ESOPHAGUS AND DIAPHRAGM
Q. Describe the methods to prevent recurrent variceal hemorrhage in a patient of esophageal varices
y Infection
y Intraperitoneal bleeding y Subcapsular hematoma y Hemobilia
y Mortality (30 day) 3 to 13%
y Stent stenosis (more common than thrombosis and is due to neointimal hyperplasia) and thrombosis (33 to 73% patients by the end of 1 year)
Emergency Surgery Indications
y Refractory variceal bleeding
y Inability to control bleeding by endoscopy
y Hemodynamically unstable patient with bleeding esophageal varices y Two attempts at endoscopic control followed by rebleeding
y Complications of endoscopy (perforation)
y Hemorrhage from gastric varices or portal hypertensive gastropathy y Failure of TIPS placement
Options
y Esophageal transection with a stapling device
y Splenectomy, portoazygos disconnection and stapling of the oesophagus (Suigura procedure)
y Portacaval shunt
y Distal splenorenal shunt in stable patients
Q. Describe the methods to prevent recurrent variceal hemorrhage in a patient
Surgical therapy of portal hypertension (include the emergency surgery role from previous question to complete this answer)
Shunt surgeries
(Remember: For splenic vein, proximal is near SMV-PV confluence and distal is near the spleen. Therefore, spleen is removed in PSRS and never in DSRS. Infact, DSRS is contraindicated in patients with prior splenectomy)
Nonselective
(Completely divert portal flow) Selective Partial
End to side portocaval shunt (Eck
fistula) Distal splenorenal shunt
(Warren) When the nonselective
shunt diameter < 10 mm Side to side portocaval shunt Coronary – caval shunt
(Inokuchi) Interposition graft [portocaval,
mesocaval (Drapanas shunt), mesorenal]
Mesenteric left portal bypass (Rex shunt) Proximal splenorenal shunt (linton
shunt)
Nonselective Shunts
y These procedures decompress the splanchnic venous circulation and intrahepatic sinusoidal network
y Side to side nonselective shunts are the most effective shunt procedures for relieving ascites as well as preventing recurrent variceal bleeding
y Both side-to-side and end to side shunts accelerate hepatic failure and lead to frequent postshunt encephalopathy
y Proximal splenorenal shunt consists of anastomosis of the proximal splenic vein (proximal splenic vein means near the superior mesenteric vein) to the renal vein.
Splenectomy is also performed
y PSRS is slightly less encephalopathic than the other nonselective shunts.
Fig. 2: DSRS and PSRS
Selective Shunts
The Distal splenorenal shunt
y Anastomosis of the distal end of the splenic vein (near the spleen) to the left renal vein with interruption of all collateral vessels (e.g. coronary vein and gastroepiploic veins), which connect the superior mesenteric vein and gastrosplenic components of the splanchnic venous circulation
y Results in separation of the portal venous circulation into a decompressed gastrosplenic venous circuit and high-pressure superior mesenteric venous system that continues to perfuse the liver
y A splenic vein diameter less than 7 mm is a relative contraindication to the procedure because of the high incidence of shunt thrombosis in these patients
y No survival benefit over nonselective shunts but a better quality of life in DSRS y Lower incidence of encephalopathy.
Hepatic Transplantation
y The only treatment for end-stage hepatic failure
y The only treatment of underlying disease in cases with variceal bleeding and portal hypertension
y Hepatic transplantation should be considered when other complications of cirrhosis develop or hepatic functional decompensation is evident clinically or by careful assessment with quantitative LFTs.
The most common cause of death in medically treated esophageal varices patients is rebleeding.
The most common cause of death in shunted esophageal varices patients is accelerated hepatic failure.
Q3. Write a note on achalasia cardia.
Describe the management of achalasia cardia.
Enumerate the differential diagnoses of achalasia cardia.
Differentiate between achalasia and pseudoachalasia.
Ans. Introduction
y It is taken from a Greek word. It means ‘failure to relax’. It is basically the failure of lower esophageal sphincter to relax during the deglutition phases.
Causes
y Syndromic association with allgrove’s disease (alacrima, achalasia, ACTH resistant adrenal insufficiency)
y Destruction of the nerves in Auerbach’s plexus to the LES is the primary abnormality.
y Neuromuscular degeneration in the body of the esophagus also occurs.
y Thus, finally both the nerves and muscles are affected.
Pathophysiology
y Selective loss of neurons in the Auerbach’s plexus that secrete NO and VIP, the inhibitory gut hormones lead to increased tone of LES and failure to relax LES.
y Also causes aperistalsis of the body of esophagus and resultant dilatation of esophagus which when advanced present as sigmoid esophagus.
