopsy. o Endoscopy: 1. Mediastinoscopy. 2. Video-assisted thoracoscopic surgery (VATS). 3. Bronchoscopy and trans-bronchial lung biopsy. 4. Esophagoscopy. o Surgery: 1. Scalene and supra-clavicular lymph node biopsy. 2. Anterior mediastinotomy. 3. Thoracotomy. 28 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma   THYMOMA  Definition: Tumor originating from thymic epithelial cells.  Clinical picture: - Most common tumor of the anterior mediastinum. - 10% of patients with myasthenia gravis have thymoma. - 40% of patients with thymoma have myasthenia gravis. - Patients may be asymptomatic, may have cough, dyspnea, chest pain, muscle fatigue characteristic of myasthenia gravis, neck and facial swelling from superior vena cava syndrome, and symptoms of other autoimmune disorders. N.B: Myasthenia Gravis occurs mostly with lymphocytic variety  Specific investigations: - CT Chest. - Acetylcholine receptor antibody levels or single fiber electromyography (EMG) for suspected myastenia gravis. - Biopsy (needle, core, open via mediastinotomy) carries the risk of seeding tumor cells. Preoperative biopsy is recommended if the tumor invades mediastinal structures, appears unresectable, associated with pleural or pericardial effusion.  Pathological cell type classification: o Epithelial predominant (most aggressive). o Lymphocytic. o Mixed. o Spindle cell.  Staging: o Stage I : No invasion of capsule microscopically or macroscopically. o Stage II : Microscopic or macroscopic invasion of the capsule or invasion into mediastinal fat or pleura. o Stage III : Invasion of lung, great vessels, pericardium. o Stage IVa: Pleural or pericardial metastases. o Stage IVb: Distant metastases.  Treatment: o Stage I Total thymectomy. o Stage II Total thymectomy + postoperative radiation. o Stage III Total thymectomy &resection of all nonvital structures invaded by the tumor + postoperative radiation & chemotherapy. If the tumor appeared unresectable give chemotherapy and radiation is followed by surgical debulking. o Stage IVa Chemotherapy followed by resection + postoperative irradiation. 29 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  o StageIVb Chemotherapy.  MYASTHENIA GRAVIS  Definition: Myasthenia Gravis (MG) is a chronic muscle disease that produces weakness and abnormally rapid fatigue of the voluntary muscles. Acetylcholine normally transmits nerve impulses to muscle fibers at the neuromuscular junction, causing the muscles to contract. Patients with MG form antibodies against the acetycholine receptors. MG is therefore an autoimmune disease. The thymus gland appears to be involved as it produces acetylcholine receptor antibodies or other substances that interfere with neuromuscular transmission.  Osserman Clinical Classification o Grade I: ocular type o Grade II: generalized type o Grade III: severely generalized o Grade IV: Myasthenic Crisis.  Diagnosis o Edrophonium test (Tensilon test): Patient response to anticholinesterase. Tensilon is most common used as it is rapid onset (30 seconds) and short duration (3minutes) o Electromyography (EMG): considered to be the most sensitive test for MG. o Antibodies against the acetylcholine receptor. The test has a reasonable sensitivity of 80–96%, but in ocular myasthenia, the sensitivity falls to 50%. o Less commonly used tests: 1. Joly Test. Repetitive simulation of peripheral nerve. 2. Ice test. Applying ice to weak muscle groups characteristically leads to improvement in strength of those muscles. Acetylcholinesterase is thought to be inhibited at the lower temperature, and this is the basis for this diagnostic test.  Management: o Treatment includes drugs and surgery: Alone or in combination. 1. Anticholinesterase agents: (Neostigmine, pyridostigmine) strengthen neuromuscular transmission. They prolong the effect of acetylcholine that signals the muscles to act. 2. Steroids (prednisone/prednisolone): For more severe cases of MG to suppress the body's immune system. Other immunosuppressive drugs, such as, cyclosporin, mycophenolate, and azathioprine can be used. People are commonly treated with a combination of these drugs with an acetylcholinesterase inhibitor. 3. Thymectomy: Removal of the thymus (especially in thymomatous MG). 30 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  4. Plasmapheresis (Blood plasma exchange process): Removes from the blood antibodies and other substances that interfere with the transmission of nerve impulses. This is highly indicated in Myasthenic crisis. 5. Intravenous immunoglobulin therapy (IVIG): Injection of immunoglobulins (There is a link between autoimmune disorders and immunodeficiency states). Patients receive between 3 and 12 infusions.  NEUROGENIC TUMORS - Most common posterior mediastinal tumors. - Asymptomatic unless large, invasive, with extension through the intervertebral foramen into the spinal canal (dumbbell tumor). - 10% are malignant - Malignant tumors occur more frequently in children.  