TITLE: DYSPHAGIA AND HYPOPHARYNGEAL DIVERTICULI
SOURCE: UTMB Department of Otolaryngology Grand Rounds
DATE: March 16, 1997
RESIDENT PHYSICIAN: Gregory Young, M.D.
FACULTY PHYSICIAN: Brian Driscoll, M.D.
SERIES EDITOR: Francis B. Quinn, Jr., M.D.

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"This material was prepared by physicians in partial fulfillment of educational requirements established for Continuing Postgraduate Medical Education activities and was not intended for clinical use in its present form. It was prepared for the purpose of stimulating group discussion in a interactive computer mediated conference setting. No warranties, either express or implied, are made with respect to its accuracy, completeness, or timeliness. The material does not necessarily reflect the current or past opinions of subscribers or other professionals and should not be used for purposes of diagnosis or treatment without consulting appropriate literature sources and informed professional opinion."

INTRODUCTION:

The diagnosis of the cause of dysphagia relies heavily on an accurate history and physical examination. Endoscopy and biopsies, as well as radiographic examinations are confirmatory. Functional alterations in swallowing may be evaluated with modified barium swallow or pharyngeal manometry with fluoroscopy. Treatment is directed towards the etiology of the dysphagia. Treatment is also directed towards compensation for the alteration in swallowing, especially if the etiology is unknown or the effect is irreversible.

PHYSIOLOGY OF SWALLOWING:

Normal swallowing is divided into four stages, the oral preparatory stage, the oral stage, the pharyngeal stage, and the esophageal stage. The first 2 stages are mechanical and under voluntary control. The last 2 stages are reflexive.

Oral Preparatory Stage:

The oral preparatory stage is essentially chewing. It involves the coordination of lip, buccal, jaw, tongue, and soft palate movements in order to prepare food for swallowing. At the end of this phase, the tongue pulls the food into a bolus, and holds it against the hard palate. The most important neuromuscular function in this phase is the lateral rolling motion of the tongue, as those without normal tongue mobility have great difficulty chewing.

Oral Stage:

The oral stage is the second stage of swallowing. It lasts about 1 second, and does not vary with age, sex, or consistency of the bolus. It moves food from the front of the oral cavity to the anterior faucial arches. Again, tongue movement is the most important aspect of this phase of swallowing, since it shapes, lifts, and squeezes the bolus upward and backward along the hard palate.

Pharyngeal Stage:

The pharyngeal stage begins when the bolus reaches the anterior faucial arches. Here, the bolus triggers the swallowing reflex which occurs via the glossopharyngeal nerve. The swallowing reflex may also be triggered by the superior laryngeal nerve at the laryngeal inlet. When a swallow reflex occurs late by this second mechanism, the patient is said to have a delayed swallowing reflex. While the first two phases of swallowing can be bypassed by using liquid feeds and syringing the bolus to the back of the mouth or extending the head, the reflexive stage of swallowing cannot be bypassed. The swallowing reflex is mediated in the reticular formation of the brainstem, adjacent to the respiratory center. It is modulated by input from the respiratory center, and cortical areas. The pharyngeal stage lasts a maximum of 1 second and does not vary with food consistency, age or sex.

Swallowing Reflex (Pharyngeal Stage):

When triggered, the swallowing reflex results in four neuromuscular functions, which occur in rapid sequence in the following order:

Laryngeal closure occurs at three sphincters:

Pharyngoesophageal segment opening depends on:

Esophageal Stage:

The fourth and final stage of swallowing is the esophageal stage. It is more variable and prolonged than the other phases of swallowing, lasting from between 8 and 20 seconds. Esophageal transit time significantly increases with age.

DIAGNOSIS OF DYSPHAGIA:

History:

Initially, it is important to determine whether dysphagia(difficulty with swallowing) or odynophagia (painful swallowing) is the chief complaint. If odynophagia is the chief complaint, the location, timing, and duration of the pain, as well as any associated ear pain, change in pain with swallowing, weight loss, change in voice, or bloody sputum should be documented. If dysphagia is present, then the duration and timing of the dysphagia should be sought. In addition, dysphagia for solids vs. liquids is important.

Physical Examination:

A complete head and neck examination should be performed. Especially important are a mirror and fiberoptic examination of the tongue base and hypopharynx, and careful palpation of the neck, oral cavity and tongue base. A cranial nerve examination, especially of CN IX, X, XI, and XII is essential. The patient should also be observed chewing and swallowing, with special attention paid to the degree of oral control, mastication ability, and the presence or absence of coughing. Aspiration is the entry of material into the airway below the true vocal cords. It is important because it may lead to acute and chronic pulmonary complications. However, the timing of aspiration in relation to swallowing can help elucidate the cause of the swallowing disorder. For example, aspiration that occurs prior to the reflexive swallow is due to either reduced tongue control or a delayed or absent swallow reflex. Aspiration during swallowing is due to inadequate airway closure. Aspiration after swallowing occurs because of retained pharyngeal residue, due to reduced laryngeal elevation, reduced pharyngeal peristalsis, unilateral pharyngeal paralysis, or pharyngoesophageal dysfunction.

