Swallowing Difficulty and Pain 12

12

Swallowing Difficulty and Pain

John P. Sutyak

Objectives

1. To distinguish between dysphagia and odynophagia.

2. To discuss the anatomy and physiology of the swallowing structures and mechanism, including the physiologic lower esophageal sphincter.

3. To discuss pertinent clinical history and physical examination findings as they relate to structural and functional pathology.

4. To develop a focused evaluation plan based on history and physical exam findings.

5. To describe various therapeutic options for patients with neurologic, neoplastic, reflex- mediated, and dysmotility-mediated disorders.

Cases

Case 1

A 58-year-old man presents to your office complaining of difficulty in swallowing. He has sustained a weight loss of 15 pounds over the past 3 months.

Case 2

A 39-year-old woman presents to your office with burning chest pain, rapidly worsening over 3 years. Recent treatment with an H2 blocker has relieved her symptoms.

Case 3

A 72-year-old woman presents to your office with difficulty in swal- lowing for decades. She describes a recent, worsening sensation of sub- sternal fullness.

200

Introduction

The swallowing mechanism is a complex interaction of pharyngeal and esophageal structures designed for the seemingly simple purpose of propelling food to the stomach and of allowing the expulsion of excess gas or potentially toxic food out of the stomach. Initial evaluation of a patient complaining of difficulty (dysphagia) or pain (odynophagia) with swallowing involves a thorough, focused history and a physical examination. Appropriate diagnostic tests then can be employed. The advent of esophageal motility and pH studies has permitted correla- tion of physiologic data to the anatomic information obtained through radiographic and endoscopic studies. Myriad primary and secondary processes may affect the swallowing mechanism. Some may be pathologic and the cause for the patient’s symptoms. Others may only confuse the diagnosis, having no relationship to the patient’s complaints. In evaluating swallowing difficulty and pain, it is extremely important to relate symptoms to diagnosis, as inappropri- ate therapy actually may worsen the patient’s symptoms or initiate new complications.

Anatomic Considerations

The esophagus is a muscular tube extending from the cricoid to the stomach. It is composed of a mucosal layer, a submucosa, and a double outer muscular layer (Fig. 12.1). No serosa is present on the esopha- gus, resulting in a structure that has less resistance to perforation, infiltration of malignant cells, and anastomotic breakdown follow-

Ganglia of submucosal plexus (Meissner)

Ganglia of myentetric plexus [Auerbach]

Epithelium

Submucosa

Muscularis mucosa

Lamina propria

Longitudinal muscle layer Esophageal gland

Muscularis externa

Figure 12.1. Cross section of the esophagus showing the layers of the wall (Reprinted from Jamieson GG, ed. Surgery of the Esophagus. Edinburgh:

Churchill Livingstone, 1988. Copyright © 1988 Elsevier Ltd. With permission from Elsevier.)

ing surgery. Three layers compose the esophageal mucosa: a stratified, nonkeratinizing squamous epithelial lining; the lamina propria (a matrix of collagen and elastic fibers); and the muscularis mucosae. The squamous epithelium of the esophagus meets the junctional columnar epithelium of the gastric cardia in a sharp transition called the Z-line, typically located at or near the lower esophageal sphincter (Fig. 12.2).

Although the upper third of esophageal muscle is skeletal and the distal portion is smooth, the entire esophagus functions as one coordi- nated structure. Contraction of the longitudinal muscle fibers of the esophageal body produces esophageal shortening. The inner circular muscle is arranged in incomplete rings, producing a helical pattern that, on contraction, produces a corkscrew-type propulsion. Muscle layers are of uniform thickness until the distal 3 to 4 cm, where the inner circular layer thickens and divides into incomplete horizontal muscu- lar bands on the lesser gastric curve and oblique fibers that become the gastric sling fibers on the greater curve. Although no complete circu- lar band exists as an anatomic lower esophageal sphincter (LES), it is the area of rearranged distal circular fibers that corresponds to the high-pressure zone of the LES. In an adult, the cricopharyngeal muscle is located approximately 15 cm from the incisors, and the gastroesophageal junction is located approximately 45 cm from the incisors.

The esophagus has abundant lymphatic drainage within a dense submucosal plexus. Because the lymphatic system is not segmental, lymph can travel a long distance in the plexus before traversing the muscle layer and entering regional lymph nodes. Tumor cells of the

Figure 12.2. Anatomic relationships of the distal esophagus and phreno- esophageal ligament. (Reprinted from Gray SW, Skandalakis JE, McClusky DA.

Atlas of Surgical Anatomy for General Surgeons. Baltimore: Williams &

Wilkins, 1985, with permission.)

upper esophagus can metastasize to superior gastric nodes, or a cancer of the lower esophagus can metastasize to superior mediastinal nodes.

More commonly, the lymphatic drainage from the upper esophagus courses into the cervical and peritracheal lymph nodes, while that from the lower thoracic and abdominal esophagus drains into the retrocar- diac and celiac nodes.

The esophagus has both sympathetic and parasympathetic innerva- tion. The sympathetic supply is through the cervical and thoracic sym- pathetic chains as well as through the splanchnic nerves derived from the celiac plexus and ganglia. Parasympathetic innervation of the pharynx and esophagus is primarily through the vagus nerve. The vagal trunks contribute to the anterior and posterior esophageal plexi.

At the diaphragmatic hiatus, these plexi fuse to form the anterior and posterior vagus nerves. A rich intrinsic nervous supply called the myenteric plexus exists between the longitudinal and circular muscle layers (Auerbach’s plexus) and in the submucosa (Meissner’s plexus).

Physiology of Swallowing

Passage of food from mouth to stomach requires a well-coordinated series of neurologic and muscular events. The mechanism of swal- lowing is analogous to a mechanical system consisting of a piston pump (tongue) filling and pressurizing a cylinder (hypopharynx) con- nected to a three-valve (soft palate, epiglottis, and cricopharyngeus) system that propels material into a worm drive (esophagus) with a single distal valve (LES). Failure of the pump, valves, or worm drive leads to abnormalities in swallowing such as difficulty in propelling food from mouth to stomach or regurgitation of food into the oral pharynx, nasopharynx, or esophagus.

The LES acts as the valve at the end of the esophageal worm drive and provides a pressure barrier between the esophagus and stomach.

Although a precise anatomic LES does not exist, muscle fiber architec- ture at the esophagogastric junction explains some of the sphincter- like activity of the LES. The resting tone of the LES is approximately 20 mm Hg. With initiation of a swallow, LES pressure decreases, allow- ing the primary peristaltic wave to propel food into the stomach. A pharyngeal swallow that does not initiate peristaltic contraction also leads to LES relaxation, permitting gastric juice to reflux into the distal esophagus. The coordinated activity of the pharyngeal swallow and LES relaxation appears to be in part vagally mediated. The intrin- sic tone of the LES can be affected by diet and medications as well as by neural and hormonal mechanisms (Table 12.1).