Clinical features
y Men and women are equally affected
y Triad – dysphagia + weight loss + regurgitation y Dysphagia is more to liquids and progresses slowly.
y Regurgitation of undigested, foul smelling food can lead to aspiration pneumonitis, lung abscess.
Investigations
y Manometry is the gold standard and shows elevated LES pressure and incomplete LES relaxation with absence of progressive peristalsis in the body of the esophagus and elevated intraesophageal pressure. Simultaneous low amplitude nonperistaltic contractions may be seen in the body.
y Barium swallow findings
– Bird beak/ pencil tip/rat tail appearance – Sigmoid esophagus/megaesophagus
– Absence of gastric air bubble in upright posture.
Differential diagnoses
y Vigorous achalasia - Achalasia with relatively high esophageal contraction amplitudes (This means that there is activity in the body of esophagus but, this is largerly ineffective spasm rather than the true co-ordinated peristalsis), often with minimal esophageal dilation and prominent tertiary contractions on radiographs and with the presence of chest pain.
y Pseudoachalasia - Distal esophageal obstruction by tumor, stricture, or surgical manipulation will result in the same functional characteristics as achalasia i.e. narrowing of the distal esophagus with aperistalsis and dilation proximal to the narrowing and has been labeled as secondary achalasia or pseudoachalasia.
Tumors causing pseudoachalasia include: Adenocarcinoma of gastric fundus (m.c.), oat cell carcinoma of lung, squamous cell cancer esophagus, lymphoma, prostatic carcinoma metastasis and pancreatic adenocarcinoma.
Achalasia Pseudoachalasia Uniform age distribution between
10–70 years More common after 3rd decade with increased incidence as age increases
In double contrast barium swallow, no morphologic abnormalities are present in classic achalasia
Morphologic abnormalities (stricture) will be present
Amyl nitrate inhalation will cause
relaxation of the narrowed part Amyl nitrate inhalation will not cause relaxation of the narrowed part
Flexible endoscope can be traversed
through LES easily without resistance Resistance is encountered when flexible endoscope is traversed through the lesion. Also, biopsy can be taken to clinch the diagnoses
Pneumatic dilatation is effective Pneumatic dilatation is ineffective and dangerous and also delays the actual treatment due to false diagnoses.
Treatment
y Medical management—tried with sublingual nitroglycerine/ nitrates/ calcium channel blockers
y Botulinium toxin injection into LES has also been tried but has a high recurrence rate of 50% within a 6 month post treatment period.
y Procedure of choice—laparoscopic Heller’s myotomy with partial Toupet or Dor fundoplication.
y Indications of esophagectomy—sigmoid esophagus, failure of 2 or more myotomy, undilatable stricture esophagus.
y Newer modality is peroral endoscopic myotomy (POEM) which is done under GA using an endoscope with a small 1 cm mucosal incision.
– It was first done by Parischa
– Indications for POEM as of now—post Heller myotomy failure and sigmoid esophagus
– After the mucosal incision, a submucosal tunnel is created using methylene blue/
indigo carmine for 10 cm on esophageal side and 5 cm on gastric side – The myotomy starts 3 cm below the mucosal incision
– Once the procedure is done, the mucosal incision is closed.
Results and prognosis
y Myotomy has a 70% success rate in reliving the symptoms
y Long standing achalasia is a risk factor for squamous carcinoma esophagus.
Q4. Describe clinical features, types and management of tracheoesophageal fistula.
Write a note on esophageal atresia.
(Important: Always Remember: Tracheoesophageal fistula is congenital as well as acquired.
If SN does not mention either specifically, write both... gives credit marks!!!) Ans. Esophageal atresia and tracheoesophageal fistula
A tracheoesophageal fistula (TEF) is an epithelialized tract between the esophagus and the trachea.
Embryological basis
y The division of foregut is the result of fusion of invaginating lateral longitudinal ridges that created a septum dividing the foregut into a dorsal digestive tract and ventral respiratory system
y The formation of tracheoesophageal septum is believed to begin caudally and end cranially y Most likely origin of TOF is heterogeneous and multifactorial and involves multiple genes
and complex gene-environment interactions.
