Oropharyngeal Dysphagia, Achalasia, and Other Esophageal Motility

Problems: Clinical Relevance and Management

Nicholas Shaheen, MD, MPHCenter for Esophageal Diseases

and SwallowingUniversity of North Carolina

Outline• Oropharyngeal Dysphagia

– Epidemiology and Clinical Impact– Pathogenesis– Work-up– Treatment

• Achalasia– Epidemiology and Clinical Impact– Pathogenesis– Work-up– Treatment

• Diffuse Esophageal Spasm (DES)• Ineffective Esophageal Motility (IEM)

All Dysphagia is Not Created Equal

• Need to differentiate thoracic vs. oropharyngealdysphagia

• DDx is different• Work-up is different

All choked up…• A 73 year old male presents to you

complaining of difficulty swallowing x 9 mos.• After eating 3-4 bites of any meal, often

gets the sensation of “choking” or “windpipe being cut off”

• Worst with drinks, better with mashed potatoes

• 10 lb weight loss, associated with voluntary food avoidance

Choked Up, cont.

• PMH – CVA 6 yrs ago with residual Left side weakness, HTN, DM

• PE – Other than weakness on left, nl• What to Do Next?

– Diagnostically– Therapeutically

Work-up of Dysphagia

• History – What Do You Want to Know?– Chronicity? Progressive or Intermittent?– Problems choking on food initially or wedged

in chest?– Liquids? Solids? Both? Which first?– Point where it is getting stuck– GERD? Wt Loss? Blood?– DM? Previous Chest RT? Meds?

What Does OropharyngealDysphagia Sound Like?

• Food “sticks in throat”• Hoarse or “Gargly” voice• Food regurgitates nasally or “strangles”• Frequent repetitive swallows• Frequent throat clearing• Cough• Pneumonia

Oropharyngeal Dysphagia, DDx

• Neurologic– CVA– MS– Parkinson’s– Huntington’s– ALS– Neuropathies/Auto-

immune

• Muscular– Poly/Dermatomyositis– MD– MG

• Lesions/Structural– Cancer– Zenker’s– Extrinsic Compression– Webs

Work-up of Oropharyngeal Dysphagia

• Involves multiple specialties:– Gastroenterologists– Neurologists– Speech pathologists– Otolaryngologists, and, – Radiologists

Work-Up of OropharyngealDysphagia

• More complicated than thoracic dysphagia, and more patient-specific– Modified BaS– FEES Study– ENT Examination– CT of the Neck and Chest– MRI of the Head– Upper endoscopy

What is the FEES Thing, Anyway?

• Flexible endoscope into nasopharynx

• Tipped back into the oropharynx to watch the patient eat

• Can tell if oral contents are inappropriately penetrating laryngeal structures

FEES vs. MBS• Indications for MBS:

– vague symptoms; – need comprehensive view – Visualize submucosal anatomy

(e.g., cervical osteophytes) – Assess oral stage/base-of-

tongue movement – UES stricture/hypertonicity? – Examine movement of multiple

structures at height of swallow – Voicing with tracheoesophageal

prosthesis : what is vibrating? – Laryngectomy

• Indications for FEES:– logistic reasons

• Fluoro not available • Transportation to radiology risky;

medically fragile patient • Positioning problematic: contractures,

quad, neck halo, obese, on ventilator • Concern about radiation

– clinical reasons• Visualize surface anatomy, mucosal

abnormalities, resection, etc. • Velopharyngeal incompetence • Visualize laryngeal movement/vocal

fold mobility • compromised pulmonary clearance • Clinical question of secretions

management • Biofeedback is desired: therapy session

SE Langmore et al, 2006.

Zenker’s Diverticulum

Multiple Sclerosis

Cervical Osteophytes

Treatment of O-P Dysphagia

• Usually directed at underlying causes– Rehabilitation and retraining by speech

pathologists• chin tuck• head rotation to the affected side• head tilt to the stronger side

– In selected cases of cricopharyngeushypertrophy, myotomy may resolve symptoms

Benign Leiomyoma

Pharyngeal Cancer

Achalasia

Pathogenesis• failure of relaxation of the lower esophageal

sphincter in response to deglutition• absence of primary peristalsis• Histologically, there is neuronal degeneration of

the myenteric plexus– preferentially effects the inhibitory, nitric oxide

producing cells– The opposing cholinergic neurons responsible for

tonic contraction are relatively spared, leading to an increased baseline tone, and inability to relax in response to swallows

Epidemiology

• Affects 0.5-1/100,000 persons• Men and women equally affected• Substantial variation in epidemiology

amongst cultures– May represent ability to diagnose the

condition

Normal E Mano

E Mano in Achalasia

Hydrostatic Dilatation of Achalasia

What is the most appropriate initial management of achalasia?

• Randomized controlled trial of pneumatic dilatation vs. open anterior myotomy– Surgery subjects had more durable relief of

symptoms– More than half of dilated patients needed re-

treatment– Late failures also more common in dilatation group

• Second RCT in Chagas patients suggested better manometric response in surgical patients.

Csendes A et al.. Gut 1989; 30(3):299-304. Felix VN, et al. Hepatogastroenterology 1998; 45(19):97-108.

DES

• EGD normal• Barium swallow

– may be normal– may show a “corkscrew”

appearance due to multiple simultaneous contractions

• E mano shows long waves that are simultaneous

DES, E Mano

DES, Treatment

• Pharmacologic– Calcium Channel Blockers– Nitrates– Anticholinergics– Centrally-acting agents for pain

• Surgical– Long myotomy is reversed for disabling,

recurrent disease

Ineffective Esophageal Motility• Characterized by:

– greater than 30% of swallows being not having complete peristalsis

– Greater than 30% of swallows having a distal peristalsis of <30 mmHg

• Often seen in association with reflux disease

• If achalasia is “esophageal asystole,”and DES is “esophageal tachycardia,”this is “Esophageal CHF”

What are the Treatment Choices in IEM?

• Metoclopramide• Erythromycin• Domperidone• Tegaserod (w/d 3 months ago)• Bethanachol

What to Do about the Subject with Functional Dysphagia

• Many gastroenterologists empirically dilate the esophagus

• More recent randomized controlled trials do not support this practice 1,2

• May consider re-assurance after work-up or a centrally-acting agent

1 K. Lavu et al, South Med J. 2004 Feb;97(2):137-40.2 JS Scolapio et al, Am J Gastroenterol. 2001 Feb;96(2):327-30.

Back to our 73 yo with O-P Dysphagia…

• Got a FEES study – showed extensive penetration of the vallecular space with food contents

• MRI of head – multiple small infarcts• Modification of diet by speech pathology

and swallowing exercises• Doing ok

Conclusions

• Dysphagia is a common symptom which requires immediate investigation

• Differentiation between thoracic and oropharyngeal dysphagia narrows the DDxand dictates the work-up

Conclusions, cont.

• First test for oropharyngeal dysphagia is more variable, and may be FEES, modified BaS, ENT eval, or head imaging. Upper endoscopy is less helpful here.

• Familiarity with the common causes of dysphagia and their treatment is essential for both PCP’s and GIs

Thank You!