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Chapter 22 Diseases of the Oesophagus CHAPTER DISEASES OF THE OESOPHAGUS ANATOMY Clinical Picture PHYSIOLOGY...
Chapter 22 Diseases of the Oesophagus CHAPTER DISEASES OF THE OESOPHAGUS ANATOMY Clinical Picture PHYSIOLOGY Special Investigations ADULT DIAPHRAGMATIC HERNIAS AND THE OESOPHAGUS Treatment Types Other Diverticulae Rolling Hernias (Para-oesophageal Hernias) Mid-Thoracic Oesophagus Sliding Hernia Distal Oesophagus OESOPHAGEAL MOTILITY DISORDERS OESOPHAGEAL STRICTURES Achalasia Classification Epidemiology Clinical Picture Pathophysiology Special Investigations Presentation Management Diagnosis OESOPHAGEAL PERFORATION Treatment Classification Other Oesophageal Motility Disorders Clinical Picture Diffuse Oesophageal Spasm Special Investigations Nutcracker Oesophagus Management CORROSIVE INJURY OF THE OESOPHAGUS OESOPHAGEAL CARCINOMA Grading Epidemiology Diagnosis Etiology Clinical Picture Risk Factors Special Investigations Histological Types Treatment Tumour Biology Prognosis Clinical Staging DIVERTICULAE OF THE OESOPHAGUS Special Investigations Classification Management Pharyngeal Pouch (Zenker’s Diverticulum) Prognosis Etiopathogenesis ANATOMY Attached to diaphragm via 3 parts phrenooesophageal o From cricoid cartilage to gastric cardia ligament – 25cm Arterial blood supply o Cervical, thoracic (upper, middle, o Neck lower), intra-abdominal Inferior thyroid artery o Traverses diaphragm at level of T10 o Thoracic 2 sphincters Bronchial arteries o Proximal Branches from thoracic aorta Cricopharyngeal muscle, o Abdomen inferior constrictor Inferior phrenic artery o Distal Left gastric artery Oesophagogastric junction Venous drainage 3-5cm high pressure o Neck area Inferior thyroid veins 217 Diseases of the Oesophagus Chapter 22 o Thoracic -Bottom 1/3 Hemiazygos – smooth Azygos muscle o Abdomen - Middle 1/3 Left gastric vein portal mixed system Longitudinal (outer) Lymph drainage Outer adventitial connective o Local lymph nodes tissue layer o Posterior mediastinal, supraclavicular, coeliac PHYSIOLOGY Nerve supply o Sympathetic Sphincter mechanism Preganglionic – T5,6 o Food tongue to oropharynx Post-ganglionic – cervical upper sphincter relaxes peristalsis vertebral, coeliac ganglia lower sphincter relaxes stomach o Parasympathetic Peristalses Glossopharyngeus o Primary Recurrent laryngeal Propulsive – superior to Vagus inferior Oesophageal wall o Secondary o Layers Not normal Mucosa (squamous Peristalsis proximal to epithelium) obstruction Distal 1-2cm – Stronger, propulsive columnar epithelium o Tertiary Division = Z-line Moves dissociated Submucosa with mucous Aimless, not propulsive glands, lymphatics Nerve supply ADULT DIAPHRAGMATIC HERNIAS AND THE OESOPHAGUS Meissner’s plexus - In Types submucosa - Secretions Type determined by position of Auerbach’s plexus oesophagogastric junction (EG junction) - Between 2 muscle layers - Peristalsis Muscles Circular (inner) – vagus responsible for peristalsis - Upper 1/3 – striated muscle Sliding hernia Rolling hernia (para-oesophageal) 218 Chapter 22 Diseases of the Oesophagus Combination o Close defect o Reduce stomach, EJ junction, Rolling Hernias (Para-oesophageal Hernia) peritoneal sac EG junction in normal position Sliding Hernia Part of stomach rolls up along oesophagus into mediastinum EG junction slides up in thorax, above 10% of cases diaphragm – no longer located in abdominal cavity Presentation EG junction covered with peritoneum anterior, posterior retroperitoneal Asymptomatic Negative pressure incompetence reflux Symptomatic injury o Dysphagia 90% of cases o Pain after meals o Hiccups, palpitations = mass in thoracic Prevention of reflux - 4 Pillars cavity, irritates pericardium and diaphragm Sphincter competence – determined by o Position of the EG junction Complications o Mucosal rosettes o Angle of His Strangulation perforation mediastinitis Emptying of oesophagus o Pain substernal Gastric emptying o Toxic/pyrexial Nature of refluxate (pH = acid/bile) o Septic shock o Death Complications o CXR Surgical emphysema Consequences and complications of reflux Pleural effusion disease and correlation with clinical picture Stasis gastritis peptic ulcer o Distal chemical oesophagitis = bleeding/perforation heartburn + bleeding Gastric volvulus – mesenterico- o Distal oesophageal ulceration = axial/organoaxial bleeding, perforation o Pain, anorexia (gastric outlet o Stricture formation obstruction) Reflux symptoms symptoms of dysphagia Special Investigations Regurgitation Weight loss Contrast swallow o Erosion Endoscopy o Ulceration o Metaplasia [Squamous adeno] = Management Barret’s oesophagus Asymptomatic Pre-malignant o Anaesthetic risk = leave o Aspiration asthma/chronic cough o No anaestetic risk = repair Diagnosis Symptomatic = as above Complicated = emergency operation Symptoms important Principles of surgical repair 219 Diseases of the Oesophagus Chapter 22 Contrast swallow cooperation/financial Endoscopy & biopsy problems] pH studies per indication [Not responding on Young PPI’s] Occupation e.g. mechanic Manometry per indication CXR o Mediastinum widened OESOPHAGEAL MOTILITY DISORDERS o Air-fluid level behind heart Achalasia Treatment Inability of the EG junction to relax due to poor Conservative (Medical) peristalsis of the oesophageal body o Pharmacological PPI’s Epidemiology o Lifestyle changes Present late 30’s, early 40’s Avoid acidic foods, alcohol Females > males If overweight, lose weight Gradual onset over years Postural South America Avoid lying flat after o Chaga’s disease meals o Infestation with Trypanosoma cruzi Bend at the knees, don’t bend back Pathophysiology Avoid tight clothing Idiopathic degeneration of Auerbach’s plexus o Possible early ischaemic incident o No vagal stimulation Presentation Stages and clinical picture o Early (Stage I) Slight oesophageal dilatation Dysphagia for fluids o Intermediate (Stage II) Obvious