Approach to Esophageal Swallowing Disorder
Dysphagia, odynophagia, retrosternal chest pain, regurgitation, and heartburn are the major symptoms of esophageal disorders. In oropharyngeal dysphagia, there is inability to initiate swallow, nasopharyngeal regurgitation, coughing or choking on attempted eating, or aspiration. Esophageal disorders can be classified as obstructive and nonobstructive etiology. Esophageal diameter less than 15 mm will lead to obstructive disorder and present with dysphagia to solids more in comparison to liquids. Nonobstructive disorders especially motor disorder present with dysphagia to both solids and liquids. Odynophagia, food impaction, or chest pain can also be seen. Dysphagia can be acute as in case of food impaction, pill, and infective esophagitis. Intermittent dysphagia is seen in rings and webs, while progressive dysphagia is seen in malignancy and strictures.
Zenker’s Diverticulum
Zenker’s diverticulum is a pulsion diverticulum in the hypopharynx. It is an outpouching of the mucosa due to a muscular weakness between cricopharyngeus and inferior pharyngeal constrictor.
There are various hypotheses which may be responsible for developing a Zenker’s diverticulum, upper esophageal sphincter [UES] dysfunction-incomplete relaxation, motility disorders of the esophagus, or gastroesophageal reflux disease causing esophageal or UES dysmotility.
This diagnosis is suspected on the history and confirmed on barium examination and upper gastrointestinal endoscopy. The barium shows features of pouch which retains the barium. The neck of the pouch is above the cricopharyngeal sphincter. On upper gastrointestinal endoscopy, the diverticulum can be visualized. The presence of carcinoma in situ or squamous cell carcinoma needs to be excluded. One must be careful and push the endoscope blindly to prevent perforation of the diverticulum.
Esophageal manometry is not required in all cases, but it may help underline an existing esophageal motility disorder like esophageal spasm or achalasia cardia or ineffective esophageal motility.
Flexible endoscopic treatment of diverticulum involves dividing the bridge between the diverticulum and the esophagus. This can be done by argon plasma coagulation, needle knife, or a monopolar forceps. The use of Zenker’s diverticuloscope or the hood or the cap over the endoscope has improved the view of the septum during the procedure and protection of the wall. A nasogastric tube in the esophagus provides better exposure of the septum and protects the anterior esophageal wall. The needle knife is used to cut from the septum from the midline towards the inferior part of the diverticulum. The direction of the cut can be from inside the diverticulum towards the posterior esophageal wall or the reverse direction. If one extends beyond the inferior part, perforation occurs. Endoclips may be applied for bleeding or to prevent micro perforation.
Argon plasma coagulation uses a noncontact method to divide the septum. The septum can be divided from below upwards or from above downwards.
Esophageal Diverticulum
Diverticula are outpouchings of one or more layers of the intestinal wall that may occur at any level of the esophagus. Diverticula are classified as true, false, and intramural types depending on the number of intestinal wall layers involved. True diverticula contain all layers of the intestinal wall, false diverticula contain only mucosal and submucosal layers, and intramural diverticula refer to outpouchings contained within the submucosa layer. Diverticula of the body of the esophagus are divided into midthoracic (parabronchial) and epiphrenic diverticula. Diverticula are also classified based on the pathogenesis as pulsion or traction diverticulum. Pulsion diverticulum is due to the high intraluminal pressure against weakness in gastrointestinal tract wall. Traction diverticulum is due to pulling forces on the outside of the esophagus from adjacent inflammatory process (mediastinal lymph node). In the lower esophagus, epiphrenic diverticula are typically considered to be pulsion diverticula. Epiphrenic diverticula are often associated with achalasia.
Mid-esophageal diverticulum may be associated with diffuse esophageal spasm or mediastinal fibrosis. Diffuse intramural diverticulosis or intramural pseudodiverticulosis is a rare entity of multiple 1- to 3-mm mucosal outpouchings associated with inflammation and esophageal wall thickening. It is due to dilation of the esophageal glands.
Midthoracic and epiphrenic diverticula are usually asymptomatic and are discovered during routine radiologic evaluations for unrelated complaints. They mostly occur in middle-aged adults and elderly. Symptoms of dysphagia and regurgitation can be reported by patients, particularly those with diverticula related to diffuse esophageal spasm or achalasia.
On barium swallow, midthoracic or traction diverticulum is typically a small, widemouth pouch located near the tracheal bifurcation. An epiphrenic diverticulum, on the other hand, appears as a large globular pouch, often associated with abnormal esophageal contractions. Endoscopy will help in visualizing the outpouching in the esophagus.