Treatment: I. Most of the tumors should be excised: o Intrathoracic disease only: Thoracotomy. o Dumbbell tumors: Laminectomy and intraspinal dissection performed initially to prevent cord injury or intraspinal hemorrhage. This is followed by thoracotomy. o Neuroblastoma: Chemotherapy& radiation. o Paragangliomas: Excision (may invade vascular structures e.g. aorta, heart). o Phaeochromocytoma: Preoperative α- and B- receptors blockade + excision. II. Neuroblastoma: Chemotherapy& radiation. Excision for residual masses.  GERM-CELL TUMORS - A germ cell tumor (GCT) is a neoplasm derived from germ cells. Germ cells normally occur inside the gonads (ovary and testis). Germ cell tumors may originate outside the gonads. The anterior mediastinum is the most common site of extragonadal germ-cell tumors. - Germ cell tumors can be cancerous or non-cancerous tumors. Germ-cell tumors 15% of primary mediastinal masses.Of all anterior mediastinal tumors, 15-20% is germ cell tumors of which approximately 50% are benign teratomas. Males aged 20-35 years are affected. - Investigations should include testicular examination with ultrasound.  Tumour markers: o ß-human chorionic gonadotrophin (β-HCG): Elevated in non-seminomatous germ-cell tumors. May be increased in seminomas (10%). o α-fetoprotein: Elevated in non-seminomatous germ-cell tumors. It is normal in pure seminoma.  Classification: 1- Teratoma: 31 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  - Tumor markers are negative in pure forms. - Pathologically it contains all components of ectoderm (skin, hair and teeth), Endoderm (blood vessels) and Mesoderm (muscle). - Encapsulated tumors are excised. - Teratoma (Benign or Malignant) include: mature teratoma, dermoid cyst, immature teratoma, teratoma with malignant transformation. - Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both. 2- Seminoma (synonyms germinoma, dysgerminoma): - Malignant. - Tumor marker (10% have elevated hCG). - Seminomatous tumors tend to grow slower than nongerminomatous tumors, and have a higher 5-year survival rate. - The survival rate for germinomatous tumors is higher in part because these tumors are very sensitive to radiation, and they also respond well to chemotherapy. - The primary management involves combination chemotherapy (cisplatin, bleomycin, etoposide, and vinblastine) + large volume radiotherapy. 3- Nonseminomatous: - Malignant - Include embryonal carcinoma, yolk sac tumour, choriocarcinoma and mixed tumor histology. - Compared to seminomatous tumors, non-seminomatous tumors tend to grow faster, have an earlier mean age at time of diagnosis, and have a lower 5-year survival rate. - The prognosis for non-germinomatous tumours has improved dramatically, however, due to the use of platinum-based chemotherapy regimens.  Tumor markers (β-HCG, α-fetoprotein) are elevated.  Tissue diagnosis is preferable but therapy may be initiated on the basis of elevated markers alone.  Management is primarily cisplatin-based chemotherapy.  Residual masses causing compressive symptoms require resection. LYMPHOMA - Lymphoma is a group of tumors that develop from lymphocytes. - There are two main types: Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). - Multinucleated Reed–Sternberg cells (RS cells) are the characteristic histopathologic finding of Hodgkin's lymphoma. - Mediastinal lymphoma is generally a part of widespread disease. 32 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  - In CXR it gives the appearance of bilateral hilar lymphadenopathy (DD. Lymphoma, TB and sarcoidosis). - Imaging is done to determine spread. - A biopsy of a lymph node (tissue core needle or surgical biopsy). Management depends on accurate tissue diagnosis. - Treatment may involve some combination of chemotherapy, radiation therapy, targeted therapy and surgery. - Chemotherapy is the mainstay of treatment, but radiotherapy or bone-marrow transplantation may be used.  Classification - Classification systems generally classify lymphoma according to: 1) Whether or not it is a Hodgkin lymphoma. 2) Whether the cell that is replicating is a T cell or B cell. - The current accepted definition is the WHO classification, based upon the "Revised European-American Lymphoma classification" (REAL). I. Mature B-cell neoplasms II. Mature T cell and natural killer (NK) cell neoplasms III. Hodgkin Lymphoma o Classical Hodgkin lymphomas: o Nodular sclerosis o Mixed cellularity o Lymphocyte-rich o Lymphocyte depleted or not depleted o Nodular lymphocyte-predominant Hodgkin lymphoma IV. Immunodeficiency-associated lymphoproliferative disorders  BRONCHOGENIC CYST 1. Located close to the main stem bronchus or trachea, just below the carina (presents with airway obstruction), in the pulmonary parenchyma especially lower lobes (presents with recurrent infection). 2. May be single, multiple or multiloculated. 3. Often communicates with the bronchial lumen. 4. Lined by ciliated columnar or cuboidal epithelium, may contain cartilage and mucous glands.  Indications for surgical excision: 1. Enlargement of the cyst. 2. Development of symptoms: Cough, dyspnea, haemoptysis). 33 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma   ENTERIC CYST - Along the esophagus, may be embedded within its wall. - Lined by any type of alimentary tract epithelium. - May compress the esophagus or respiratory tree and may ulcerate. - CXR and CT show cystic lesion of low density.  