oesophageal dilatation Food lodged in oesophagus Surgical Regurgitation/aspiration/halito o Nissen fundoplication (360 wrap) sis o Repair defect in diaphragm o Late (Stage III) o Indications for surgery Sigmoid oesophageal Complications of reflux dilatation disease e.g. strictures, ulcers, As above + associated weight respiratory loss Aspiration Bile reflux Failure of medical management [patient 220 Chapter 22 Diseases of the Oesophagus Progressive vs Non-progressive Dysphagia o NB!! Endoscope passes with ease Manometry Progressive Non-progressive o Sometimes performed Mechanical problem Motility problem o Poor peristalsis in oesophageal body No pain Pain Treatment 4 grade which follow Dysphagia erratic each other Balloon dilatation (temporary) o Grade 1 – o Allows time to improve nutritional status Unable to prior to surgery tolerate solids Botox injection in distal sphincter (temporary) o Grade 2 – Surgery = only long-term solution Unable to o Principles tolerate semi- Early stages solids Heller myotomy + o Grade 3 – 180 Watson Unable to anterior tolerate liquids fundoplication = o Grade 4 – partial sphincter Unable to competence swallow air Late stages Lesion obstructing Oesophagectomy + lumen colon interposition Other Oesophageal Motility Disorders o End-stage (Stage IV) Paralysed mediastinal Presents with intermittent dysphagia + oesophageal sac atypical chest pain = mimic angina Not able to take anything in o Presents to cardiologist Diagnosis Diffuse Oesophageal Spasm CXR Corkscrew oesophagus o Fluid level in oesophagus (behind heart) Treatment o Dilated oesophagus – widened Ca2+-channel blockers mediastinum Severe Contrast swallow o Myotomy of oesophageal body o Dilated oesophagus o Fluid level in oesophagus Nutcracker Oesophagus o Groundglass appearance (contrast surrounding foodrests above fluid level) Peristalsis normal, but high amplitude o Bird’s beak appearance o Very little contrast enters stomach Treatment o Absent gastric air bubble Endoscopy Ca2+-channel blockers o Stasis esophagitis Severe o Mainly to rule out mechanical problem o Myotomy v oesophageal body 221 Diseases of the Oesophagus Chapter 22 CORROSIVE INJURY OF THE OESOPHAGUS Mediastinitis Pleural effusion Incidences Shocked o Toddlers = accidental Multi-organ failure o Adults = suicide Death Types of chemicals Stomach and duodenum o Alkalines e.g. cleaning detergents o Gastritis/duodenitis Toddlers o Necrotic stomach/duodenum Burn oesophagus more Acute abdomen o Acids e.g. battery acid Lungs Suicide o Dyspnoea = pleural effusion Burn stomach more o Chemical pneumonitis Grading Special Investigations Zargar classification – determined If not shocked endoscopically o CXR Surgical emphysema Zargar Classification of Caustic Injuries Air in mediastinum Grade 0 Normal mucosa Pneumoperitoneum Grade I Oedema and erythema of the mucosa Pleural effusions Grade IIA Haemorrhage, erosions, blisters, o Gastrograffin swallow to rule out superficial ulcers oesophageal and Grade IIB Circumferential lesions gastric perforations Grade IIIA Focal deep gray or brownish-black o Early endoscopy to determine extent ulcers and severity of injury Grade IIIB Extensive deep gray or brownish-black If shocked ulcers o Resuscitate first before embarking on Grade IV Perforation special investigations Treatment Diagnosis Management Clinical picture o Not shocked Keep nil per mouth initially Oral cavity and pharynx Peripheral IV line – Dextrose o Stomatitis & pharyngitis in Saline o Irritation/pain Sucralfate o Drooling/agitated Sedate patient o Not shocked Start feeding after 24-48 hours o Recover completely Discharge if eating properly Oesophagus Rescope after ± 6 weeks if o Oesophagitis oesophagus was injured to Mediastinal pain evaluate for strictures Haematemesis o Shocked Not shocked Admit in ICU Stricture formation Intubate and ventilate o Necrotic oesophagus Urine catheter 222 Chapter 22 Diseases of the Oesophagus 2 Peripheral and 1 central IV line Resuscitate aggressively with fluid IV antibiotics – 3rd generation cephalosporin Theatre After successful resuscitation or if patient remains shocked Scope in theater Pharyngeal Pouch (Zenker’s Diverticulum) Necrotic oesophagus/ Etiopathogenesis stomach – do emergency Pseudo, pulsion diverticulum through Kilian’s oesophagectomy & triangle (weak spot) gastrectomy o Mucosal outpouching in Kilian’s Reconstruction later triangle, mostly to the left because patient too Upper sphincter dysmotility – increased ill = after 3-6 months pressure in distal pharynx Prognosis Clinical picture Necrotic oesophagus = mortality rate >50% Older individuals Pre-malignant condition Swelling in neck mostly to the left - especially after meals DIVERTICULAE OF THE OESOPHAGUS Regurgitation/halitosis Noise in neck Classification Weight loss By position Special investigations By wall o True diverticulum Contrast swallow Involved all 3 layers of the wall o See contrast spot in neck o Pseudo diverticulum o Rest of oesophagus normal Involves only mucosa Endoscopy By mechanism o Careful of perforation o Pulsion o Exclude other pathology Increased intraluminal pressure from sphincters Treatment Peridiaphragmatic/pharyngeal o Traction Small (5cm) o Laser septotomy in stages 223 Diseases of the Oesophagus Chapter 22 o Surgery Management Fix sac invertedly to prevertebral fascia Single and 50 years o Grow into lumen M:F = 3:1 Polypoid/fungating mass Squamous > adeno Obstruction = progressive Most often in thoracic oesophagus dysphagia Growth in submucosa Etiology o Up and down o Circumferential Premalignant conditions o Malignant stricture o Leukoplakia o Progressive dysphagia o Plummer Vinson syndrome Growth through wall – invasion of nearby Chronic iron deficiency structures (locally advanced) anaemia in females o Recurrent laryngeal nerve = Squamous hyperplasia hoarseness Stricture high in oesophagus o Trachea/bronchus = fistula o Barrett’s oesophagus – reflux disease Coughing when eating or o