History and Physical Examination

A precise medical history is essential to obtaining an accurate diag-

nosis of swallowing difficulties. Does the patient suffer from difficulty

in swallowing (dysphagia) alone, or is pain with swallowing

(odynophagia) a primary or associated complaint? If pain is the

primary complaint, elucidate its nature (squeezing, burning, pressure), aggravating factors (temperature and type of food, liquids and/or solids, medications, caffeine, alcohol, position, size or time of meals), relieving factors (medications, position, eructation, emesis), time course (lifelong, several years, slow progression, worsening, stable, episodic, constant), and associated factors (patient age, weight gain or loss, presence of a mass in the neck, preexisting disease processes, chronic cough, asthma, recurrent pneumonia, tobacco and alcohol use).

When dysphagia is not associated with pain or with pain as a minor complaint, questioning should still follow the pattern above (nature, aggravating factors/relieving factors/time course/associated factors) and include questions focusing on disease progression (difficulty with solids at first, then difficulty with liquids, or difficulty with both solids and liquids).

Appropriate identification and evaluation of esophageal abnor- malities rely on a thorough understanding of the patient’s symptoms and of how these symptoms relate to various disorders. Table 12.2 lists symptoms that may be attributable to esophageal disorders. Occa- sional symptoms are common and of no pathologic significance.

However, frequent and persistent symptoms should prompt further investigation. A useful method is to determine how much the symp- toms have affected the patient’s lifestyle in terms of activity, types of food eaten, interruption of employment, and effects on family life.

A precise relationship of symptoms to diagnosis is essential in order to avoid inappropriate and dangerous treatment.

Table 12.1. Neural, hormonal, and dietary factors thought to affect lower esophageal sphincter (LES).

Increase LES pressure Cholinergics Prokinetics a-Agonists b-Blockers Gastrin Motilin Bombesin Substance p

Decrease LES Pressure a-Blockers

b-Blockers

Calcium channel blockers Cholecystokinin

Estrogen Progesterone Somatostatin Secretin

Caffeine (chocolate, coffee) Fats

Source: Reprinted from Smith CD. Esophagus. In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.

Although the majority of preliminary diagnostic information is obtained through a focused history, physical examination can add important clues to the diagnosis, particularly when malignancy is of concern. Signs of chronic or acute weight loss, lymphadenopathy, tobacco abuse, ethanol abuse, portal hypertension, and any abnormal neck or abdominal masses should be noted on physical examination.

Further history and examination findings are covered under the spe- cific diagnoses that follow later in this chapter.

Diagnostic Tests for Evaluation of Esophageal and Swallowing Disorders

Several diagnostic tests are available to evaluate patients with dys- phagia/odynophagia. These tests, listed in Table 12.3, can be divided into tests to assess structural abnormalities, tests to assess functional abnormalities, tests to assess esophageal exposure to gastric content, and tests to provoke esophageal symptoms. Gastric motility and biliary disease may need to be evaluated as well to rule out gastroparesis or gallbladder disease. See Algorithm 12.1 for swallowing evaluation.

Assessment of Structural Abnormalities

Plain chest x-ray films may reveal changes in cardiac silhouette or tra- cheobronchial location, suggesting esophageal disorders. Herniation of Table 12.2. Patient symptoms and likely etiologies.

Symptom Definition Likely etiology

Heartburn Burning discomfort behind breast bone Gastroesophageal reflux Bitter acidic fluid in mouth (GER)

Sudden filling of mouth with clear/salty fluid

Dysphagia Sensation of food being hindered in Motor disorders passage from mouth to stomach Inflammatory process

Diverticula Tumors

Odynophagia Pain with swallowing Severe inflammatory process

Globus sensation Lump in throat unrelated to swallowing

Chest pain Mimics angina pectoris GER

Motor disorders Tumors

Respiratory symptoms Asthma/wheezing, bronchitis, hemoptysis, GER

stridor Diverticula

Tumors ENT symptoms Chronic sore throat, laryngitis, halitosis, GER

chronic cough Diverticula

Rumination Regurgitation of recently ingested food Achalasia

into mouth Inflammatory process

Diverticula Tumors

Source: Reprinted from Smith CD. Esophagus. In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.

the stomach and other structures above the diaphragm often is identi- fied by abnormal gas patterns on chest radiographs. A simple and often specific diagnostic test for esophageal disease is a contrast esopha- gogram or barium swallow. Structural abnormalities, including diver- ticula, narrowing or stricture, ulcers, and hiatal or paraesophageal hernias, all can be demonstrated. Use of fluoroscopy with videotaped recordings of both a liquid and solid contrast swallow increases accu- racy in identifying subtle abnormalities. Abnormalities of esophageal motility or gastroesophageal reflux can be seen during a barium swallow, but these disorders are more appropriately diagnosed using other tests. The value of attempting to elicit reflux is questionable because 20% of normal individuals have radiologic reflux. The timed barium esophagogram is a simple test of esophageal function. After ingestion of a premeasured amount of barium, spot films are taken at 1-, 2-, 5-, 10-, and 20-minute intervals. This test allows quantification of esophageal emptying and is useful for the evaluation of motility dis- orders. Computed tomography (CT) scan of the chest also may be useful in assessing lesions identified with barium swallow or endoscopy thought to be malignancies and in assessing the presence of complex paraesophageal hernias. A modified fluoroscopic barium study in the lateral projection may be useful in identifying mechani- cal disorders of the pharyngeal swallowing mechanism.

Endoscopy

Most patients with swallowing disorders or pain should undergo esophagoscopy. Patients with dysphagia should undergo esoph- agoscopy, even in the presence of a normal barium swallow. A barium swallow performed before esophagoscopy helps the endoscopist to focus on any subtle radiographic findings and helps to prevent Table 12.3. Assessment of esophageal function.

Condition Diagnostic test

Structural abnormalities Barium swallow Endoscopy Chest x-ray CT scan

Cinefluoroscopy Endoscopic ultrasound

Functional abnormalities Manometry (stationary and 24 hour) Transit studies

Esophageal exposure to 24-hour pH monitoring gastric content

Provoke esophageal Acid perfusion (Bernstein)

symptoms Edrophonium (Tensilon)

Balloon distention

Others Gastric analysis

Gastric emptying study Gallbladder ultrasound

Source: Reprinted from Smith CD. Esophagus. In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.

endoscopic misadventures with anatomic abnormalities such as esophageal diverticula.

For the initial assessment, the flexible esophagoscope allows a safe, thorough assessment that can be performed quickly in an outpatient setting with high patient tolerance and acceptance. The mucosa of the entire esophagus, stomach, and duodenum should be inspected care- fully. Any areas of mucosal irregularity or abnormality should be photodocumented and biopsied. Retroflex views within the stomach of the gastroesophageal junction should note the presence of hiatal hernia.

The location of the transition from squamous mucosa to columnar gastric mucosa (Z-line) should be noted as the distance from the incisors to this point of transition. Known esophageal diverticula can be investigated endoscopically; however, great care should be taken because diverticula can be perforated easily.

Identify if oropharyngeal dysphagia is likely History

Symptoms Metabolic disease

Medications (anticholinergics, phenothiazines, etc.) Physical exam

Identify features of underlying systemic or metabolic disorders Determine neurologic status

Identify pulmonary and nutritional sequelae of dysphagia

Identify structural etiologies: Zenker’s diverticulum, neoplasm, infection, etc.