Diagnostic Tests:

Barium Swallow:
Barium swallow is commonly ordered to evaluate a patient’s complaint of dysphagia. Structural abnormalities such as webs, stenoses, diverticuli, and cancer may be found. However, functional abnormalities are more difficult to evaluate with this study.
Direct Laryngoscopy and Esophagoscopy:
Direct laryngoscopy and esophagoscopy should be performed when malignancy is suspected, or the diagnosis is unclear.
Modified Barium Swallow:
The modified barium swallow is a radiographic videofluoroscopic technique that is used to evaluate the oral and pharyngeal stages of swallowing. It is the method of choice to evaluate suspected aspiration, and is helpful is diagnosing functional deficits such as those caused by neurologic disorders. The swallowing mechanism with both small and large boluses, and both liquids and solids can be evaluated. From information obtained through the modified barium swallow, the etiology of the swallowing dysfunction can often be found. In addition, recommendations can be made regarding compensatory maneuvers to facilitate swallowing without aspiration. These compensatory maneuvers can then be tested during the modified barium swallow to rule out silent aspiration(i.e. aspiration without associated cough). The modified barium swallow also provides radiographic support when procedures such as vocal cord medialization or cricopharyngeal myotomy may be indicated.
Manometry:
Manometry is useful in evaluating disorders of esophageal motility. Water infusion manometers are sometimes used to evaluate esophageal motility. The following disorders are associated with specific manometric findings: Achalasia- Absent peristalsis, increased LES pressure, incomplete LES relaxation Diffuse Esophageal Spasm- Simulataneous contractions, Intermittent normal peristalsis Nutcracker esophagus- Increased amplitude of peristalsis Hypertensive LES- Elevated LES pressure, normal LES relaxation
Manofluorography:
Pharyngeal function involves rapid changes in pressure and motion, and so requires simultaneous fluoroscopy and solid state manometry, or manofluorography. Pressure, anatomic events, and bolus transit are simultaneously recorded in manofluorography. During swallowing, pharyngeal pressures are generated by the tongue, palate, larynx, and the pharyngeal walls. Manofluorography can quantitate the pressure applied to the bolus from each of these structures. Therefore, the structure(s) responsible for the swallowing dysfunction may be identified. Determination of intrabolus forces is the most sensitive method for determining pharyngeal dysfunction. The mechanism of pressure generation during swallowing has been described as a two pump system, the oropharyngeal propulsion pump and the hypopharyngeal suction pump. The tongue and upper pharynx form the oropharyngeal propulsion pump, with the tongue being the piston, and the pharynx being the chamber. Any pathology that affects mobility or control of the tongue or pharyngeal wall may compromise the oropharyngeal propulsion pump. It is also affected by changes in the pharyngo-esophageal sphincter. An increased oropharyngeal pump pressure is measured when the PE sphincter is obstructed. The oropharyngeal propulsion pump is best measured in the pharynx just superior to the arytenoids.

The hypopharyngeal suction pump is formed by the larynx and hypopharynx. Laryngeal elevation combined with inferior constrictor relaxation results in a negative pressure exerted on the bolus. The following disorders are associated with changes in the oropharyngeal propulsion and/or hypopharyngeal suction pumps.

Manofluorography has been used to study the abnormal swallowing characteristics of several disorders that cause dysphagia. Examples include Wallenburg's syndrome, dermatomyositis, Guillian-Barre, oculopharyngeal muscular dystrophy, and irradiation fibrosis.

Wallenberg's syndrome (lateral medullary syndrome) is due to thrombosis of the posteroinferior cerebellar artery which results in ischemia of the lateral medullary region of the brain stem. In addition to vertigo, nystagmus, nausea, vomiting, Horner's syndrome, dysphonia, ataxia, falling to the side of the lesion, and loss of pain and temperature sensation to the ipsilateral head and contralateral body, this syndrome also leads to dysphagia. It is the most common brain stem vascular disorder. Ipsilateral palate paralysis occurs. It differs from many other types of dysphagia in that the tongue driving force and oropharyngeal propulsion pump force are greatly increased, compared to normal values. This is in part due to the failure of pharyngoesophageal sphincter opening during swallowing. The hypopharyngeal suction pump is decreased, and the pharyngeal transit time is mildly increased. There is some bolus residue after swallowing. Aspiration is not a problem.