Achalasia drinking o Corrosive injury o Vertebrae = back pain Nitrosamines o Weight loss/cachectic 225 Diseases of the Oesophagus Chapter 22 Distant metastases Mediastinal sinus o Lymphogenic Axis deviation Neck nodes (Virchow- Angulation Trossier) Shoulder formation Mediastinal nodes o Endoscopy & Biopsies Pre-aortic nodes abdomen Position Epigastric mass Length o Haematogenic Degree of obstruction Lung, liver, bone o CT-scan for surgical planning Malignant pleural effusion o Sometimes bronchoscopy Liver mass To look for metastatic disease Localized bone pain o CXR Lung lesions – cannon balls Clinical Staging Pleural effusions – tap for cytology Early (Still confined to wall) - ±5% o Liver sonar o Potentially curative group Liver lesions o No total obstruction – grade I dysphagia o Bone scan o Minimal weight loss Hot spots o No metastases Locally advanced (outside wall but no Management peripheral metastases) - ±90% o Palliative group Early o Grade II + III dysphagia o Try to cure thus aggressive treatment o Moderate weight loss/cachectic o Radical oesophagectomy + o VT node/neck nodes reconstruction o Broncho-oesophageal fistula Remove oesophagus + o Back pain mediastinal lymph nodes and o Hoarseness reconstruct with gastric pull-up Metastatic (Peripheral metastatic disease) - o External beam radiotherapy – 5 gray = ±5% 5000 rad o Moribund group Locally advanced o Pleural effusion o Palliate dysphagia o Liver mass o No curative intent o Bone pain o Endoscopic dilatation + placement of a o Severely cachectic and weak covered self-expandable stent through tumour Special Investigations o Radiotherapy as above o Sometimes laser ablation or To investigate primary lesion brachytherapy o Contrast swallow Metastatic Signs of locally advanced o Tender love and care tumour >5cm long (2 Prognosis vertebral bodies) Total/severe Early obstruction o 80% 2-year survival rate Broncho- Others oesophageal fistula o Live only months or weeks 226 Chapter 23 Diseases of the Stomach and Duodenum CHAPTER DISEASES OF THE STOMACH AND DUODENUM PEPTIC ULCER DISEASE GASTRIC OUTLET OBSTRUCTION Introduction Causes Etiology Benign Duodenal Ulcer Malignant Clinical Features Pathophysiology Diagnosis Clinical Features Medical Treatment Differential Diagnosis Surgery Management Gastric Ulcer Investigations Clinical Features Treatment Diagnosis GASTRIC NEOPLASMS Medical Treatment Introduction Surgery Gastric Adenocarcinoma Complications of Ulcer Disease Epidemiology Bleeding Pathogenesis Perforation Diagnosis Obstruction Staging Zollinger-Ellison Syndrome Treatment Clinical Features Gastric Lymphoma Diagnosis Gastric GIST Treatment o Antrum of the stomach PEPTIC ULCER DISEASE o Lower end of oesophagus in patients Introduction with gastro-oesophageal reflux o Efferent limb of gastroenterostomy Common condition o Inside Meckel's diverticulum if there is o 10% of population suffer from it at some ectopic gastric mucosa time or another Acid hypersecretion Incidence and severity of peptic ulcer disease o Thought to be most important factor in decreasing in the Western world, increasing in causation of ulcers, in particular developing countries duodenal ulcers. Incidence increasing in elderly Helicobacter pylori o Widespread use of non-steroidal anti- o Gram-negative organism inflammatory drugs (NSAID) o Resides in mucus layer of antrum o Real culprit in majority of cases Etiology o Found in >90% of patients with duodenal ulcers, 70% of patients with Occur when balance between acid pepsin gastric ulcers digestion and defence mechanism of mucosa o Exact mechanism through which is disturbed bacteria cause peptic ulceration still Classical sites for peptic ulcers conjectural o First part of the duodenum o Eradication of the bacteria heals ulcer o Angula incisura and prevents recurrence 227 Diseases of the Stomach and Duodenum Chapter 23 Discovery revolutionised o Non-secretors of blood group antigens understanding of the in the saliva pathogenesis and o High circulating pepsinogen management of peptic ulcers Mucosal defence against acid-pepsin Clinical Features digestion o Mucous layer on the mucosa serving as Cardinal symptom is pain barrier between lumen and epithelial o Typically localised to the epigastrium surface o Dull or burning in character o Secretion of bicarbonate o Starts several hours after a meal o Rapid turnover of mucosal cells o Wakes the patient at night Mucosal defence weakened by o Relieved by food or antacids o Helicobacter infection Nausea and vomiting o NSAID intake May be present during acute exacerbation but o Reflux of bile into stomach are not prominent features. o Mucosal ischaemia Apart from mild tenderness in the epigastrium, “Stress ulcers” patients with uncomplicated ulcer disease do o Common in severely ill patients not have physical signs. o Caused by Course of duodenal ulcer disease one of Increased acid production in relapses and remissions stressed patients o Patient complains of episodes of Mucosal ischaemia as a result severe pain lasting for weeks, of splanchnic hypoperfusion interspersed by months of remission o Cushing's ulcers o Pattern repeating itself over several Stress ulcers occurring after years head injuries o May burn itself out after 10–15 years o Curling's ulcers Diagnosis Stress ulcers occurring after severe burns Difficult to differentiate from other causes of dyspepsia Duodenal Ulcer o Gastric ulcer Practically all duodenal ulcers occur in o Non-ulcer dyspepsia duodenal bulb o Reflux oesophagitis o Direct path of the acid contents of the o Gastric cancer stomach o Gallstones o Alkaline pancreatic juice and bile, which Contrast meal may show an ulcer crater enter duodenum in 2nd part, have not o May be difficult to differentiate active yet had an opportunity to neutralise ulceration from scarring gastric acid Flexible endoscopy is most accurate More common than gastric ulcers diagnostic method Occur in younger patients o The oesophagus, stomach and