Midthoracic or epiphrenic diverticula that are asymptomatic need not be treated. In symptomatic patients, treatment should be aimed at the underlying esophageal motility disorder or stricture. Surgical management of midthoracic or epiphrenic diverticula depends on the underlying motility disorder which can involve extended myotomy with a diverticulectomy.
Achalasia Cardia
It is defined as a primary motility disorder of the esophagus, in which there is a loss of esophageal peristalsis and insufficient lower esophageal sphincter relaxation.
Achalasia has an equal incidence in men and women and can affect all age groups but is more commonly seen in the third to sixth decade of life. The exact etiology may be autoimmune, viral, or neurodegenerative in nature. In areas where Trypanosoma cruzi is endemic, achalasia presents as a manifestation of Chagas disease.
The ganglion cells of the myenteric plexus are degenerated which causes an imbalance in the inhibitory and excitatory neurons of the lower esophageal sphincter. The end result is unopposed action of the cholinergic activity which leads to insufficient relaxation of the lower esophageal sphincter. The manifestations of achalasia are more due to the incompletely relaxation of the lower esophageal sphincter rather than the aperistaltic esophagus. The aperistaltic esophagus usually causes symptoms when it gets massively dilated to form a sigmoid esophagus. Thus, all the treatment modalities are directed towards the lower esophageal sphincter.
Achalasia should be suspected in all patients who present with dysphagia to solids and liquids and in those patients in whom the regurgitation does not respond to proton pump therapy. The disorder starts insidiously and gradually progressive over weeks and months.
Dysphagia to solids and liquids and regurgitation are common symptoms during presentation. Regurgitation usually gets worse during supine posture, and some patients may self-induce vomiting to relieve the symptoms. Many patients will describe the heartburn to be bland in nature because acid does not mix the retained food in the esophagus. Patients may use liquids to push food down and may change posture by arching their neck or pushing their shoulders backwards to help the food go down into the stomach. At times they may complain of heartburn due to residual food in the esophagus. They also complain of chest pain which is usually substernal. Weight loss commonly occurs in patients with achalasia, but if significant weight loss occurs especially in an elderly patient, then secondary achalasia should be excluded.
Endoscopically, the findings show saliva, liquid, and food in the esophagus. The esophagus appears dilated and roomy (Fig. 5.1). There is no evidence of mechanical obstruction seen. If there is long-standing stasis of food, then erythema and erosions or candida infection may be seen. There is some resistance in passage of the endoscope from the esophagus into the stomach; a sensation described by endoscopists as a “give way” may be appreciated. This is not mandatory for the diagnosis, but if present in a non-dilated esophagus without residue, it helps the clinician to pursue his/her suspicion of achalasia by asking for a manometry. If there is a massively dilated esophagus like sigmoid esophagus, the lower esophageal sphincter opening may be hidden under the residual food and maybe displaced laterally. Rarely, the endoscopists may find it difficult to intubate the stomach. Endoscopy is recommended in all patients with suspected achalasia to exclude secondary achalasia like a gastroesophageal junction tumor or a fundal tumor infiltrating the gastroesophageal junction.
Fig. 5.1
Achalasia cardia dilated esophagus
Manometry is now recommended in all patients before starting therapy. Earlier conventional manometry described achalasia to have aperistaltic esophagus, elevated lower esophageal sphincter resting pressures, and an elevated nadir [residual] pressure of the lower esophageal sphincter of >8 mmHg. Now with the advent of high-resolution manometry, achalasia can be classified into three types by the Chicago classification, which help the clinician decide the therapy that will best benefit the patient.
Type 1 achalasia cardia (Fig. 5.2)
Hundred percent failed peristalsis, mean integrated relaxation pressures [IRP] of the lower esophageal sphincter >15 mmHg
Fig. 5.2
Type 1 achalasia
Type 2 achalasia cardia (Fig. 5.3)
No normal peristalsis, panesophageal pressurization in >20 % of swallows, mean integrated relaxation pressures of the lower esophageal sphincter >15 mmHg
Fig. 5.3
Type 2 achalasia
Type 3 achalasia cardia (Fig. 5.4)
No normal peristalsis, preserved fragments of distal peristalsis or premature [spastic] contractions in >20 % of swallows, mean integrated relaxation pressures of the lower esophageal sphincter >15 mmHg
Fig. 5.4
Type 3 achalasia
Other Tests
Barium esophagogram will show a dilated esophagus, bird-beak appearance at the lower esophageal sphincter, and holdup of barium above the lower esophageal sphincter with a delay in passage of the barium into the stomach (Fig. 5.5). There will be absent peristalsis in the body of the esophagus. A sigmoid-like esophagus may occur in patients with long-standing untreated disease.