Endoscopic examination Radiologic examination

Videofluoroscopy Cineradiography

Determine if dysphagia is amenable to therapy Surgical correction

Swallowing modification techniques Diet modification

No Yes

Yes

Algorithm 12.1. Algorithm for swallowing evaluation (dysphagia).

Rigid esophagoscopy rarely is indicated and remains a tool used primarily in the operating room when cricopharyngeal or cervical esophageal lesions prevent passage of a flexible scope, when biopsies deeper than those obtainable with flexible endoscopy are needed to stage disease and plan resective therapy, and for the removal of foreign bodies.

Endoscopic ultrasound (EUS) is a newer technique. It allows char- acterization and staging of esophageal lesions by imaging the layers of the esophageal wall and surrounding structures in order to identify depth of tumor invasion and periesophageal lymphadenopathy, and it allows EUS-guided fine-needle aspiration of lymph nodes.

Assessment of Functional Abnormalities

Esophageal manometry has become widely available to examine the motor function of the esophagus and the LES (Fig. 12.3). Manometry is indicated when a motor abnormality is suspected on the basis of symptoms of dysphagia or odynophagia and when the barium swallow and esophagoscopy do not show an obvious structural

mm Hg

Figure 12.3. Esophageal manometry. Swallowing requires coordinated relax- ation and contraction of the upper and lower esophageal sphincters (UES, LES) and adequate peristalsis of the esophageal body. (Reprinted from Rice TW.

Esophagus. In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery:

Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.)

abnormality. Manometry also is indicated when esophageal dys- motility or LES dysfunction is either associated with or a conse- quence of other structural abnormalities such as hiatal and paraesophageal hernias. Manometry is essential to confirm diagno- sis of primary esophageal motility disorders such as achalasia, diffuse esophageal spasm, nutcracker esophagus, and hypertensive lower esophageal sphincter. It may be useful in identifying nonspecific esophageal motility disorders and motility abnormalities secondary to systemic diseases of scleroderma, dermatomyositis, polymyositis, or mixed connective tissue disease. In patients with symptomatic gas- troesophageal reflux, manometry is useful particularly in confirming inadequate function of the LES, assessing preoperative esophageal clearance mechanisms, and determining operative or nonoperative therapy. Esophageal manometry is performed by passing a catheter nasally into the stomach while measuring pressure through a pressure- sensitive transducer. Pharyngeal, esophageal body, and LES function can be recorded and assessed using computer-based analysis software.

Assessment of Esophageal Exposure to Gastric Content

Ambulatory 24-hour esophageal pH monitoring has become the stan- dard for quantitating esophageal exposure to acidic content and relat- ing symptoms to esophageal pH. A multichannel pH electrode is positioned with at least two sensors proximal to the manometrically identified LES. Gastric pH can be measured along with esophageal pH.

While the patient continues a normal routine, including eating and the usual activities, the pH is recorded throughout a 24-hour cycle. The patient maintains a diary, recording body positions, meals, and symp- toms, so that esophageal pH can be correlated with symptoms. At the completion of the test, the results are tallied and compared to normal values for esophageal acid exposure. The study can be performed in the presence or absence of acid-reducing medications in order to deter- mine the effectiveness of the medication.

Twenty-four-hour pH monitoring is indicated for patients who have typical symptoms of gastroesophageal reflux, for patients for whom other diagnostic tests are equivocal, for patients with atypical symptoms of gastroesophageal reflux such as noncardiac chest pain, persistent cough, wheezing, and unexplained laryngitis, or for patients with previously failed esophageal or gastric surgery with recurrent symptoms.

Provocation of Esophageal Symptoms

Three tests previously were used to identify a relationship between symptoms and esophageal exposure to acid or motor abnormalities:

the acid perfusion test (Bernstein, 0.1 N HCl infusion), edrophonium (Tensilon, acetylcholinase inhibitor precipitates contractions), and balloon distention tests. Perfusion tests are placebo controlled.

Ambulatory pH testing and esophageal manometry have made these

tests primarily of historical and academic interest.

Evaluation of Gastric Motility and Biliary Disease

In evaluating a patient with esophageal symptoms, it also is impor- tant to consider the impact of gastroduodenal dysfunction on lower esophageal function as well as other common gastrointestinal prob- lems that can mimic esophageal disease. A gastric emptying study and/or right upper quadrant ultrasound may be indicated in patients with symptomatology suggestive of esophageal disorders in order to rule out gastroparesis or gallbladder disease.

Specific Conditions

Tumors

Malignant Esophageal Tumors

Overview: The majority of esophageal neoplasms are malignant. Less than 1% of tumors are benign. Esophageal cancer is among the top 10 leading causes of cancer deaths in the United States and is increasing in incidence. Late presentation and early spread lead to a poor prog- nosis. The overall 5-year survival rate is 8% to 12%. Although squa- mous cell carcinoma previously accounted for 90% to 95% of reported esophageal malignancies, the incidence of adenocarcinoma has increased dramatically in the past two decades and now accounts for at least 40% of all malignancies. This relative change may reflect the increased use of flexible endoscopy and closer surveillance of asymp- tomatic patients who are at risk of developing esophageal carcinoma.

Other cell types of esophageal malignancy are extremely rare.

Squamous cell carcinomas are distributed equally among the upper, middle, and lower thirds of the esophagus. Adenocarcinomas most commonly are found in the lower third of the esophagus. Most ade- nocarcinomas are thought to arise in columnar cell Barrett’s mucosa.

Alcohol consumption and tobacco use are well-established factors for the development of esophageal carcinoma. Other risk factors for esophageal cancer include achalasia, radiation esophagitis, caustic esophageal injury, infection (human papilloma virus), Plummer–

Vinson syndrome, leukoplakia, esophageal diverticula, ectopic gastric mucosa, and the inherited condition of familial keratosis palmaris et plantaris (tylosis).

Diagnosis: The vast majority of esophageal carcinomas are clinically occult and present well after disease progression prevents cure. As in Case 1, the classic history is a patient who presents with dysphagia and weight loss; chest pain, abdominal pain, and gastrointestinal (GI) blood loss are described less frequently (Table 12.4). Most patients experience dysphagia an average of 2 to 4 months before presentation.

Unfortunately, dysphagia almost uniformly indicates extensive disease and incurability.

The initial study should be a barium swallow; this most frequently

reveals distinct mucosal irregularity, stricture, a shelf in the lower

esophagus, or rigidity. Upper esophageal endoscopy allows visualiza-

tion of the affected area and biopsy to confirm the diagnosis.

Staging: The stage of esophageal cancer is determined by the depth of penetration of the primary tumor (T) and the presence of lymph node (N0, N1) and distant organ metastasis (M0, M1). The TNM descrip- tors can be grouped into stages with similar behavior and prognosis.

Clinical (cTNM) preoperative staging determines the intent and extent of subsequent treatment, either curative or palliative. Initial staging includes a careful physical examination for the sequelae of esophageal cancer (weight loss, supraclavicular adenopathy, pleural effusion), routine blood tests, and CT scan of the chest and abdomen.

Bronchoscopy is indicated for midesophageal tumors because of their propensity to invade the trachea and left mainstem bronchus.

The recent development of EUS has improved staging by allowing the depth of invasion into the esophageal wall to be determined accu- rately and by allowing the surrounding lymph node involvement to be identified and biopsied.