the first More common in men than women and second part of the duodenum can Genetic predisposition be clearly seen using this technique o More common in family members of Helicobacter pylori index cases o Can be demonstrated by o Blood group O Serology Urea breath test 228 Chapter 23 Diseases of the Stomach and Duodenum Urea labelled with a simple to conduct as only drop non-radioactive of blood is necessary (C13) or radioactive Accuracy of the commercially (C14) carbon isotope available test kits have is given by mouth improved but their validity Helicobacter must be confirmed for each produces urease, country as there are which split the urea geographical differences in into ammonia and genetic makeup of the carbon dioxide bacteria If C13 or C14 is Serology cannot be reliably detected in the used to assess the success of exhaled breath the eradication therapy, as presence of antibody levels can remain Helicobacter is raised for prolonged periods confirmed even after successful The urea breath test eradication is a reliable non- Proton pump inhibitors lead to invasive way of a suppression of organism confirming the numbers in the gastric success of mucosa eradication therapy Can lead to false negative Antral biopsies using results in patients undergoing microscopy urea breath test, urease test Rapid urease test and even histology Antral biopsies are It is recommended that embedded into a gel patients on proton pump containing urea and inhibitors have these ceased an indicator dye at least 2 weeks prior to urea (neutral red) breath testing or, if testing is to The ammonia be performed at endoscopy, produced as a result that biopsies are taken for of urease is alkaline histology from both antrum and changes the and body of stomach to indicator dye to a red increase the yield colour Medical Treatment This gives a rapid result—often within The aim of treatment is to an hour—and is o Alleviate ulcer pain cheaper than o Heal the ulcer histology o Prevent recurrence Culture of organisms is o Forestall complications difficult and rarely performed With powerful anti-secretory drugs and in clinical practice effective regimens to eradicate Helicobacter, Serology detects past as well these aims can be achieved by medical as current infection and is therapy in the great majority of patients 229 Diseases of the Stomach and Duodenum Chapter 23 Apart from giving up smoking and avoiding, if Sucralfate possible, ulcerogenic drugs, lifestyle o Not absorbed but exerts its action by modification such as a change in diet or physically covering the ulcer base avoidance of stress is not necessary o Ulcer healing rates are similar to H2 Such changes are difficult to make and there blockers. is little evidence that they accelerate ulcer healing Eradication of Helicobacter Pylori Nowadays, elective surgery for uncomplicated ulcer disease is rarely, if ever, indicated Cornerstone of ulcer treatment Nearly all ulcer surgery is performed as an Main reason why elective surgery for peptic emergency procedure for complications. ulcer disease is now very rare The bacteria dwells in, and is protected by, the Commonly Used Drugs mucous layer of the gastric pits and are difficult to eradicate Antacids Multiple drugs need to be administered o Tablet or liquid form simultaneously o Magnesium based (liable to cause Effective combinations should result in diarrhoea) or aluminium based (may eradication in more than 90% of patients, if cause constipation). patient compliance is satisfactory o Give rapid relief of ulcer pain but do not Combinations are 1–2 weeks of triple therapy heal ulcers unless taken in very high using bismuth, tetracycline and metronidazole doses o Side effects, especially gastrointestinal Histamine-2 Receptor Antagonists upsets, are common o First group of drugs that can effectively Combinations of a proton pump inhibitor and block gastric acid secretion two other antibiotics (e.g. amoxycillin and o Examples are cimetidine, ranitidine and clarithromycin) taken for 1 week are equally famotidine effective o Relief of pain occurs after a few days o Fewer side effects and up to 90% of ulcers heal after a 6- o This combination has become first line week course therapy in most countries. o Once the drug is stopped, however, acid production returns to normal and Surgery ulcer recurrence is highly likely Proton Pump Inhibitors The classical operations for duodenal ulcer o Complete inhibition of gastric acid disease are designed to reduce gastric acid production production o Examples such as omeprazole or Nowadays their use is almost entirely confined lansoprazole to the management of ulcer complications o Leads to symptomatic relief and ulcer Essentially one can remove the acid- healing is even more impressive than producing part of the stomach or sever the H2 blockers vagus nerve, which controls acid secretion o Once the drug is stopped ulcer recurrence is common o Shown to both prevent and heal ulcers associated with NSAID use 230 Chapter 23 Diseases of the Stomach and Duodenum Operations for Duodenal Ulcers If the whole vagal trunk is cut, delay in gastric (%) Side Recurrence emptying occurs in a significant number of Mortality Effects rate (%) patients because the motor supply to the Pólya's antrum is also severed 2–5 +++ 5 gastrectomy A drainage operation, either a pyloroplasty or Vagotomy gastro-enterostomy, is necessary 1 + 5–10 These operations are relatively easy to and drainage Highly perform and are useful in emergency selective 15 nodes involved gastroenterologist, oncologist, nurse and M (Metastasis) M0 No distant metastases pathologist M1 Distant metastases The team should also include when appropriate a palliative care specialist The information can also be used to place the Endoscopic treatment patient's tumour into a stage group, I through IV. A small number of patients will be diagnosed o For example a T3 N0M0 tumour is in with early stage T1 tumours - of these a stage