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    CONTINUING EDUCATION Esophageal Motor Disorders: Achalasia and Esophageal Spasm Column Editor: Lorna Schumann, PhD, APRN-BC, NP-C, CCRN, ARNP, FNP, ACNP, FAANP. Purpose ‘To define the esophageal motor disorders of achalasia and esophageal spasms and describe their presentation in the clinical seting. Data Sources Selected research-based articles, textbooks, and expert opinion. A case study is presented. Conclusions ‘The presentation of esophageal motor disor- ders may not be clear, particularly when the presenting symptom is chest pain. Determining whether the pain is cardiac or igestive in origin is crucial Implications for Practice Progressive dysphagia for both solids and liq- uids is the major symptom of achalasia; other symptoms include regurgitation, chest pain, and nocturnal cough. Diffuse esophageal spasm typically causes substernal chest pain with nonprogressive dysphagia and odynopha- gia for both liquids and solids. Dysphagia related to esophageal motility is characterized by a sensation of swallowed food “sticking” in the throat or chest: there is no problem initat- ing the act of swallowing. Key words Esophageal motor disorders; achalasia; diffuse esophageal spasms; esophageal chest pain. | Author Susan Lee Strader, RN, MSN, graduated May ‘of 2000 from Florida Atlantic Universiy in Boca Raton, Florida. She is a Ceriied Family Nurse Practitioner in private practice in Vero Beach, Florida. Contact Ms. Strader by E- ‘mail: SLeestrader@aol.com VOLUME 13, ISSUE 11, NOVEMBER 2001 Susan Lee Strader, RN, MSN, FNP-C INTRODUCTION The immediate thought when a patient complains of chest pain in the clinical seting is angina or possible myocardial infarction. ‘The nurse pract tioner (NP), however, must bear in mind other possible conditions in the f= ferential diagnosis of chest pain. Two such possibilities are the esophageal ‘motor disorders of achalasia and esophageal spasms. The purpose of this arti- cle isto define these conditions and theie presentations in the clinical setting and alert NPs to be aware of the defining characteristics of esophageal motor disorders in patients with chest pain. By understanding the pathophysiology of this disorder, the NP can provide prompt relief for the discomfort associ- ated with esophageal motor disorders and also spare the patient from unncc- cesary testing, financial strain and worry. Substernal chest pain or rightness that radiates to the arm or back and responds promptly to nitroglycerin is a common presentation of esophageal motor disorders. Although dysphagia is typically considered a hallmark of any condition involving the esophagus, his classic anginal type pain may be the only presenting symptom carly in the course of esophageal motor disorders. There are a number of esophageal motor disorders; achalasia and diffuse «esophageal spasms are the most common that are frequently confused with coronary events. Achalasia is a disorder of esophageal motility involving the body of the «sophagus and the lower esophageal sphincter (LES). It is characterized by a decreased number of ganglion cells in Aucrbach’s myenteric plexus, which causes diminished or absent peristalsis and failure of the LES to relax. As the disease progresses, the esophagus becomes increasingly dilated until an entire meal may be lodged in its lumen. The onset of achalasia is usually insidious; the most common sensation is that of food “sticking” in che lower esophagus Pain and regugitation are common (Gorvoll, & Mulley, 2000). Achalasia is a rare disease of uncertain etiology with a variety of treatment options. It has been reported to occur in all age groups with no. gender predilection and has been known to exist for hundreds of years. The term achalasia, which means lack of relaxation, was used originally by Hurst in 1914, and caught on in che late 1950s, Sit Thomas Willis performed the frst dilation of a patient with achalasia in 1674 using a whalebone. Heller per formed the frst surgical procedure for achalasia in 1913. Pneumatic dilation has been available since the 1920s (Waring, 1999). Current treatment consists of dilation of milder cases and an esophagomyoromy for more intractable cases (Rakel, 1995). This condition has also been known as simple ec cardiospasm, esophageal aperistasis, and megaesophagus (Merck Manual, 1999), 502 Diffse esophageal spasm is a generalized neurogenic disorder of esophageal motility in which phasic nonpropulsive contractions replace normal peristalsis and, in some cases, LES malfunctions ‘oceut, The etiology is unclear, but