Theories of EA and TEF
y Esophageal occlusion and failure of recanalization y Spontaneous deviation of tracheoesophageal septum y Abnormal migration of tracheoesophageal septum y Teratogen: doxorubicin
“VOGT classification”
y Type 1: Absent esophagus.
y Type 2: EA without TEF (TYPE A – 8%)
y Type 3: EA with fistula:
– a: EA with proximal TEF (TYPE B – 1-2%) – b: EA with distal TEF (m.c.) (TYPE C – 84%) – c: EA with proximal and distal TEF (TYPE D – 4%) y Type 4: Isolated TEF with intact esophagus (TYPE E – 3%)
A B C D
Fig. 3: Tracheoesophageal fistula
Associations (incidence – 50–70%) m.c. - Cardiovascular 35%
y Vacterl association: 20%
– Vertebral: 17%
– Anal 12%
– Cardiac 20% (m.c. VSD) – Renal 16%
– Limb 5%
y Syndrome association – DiGeorge sequence – Polysplenia sequence – Holt-Oram syndrome – Peirre Robin sequence – Feingold syndrome – Fanconi syndrome.
Prenatal detection
y USG - Anechoic area in the middle of the neck, small or absent stomach bubble and associated maternal polyhydroamnios, predictive value 20 to 40%
y Fetal MRI may be a useful adjunct for diagnosis.
Postnatal
y Earliest sign is excessive salivation, typically first feeding is followed by regurgitation, choking and coughing
y Cyanosis with or with out feeding, respiratory distress, inability to swallow and inability to pass a feeding tube in the stomach
y If distal fistula present the abdomen distends.
Investigations
y Plain chest X-ray with a nasogastric tube: Coiled at upper pouch.
y A few ml of air can be injected through the tube and used as contrast to distend upper pouch as frontal and lateral films taken.
y If necessary 0.5 to 1 ml of diluted barium can be used, if barium used fluoroscopy is advisable to detect proximal fistula. Air in the stomach and bowel confirm distal TEF, absence of air typically represent isolated TEF.
y Additional testing:
– Echocardiography – Renal ultrasound – Chromosomal analysis Preoperative treatment
y NPO and IVF 10% D and hypotonic saline
y Start broad spectrum antibiotic and chest physiotherapy
y A sump catheter/double lumen catheter for continuous low pressure negative suction y Positioning of infants to prevent reflux: Upright sitting/head-up prone position y Vitamin K analogue should be given.
Routine ET intubation with elective positive pressure ventilation and elective gastrostomy are to be avoided.
Surgery Type of Surgery
y Thoracotomy: Extra-pleural/trans-pleural y VATS (Video assisted thoracoscopic surgery)
Approach
Right sided in case of left aortic arch and left sided in case of right aortic arch Techniques
y Primary closure (single layer increases leaks and double layer increase stricture rates.), if adequate length of esophagus is available
y In case length is not adequate to allow a tension free anastomosis – Circular/spiral esophagomyotomy
– Mobilization of upper pouch and anastomosis
– Traction sutures and delayed anastomosis after tacking the distal pouch to prevertebral fascia to allow lengthening
– Cervical esophagostomy with gastrostomy and gastric pull up or colon interposition at an age of 1 year.
Complication y Early:
– Anastomotic leak – Anastomotic stricture – Recurrent TEF y Late:
– Gastroesophageal reflux – Tracheomalacia
– Disordered esophageal peristalsis
A word about acquired tracheoesophageal fistula:
Causes
y Intubation and tracheostomy tube cuff-related tracheal injury y Malignant tumors of esophagus and trachea
y Blunt and penetrating trauma, radiation, surgery and caustic ingestion y Infections, steroid use, hypotension and diabetes are all associated risk factors.
Persistent coughing with meals, frequent respiratory infections including pneumonia are common with larger fistulas.
Investigations include endoscopy, bronchoscopy, and barium esophagogram.
Management
y Initial: IV antibiotics, gastrostomy for feeding and esophageal stent to decrease contamination of lungs.
y Surgery: Three main steps – Step 1: Exposure of fistula tract
– Step 2: Excision of the involved trachea and primary repair of esophagus
– Step 3: Muscle flap interposition between trachea and esophagus to prevent recurrence and primary trachea repair
Q5. Write a note on Barrett’s esophagus.
Discuss management of gastroesophageal reflux disease.
Ans. Gastroesophageal reflux disease and Barrett’s esophagus
It is a disease characterized by classic triad of epigastric pain, retrosternal pain and regurgitation.