Fig. 5.5
Barium achalasia cardia
Chest X-ray will usually show a widened mediastinum due to the dilated esophagus and absent gastric air bubble which occurs due to a non-relaxing lower esophageal sphincter.
Treatment
The treatment of achalasia is now influenced by the type of achalasia found on manometry. Type 1 achalasia responds best to laparoscopic Heller’s myotomy, and type 2 achalasia has equal response rates to laparoscopic Heller’s myotomy and pneumatic dilatation, and type 3 does poorly to pneumatic dilatation, botulinum toxin, or laparoscopic Heller’s myotomy.
Drug treatment with calcium channel blockers and nitrates has poor response rates up to 10 %. They are usually reserved for patients who are unwell due to comorbidities or refuse treatment. They act by reducing the lower esophageal sphincter pressures.
Botulinum toxin can be injected endoscopically into the lower esophageal sphincter. It acts by blocking the release of acetylcholine from the presynaptic receptors and restores the balance between the inhibitory and excitatory neurotransmitters. It is injected in four quadrants, 1 cm above the Z-line of the gastroesophageal junction in a dose of 25 units [1 ml] in each quadrant. The response is seen in 70–90 % of patients, but most of them would relapse in 6–12 months and will require a second injection. The relapse rate is higher and the time to relapse is earlier when compared to pneumatic dilatation or laparoscopic Heller’s myotomy.
Pneumatic dilatation and laparoscopic myotomy are the standard of care for achalasia cardia. The main aim of pneumatic dilation is to disrupt the lower esophageal sphincter. This reduces the gradient across the lower esophageal sphincter and helps the esophagus to empty the food and liquids by gravity. Pneumatic dilatation is done with a Rigiflex dilator after placing a guidewire with the endoscope. Most endoscopists would start with a 30 mm size of the balloon, and if it does not respond, then they would use a high diameter of 35 or 40 mm. The dilator is inflated with air till one can visualize “waist” obliteration of the balloon on fluoroscopy. This balloon is kept inflated for 1 min after which it is rapidly deflated. Some endoscopists would reinflate the balloon once more and note the pressure required for waist obliteration, which will be less than that required the first time. This procedure has a success rate of 70–90 % in expert hands as the initial success, but 50 % of patients would relapse over 5 years. They will then be subjected to another pneumatic dilatation or be sent for laparoscopic Heller’s myotomy.
For laparoscopic Heller’s myotomy, please refer to the chapter ahead.
Diffuse Esophageal Spasm
This is a motility disorder of the esophagus. The clinical significance of this disorder is not known. It was also called as distal esophageal spasm because the findings usually occurred in the distal esophagus [smooth muscle]. There is no clarity on the fact if symptoms are produced due to the impaired [rapid] contraction or due to the elevated amplitude that may be observed in some of them.
The common presentation is unexplained chest pain or dysphagia. Gastroesophageal reflux disease [GERD] can also present with the similar symptoms but usually have heartburn as one of the major symptoms. GERD can be present in two-thirds of patients with motility disorders, and a 24 h ph study can differentiate primary from secondary esophageal motility disorders. It would be worthwhile doing an endoscopy to exclude any obstructive pathology if dysphagia is a presenting symptom. Cardiac cause for the chest pain should always be excluded before pursuing this diagnosis.
The pathophysiology of diffuse esophageal spasm is not exactly known. But since treatment with nitrates has shown benefit, a defect in the nitric acid synthesis has been postulated.
CT scan may show increase thickness of the esophageal wall to >3 mm in some patients. Since cancer of the esophagus can also present with hypertrophy of the esophageal wall, further investigations to exclude a malignancy are warranted.
The earlier conventional manometry defined diffuse esophageal spasm as:
1.
Simultaneous [synchronous] contractions in >20 % of swallows with amplitude >30 mmHg in the distal esophagus
2.
Repetitive contractions >3 peaks in the distal esophagus
3.
Distal peristaltic velocity >8 cm/s in the distal esophagus
The current Chicago classification for high-resolution manometry classifies diffuse esophageal spasm as:
1.