II whereas a T3 N2 M0 tumour proportion are suitable for endoscopic would be stage III mucosal resection 241 Diseases of the Stomach and Duodenum Chapter 23 In Japan nearly half of all cancers are treated Early Complications in this way and as a consequence there are carefully derived systems for deciding which The post-operative course for a patient having tumours are appropriate for such treatment a gastric resection for cancer can be stormy, There are clearly considerable advantages for especially if the patient has a total the patient if surgery can be appropriately and gastrectomy safely avoided Potential complications include the usual cardiac, respiratory and wound complications Surgery that may occur in any patient undergoing abdominal surgery The majority of patients with potentially There is a risk of anastomotic leakage, curable disease will have a surgical resection especially after total gastrectomy and as the mainstay of their treatment oesophagojejunal anastomosis The operation may involve resection of the The suture or staple line where the duodenum distal stomach or the entire stomach has been divided may also break down There has been very considerable debate Fluid collections or abscesses are common, around whether the operation should involve particularly if extensive lymph node dissection extensive resection of the lymph nodes has been performed draining the stomach Dissection of lymph nodes from the pancreas The Japanese with their very high incidence risks causing acute pancreatitis of gastric cancer are convinced that such a Efferent loop obstruction lymph node dissection adds to the chance of o Sometimes the gastric remnant does achieving a cure not drain well into the jejunum and the Two large randomised controlled trials and patient may have prolonged several smaller trials carried out in Western nasogastric drainage or vomiting countries have failed to show a conclusive o This may be due to a mechanical benefit for the more radical lymph node obstruction (efferent loop obstruction) dissections although further analysis has or to poor motility of a partially shown potential benefit for patients with stage denervated gastric remnant II and early stage III disease Afferent loop obstruction Following gastric resection intestinal o Obstruction of the afferent loop can continuity is usually restored with a Roux-en - occur early in the post-operative course Y type reconstruction that reduces the risk of and lead to disruption of the duodenal bile reflux into the remaining stomach or stump oesophagus o It can also occur late and lead to post- There is some evidence from meta-analysis prandial pain and nausea commonly that the use of a jejunal pouch may improve relieved by vomiting the functional outcome after total gastrectomy o Afferent loop obstruction is due to a When informing patients about major gastric poorly constructed afferent loop, and resection it is important that they are aware usually requires surgical correction that there is a 2–5% associated mortality and a 20–30% incidence of significant Late Complications complications The risk of mortality is related to the volume of Late complications are due to changes in the operations carried out in the surgical unit and anatomy and the physiology of the upper high volume units report mortality rates of 1% gastrointestinal tract or less The complications include o Reflux gastritis and/or oesophagitis 242 Chapter 23 Diseases of the Stomach and Duodenum Due to loss of the pylorus and May result from changes in easy passage of alkaline appetite, from gastritis or biliary and pancreatic fluid into dumping syndromes, or from the stomach recurrence or progression of There is endoscopic evidence the cancer of gastritis in most patients It is normal for patients to lose who have had a loop about 10% of their jejunostomy as a preoperative weight after a reconstruction, but only a total gastrectomy and 5% after small proportion have distal gastrectomy significant symptoms Both osteoporosis and Medical therapy is not very osteomalacia are more effective, and some patients common after gastrectomy require surgery to divert the The reasons for this small bowel fluid from the are not entirely clear stomach via a Roux-en- Y but may result from gastrojejunostomy reduced absorption o Dumping syndromes of calcium and/or Refers to an array of vitamin D gastrointestinal and o Anaemia vasomotor symptoms Common after gastrectomy attributed to rapid gastric May be due to vitamin B12 emptying deficiency from loss of intrinsic The symptoms include factor after total gastrectomy fullness, abdominal pain, and/or poor iron absorption nausea, vomiting and due to failure of conversion of diarrhoea iron from the ferric to the The vasomotor symptoms are ferrous form through the due to rapid fluid shifts into the absence of acid bowel lumen, and are the After total gastrectomy typical symptoms of patients require regular hypovolaemia vitamin B12 injections ‘Late’ dumping is due to an o Most of the problems are most marked insulin surge soon after a in the few months after surgery and meal, followed by reactive most fade within about one year hypoglycaemia The treatment of dumping is Adjuvant therapy dietary Patients should eat small Even with potentially curative surgery with frequent meals, try to separate removal of all visible tumour and clear dry foods from liquids, and resection margins most series report 5-year avoid simple sugars survival between 30 and 40% The severity of symptoms Therefore there is a clear need to look at settles with time whether additional adjuvant therapy can offer o Diarrhoea survival benefit o Nutritional deficiencies 243 Diseases of the Stomach and Duodenum Chapter 23 The literature in this area is not clear but important that soon after the diagnosis is trends are emerging which will guide future made a clear management plan is established research with the palliative care team