Weight loss greater than 10% has been shown to be associated with a significantly poorer outcome in patients with operable esophageal cancer. Clinical staging categorizes patients into two groups: those with potentially curable disease and those with metastatic disease (disease outside of the local or regional area) in whom palliation is currently the only treatment option.

Treatment: The overall prognosis for esophageal carcinoma is bleak. An overall 5-year survival for esophageal cancer patients was reported in only 4% after surgical resection (surgical mortality, 29%) and in only 6% after radiation therapy. The treatment of esophageal cancer is gen- erally a palliative practice, and cure is a chance occurrence. However, precise clinical staging allows treatment modification of patients with carcinoma of the esophagus. Surgical, radiation, and chemotherapy therapies are possible, with optimal outcomes often utilizing a combi- nation approach.

Based on reviews of current literature available on the multimodal- ity management of patients with esophageal carcinoma, treatment pro-

Table 12.4. Presenting symptoms of esophageal carcinoma.

Symptom Incidence (%)

Dysphagia 87

Weight loss 71

Substernal or epigastric pain/ 46 burning

Vomiting or regurgitation 28

Aspiration pneumonia 14

Palpable cervical nodes 14

Hoarseness 7

Coughing and choking 3

Source: Reprinted from Smith CD. Esophagus. In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.

tocols have been developed and are the basis of published practice guidelines for esophageal cancer (see Algorithms 12.2 and 12.3).

Surgery: Esophagectomy with gastric or colon replacement is the treatment of choice for small tumors confined to the esophageal mucosa or submucosa. Surgical treatment for resectable esophageal cancers results in 5-year survival rates of 5% to 20% and an operative

Technically resectable esophageal cancer (Stage 0–III)

If available, entry into clinical trial Surgery: esophagectomy

Curative resection Yes

(negative margins, no distant mets)

No (positive margins,

distant mets)

Adjuvant therapy:

Radiotherapy (5000 – 6000 cGy+

external boost)

Radiotherapy (4500 cGyt) +/–

+

Chemotherapy (2 cycles platinum/5-FU)

Palliative therapy:

Chemotherapy (2 cycles platinum/5-FU)

Algorithm 12.2. Management of technically resectable esophageal cancer, 5-Fu, 5-fluorouracil; mets, metastases. (Reprinted from Smith CD. Esophagus.

In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.)

1DeMeester TR, Bonavina L, Albertucci M. Evaluation of primary repair in 100 con- secutive patients. Ann Surg 1986;204:9–20. Donohue PE, Samelson S, Nyhus LM, et al.

The Nissen fundoplication. Effective long-term control of pathologic reflux. Arch Surg 1985;120:663–667. Ellis FH. The Nissen fundoplication. Ann Thorac Surg 1992;54:1231–1235. Grande L, Toledo-Pimentel V, Manterola C, et al. Value of Nissen fun- doplication in patients with gastro-oesophageal reflux judged by long-term symptom control. Br J Surg 1994;81:548–550. Johansson J, Johnsson F, Joelsson B, et al. Outcome 5 years after 360 degree fundoplication for gastro-oesophageal reflux disease. Br J Surg 1993;80:46–49. Luostarinen M, Isolauri J, Laitinen J, et al. Fate of Nissen fundoplication after 20 years. A clinical, endoscopical, and functional analysis. Gut 1993;34:1015–1020.

Macintyre IM, Goulbourne IA. Long-term results after Nissen fundoplications: a 5–15- year review. J R Coll Surg Edinb 1990; 35:159–162. Martin CJ, Cox MR, Cade RJ. Collis- Nissen gastrooplasty fundoplication for complicated gastrooesophageal reflux disease.

Aust N Z J Surg 1992;62:126–129. Mira-Navarro J, Bayle-Bastos F, Frieyro-Segui M, et al.

Long-term follow-up of Nissen fundoplication. Eur J Pediatr Surg 1994;4:7–10.

mortality of 2% to 7%. Once symptoms appear, most esophageal cancers have invaded adjacent structures or have spread to distant organs. In those cases in which significant obstructive symptoms exist, operative management often is the most effective means of relieving dysphagia and providing long-term palliation. In general, because esophageal cancer can have extensive and unpredictable spread longi- tudinally, it seems prudent to perform total esophagectomy, especially for those proximal- and middle-third lesions. Distal small lesions may be approached through the abdomen only, or resection for palliation alone can avoid total esophagectomy and its associated morbidity.

Nonsurgical Therapy: A randomized phase III study of chemotherapy with cisplatin, fluorouracil, and radiotherapy versus radiation therapy alone in patients with T1–3, N0–1, M0 esophageal carcinoma has demonstrated a survival advantage for patients receiving combined therapy (Table 12.5). Long-term follow-up of these patients reported a 5-year survival of 26% for combined therapy, while no patient receiv- ing radiation alone survived 5 years. A 5-year survival of 14% was seen

Unresectable esophageal cancer (Stage IV)

If available, entry into clinical trial

Palliative strategies

Chemotherapy if : a) no dysphagia

b) poor operative candidate c) locally advanced

d) metastatic disease e) good performance status

Palliative esophagectomy if:

a) dysphagia

b) good performance status c) locally resectable

Radiotherapy:

3000 cGy +/– 5-FU radiosensitizer

Endoscopic stent Laser ablation Photodynamic therapy

Algorithm 12.3. Management of stage IV esophageal cancer. (Reprinted from Smith CD. Esophagus.

In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.)

in patients receiving combined therapy after randomization was closed.

Palliation: Patients with distant metastases or contraindications to chemoradiotherapy or surgery should be considered for palliative interventions. Local and regional treatment modalities are the corner- stones of symptomatic control. Palliative radiation therapy is a key component and is associated with significant, albeit short-term, suc- cess in maintaining adequate swallowing. The value of systemic chemotherapy is limited. Concurrent chemotherapy and radiation have been used in the palliation of patients with metastatic tumors.

Table 12.5. Phase III treatment trials for esophageal carcinoma.

Author Cell type R1 R2 Survival Positive findings

Cooper et al^a Both Rad Che/Rad 0% vs. 26% ≠ Toxicity, R2 (5 years)

Law et al^b Squ Surg Che/Surg Same ≠ Downstaging, R2

≠ Curative resection, R2

Kelsen et al^c Both Surg Che/Surg Same

Walsh et al^d Adeno Surg Che/Rad/Surg 6% vs. 32% Ø N1and M1R2 (3 years)

Bosset et al^e Squ Surg Che/Rad/Surg Same ≠ DF survival, R2

≠ Curative resection, R2

Ando et al^f Squ Surg Surg/Che Same

Adeno, adenocarcinoma; Che, chemotherapy; Rad, radiation therapy; Squ, squamous cell carcinoma; Surg, surgery.

aCooper JS. Guo MD. Herskovic A, et al. Chemoradiotherapy of locally advanced esophageal cancer. Long-term follow-up of a prospective randomized trial (RTOG 85-01). JAMA 1999;281:1623–1627.

b Law S, Fok M, Chow S, et al. Preoperative chemotherapy versus surgery alone for squamous cell carcinoma of the esophagus: a prospective randomized trial. J Thorac Cardiovasc Surg 1997;114:210–217.

c Kelsen DP, Ginsberg R, Pajak TF, et al. Chemotherapy followed by surgery compared to surgery alone for local- ized esophageal cancer. N Engl J Med 1998;339:1979–1984.

d Walsh TN, Noonan N, Hollywood D, et al. A comparison of multimodality therapy and surgery for esophageal adenocarcinoma. N Engl J Med 1996;335:462–467.

e Bosset JF, Gignoux M, Triboulet JP, et al. Chemoradiotherapy followed by surgery compared with surgery alone in squamous cell cancer of the esophagus. N Engl J Med 1997;337:161–167.

f Ando N, Iizuka T, Kakegawa T, et al. A randomized trial of surgery with and without chemotherapy for localized squamous cell carcinoma of the thoracic esophagus. J Thorac Cardiovasc Surg 1997;114:205–209.