IRP [integrated relaxation pressures] <15 mmHg
Integrated relaxation pressure [IRP] is measured in mmHg and defined as mean esophageal gastric junction pressure measured for four contiguous or non-contiguous seconds of relaxation in the 10-s window following deglutitive UES relaxation.
2.
Distal latency <4.5 s
Distal latency [DL] is the interval between upper esophageal sphincter relaxation and the contractile deceleration point. The distal latency is >4.5 s in normal peristalsis.
3.
Contractile forward velocity >9 cm/s
Contractile front velocity (cm s −1 ) [CFV] is the slope of the tangent approximating the 30 mmHg isobaric contour between the proximal trough of the distal esophageal contraction and the contractile deceleration point.
For the management of diffuse esophageal spasm, see below in nutcracker esophagus.
Nutcracker Esophagus
This is a motility disorder of the esophagus in whom there is an elevated amplitude of peristalsis [>180 mmHg] in the distal smooth muscle of the esophagus on conventional manometry (Figs. 5.6 and 5.7).
Fig. 5.6
Diffuse esophageal spasm
Fig. 5.7
Nutcracker esophagus
In the Chicago classification, it is classified as a distal contractile integral >5,000 mmHg-s-cm with an integrated relaxation pressure <15 mmHg.
Distal contractile integral (mmHg-s-cm) [DCI] is defined as amplitude x duration x length of the distal esophageal contraction >20 mmHg from proximal to distal pressure troughs.
The presentation is commonly chest pain, rarely dysphagia since there are no bolus transit abnormalities seen. It may be associated with a hypertensive or hypotensive lower esophageal sphincter. The symptoms usually poorly correlate with the timing of the elevated amplitudes, i.e., not all high-amplitude peristalses cause pain. Visceral hypersensitivity may be responsible in some patients with chest pain.
Endoscopy in Spastic Disorders of the Esophagus
Endoscopy is usually normal and there are no characteristic signs.
Barium studies may show a corkscrew or rosary bead appearance due to non-propulsive peristalsis.
Management of Diffuse Esophageal Spasm and Nutcracker Esophagus
Drug treatment provides varied degree of relief for spastic disorders of the esophagus.
Reassurance and alleviation of anxiety is important. The fact that a diagnosis has been reached itself is reassuring to many patients. Counseling the patient that it is usually a non-progressive disease and without any long-term adverse outcome helps in alleviation of their anxiety.
Proton Pump Inhibitors
Gastroesophageal reflux disease may also contribute to the chest pain. A trial of proton pump inhibitors in twice daily dosage for 3 months may benefit such patients.
Calcium Channel Blockers
Nifedipine [10–20 mg three times daily] and diltiazem [30–60 mg three to four times daily] both have been used with variable results. They help in alleviating chest pain and dysphagia. Nifedipine can cause pedal edema and diltiazem can cause bradycardia and should not be used with other rate-slowing drugs like beta blockers.
Nitrates
Isosorbide dinitrate can be used before meals in patients with dysphagia or at the time of the chest pain. Headache is a common side effect and tolerance may develop if used for a long duration.
Tricyclic Antidepressants
Imipramine [50 mg/OD] or amitriptyline can be used for chest pain. They help by modifying the visceral hypersensitivity.
Phosphodiesterase Inhibitors
Sildenafil has been shown to help patients with chest pain in nutcracker esophagus. Its action is due to its smooth muscle relaxant effect which can reduce esophageal spastic contractions and the lower esophageal sphincter pressures.
Botulinum Toxin
In patients not responding to drug treatment, botulinum toxin can be used. Its effect is short term [up to 6 months] when injected in the lower esophagus just above the lower esophageal sphincter. Its mechanism of action is by binding to receptors and reducing the amount of acetylcholine release.
Balloon Dilatation
This has been used in diffuse spasm and nutcracker esophagus with partial relief and recurrence of symptoms. Its usual role is in the treatment of achalasia cardia.
Non-pharmacological Treatment
Hot water to be taken with meals has been found to reduce the amplitude of esophageal contraction and improve esophageal clearance.
Peppermint oil has been tried in patients of diffuse esophageal spasm. It was found to improve symptoms of chest pain and improve manometric findings.
Scleroderma
The esophagus is unique by having skeletal muscle in the upper third and smooth muscle in the lower two-third. Scleroderma causes fibrosis and atrophy in the smooth muscle of the esophagus. The disease involves the esophageal body to cause aperistalsis [in the lower two-third of the esophagus] and weakens the lower esophageal sphincter [hypotensive]. The motility of the skeletal muscle [upper third of the esophagus] is unaffected.
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