Dermatomyositis causes dysphagia that is similar to Wallenberg's syndrome. In contrast, those with Guillian-Barre, oculopharyngeal muscular dystrophy, and irradiation fibrosis have very low tongue driving force and oropharyngeal propulsion pump, greatly prolonged pharyngeal transit time, mild(Oculopharyngeal muscular dystrophy) to severe(irradiation fibrosis) aspiration, and high bolus residue.

TREATMENT:

Swallowing Therapy:

Patients with deficits of the oral or pharyngeal phases of swallowing may benefit from swallowing therapy. This may include strengthening and coordination exercises, or compensatory maneuvers to aid in swallowing. The supraglottic swallow, Mendelsohn maneuver, and neck turning with swallowing are examples of compensatory maneuvers. The supraglottic swallow involves voluntarily holding the breath at the height of inspiration before and during swallowing, thereby closing the vocal fords before and during the swallow. The patient coughs after the swallow to clear any residual food from the larynx. The Mendelsohn maneuver is performed by voluntarily elevating the larynx during the swallow, which tucks the laryngeal inlet anteriorly and opens the PE segments, thereby facilitating the swallow. Patients with unilateral pharyngeal paralysis can turn their head towards the paralyzed side, which diverts the bolus down the functioning side of the pharynx.

Medical Treatment:

If possible, treatment for dysphagia should be directed towards the etiology of the symptom. Dysphagia secondary to thyroid disease, myasthenia gravis, and Parkinson's disease are potentially treatable. Gastroesophageal reflux, the most common medically treated cause of dysphagia, is treated with H2 blockers and GERD precautions. Esophageal motility disorders causing chest pain are the second most commonly medically treated cause of dysphagia, and these are often treated with nitrates or calcium channel blockers.

Surgical Treatment:

Surgical treatment for dysphagia includes dilation and cricopharyngeal myotomy. Strictures, webs, Schatzki's rings, and achalasia are often treated with dilation. Dilation is the most common procedure performed for dysphagia. Cricopharyngeal myotomy is the second most common procedure performed for dysphagia. This procedure involves cutting the lower half of the inferior constrictor, the cricopharyngeus, and the circular muscles of the upper cervical esophagus.

The indications for cricopharyngeal myotomy are controversial. This is in part due to the finding that pharyngoesophageal (PE) segment opening depends on both relaxation of the PE segment and elevation of the larynx. These two functions cana be evaluated separately with manofluorography. Cricopharyngeal myotomy would likely improve only those disorders with PE segment relaxation problems. For this reason, current recommendations are that cricopharyngeal myotomy should be considered for disorders in which there is incomplete PE segment relaxation(pressure greater than 0mm Hg) or abnormal muscular contractions during the relaxation period. In practice, however, is manofluorography is not yet widely available and so the exact anatomic cause of dysphagia may be difficult to elucidate.

Lower esophageal sphincter incompetence with reflux is a contraindication to cricopharyngeal myotomy, because this procedure may lead to worsening reflux with significant aspiration.

Treatment of dysphagia must include treatment of any significant aspiration. If Oral feeding should be suspended is aspiration is significant, and a feeding tube should be placed. If NPO status is predicted to be required more than 2 months, a percutaneous or open gastrostomy tube should be considered. A feeding tube and/or gastrostomy tube are also employed if oral nutrition cannot be maintained.

SPECIFIC PROBLEMS:

Aspiration:

Aspiration may require surgical management if chronic in duration and unresponsive to swallowing therapy. Surgery can sometimes completely correct aspiration. Examples are aspiration due to a paralyzed abducted vocal cord, and aspiration due to Zenker's diverticulum. A paralyzed abducted vocal cord can be corrected with Teflon injection or surgical medialization.

In patients with unremitting chronic aspiration, a tracheostomy may be indicated. However, tracheostomy does not prevent aspiration, and may actually increase it. If patients with this severe form of aspiration desire to resume oral feeding, some type of laryngeal closure procedure may be required. These include supraglottic or glottic closure, laryngeal stints, cricoid resection, laryngoplasty, laryngotracheal diversion, or laryngectomy. None of the procedures is appropriate in all cases of refractory aspiration, and treatment must be individualized.