Source: Reprinted from Rice TW. Esophagus. In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.

2Cooper JS, Guo MD, Herskovic A, et al. Chemoradiotherapy of locally advanced esophageal cancer. Long-term follow-up of a prospective randomized trial (RTOG 85-01).

JAMA 1999;281:1623–1627. Law S, Fok M, Chow S, et al. Preoperative chemotherapy versus surgery alone for squamous cell carcinoma of the esophagus: a prospective ran- domized trial. J Thorac Cardiovasc Surg 1997;114:210–217. Kelsen DP, Ginsberg R, Pajak TF, et al. Chemotherapy followed by surgery compared to surgery alone for localized esophageal cancer. N Engl J Med 1998;339:1979–1984. Walsh TN, Noonan N, Hollywood D, et al. A comparison of multimodality therapy and surgery for esophageal adenocarci- noma. N Engl J Med 1996;335:462–467. Bosset JF, Gignoux M, Triboulet JP, et al. Chemora- diotherapy followed by surgery compared with surgery alone in squamous cell cancer of the esophagus. N Engl J Med 1997;337:161–167. Ando N, Iizuka T, Kakegawa T, et al. A randomized trial of surgery with and without chemotherapy for localized squamous cell carcinoma of the thoracic esophagus. J Thorac Cardiovasc Surg 1997;114:205–209.

While efficacious in improving local and regional control, this treat- ment comes with a significantly increased risk of toxicity and may not be appropriate in most patients.

A number of local measures can preserve swallowing and avoid the toxicity of chemotherapy and radiotherapy. Dilation of malignant strictures with bougies or endoscopic balloon dilators temporarily can relieve dysphagia. Dilation is typically performed not as a sole therapy but as a prelude to other, more definitive measures. Injection with alcohol causes tumor necrosis and a decrease in the exophytic portion of the tumor. However, dysphagia usually recurs within 30 days, necessitating retreatment.

Laser therapy is reserved for patients with severe obstruction of the esophagus requiring palliation until chemotherapy and radiotherapy take effect. It also is used in patients who are not candidates for prosthesis placement because of an anticipated short life expectancy.

Photodynamic therapy provides longer relief of dysphagia than neodymium:yttrium-aluminum-garnet (Nd:YAG) laser therapy, but patients must avoid sunlight up to 30 days after injection because of dermal photosensitivity. This is not a desirable method of palliation for patients whose life expectancy is measured in weeks or months.

Newer, self-expanding metal stents are easier to place and require much less tumor dilation before placement. Varied lengths and config- urations are available. Silicone-covered stents prevent tumor ingrowth but are more apt to migrate than noncovered stents; they are the pros- theses of choice in the treatment of malignant fistula between the airway and esophagus. Stent placement after chemotherapy or radio- therapy may be associated with increased complications. Modern stents provide effective, long-lasting palliation with little morbidity and are the first mode of palliation considered for patients with esophageal carcinoma.

Benign Esophageal Tumors

Leiomyomas, Cysts, and Polyps: Benign tumors of the esophagus are uncommon, with three histologic types accounting for 87% of benign esophageal tumors: leiomyomas, cysts, and polyps. These three tumors have distinct locations in the esophagus that reflect their cells of origin.

Polyps occur almost exclusively in the cervical esophagus, while leiomyomas and cysts tend to occur in the distal two thirds.

Leiomyomas constitute 50% of benign tumors of the esophagus, with an average patient age at presentation of 38 years, in contrast to esophageal malignancy, which typically presents at a more advanced age.

Esophageal cysts are commonly congenital and are lined by colum- nar epithelium of the respiratory type, glandular epithelium of the gastric type, squamous epithelium, or transitional epithelium. Enteric and bronchogenic cysts are the most common. Treatment is similar to that for leiomyoma, with resection for large or symptomatic lesions.

The cyst wall should be removed completely. Search for a fistulous tract

to the respiratory tract should be carried out, especially in patients who have had recurrent respiratory tract infections.

Gastroesophageal Reflux Disease (GERD)

Gastroesophageal reflux (GER) is defined as failure of the antireflux barrier, allowing abnormal reflux of gastric contents into the esoph- agus. It is a mechanical disorder that is caused by an inadequate LES, which may be associated with a gastric emptying disorder or failed esophageal peristalsis. These abnormalities result in a spectrum of symptoms and diseases ranging from “heartburn” to esophageal tissue damage with subsequent complications of ulceration and stricture for- mation. A host of extraesophageal manifestations of GER, such as asthma, laryngitis, and dental breakdown, also are being increasingly identified. Gastroesophageal reflux is an extremely common condition, accounting for nearly 75% of all esophageal pathology. Nearly 44% of Americans experience monthly heartburn, and 18% of these individu- als use nonprescription medication directed against GER.

Pathophysiology

Antireflux Mechanism: Although our understanding of the antireflux barrier is incomplete and has evolved over many years, a current view is that the LES, diaphragmatic crura, and phrenoesophageal ligament are key components, and LES dysfunction is the most common cause of GER. Three factors determine the competence of the LES: resting LES pressure, resting LES length, and abdominal length of the LES.

Lower esophageal sphincter dysfunction may be either physiologic and transient or pathologic and permanent. Nearly everyone experiences physiologic reflux, most commonly related to gastric distention fol- lowing a meal. Postprandial gastric distention results in pressure against the LES, which stretches and pulls the sphincter open while shortening the LES length. The resulting incompetence of the LES leads to transient periods of reflux. These transient episodes of reflux are relieved with gastric venting (belching) or when the stomach empties normally. Overeating exacerbates these episodes, and a high-fat Western diet may delay gastric emptying, thereby extending the dura- tion of these transient episodes. Evidence is accumulating that chronic, gastric-related, transient physiologic reflux leads to suffi- cient esophageal injury to cause dysfunction of the antireflux barrier;

this then progresses to more permanent and pathologic reflux.