Pathophysiology
y Lower esophageal sphincter (LES) Mainly formed by
– Intrinsic musculature of esophagus – Sling fibers of cardia
– Crura of diaphragm
– Intra-abdominal length of esophagus y Normal values at LES
– Total length—> 2 to 5 cm – Intra-abdominal length—>1cm
– Resting pressure—> 6 mm Hg to 26 mm Hg – Acid reflux episodes—4–7%
y Defective LES – Total length <2 cm
– Intra-abdominal length <1cm – Resting pressure < 6 mm Hg
– Acid reflux episodes—12% in esophagitis and upto 26% in Barrett’s esophagus Chain of progression
GERD – Intestinal type metaplasia (Barrett’s esophagus) low grade dysplasia, high grade dysplasia, 40 times increased risk of adenocarcinoma in this 10% population (incidence of Barrett’s)
Clinical features
y Classic triad of symptoms or any combination of it can be seen.
y Occasional patient may present with hoarseness and change in voice and in severe cases, complete loss of voice due to severe reflux induced laryngeal edema and laryngitis. These symptoms when mild characteristically show diurnal variation with symptoms more in morning and decreased by evening.
Diagnoses
y UGI endoscopy and biopsy of suspicious areas
y Endoscopic ultrasound and biopsy, chromoendoscopy (indigo carmine, methylene blue, toluidine blue), laser induced endoscopy (5-aminolevulinate), optical coherence tomography can also be used
y Manometry
y 24 hour pH monitoring (Gold Standard)
– Document number of reflux episodes (pH < 4) – Longest reflux episode
– Number of reflux episodes > 5 minutes – Extent of reflux
y Demeester score (N < 14.7)
Management GERD
General measures
y Lifestyle modifications
y Cessation of smoking and alcohol
y Avoid large, fatty meals at sleep time atleast before 2 hrs
y Medical management include double dose proton pump inhibitors.
Indications of surgery
y Failure of medical therapy
y Complicated GERD (Barrett’s, peptic stricture, esophagitis, ulcer) y Persistent symptoms at a young age.
Indications of primary surgery in GERD (contraindications to medical management) y Supine reflux/bile reflux
y Structurally defective sphincter y Erosive esophagitis
y Poor esophageal contractility.
Procedures
y Allison procedure—anatomical repair
y Angelchick prosthesis—horseshoe shaped prosthesis at GE junction y Transthoracic procedures—Nissen, belsey, collis gastroplasty Types of fundoplication
y Watson (90°anterior) y Toupet (180°anterior)
y Dor (180°posterior, now 270°posterior) y Belsey mark 4 (270°anterior)
y Nissen (360°)
y Rosette and hill modification of nissen (360°using only the anterior stomach) y Laparoscopic Nissen (Gold Standard)
Long term failure rate—25%
Indications for a partial fundoplication in GERD
When peristalsis is absent or severely disordered or when amplitude < 20 mmhg, 2/3 fundoplication should be done.
Indications of esophageal resection
y Global absence of contractility with short esophagus y Several failed antireflux procedures
y >50% interrupted or dropped contractions with short esophagus Management of Barrett’s esophagus
y Columnar epithelium lining distal esophagus y Biopsy showing columnar metaplasia
Intestinal metaplasia Endoscopy and biopsy every 2 years (This is a 4 quadrant biopsy at 2 cm intervals starting 1 cm from GE junction) Low grade dysplasia Endoscopy and biopsy every 6 months (This is a 4 quadrant
biopsy at 2 cm intervals starting 1 cm from GE junction) High grade dysplasia Endoscopic mucosal resection > esophagectomy
Other options – photodynamic therapy, thermal ablation, electrocoagulation
Q6. Describe the clinical features and management of a patient with zenker’s diverticulum.
Classify esophageal diverticula and describe zenker’s diverticulum.
Ans. Esophageal diverticula
The three most common sites of occurrence are:
y Pharyngoesophageal (zenker’s) y Parabronchial (midesophageal) y Epiphrenic (supradiaphragmatic)
Classifications
1. Congenital and acquired 2. True
False Contain all layers
Only mucosa and submucosa 3. Pulsion
Traction
Occur because of elevated intraluminal pressure and are false For example – Zenker/Epiphrenic
External inflammatory mediastinal lymph nodes adhering to esophagus
Pharyngoesophageal (Zenker’s) diverticulum y Also called cricopharyngeal achalasia y Most common of all esophageal diverticula
y Found herniating into killian’s triangle, between the oblique fibers of the thyropharyngeus muscle and the horizontal fibers of the cricopharyngeus muscle
y As the diverticulum enlarges, the mucosal and submucosal layers dissect down the left side of the esophagus into the superior mediastinum posteriorly along the prevertebral space.