to maximise the There is some evidence that neo-adjuvant quality of the patients remaining life chemotherapy can downstage gastric cancers although the post-operative survival benefits Gastric Lymphoma are not yet defined Post-operative adjuvant chemotherapy has Lymphoma of the stomach accounts for 2–5% been shown to be of benefit in several of the of all gastric neoplasms randomised controlled trials carried out and a In general they are of B cell origin although T meta-analysis of all trials to date has shown a cell lymphomas do occur significant benefit in favour of chemotherapy The commonest gastric lymphoma arises However despite the meta-analysis because from mucosa associated lymphoid tissue of the very heterogeneous nature of the trials (MALT) rather than lymph nodes included in the analysis such treatment is not o The occurrence of MALT in the usual in the majority of centres stomach is thought to be a response to A recent large multicentre study looked at the chronic inflammation most frequently use of post-operative chemoradiotherapy occurring as a result of H. pylori following surgery for gastric cancer and infection showed a significant survival benefit o There is no longer any doubt that H. Whilst further studies are needed it does pylori is a very important cause of appear that adjuvant therapy offers some gastric lymphoma benefit for patients with gastric cancer and B cell lymphomas of the stomach can be such treatments should be considered classified as either low grade or high grade depending upon their histological Palliation characteristics Following diagnosis patients with gastric Unfortunately the majority of patients with lymphoma should be appropriately staged gastric cancer either present with advanced with a combination of CT scan and disease or develop recurrence following endoscopic ultrasound surgery, therefore palliation is very important The treatment of low grade MALT lymphoma Whilst not universally accepted there seems is eradication of Helicobacter infection if to be little benefit at all for patients with present advanced gastric cancer undergoing surgery This results in tumour resolution in 70–100% where all of the detectable tumour is not of cases removed Treatment of those patients who do not The overall survival in such patients is not respond or those with high grade tumours is different from those who do not undergo with chemoradiotherapy resection Surgery is generally reserved for the One of the commoner indications for palliative treatment of tumour or treatment related surgery is gastric outlet obstruction from a complications such as haemorrhage or stenosing distal gastric cancer and even in bleeding this situation the use of expandable metal mesh stents can offer better palliation in a Gastric GIST significant proportion The median survival in patients with non- Gastrointestinal stromal tumours were until curable gastric cancer is 4–6 months and it is the last decade described as leiomyomas or leiomyosarcomas and it is only with advances 244 Chapter 23 Diseases of the Stomach and Duodenum in molecular biology that this particular tumour has been recognised as a discrete entity GI stromal tumours can arise anywhere in the GI tract but the stomach is the commonest site They are mesenchymal tumours believed to originate from the interstitial cells of Cajal They generally present as elevated submucosal swellings that ulcerate or bleed; a number are discovered as incidental findings at endoscopy GIST's behave in a somewhat unpredictable manner with many appearing to be completely benign and others as an aggressive malignancy Increased risk of malignant behaviour is associated with increasing size and the number of mitoses seen within pathological specimens GIST tend to spread through local invasion and haematogenous spread, lymph node metastases are unusual Once diagnosed and staged through a combination of CT scanning and if available endoscopic ultrasound treatment is surgical resection There is no role for associated lymph node dissection At a molecular level GIST's are characterised by an abnormality of one of several transmembrane growth factor receptors, the commonest (85–90%) being the molecule c- kit (CD117) Recently it has been demonstrated that the c- kit antagonist imatinib can produce very significant tumour regression of advanced GIST's. There is no current data to support the use of imatinib in the adjuvant setting 245 Chapter 26 Diseases of the Appendix CHAPTER DISEASES OF THE APPENDIX By Dr. E Brits Revised January 2020 INTRODUCTION Differential Diagnosis Anatomy Management Possible Function Approach Diseases Preoperative APPENDICITIS Operative Epidemiology Postoperative Pathogenesis Interval Appendicectomy Work-up and Diagnosis Outcomes History (Symptoms) Mortality Physical Examination (Signs) Morbidity Laboratory and Side Room Investigations Recurrent Appendicitis Alvarado Score Stump Appendicitis Imaging APPENDIX NEOPLASMS The appendix is a true diverticulum of the cecum INTRODUCTION containing all the layers of the colonic wall in contrast to Appendicitis is one of the most common causes of an acquired diverticular disease, which consists of a acute abdomen and most frequent indications for an protuberance of a subset of the enteric wall layers. emergency abdominal surgical procedure worldwide. Possible Function Anatomy The appendix is a vestigial structure that lost all or most The appendix is located in the right lower quadrant (RLQ) of its original function through the process of evolution. of the abdomen at McBurney’s point (1/3rd of the distance laterally on a line drawn from the umbilicus to the right The appendix has a possible immunological function. The anterior superior iliac spine (ASIS)). In the case of presence of B and T lymphoid cells in the mucosa and intestinal malrotation or situs inversus the appendix can submucosa of the lamina propria make the appendix be found at various anatomical