is thought to be a disturbance of vagal tone. Histologically, there is an extreme degree of mus- cular hypertrophy. Diagnosis is based largely on radiologic stud~ ies that document the functional disturbance, Patients complain of dysphagia and substernal pain that may radiate to the jaw or arms, in some cases mimicking angina. Treatment is similar to that for achalasia (Rakel, 1995). ‘There are also motor disorders of the esophagus that are vari ants of achalasia and diffuse spasms. Some patients show symp- tom complexes that ft neither typical achalasia nor typical diffuse spasm. Some of the complexes have been called “vigorous achala- sia" because they have both the fluid retention and aspiration of achalasia and the severe pain and spasm of diffuse spasm. This phrase was introduced in the 1960s to describe this arypical clin- al presentation of the disease. Vigorous achalasia was defined by the occurrence of retrosteral pain, demonstration of strong, repetitive contractions on esophageal manometry, and radi- ‘ographic findings indicative of diffuse esophageal spasm. Debate continues as to whether this atypical presentation is indeed a dis- inct entity, whether concomitant chest pain represents an carly ot late manifestation of disease, or whether therapy can alter its clinical course (Eckardt, Seauf, & Bernhard, 1999). PATHOPHYSIOLOGY Normally, the LES maintains its tone through myogenic properties. Lower esophageal sphincter relaxation is related to inhibition by nonadrenergic, noncholinergic neurons. Nitric oxide is largely, if not entirely, responsible for this (Waring, 1999). Thete is a loss of inhibitory ncurons in the LES that release nitric oxide as the primary neurotransmitter (Pasticha, 1999), Esophageal body peristalsis is created by @ combination of ‘excitatory input from cholinergic neurons, as well as inhibition by the nonadrenergic, noncholinergic neurons. In achalasia, there is a loss of these inhibitory neurons, leading to impaired relaxation of the LES and loss of peristaltic contractility in the «esophageal body; this change may be caused by a malfunction of the myenteric plexus of the esophagus that results in denervation of esophageal muscle. Histopathologically, myenteric inflamma- tion, loss of the ganglion cells, loss of the interstitial cells of Cajal, and fibrosis of the myenteric nerves are found. Clinically, there is an absence of peristalsis in the esophageal body. This is now the sole erterion needed to make the diagnosis of achalasia although impaired LES relaxation is common and often tespon- sible for the symproms of dysphagia (Waring, 1999), Diffuse esophageal spasm is characterized by simultaneous contractions throughout the entire esophagus intermixed with periods of normal peristaltic activity (Goroll & Mulley, 2000) ‘Transient spasms may be related to psychological distress. The condition i also commonly an early symptom of neuromuscular disease, in which case it progresses to achalasia 503 DIFFERENTIAL DIAGNOSIS Esophageal motor disorders can be related so transfer dys- phagia, myasthenia gravis, multiple sclerosis, Parkinson's disease, amyotrophic lateral sclerosis (ALS), scleroderma, and diffuse ‘esophageal spasm. When the presenting symptom is substernal chest pain, the differential diagnosis would also include coronary artery disease, mitral valve prolapse, hypertrophic cardiomyopa~ thy, pericarditis, angina, and myocardial infarction (Seller, 1996). Gastrointestinal conditions can also cause similar symp- toms, most commonly gas entrapment syndrome or peptic «esophagitis, which is often precipitated by overeating and recum- bency. ‘The pain frequently awakens the patient at night. Scleroderma is usually accompanied by a history of Raynaud’ phenomenon and symptoms of gastroesophageal reflux disease (GERD). ‘Chest pain due to esophageal spasm can be precipitated by swallowing ccrtain substances, particularly cold liquids and any- thing thar stimulates the production of endogenous gastrin. Thus, chest pain that follows ingestion of alcohol should suggest the possibility of esophageal spasm. If che pain is exacerbated or reproduced when pressure is applied over the painful area, cos- tochondriis, Tietze syndrome, rib fractures, chest wall syn: drome, and nonspecific myositis are more likely. Reproduction or exacerbation of pain with chest wall pressure does not usually ‘occur in patients with ischemic eardiac pain or psychiatric disor- ders (Selle, 1996). SIGNS AND SYMPTOMS Achalasia occurs at any age but usually begins between ages 20 and 40. Onset is insidious and progression