Clinical features
y Older patients, seventh decade of life y Initially asymptomatic
y Chronic cough, excessive salivation and intermittent dysphagia y Regurgitation of foul-smelling, undigested material
y Voice changes, retrosternal pain and respiratory infections
y The most serious complication from an untreated Zenker’s diverticulum is aspiration pneumonia or lung abscess.
Diagnosis
y Barium esophagraphy (lateral views)
y At the level of the cricothyroid cartilage, the diverticulum can be seen filled with barium resting posteriorly alongside the esophagus.
Treatment
y Endoscopic or open surgical Dohlmans procedure.
y Small (<2 cm diverticulum) – myotomy (thyropharyngeus and cricopharyngeus muscles) alone
y In frail patients—Diverticulopexy without resection with myotomy
y Large sac (>5 cm)—excision of the sac (Diverticulectomy) with myotomy is indicated or Endoscopic division of the common wall between the esophagus and diverticulum using a laser or stapler has also been successful. The endoscopic technique has gained favor and is advocated for patients with diverticula between 2 and 5 cm
y For diverticula 3 cm or less in size, surgical repair is superior to endoscopic repair in eliminating symptoms
y For any diverticulum larger than 3 cm, the results are the same.
Midesophageal traction diverticula
y Inflamed mediastinal lymph nodes from an infection with tuberculosis, histoplasmosis and resultant fibrosing mediastinitis exerts traction on the wall of the esophagus and leads to the formation of a true diverticulum in the midesophagus
y Some may also be caused by a primary motility disorder, such as achalasia, diffuse esophageal spasm (DES), or nonspecific esophageal motility (NEM) disorder
y Symptomatic / >2 cm size – diverticulopexy with a long esophagomyotomy if patient has a concomitant motility disorder.
Epiphrenic Diverticula
y Found adjacent to the diaphragm in the distal third of the esophagus, within 10 cm of the GEJ
y Pulsion, or false, diverticula that are often associated with DES, achalasia y More common on the right side and tend to be wide-mouthed
y Similar to that of a midesophageal diverticulum
y If a large hiatal hernia is also present, the diverticulum is excised, a myotomy performed, and the hiatal hernia repaired. Failure to repair the hernia results in a high incidence of postoperative reflux.
Q7. Discuss the management of esophageal perforation.
Enumerate the causes of esophageal perforation and discuss its treatment.
Ans. Esophageal perforation Causes
y Most common cause – Iatrogenic perforation (endoscopic instrumentation for a diagnostic or therapeutic procedure, endoscopic dilation, stent placement, difficult endotracheal intubation, blind insertion of a minitracheostomy and injury during dissections in the neck, chest, and abdomen)
y Boerhaave’s syndrome (Postemetic rupture of the esophagus. Other predisposing factors include blunt thoracic trauma, epileptic seizures, defecation and childbirth)
y Foreign body ingestion y Trauma
Most common site of perforation is left lateral posterior into mediastinum or just above GE junction
Symptoms and signs
y Neck, substernal or epigastric pain y Vomiting, hematemesis
y Cervical perforations may present with neck ache and stiffness
y Thoracic perforations present with shortness of breath and retrosternal chest pain lateralizing to the side of perforation
y Abdominal perforations present with epigastric pain that radiates to the back if the perforation is posterior
y Hemodynamic instability and shock y Subcutaneous air in the neck or chest y Shallow decreased breath sounds Diagnoses
y Elevated white blood cell count and an elevated salivary amylase level in the blood or pleural fluid
y Chest roentgenogram may demonstrate a hydropneumothorax
y Contrast esophagography with a film in the left lateral decubitus position is performed using barium for a suspected thoracic perforation and Gastrografin for an abdominal perforation.
(IMP : water soluble nonionic contrast media (iopamidol) is used in thoracic perforation which is inert and nontoxic to thorax. If no leak here, barium is done to confirm. In abdominal perforations, barium is contraindicated as it causes barium peritonitis. Here, ionic water soluble contrast media like gastrograffin is used whenever perforation is suspected in abdomen. This is not used in thorax as in thorax it can cause chemical pneumonitis and mediastinitis. Whereas barium even if aspirated causes only inert granulomas and is therefore safe to use in thorax. The same ionic contrast media is used in cases of meconium syndromes) y Chest CT scan—mediastinal air and fluid at the site of perforation
y Surgical endoscopy—if the esophagram is negative or if operative intervention is planned. Mucosal injury is suggested if blood, mucosal hematoma or a flap is seen or if the esophagus is difficult to insufflate.