positions and may histologically distinct form the cecum. These cells are part complicate the diagnosis. of the gut-associated lymphoid tissue system (GALT). Lymphoid hyperplasia can cause obstruction of the The appendix attachment is at the base of the cecum, appendix lumen near its base and lead to appendicitis. near the ileocecal valve, where the taenia coli converge. The lymphoid tissue undergoes atrophy with age. The tip of the appendix can vary from being retro-caecal, sub-caecal, pre-ileal, post-ileal, or pelvic positions. The The appendix provide a “safe house” for intestinal average length is 11cm (range 2 – 20cm) and the bacteria/flora. It is proposed that it serves as a haven for diameter is usually 7 – 8 mm. the bacteria when illness flushes it from the intestines e.g. diarrhoea. The blood supply is from the appendiceal artery (a Diseases terminal branch of the ileocolic artery) which traverse the mesoappendix and terminates at the tip of the appendix. The most common diseases of the appendix are appendicitis and carcinoid tumours. Appendix cancer 261 Diseases of the Appendix Chapter 26 accounts for 1 in 200 of all gastro-intestinal (GIT) Work-up and Diagnosis malignancies. The goal is to identify patients as early in their clinical APPENDICITIS course as possible, while minimizing the negative or non- therapeutic laparoscopy/laparotomy rates. Missed Epidemiology diagnosis of appendicitis (especially when perforated), can result in adverse patient outcomes, while negative or Appendicitis is inflammation of the appendix. It is common non-therapeutic operations incur surgical morbidity and seen in up to 1 in 10 individuals over a lifetime. Most without treating the underlying condition. cases present between ages 10 and 30 years. There is a slight male preponderance (male: female ration of 3:2). Evaluation for appendicitis can be particularly challenging in several populations, including: Pathogenesis Women of reproductive age: o Gynaecologic pathologies can mimic Appendiceal luminal obstruction is proposed to be the appendicitis clinically (e.g. pelvic primary cause of appendicitis. This can be caused by inflammatory disease, ovarian torsion, numerous objects e.g. fecaliths, lymphoid hyperplasia, ectopic pregnancy etc.) benign or malignant tumours, seeds, worms, foreign bodies etc. The elderly, the young, immunosuppressed, multiple comorbidities: The obstruction leads to build up of mucous and the o These patients present with non- appendix distends. There is an increase in luminal and classical or non-specific clinical intramural pressure, causing thrombosis and occlusion of features and often late in the clinical the small vessels in the appendiceal wall and stasis of course of the disease. lymphatic flow. This engorgement of the appendix stimulates visceral afferent nerves at spinal level T8-T10, History (Symptoms) causing vague peri-umbilical abdominal pain. The cause of luminal obstruction varies depending upon the patient’s The clinical presentation of acute appendicitis has the age. In the young lymphoid hyperplasia and in the elderly following classic symptoms: a neoplasm may be the cause. Peri-umbilical abdominal pain “migrating” to the right iliac fossa (RIF) area. Bacterial overgrowth occurs within the appendix. This is a Anorexia mixed infection with aerobic (gram + and gram -) and Nausea and vomiting anaerobic organisms. Intra-luminal bacteria invade the appendiceal wall and further propagate a neutrophilic Fever generally occur later in the course of the illness. exudate causing fibro-purulent reaction on the serosal surface, irritating the surrounding parietal peritoneum. If the patients present early, the initial features of acute This infection and inflammation results in stimulation of appendicitis are atypical or nonspecific/subtle and can somatic nerves of the adjacent parietal peritoneum include: causing localized tenderness. Indigestion Flatulence The wall of the appendix becomes ischemic and then Bowel irregularity necrotic as vascular and lymphatic compromise Diarrhoea progresses. The appendix is at risk of perforation, which Generalized malaise can lead to localized abscess formation or diffuse peritonitis. 262 Chapter 26 Diseases of the Appendix Symptoms of acute appendicitis may vary depending o Extending the hip causes pain because upon the location of the tip of the appendix: it stretches out these muscles, while An anterior appendix produces marked localized flexing the hip activates them. pain in the RIF. Obturator sign A retrocecal appendix may cause a dull o If an inflamed appendix is in contact abdominal ache. with the right obturator internus muscle, An appendix tip in the pelvis may cause spasm of the muscle can be tenderness below McBurney’s point with urinary demonstrated by flexing and internal frequency, dysuria, tenesmus or diarrhoea. rotation of the hip, which elicits RLQ abdominal pain. Physical Examination (Signs) Blumberg sign o Also known as rebound tenderness, Early signs of appendicitis: indicating peritonitis It is often very subtle. o Pain is elicited with palpation over the Low-grade fever may be present. inflamed peritoneal area and sudden Abdominal examination may be unrevealing release of the pressure. because the visceral organs does not have somatic pain fibres. Laboratory and Side Room Investigations Later signs of appendicitis: Full blood count (FBC) Inflammation of the overlying parietal peritoneum o A mild leucocytosis with a left shift is causes localized tenderness in the RIF present most of the time (slightly raised o There may be involuntary guarding white blood cell count (WBC >10 x (rigidity) of the abdomen 109/L) with bands (immature In the case of a retro-caecal or pelvic appendix, neutrophils) and neutrophils) tenderness with rectal exam or right adnexal o In very early appendicitis the WBC tenderness with female pelvic exam may be count may be normal present with less severe