is gradual over ‘months or years. Progressive dysphagia for both solids and lig- uids is the major symptom; other symproms include regurgita- tion, chest pain, and nocturnal cough. Increased pressure at the LES produces obstruction with secondary dilation of the esoph- agus. Nocturnal regurgitation of undigested food occurs in about 33% of patients and may cause cough, pulmonary aspira- ion with lung abscess, bronchiectasis, or pneumonia. Chest pain is less common but may occur on swallowing or spontaneously, Weight loss is usually mild to moderate; when weight loss is pro- nounced, particularly in the elderly patient in whom the symp- coms of dysphagia have developed rapidly, achalasia secondary to a tumor of the gastroesophageal junction should be considered (Rakel, 1995). Diffuse esophageal spasm typically causes substernal chest pain with nonprogressive dysphagia and odynophagia (painful swallowing) for both liquids and solids (Goroll, & Mulley, 2000). The pain is similar to that of coronary artery spasm (i... the tightness of anginal type pain); it may be severe enough t0 awaken the patient from sleep: it may be triggered or aggravated by swallowing very hot or cold liquids. Thus, in addition to the quality of the pain, the chronology of the symptoms isan impor- tant aspect of the history. Prompt treatment is needed because cover time, this disorder may evolve into achalasia (Rakel, 1995). JOURNAL OF THE AMERICAN ACADEMY OF NURSE PRACTITIONERS, ‘The etiology of this pain is unknown. Occasionally, such episodes of chest pain may actually mask mote characteristic symptoms, such as dysphagia, leading to a delay in diagnosis. In a study of chest pain in 101 patients diagnosed with acha- lasia, angina-like retrosternal pain was present in 50% of the patients (Eckerde et al., 1999). Over the clinical course of the disease, neither manometric nor radiographic studies were able to predict the occurrence of this symptom. Moreover, while pneumatic dilatation or myotomy effectively reduced dysphagia, such conventional cherapy had litle effect on the incidence of retrosternal pain. Chest pain did diminish in frequency over the course of a patient lifetime, suggesting the occurrence of this symptom will likely spontancously decrease with advancing age in many patients, When chest pain is the presenting symptom, the history should be focused on eliminating cardiae pathology and clarify ing risk factors. When the presenting symptom is dysphagia, the key point for clarification is whether or not the problem occurs im the esophageal phase of swallowing. Dysphagia related co «sophageal motility is characterized by a sensation of swallowed food “sticking” in the throat or chest; there is no problem ini ating the act of swallowing (Spickcr, 2000). ‘The physical examination should include skin assessment for lesions of scleroderma, and careful assessment of the mouth, neck, and oropharynx for signs of masses or lesions that would interfere with swallowing. A cardiopulmonary assessment should focus on cardiac status. Palpation of the chese wall and ribs is done ro rule out musculoskeleral involvement. Because of the common association with neurological conditions, a thorough neurological examination, particularly focused on motor func: tions, is essential. Finally, a stool for occult blood should be done. Laboratory studies are usually necessary and based upon find- ings in the history and physical examination. If ischemic cardiac disease is suspected, ruling out an acute coronary event is the highest priority. Once candiae disease is ruled out, diagnostic studies should be done to evaluate Famine function, ‘The most sensitive test to view the esophagus and demon- strate the absence of an obstruction is a barium sallow (Goroll & Malley, 2000). Although evidence characteristic of motor dis- orders (absent or abnormal peristaltic contractions during swal- lowing) may be seen in fluoroscopic studies, the specificity of this testis low, especially in early disease. The classic radiologic sign of achalasia is a dilated esophagus, frequently enormous, with a narrowed and beak-like shape at the LES. ‘The hallmark of esophageal spasms on a barium swallow includes poor pro- gression of a bolus and disordered, simultancous contractions or tertiary contractions. Severe spasms may mimic diverticula but will vary in size and position If the Nuoroscopic study is inconclusive, expphagea! maname- sry might be suggested. Characteristic findings include aperistal- sis, increased LES pressure, and incomplete sphincteric relax- VOLUME 13, ISSUE 11, NOVEMBER 2001 