Treatment is surgical excision.  PERICARDIAL CYST - Located near the cardiophrenic angles. - 75% are on the right side. - 10% communicate with the pericardial sac. - Diagnosis is accurate by CT scan, echocardiography or needle aspiration if possible (clear spring-water fluid). - Exision is required to obtain tissue diagnosis or if the patient is symptomatic.  RETROSTERNAL GOITRE - More than 50% of the thyroid is below the thoracic inlet. - Indications for excision are: Respiratory distress (sudden or chronic with tracheomalacia), dysphagia, SVC obstruction and hyperthyroidism. - Retrosternal goiter is removed using a collar incision with or without sternotomy. - True ectopic goiter (has a thoracic vascular supply), goiter with posterior mediastinal extension or malignant retrosternal goiter are removed using thoracotomy.  MEDIASTINITIS  Etiology: 1. Median sternotomy wound infection (following open heart surgery and sternal dehiscence). 2. Esophageal perforation. 34 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  3. Local spread of infection (from lung, pleura, subphrenic, ribs, vertebrae, head and neck or penetrating injury). 4. Hematogenous and lymphatic spread (from distant septic sources). 5. Idiopathic.  Clinical Picture: - Pain, dyspnea, fever with high WBC count. - Sternal dehiscence (fullness and erythema of the wound, local tenderness, discharge, palpable separation of the sternal edges on coughing).  Investigations: 1. Laboratory: Leucocytosis, … 2. CXR, CT chest: o Broad mediastinum. o Mediastinal abscess with fluid level. o Evidence of sternal dehiscence. o Associated pathology.  Treatment: 1. Systemic antibiotics. 2. Surgical debridement. 3. Irrigation of the mediastinum with Povidone iodine solution and antibiotics. 4. Drainage of any mediastinal collection. 5. Deal with underlying pathology. 6. Reclosure of the sternum in sternal dehiscence.  SUPERIOR VENA CAVA SYNDROME  Etiology: 1. Malignant diseases: Cancer lung (75%), lymphoma (10%), mediastinal tumors as germ- cell tumors, malignant thymoma and metastases (5%). 2. Benign diseases: Fibrosing mediastinitis, retrosternal goitre and aneurysms of great vessels (5%). 35 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  3. Iatrogenic causes: Thrombosis secondary to transvenous pacemakers or central venous lines as CVP lines, portcath, Denver & leVeen shunts and renal dialysis catheters (5%).  Clinical Picture: 1. Symptoms: Dyspnea, cough, face and/or arm swelling, chest pain, syncope, headache, confusion. 2. Signs: Edema and prominent veins of the head, neck, upper trunk and arms, cyanosis and plethora, papilledema, Horner’s syndrome, vocal cord paresis.  Investigations: o CXR: Mediastinal mass. o CT scan chest with IV contrast: Level of SVC obstruction, extent of mediastinal disease and venous collaterals. o MRI: Mediastinal vascular anatomy. o Venography: Site of SVC obstruction, presence of intravascular thrombus and pattern of collateral vessels. o Serum markers -HCG or -fetoprotein: Germ-cell tumor. o Biopsy: Percutaneous needle biopsy, bronchoscopy, mediastinoscopy, mediastinotomy, VATS, thoracotomy.  Treatment: 1. Medical: Elevation of the head, oxygen, diuretics, salt restriction, steroids for laryngeal edema or brain metastases, anticoagulants for venous thrombosis. 2. Radiotherapy: For thoracic malignancy. 3. Chemotherapy: For lymphoma or small cell lung cancer. May be combined with radiotherapy. 4. Intravascular stents. 5. Surgery: a) Bypass using venous autografts or synthetic tube grafts for patients with benign disease and selected patients with malignancy. b) Resection and vascular reconstruction: For localized tumors 36 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma  The Chest Wall  Congenital abnormalities: 1) Bifid rib. 2) Cervical rib. 3) Pectus excavatum: the sternum is depressed below the level of the ribs. 4) Pectus carinatum (pigeon chest): the sternum is elevated above the level of the ribs. 5) Poland syndrome: is a congenital malformation affecting the chest muscle and hand on one side of the body. The pectoralis major is absent. The fingers are webbed on the same side of the body. In girls, the breast on that side may be absent. Treatment includes reconstructive surgery.  Chest wall tumours:  Pathology: I. I-benign: Osteoma, chondroma. II. II-Malignant: a) Secondary: are the commonest chest wall tumors, as direct invasion or hematogenous. b) Primary: originating from any mesenchymal structure of the chest wall e.g. osteosarcoma, chondrosarcoma, fibrosarcoma, etc.  Investigations: a) Radiological: b) Plain CXR (PA and Lateral), CT scan, MSCT scan (skeleton view) and isotope bone scan. c) Biopsy: Needle biopsy, incisional or excisional biopsy.  Treatment:  Excision with safety margin.  Chest wall reconstruction: if resection involves more than two successive ribs. Using: Prosthetic material as Prolene mesh or myocutaneous flaps.  Chemo or radiotherapy: for advanced tumors. 37 | P a g e W a l i d A b u A r a b Cardiothoracic Trauma