abdominal tenderness. C-reactive protein (CRP) o This acute phase response protein No physical finding taken alone, definitively confirms a raises in response to any infection or diagnosis of appendicitis. Commonly described physical inflammation in the body signs include: Pregnancy test Rovsing’s sign o Pregnancy need to be excluded in all o Palpation in the left iliac fossa upwards females of childbearing age (counter clockwise along the colon) Urine dipsticks causes pain in the RIF o Lower urinary tract symptoms (LUTS) o This is due to bowel content being may be present due to irritation of the pushed towards the ileo-caecal valve bladder caused by appendicitis with and thus increasing pressure around resultant leukocytes in the urine the appendix. o Look for nitrates in the urine to Psoas sign diagnose a urinary tract infection (UTI) o RLQ pain produced with passive extension of the patient’s right hip (lying No laboratory test can safely and sufficiently confirm or on the left side) or by active flexion of exclude the suspected diagnosis of acute appendicitis. the right hip (lying supine) by the patient o The pain is due to inflammation of the peritoneum overlying the ilio-psoas muscles 263 Diseases of the Appendix Chapter 26 Alvarado score o CT without IV contrast is acceptable when IV contrast is contra-indicated. The Alvarado score is a clinical (signs and symptoms) and The use of oral or rectal contrast is laboratory based scoring system used to assist in preferable. diagnosing appendicitis. A low Alvarado score (6mm) Table 1: The Alvarado Score Appendiceal wall thickening Points (>2mm) Symptoms Peri-appendiceal fat stranding Migratory RIF pain 1 Appendiceal wall Anorexia 1 enhancement with IV contrast Nausea or vomiting 1 Appendicolith Signs Tenderness in RIF 2 Ultrasound Rebound tenderness in RIF 1 US is the preferred imaging exam in children and Fever (>37.5oC) 1 pregnant women Biochemistry In other populations, US is an alternative to CT if Leucocytosis (WBC >10 x 109/L) 2 the latter is not available Total score 9 The ability of US to diagnose appendicitis has a sensitivity of 85% and specificity of 90% The rate of non-diagnostic exams are high, with Imaging 50 – 85% of normal appendices not visualized Selection of modality / imaging approach Imaging features of appendicitis on US include: o Imaging is used to increase the o Non-compressible appendix with a specificity of the diagnostic evaluation cross sectional diameter >6mm for acute appendicitis and decrease the o Focal pain over the appendix with negative appendectomy rate. compression using the US probe o Computed tomography (CT) is the o Increased echogenicity of inflamed preferred modality, with ultrasound peri-appendiceal fat (US) and magnetic resonance imaging o Fluid in the right lower quadrant (MRI) reserved for radiosensitive o Appendicolith pregnant women and children. Magnetic Resonance Imaging Computed tomography o Abdominopelvic CT with intravenous MRI should be used in pregnant woman and (IV) contrast is recommended older children who can cooperate with the exam 264 Chapter 26 Diseases of the Appendix MRI with IV contrast has the diagnostic accuracy Elderly comparable to CT and is better than US Diverticulitis Mesenteric ischemia It has a sensitivity of 95% and specificity of 92% Intestinal obstruction Leaking aortic aneurysm to diagnose appendicitis Colonic carcinoma The rate of non-diagnostic exams is higher than with CT but lower than with US, with 20 – 40% of Adults normal appendices not visualized Inflammatory bowel Enteritis disease – new onset Plain Radiography Pancreatitis Renal colic Plain abdominal X-ray (AXR) is not Perforated peptic ulcer Testicular torsion recommended in the work-up of suspected appendicitis due to its limited diagnostic value Management Imaging features that might be present with acute appendicitis are: Approach o Fecalith (pathognomonic) o Loss of psoas muscle shadow on the Uncomplicated- /Non-perforated/Early/Simple right side Appendicitis o Scoliosis to the right side o Air fluid levels in RLQ area This includes erythematous- (grade 1), exudative- (grade 2) and purulent- (grade 3) Differential Diagnosis appendicitis. Timely appendectomy (open or laparoscopic) is Differential diagnosis of RLQ abdominal pain includes recommended as the treatment of choice for inflammatory disease processes, infectious diseases and adult patients. obstetrical conditions all specific to age and gender of the Antibiotics and supportive care only (non- patient. Examples of differential diagnosis according to operative treatment) are an option for those who age and gender: are unfit for surgery or refuse surgery. o Rescue appendectomy is needed with Children clinical deterioration of the patient. Gastroenteritis Urinary tract infection o If a fecalith is present on imaging, non- Mesenteric adenitis Lobar pneumonia operative treatment is not Intussusception Pancreatitis recommended. Distal intestinal o There is a potential recurrence rate of Typhlitis – Leukaemia obstruction syndrome appendicitis of 15 – 25% depending on Meckel’s diverticulitis Trauma the length of follow-up. Inflammatory bowel Henoch-Schönlein o In high risk patients (the elderly, disease – new onset purpura immunocompromised, multiple co- morbidities) the severity of disease may Young women be underestimated. Menarche Mittelschmerz o The risk of a missed appendiceal Pelvic inflammatory neoplasm may be higher. Although this Dysmenorrhea group could potentially benefit from a disease Severe menstrual cramps Ectopic pregnancy non-operative treatment approach the Torsion/rupture of ovarian efficacy thereof is still unknown. Endometriosis cyst 265 Diseases of the Appendix Chapter 26 Complicated/Perforated Appendicitis/Advanced Immediate appendectomy is appendicitis not advised Serial clinical examinations This includes necrotic appendicitis (grade 4), and/or repeat imaging is appendix abscess and appendix