Consequences of Reflux: Gastroesophageal reflux may lead to symptoms

related to the reflux of gastric content into the esophagus, lungs, or

oropharynx, or to damage to the esophageal mucosa and respiratory

epithelium with subsequent changes related to repair, fibrosis, and

reinjury. Manifestations of GER typically are classified as esophageal

and extraesophageal. Esophageal symptoms of GER include heart-

burn, chest pain, water brash, or dysphagia. Dysphagia often impli-

cates complicated GER with esophagitis and ulceration, stricture, or

Barrett’s metaplastic changes. Extraesophageal manifestations gener-

ally are pulmonary, resulting from pulmonary aspiration or bron- chospasm induced when reflux stimulates a distal esophageal vagal reflex. Extraesophageal symptoms and signs include chronic cough, laryngitis, dental damage, and chronic sinusitis. With a better under- standing of GER and new therapies for eliminating symptoms, fewer patients are presenting with severe complications of GER. However, those with complicated GER (high-grade esophagitis, stricture, or Barrett’s mucosa) have more severe reflux, suggesting a mechanically defective LES as a major etiologic factor. Reconstruction of the defec- tive LES has been the basis of offering operative therapy for compli- cated GER.

Diagnosis

The clinical diagnosis of GER is fairly straightforward if the patient reports the classic symptom of heartburn that is readily relieved after ingesting antacids. As in Case 2, many patients with this classic pre- sentation will have been treated with an empiric trial of H2 blockers or proton pump inhibitors (PPIs). Other typical symptoms of GER include regurgitation or dysphagia. Chest pain, asthma, laryngitis, recurrent pulmonary infections, chronic cough, and hoarseness may be associated with reflux. Increasing numbers of patients with these atypical GER symptoms are being evaluated for reflux.

A careful history should confirm both typical and atypical symptoms of GER and any response to medical therapy. Atypical symptoms, no response to high-dose medication, dysphagia, odynophagia, GI bleeding, or weight loss suggests complications of GER or another disease process entirely and should prompt a more thorough evalu- ation. In a patient with typical symptoms, endoscopic findings of esophageal erosions, ulcers, or columnar-lined esophagus are fairly specific for GER. During esophagogastroduodenoscopy (EGD), an esophageal mucosal biopsy should be obtained to confirm esophagitis, and esophageal length and the presence of a hiatal hernia or stricture can be assessed. This eliminates the need for a confirmatory barium swallow. With typical findings, no other tests beyond EGD are neces- sary to diagnose GER. However, in many patients, the EGD will be normal due to empiric treatment of symptoms. In this setting, 24-hour pH testing is necessary to objectively establish the diagnosis of GER.

Ambulatory 24-hour pH monitoring has been regarded as the gold standard in diagnosing GER and is of unquestionable benefit in patients where the diagnosis is unclear or in those with nonerosive esophagitis on EGD. Ambulatory pH monitoring is not mandatory in patients with typical reflux symptoms and erosive esophagitis on EGD.

Barium swallow is the test of choice in evaluating the patient with dysphagia, suspected stricture, paraesophageal hernia, or shortened esophagus. Other studies may be helpful in difficult cases, such as gastric emptying studies in patients with significant bloating, nausea, or vomiting.

Treatment

There is considerable debate regarding optimal treatment of GER. With

many Americans experiencing daily heartburn and the established

impact this condition has on an individual’s quality of life, there is tremendous amount of interest and effort devoted to understanding this condition and establishing treatment algorithms that are effective and cost efficient. See Algorithms 12.4 and 12.5 for treatment algo- rithms for uncomplicated and complicated GER.

Medical: The principles of nonoperative management of GER include lifestyle modifications, medical therapy for symptomatic control, and identification of those who would be best served with an antireflux operation. Although lifestyle modifications always have been the initial step in therapy, only those patients with mild and intermittent symptoms seem to benefit from lifestyle changes alone. Most patients who seek medical advice are best treated with either medication or an operation. Selection of a particular medical regimen depends on the

Trial of H₂RA

Responds to H₂RA Suboptimal response to H₂RA

Maintenance therapy vs.

Treat on demand

Titrate H₂RA dose or Begin PPI

Suboptimal response Responds

Confirm diagnosis Maintenance therapy

No GER GER

Further workup Refer for surgery

Algorithm 12.4. Management algorithm for treatment of uncomplicated gas- troesophageal reflux (based on endoscopic findings). H2RA, H2 receptor antag- onist; PPI, proton pump inhibitor; GER, gastroesophageal reflux. (After Fennerty MB, Castell D, Fendrick AM, et al. The diagnosis and treatment of gastroesophageal reflux disease in a managed care environment. Suggested disease management guidelines. Arch Intern Med 1996;156:477–484, with permission. Copyright © 1996 American Medical Association. All Rights Reserved. Reprinted from Smith CD. Esophagus. In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York:

Springer-Verlag, 2001, with permission.)

severity of GER, effectiveness of the proposed therapy, cost, and con- venience of the regimen.

Numerous trials have shown that short-term treatment of GER with acid-suppression regimens can effectively relieve symptomatic GER and heal reflux esophagitis in approximately 90% of cases with intensive therapy. Levels of success depend on the type, duration, and dosage of antisecretory therapy. The PPIs have profoundly changed the medical treatment of GER. Rates of healing of esophagitis have improved dramatically with PPIs when compared to H2 receptor antagonists. The cost of PPIs has led many to recommend their use in only complicated or refractory GER. It appears that reflux symptoms permanently disappear only in a minority of patients with GER when they are taken off medications. Recurrence of symptoms and esophagitis is observed frequently, and thus treatment strategies based on effectiveness and outcome must be based on long-term follow-up. In fact, reflux disease must be considered a lifelong disease that requires a lifelong treatment strategy.

Surgical: Treatment algorithms for both uncomplicated (see Algorithm 12.4) and complicated (see Algorithm 12.5) reflux esophagitis begin

Trial of PPI

Responds

Maintenance therapy

Suboptimal response

Increase PPI

Suboptimal response

Confirm diagnosis

No GER GER

Further workup

Responds

Maintenance therapy

Refer for surgery

Algorithm 12.5. Management algorithm for treatment of complicated gastro- esophageal reflux (based on endoscopic findings). PPI, proton pump inhibitor;

GER, gastroesophageal reflux. (After Fennerty MB, Castell D, Fendrick AM, et al. The diagnosis and treatment of gastroesophageal reflux disease in a managed care environment. Suggested disease management guidelines. Arch Intern Med 1996;156:477–484, with permission. Copyright © 1996 American Medical Association. All Rights Reserved. Reprinted from Smith CD. Esopha- gus. In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.)

with medical therapy. The expense and psychological burden of a life- time of medication dependence, undesirable lifestyle changes, the uncertainty as to the long-term effects of some newer medications, and the potential for persistent mucosal changes despite symptomatic control all make surgical treatment of GER an attractive option. Surgi- cal therapy, which addresses the mechanical nature of this condition, is curative in 85% to 93% of patients. Chronic medical management may be most appropriate for patients with limited life expectancy or comorbid conditions that would prohibit safe surgical intervention.

Historically, antireflux surgery was recommended only for patients with refractory or complicated gastroesophageal reflux. Recent devel- opments have affected the long-term management of patients with GER. The rapid postoperative recovery seen with laparoscopic surg- ery is now feasible following antireflux procedures. The widespread availability and use of ambulatory pH monitoring has improved recognition of true GER and selection of patients for long-term therapy. Physicians now recognize that patients with GER have a greatly impaired quality of life, which normalizes with successful treatment.