ation with swallowing. Manometry is often inconclusive and its routine use has been questioned (Goroll, & Mulley, 2000). In the case of suspected obstruction, exephagoscopy and biopsy are necessary to determine the location and extent of obstruction, as, well as to rule out malignancy. Achalasia must be differentiated from a distal stenosing carcinoma and a peptic stricture, partic~ ularly in the patient with scleroderma in whom esophageal manometry may also show aperistalsi Esophageal scintigraphy may be a sensitive method of detect- ing impaited bolus transport, but esophageal manometry pro- vides the most sensitive and specific description of the spasms. Contractions are usually simultaneous, prolonged or multipha- sic, and possibly of very high amplitude. For evaluating patients with nondiagnostic baseline studies, use provocative tess with drugs (eg edrophonium chloride 10 mg intravenously) or food, which may disclose a propensity 1o symptomatic spasms. Studies show lower esophageal sphincter pressure of relaxation, impairment in 30% of patients (Merck Manual, 1999) Hypertensive lower esophageal sphincter (HLES) was first described in 1960 by Code, Schlegel, and Kelley. One of the ‘common causes of the HLES is achalasia; however, HLES not associated with achalasia is occasionally found, but its clinical implications are poorly understood (Code et al., 1960). The pre~ dominant symptoms in nonachalasia HLES include chest pain (64%%-100%) and dysphagia (18%-73%; Bassotti, Alumni, & Cocchien 1992). Psychological symproms have been examined as possible causes or contributors to HLES (Waterman, Dalton, & Ott, 1986), Seventy-five percent of HLES patients without a diagnosis of achalasia had abnormal psychological scores when assessed for levels of amxiety and somatization. A study by Katada et al. (1996) attempted to determine whether HLES was metely an expression of the outer range of the normal distribution curve of LES pressure, or whether it has clinical or pathophysiological significance, as well as the rela tionship between the HLES and GERD or achalasia. ‘The rela- tionship of HLES and achalasia was measured through ‘sophageal manometry and pH monitoring and their changes in response to treatment. Findings indicated that HLES is a mano- metic finding that may present with either dysphagia ot chest pain i¢ may be an isolated finding or may be associated with achalasia, disordered esophageal body motility, previous Nissen, fundoplication, GERD, or carcinoma at the cardia. Esophageal ‘manometry is essential for diagnosis and 24-hour pH studi are useful in the evaluation of these patients so that appropriate therapy can be initiated. Only a small proportion of the patients, in this study required surgical treatment. Achalasia was the most commonly associated finding: however, 74% of all HLES patients did noc have achalasia, ‘The “nutcracker” esophagus, characterized by very long and very high amplitude contractions in the distal esophagus, was found in 13% to 56% of HLES patients Ina case report (Triadafilopoulos, & Tsang, 1996), a patient with diffuse esophageal spasm predictably developed dysphagia and chest pain upon inhaletion of perfume of other strong ‘odors. Through esophageal scintigraphy, the authors determined that olfictory stimulation eriggered the spasms, which are medi- 508 ated through the vagus nerve. Furthermore, the spasms were ameliorated by the administration of ipratropium bromide, an anticholinergic agent. This case provided hope for a better understanding of the pathophysiology of diffuse esophageal spasm and the porential for a new and safe therapy in the man- agement of this challenging condition. Regular use of anti- cholinergics, however, has proved disappointing (Goroll & Mulley, 2000), REVIEW OF LITERATURE RELATED TO CURRENT MANAGEMENT AND TREATMENT Current research into treatment approaches is varied. ‘The aim of treatment is to reduce the pressure and thus the obstruc- tion at the LES. Referral for forceful or pneumatic dilation of the sphincter is indicated initially. Although invasive and not a cure, dilation has been the most effective treatment. Sustained response is obtained! in 50% of patients intially; with a second dilatation, 70% will achieve sustained response. The remaining 30% will ope for surgery or silent suffering (Pasricha, 1999). Complications of esophageal ruprure and secondary medisinitis requiring surgery occur in less than 19% of patients (Merck Manual, 1999), Heller myotomy, in which the muscular fibers in the lower

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