Hypopharyngeal Diverticuli:

A Zenker's diverticulum is a pulsion diverticulum through Killian's triangle that occurs primarily in middle and older age. 40% of these patients also have a hiatal hernia, compared to 15% of the general population. Complaints and symptoms range from dysphagia, weight loss, and coughing in the supine position, to severe aspiration with recurrent pneumonia. Regurgitation of undigested food boluses, rather than liquid, should raise suspicion of Zenker's diverticulum. A positive Quinn's sign is virtually diagnostic of Zenker's diverticulum. This is the regurgitation of food into the mouth upon external decompression of the pouch (by pressing the left side of neck near the cricoid cartilage). A barium swallow is the gold standard for diagnosis, because it shows the size and location of the diverticulum, and may document aspiration. In addition, other esophageal pathology may be identified. Treatment

Controversy exists concerning the removal of asymptomatic Zenker's diverticuli. Due to the potential for complications from this or any surgical procedure, some authors prefer in many cases to wait for symptoms to develop. Others site potentially silent aspiration as a reason to proceed with surgery for even asymptomatic diverticuli. Surgical treatment options for Zenker's diverticuli include endoscopic procedures, diverticulopexy, imbrication, diverticulectomy. Each of these procedures should be performed in conjunction with cricopharyngeal myotomy, in order to prevent recurrence. Endoscopic diverticulectomy involves transecting the party wall between the esophagus and the diverticulum and sectioning of the cricopharyngeal muscle. With this procedure, hospitalization is decreased and the risk of mediastinitis is reduced. However, specialized equipment is required, and cannot be performed on small or oblique pouches. In elderly or debilitated patients, a diverticulopexy and cricopharyngeal myotomy may be indicated. With this procedure, patients may be fed sooner, hospital stay is shorter than with diverticulectomy, and there is no risk of salivary leak from the resected sac neck.

In this procedure, a esophageal bougie is placed, then the external cricopharyngeal myotomy is made, including division of fibers from the distal inferior constrictor, cervical esophageal fibers, and cricopharyngeus. (Note that with the endoscopic technique, only the cricopharyngeus is divided.) This sectioning is performed posterior to the cricothyroid joint in order to avoid injury to the recurrent laryngeal nerve. The apex of the sac is then suspended to the prevertebral fascia with 3-0 silk ties.

For small pouches, imbrication along with cricopharyngeal myotomy has been successful. With imbrication, the mucosa is not violated, so there is minimal risk of salivary leak. Larger pouches cannot be imbricated due to problems with esophageal obstruction.

Diverticulectomy is usually performed in conjunction with cricopharyngeal myotomy, and the surgical approach is the same. An esophagoscopy and laryngoscopy are performed first, and the diverticulum is identified either posteriorly or left posterolaterally in most cases. The pouch is examined to rule out coincidental carcinoma, and then is packed with 1 inch plain gauze. Packing the pouch facilitates its identification and dissection within the neck. A #40 bougie is inserted into the esophagus to prevent overly aggressive resection of the pouch, which can lead to stenosis. In addition, it provides a stable structure to facilitate performing the myotomy without inadvertent entry in the lumen. Dissection should occur along the posterior aspect of the cricoid, in order to minimize risk of recurrent laryngeal nerve injury. The wound is closed in 2 layers, either with suture, or with suture and staples. Closures are tested by filling the mouth and pharynx with saline. A #12 soft Silastic feeding tube is then placed under direct visualization. A penrose drain is placed in the neck wound, and oral feeds are not started for 5 days. Complications include mediastinitis, pharyngocutaneous fistula, stricture, and recurrent laryngeal nerve injury.

Regardless of the type of surgical treatment chosen for Zenker's diverticulum, cricopharyngeal myotomy is the key to prevent recurrence. Smaller diverticuli have been treated successfully with cricopharyngeal myotomy alone.

Discussion by Dr. Faculty (Dr. Driscoll):

Dr. Driscoll pointed out that a great deal can be learned about the patient's swallowing difficulty during the physical examination. Function of cranial nerves VII, IX, and XII influences the oral phase of swallowing. Inserting the examining finger into the mouth and having the patient press his tongue against the hard palate gives one an appreciation of any lingual muscular weakness. Asking the patient to say repeatedly the phrase, "putaka putaka putaka...." allows an estimate of fine motor coordination mediated by the VIIn and XIIn. Mirror examination will reveal puddling of secretions in the valleculae and/or pyriform sinuses, as well as vocal cord function.

(Ed. note: "Quinn's sign" referred to here is actually "Quinn's sign (1)" wherein as the patient protrudes his tongue for mirror laryngoscopy, a bubble of air escapes the diverticulum into the hypopharynx producing a characteristic mini-belch. This can be elicited as well by manipulating the larynx and upper trachea with the examiner's hand while the patient breathes through his mouth. "Quinn's sign(2)" is "burpotoalgia," a sudden sharp pain the the ear upon belching or burping, characteristic of the initial stage of bullous myringitis. It is sometimes seen in fungal external otitis in which myringitis may figure prominently.)

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