The management goals of GER have changed. Rather than focus- ing therapy only on controlling symptoms, modern treatment aims to eliminate symptoms, improve a patient’s quality of life, and institute a lifelong plan for management. Controlled trials that compared medical and surgical therapy of GER have favored surgical therapy.

Surgical treatment was significantly more effective in improving symp- toms and endoscopic signs of esophagitis for as long as 2 years. Other longitudinal studies report good to excellent long-term results in 80%

to 93% of surgically treated patients (Table 12.6).

Table 12.6. Medical versus surgical treatment of Barrett’s esophagus.

No. patients Symptom control Stricture/esophagitis

Author Medical Surgical Medical Surgical Medical Surgical

Attwood 1992^b(p)^a 26 19 22% 81% 38% 21%

Oritz 1996^c(pr) 27 32 85% 89% 53%/45% 5%/15%

Sampliner 1994^d(p) 27 — 70% — 50% —

Csendes 1998^e(pr) — 152 — 46% — 64%

pr, prospective randomized; p, prospective; ret, retrospective.

aBefore availability of proton pump inhibitors.

b Attwood SE, Barlow AP, Norris TL, et al. Barrett’s oesophagus: effect of antireflux surgery on symptom control and development of complications. Br J Surg 1992;79:1050–1053.

c Ortiz A, Martinez de Haro LF, Parrilla P, et al. Conservative treatment versus antireflux surgery in Barrett’s oesoph- agus: long-term results of a prospective study. Br J Surg 1996;83:274–278.

d Sampliner RE. Effect of up to 3 years of high-dose lansoprazole on Barrett’s esophagus. Am J Gastroenterol 1994;89:1844–1848.

e Csendes A, Braghetto I, Burdiles P, et al. Long-term results of classic antireflux surgery in 152 patients with Barrett’s esophagus: clinical, radiologic endoscopic, manometric, and acid reflux test analysis before and late after operation.

Surgery (St. Louis) 1998;126:645–657.

Source: Reprinted from Smith CD. Esophagus. In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.

Indications: Antireflux surgery should be considered in patients in whom intensive medical therapy has failed. With the advent of proton pump inhibitors, true medical failures are unusual. Antireflux surgery also should be offered to patients whose symptoms recur immedi- ately after stopping medications and who require long-term daily medication. Many patients want to avoid the cost, inconvenience, and side effects of long-term medication and want to preserve their quality of life. Complications of GER, such as Barrett’s esophagus and esophageal stricture, should not alter the approach to long-term man- agement. However, patients with these complications usually have more severe disease, require more intensive medical therapy, and are referred for surgical evaluation. Occasionally, GER presents atypically with chest pain, asthma, chronic cough, or hoarseness. Patients with these atypical symptoms usually improve with surgery. However, appropriate patient selection can be very difficult. Ambulatory pH monitoring has been thought to provide the most objective way to select these patients for surgery, but an abnormal pH study does not correlate well with symptom relief following antireflux surgery. There- fore, a trial of medical therapy with resolution of symptoms remains the best way to prove an association between GER and an individual’s atypical symptoms. When such an association exists, antireflux surgery is indicated.

Preoperative Evaluation: The preoperative evaluation should both justify the need for surgery and direct the operative technique to opti- mize outcome. At a minimum, all patients being considered for surgery should undergo a thorough history and a physical exam, EGD, and esophageal manometry. Esophageal manometry allows evaluation of the lower esophageal sphincter and is diagnostic in differentiating GER from achalasia. Equally important is its use in assessing esophageal body pressures and identifying individuals with impaired esophageal clearance who may not do as well with a 360-degree fundoplication.

Procedures: To establish an effective antireflux barrier, operative procedures for GER are designed to restore adequate LES pressure, position the LES within the abdomen where it is under positive (intraabdominal) pressure, and to close any associated hiatal defect.

Advances in laparoscopic technology and technique allow the repro- duction of “open” procedures while eliminating the morbidity of an upper midline incision. Open antireflux operations remain indicated when the laparoscopic technique is not available or is contraindicated.

Contraindications to laparoscopic antireflux surgery include uncor- rectable coagulopathy, severe chronic obstructive pulmonary disease (COPD) such that CO

elimination is impeded during laparoscopy, and advanced pregnancy. Only a very experienced laparoscopic surgeon should attempt the minimally invasive approach in the presence of previous upper abdominal operation or prior antireflux surgery.

In patients with normal esophageal body peristalsis, laparoscopic

Nissen fundoplication (Fig. 12.4) has emerged as the most widely

accepted and applied antireflux operation. Thousands of laparoscopic

Nissen fundoplication patients have been reported in the world litera-

ture, with 93% of patients symptom free at 1 year postoperatively. Only 3% require some medical therapy for symptom control. Overall, 97%

of patients are satisfied with their results. Transient dysphagia occurs in nearly 50% and resolves within 3 weeks of surgery.

Summary: Antireflux surgery is indicated in any patient with GER refractory to medical management or in any patient who has symptom recurrence when medicine is withdrawn. In many patients with classic symptoms, an EGD and esophageal manometry are all the preoperative testing necessary. Additional tests are confirmatory in dif- ficult cases. The laparoscopic Nissen fundoplication is both safe and effective in the long-term management of nearly all patients with chronic GER. The Toupet fundoplication may be best used in patients with impaired esophageal body peristalsis.

Hiatal Hernias: Sliding and Paraesophageal Hernias

The majority of patients with hiatal hernia are asymptomatic, and the diagnosis often is made incidentally during investigation of other gas- trointestinal problems. The underlying etiology of hiatal hernias remains unclear.

Hiatal hernias can be classified into four types. A type I hiatal hernia (Fig. 12.5) also is known as a sliding hiatal hernia. It consists of a simple herniation of the gastroesophageal junction into the chest. The phrenoesophageal ligament is attenuated, and there is no true hernia sac. This is the most common hiatal hernia and is frequently diagnosed in women and in the fifth and sixth decades of life. Type II hiatal hernias (Fig. 12.5) are commonly referred to as paraesophageal hernias. The gastroesophageal junction remains at the esophageal

Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.)

hiatus while the gastric fundus herniates alongside the esophagus, through the hiatus, and into the chest. Type III hiatal hernias are a combination of type I and type II hernias, with the esophagogastric junction being displaced into the chest along with the gastric fundus and body. Paraesophageal hernias (types II and III) have a true hernia sac accompanying the herniated stomach. Type IV hernias are an advanced stage of paraesophageal hernia in which the entire stomach and other intraabdominal contents (colon, spleen) are herniated into the chest. As in Case 3, paraesophageal hernias are found predomi- nantly in older individuals.

Diagnosis

When symptoms are present, sliding hernias have a different pre- sentation from paraesophageal hernias. Paraesophageal hernias tend to produce more dysphagia, chest pain, bloating, and respiratory prob- lems than do sliding hernias. Symptoms associated with a sliding hernia more often are related to LES dysfunction and include classic reflux symptoms, heartburn, regurgitation, and dysphagia.

Squamo- columnar junction Stomach

Esophageal mucosa Esophageal muscle

Phrenoesophageal membrane

Endothoracic fascia Diaphragm Endoabdominal fascia Peritoneum

Type I hiatal hernia

Esophageal muscle Phrenoesophageal membrane Squamocolumnar junction

Stomach

Peritoneal sac Endothoracic fascia

Diaphragm Endoabdominal fascia Peritoneum

Type II hiatal hernia

Figure 12.5. Classification of hiatal hernia. (Reprinted from Smith CD. Esoph- agus. In: Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.)

Treatment

Because a hiatal hernia is a purely mechanical abnormality, nonop- erative treatment does not exist. The risk of bleeding, incarceration, strangulation, perforation, and death with paraesophageal hernias is such that when a type II or greater hernia is identified, operative repair should be performed. In contrast, a significant number of patients with type I hiatal hernias are asymptomatic and remain so throughout the remainder of their life. Therefore, the presence of a sliding (type I) hiatal hernia alone does not mandate intervention.

However, patients with a type I hernia and gastroesophageal reflux, chest pain, dysphagia, regurgitation, or other symptoms referable to their hernias should undergo symptom-specific workup and may be best treated with an operative repair. Occult gastrointestinal bleeding is a complication of hiatal hernia thought to result from the mechanical trauma of the stomach moving into and out of the chest, causing subtle erosions in the stomach that slowly bleed and lead to anemia.

The operation can be performed through the chest or abdomen and via “open” or minimally invasive techniques. Most sliding type I hernias are repaired on the basis of GER symptoms, and an antireflux procedure is critical to successful treatment. Routine addition of a fun- doplication to the repair of the other three types of hiatal hernia is con- troversial. Most patients with type II hernias do not have reflux symptoms, and an antireflux operation for these patients may add little benefit. With careful questioning, patients with type II hernias may give a history of GER symptoms that spontaneously abated, suggest- ing an anatomic change (perhaps hernia development) leading to symptom resolution.

Barrett’s Esophagus

Barrett’s esophagus is a condition in which the normal squamous epithelium of the esophagus is partially replaced by metaplastic columnar epithelium, placing patients at risk for developing adeno- carcinoma. Intestinal metaplasia (not gastric-type columnar changes) constitutes true Barrett’s esophagus, with a risk of progression to dys- plasia and adenocarcinoma. Barrett’s esophagus occurs in 7% to 10%

of people with GER and may represent the end stage of the natural history of GER. Barrett’s esophagus is associated with a more pro- found mechanical deficiency of the LES, severe impairment of esophageal body function, and marked esophageal acid exposure.

Only a small number of patients with Barrett’s esophagus develop

carcinoma. The estimated incidence of adenocarcinoma in patients

with Barrett’s esophagus is 0.2% to 2.1% per year. Only patients with

specialized columnar epithelium are at an increased risk of developing

Barrett’s adenocarcinoma. The presence of epithelial dysplasia, partic-

ularly high-grade dysplasia, is a risk factor for adenocarcinoma, and

the progression of specialized columnar epithelium to dysplasia and

invasive carcinoma is well documented.

Diagnosis

Heartburn, regurgitation, and—with stricture formation—dysphagia are the most common symptoms. Heartburn is milder than in the absence of Barrett’s changes, presumably because the metaplastic epithelium is less sensitive than squamous epithelium. The diagnosis often is suggested by the esophagoscopic finding of a pink epithelium in the lower esophagus instead of the shiny gray-pink squamous mucosa, but every case should be verified by biopsy. Radiographic findings consist of hiatal hernia, stricture, ulcer, or a reticular pattern to the mucosa—changes of low sensitivity and specificity.

Treatment

Treatment goals for patients with Barrett’s esophagus are relief of symptoms and arrest of ongoing reflux-mediated epithelial damage.

Patients with Barrett’s have more severe esophagitis and frequently require more intensive therapy for control of reflux. Regardless of medical versus surgical treatment, patients with Barrett’s esophagus require long-term endoscopic surveillance with biopsy of columnar segments for progressive metaplastic changes or progression to dys- plasia. Esophagectomy, if performed with a low operative mortality, is indicated in patients with a diagnosis of high-grade dysplasia. Several studies have compared medical and surgical therapy in patients with Barrett’s esophagus.

Current evidence suggests that neither medical nor surgical therapy result in regression of Barrett’s epithelium. There is evidence suggest- ing that antireflux surgery may prevent progression of Barrett’s changes and protect against dysplasia and malignancy. These are very strong data in support of the favorable impact of operative therapy on the natural history of Barrett’s esophagus.

Achalasia

This idiopathic degenerative disorder results in aperistalsis of the esophageal body and abnormal to absent LES relaxation. It has been described in all age groups. The majority of patients present at between 20 and 40 years of age. There is no gender partiality. Achalasia is a risk factor for esophageal malignancy. Squamous cell carcinoma is esti- mated to develop in approximately 5% of patients at an average of 20 years after initial diagnosis. Carcinoma presents approximately 10

3 Ortiz A, Martinez de Haro LF, Parrilla P, et al. Conservative treatment versus antireflux surgery in Barrett’s oesophagus: long-term results of a prospective study. Br J Surg 1996;83:274–278. Martinez de Haro LF, Ortiz A, Parrilla P, et al. Long-term results of Nissen fundoplication in reflux esophagitis without strictures. Clinical, endoscopic, and pH-metric evaluation. Dig Dis Sci 1992;37:523–527. Sagar PM, Ackroyd R, Hosie KB, et al. Regression and progression of Barrett’s oesophagus after antireflux surgery. Br J Surg 1995;82:806–810. Putnam JBJ, Suell DA, Natarajan G. A comparison of three tech- niques of esophagectomy for carcinoma of the esophagus from one institution with a res- idency training program. Ann Thorac Surg 1994;57:319–325.

years earlier than in the general population and is associated with a worse prognosis, possibly due to delayed diagnosis.

Diagnosis

Patients typically describe dysphagia for solids and, to varying degrees, for liquids. Exacerbation of dysphagia may occur with inges- tion of cold liquids or during emotional stress. Symptoms onset is gradual. The average duration of dysphagia before presentation is 2 years. Regurgitation is reported in 60% to 90% of patients and chest pain in about 50%. Recurrent respiratory infections, aspiration pneu- monia, and lung abscess also may be initial presentations.

Barium swallow reveals the typical distal esophageal bird’s- beak deformity and proximal esophageal dilatation in 90% of patients (Fig. 12.6). This typical esophagogram also may be found with

“pseudoachalasia,” typically seen with gastroesophageal malignancies or as part of a paraneoplastic syndrome. Vigorous achalasia, a very early stage of achalasia, may present with strong tertiary esophageal contractions resulting in a radiographic appearance similar to diffuse esophageal spasm. Even with a typical presentation, esophagoscopy is essential to investigate the esophageal mucosa and exclude a malig- nancy. Esophageal manometry is diagnostic and demonstrates absent peristalsis in the distal esophagus with incomplete or failed LES relax-

Figure 12.6. Barium esophagogram. Contour: multiple rapid swallows of low- density barium provide a full-column technique that demonstrates esophageal contour in a patient with achalasia. (Reprinted from Rice TW. Esophagus. In:

Norton JA, Bollinger RR, Chang AE, et al, eds. Surgery: Basic Science and Clinical Evidence. New York: Springer-Verlag, 2001, with permission.)