Esophagus and Diaphragmatic Hernia
BASIC SCIENCE QUESTIONS
1. The esophagus has three normal points of narrowing. Which of the following is NOT one of the normal points of narrowing?
A. At the cricopharyngeus muscle
B. At the level of the aortic arch
C. At the level of the carina
D. At the level of the diaphragm
Three normal areas of esophageal narrowing are evident on the barium esophagogram or during esophagoscopy. The uppermost narrowing is located at the entrance into the esophagus and is caused by the cricopharyngeal muscle. Its luminal diameter is 1.5 cm, and it is the narrowest point of the esophagus. The middle narrowing is due to an indentation of the anterior and left lateral esophageal wall caused by the crossing of the left main stem bronchus and aortic arch. The luminal diameter at this point is 1.6 cm. The lowermost narrowing is at the hiatus of the diaphragm and is caused by the gastroesophageal sphincter mechanism. The luminal diameter at this point varies somewhat, depending on the distention of the esophagus by the passage of food, but has been measured at 1.6 to 1.9 cm. (See Schwartz 9th ed., p 805.)
2. The geometry of the circular muscle in the esophagus is
The circular muscle layer of the esophagus is thicker than the outer longitudinal layer. In situ, the geometry of the circular muscle is helical and makes the peristalsis of the esophagus assume a wormlike drive, as opposed to segmental and sequential squeezing. As a consequence, severe motor abnormalities of the esophagus assume a corkscrew-like pattern on the barium swallow radiogram. (See Schwartz 9th ed., p 808.)
3. The components that contribute to the function of the LES include all of the following EXCEPT
A. Length of the intra-abdominal esophagus
B. Width of the diaphragmatic hiatus
C. Resting pressure (tone) in the lower esophageal muscle
D. Length of the area of increased tone in the lower esophageal muscle
As defined by esophageal manometry, there are three characteristics of the LES that work in unison to maintain its barrier function. These characteristics include the resting LES pressure, its overall length, and the intra-abdominal length that is exposed to the positive pressure environment of the abdomen.
Therefore, a permanently defective sphincter is defined by one or more of the following characteristics: An LES with a mean resting pressure of less than 6 mmHg, an overall sphincter length of 2 cm, and intra-abdominal sphincter length of 1 cm. When compared to normal subjects without GERD these values are below the 2.5 percentile for each parameter. The most common cause of a defective sphincter is an inadequate abdominal length. (See Schwartz 9th ed., p 828, and Table 25-1.)
TABLE 25-1 Normal manometric values of the distal esophageal sphincter, n = 50
4. The primary reason that reflux is common after a big meal is
A. Shortening of the LES
B. Increased acid production
C. Hyperperistalsis of the stomach
D. Increased gastrin production
The resistance to gastroesophageal reflux is a function of both the resting LES pressure and length over which this pressure is exerted. Thus, as the sphincter becomes shorter, a higher pressure will be required in order to prevent a given amount of reflux. Much like the neck of a balloon as it is inflated, as the stomach fills and distends, sphincter length decreases. Therefore, if the overall length of the sphincter is permanently short from repeated distention of the fundus secondary to large volume meals, then with minimal episodes of gastric distention and pressure, there will be insufficient sphincter length for the barrier to remain competent, and reflux will occur. (See Schwartz 9th ed., p 851, and Fig. 25-1.)
FIG. 25-1. A graphic illustration of the shortening of the lower esophageal sphincter that occurs as the sphincter is “taken up” by the cardia as the stomach distends.
5. Which of the following INCREASES lower esophageal sphincter (LES) pressure?
The LES has intrinsic myogenic tone, which is modulated by neural and hormonal mechanisms. Alpha-adrenergic neurotransmitters or beta blockers stimulate the LES, and alpha blockers and beta stimulants decrease its pressure. It is not clear to what extent cholinergic nerve activity controls LES pressure. The vagus nerve carries both excitatory and inhibitory fibers to the esophagus and sphincter. The hormones gastrin and motilin have been shown to increase LES pressure; and cholecystokinin, estrogen, glucagon, progesterone, somatostatin, and secretin decrease LES pressure. The peptides bombesin, l-enkephalin, and substance P increase LES pressure; and calcitonin gene-related peptide, gastric inhibitory peptide, neuropeptide Y, and vasoactive intestinal polypeptide decrease LES pressure. Some pharmacologic agents such as antacids, cholinergics, agonists, domperidone, metoclopramide, and prostaglandin F2 are known to increase LES pressure; and anticholinergics, barbiturates, calcium channel blockers, caffeine, diazepam, dopamine, meperidine, prostaglandin E1 and E2, and theophylline decrease LES pressure. Peppermint, chocolate, coffee, ethanol, and fat are all associated with decreased LES pressure and may be responsible for esophageal symptoms after a sumptuous meal. (See Schwartz 9th ed., p 812.)
6. Which of the following is NOT part of the arterial blood supply to the esophagus?
A. Inferior thyroid artery
B. Bronchial arteries
C. Inferior phrenic artery
D. Right gastric artery
The cervical portion of the esophagus receives its main blood supply from the inferior thyroid artery. The thoracic portion receives its blood supply from the bronchial arteries, with 75% of individuals having one right-sided and two left-sided branches. Two esophageal branches arise directly from the aorta. The abdominal portion of the esophagus receives its blood supply from the ascending branch of the left gastric artery and from inferior phrenic arteries. On entering the wall of the esophagus, the arteries assume a T-shaped division to form a longitudinal plexus, giving rise to an intramural vascular network in the muscular and submucosal layers. As a consequence, the esophagus can be mobilized from the stomach to the level of the aortic arch without fear of devascularization and ischemic necrosis. Caution should be exercised as to the extent of esophageal mobilization in patients who have had a previous thyroidectomy with ligation of the inferior thyroid arteries proximal to the origin of the esophageal branches. (See Schwartz 9th ed., p 808, and Fig. 25-2.)
FIG. 25-2. Arterial blood supply of the esophagus. (Reproduced with permission from Rothberg M, DeMeester TR: Surgical anatomy of the esophagus, in Shields TW (ed): General Thoracic Surgery, 3rd ed. Philadelphia: Lea & Febiger, 1989, p 84.)
1. In a patient with Barrett’s esophagitis, a fundoplication will
A. Heal the mucosal injury
B. Prevent Barrett’s progression
C. Improve symptoms
D. All of the above
The long-term relief of symptoms remains the primary reason for performing antireflux surgery in patients with BE. Healing of esophageal mucosal injury and the prevention of disease progression are important secondary goals. In this regard, patients with BE are no different from the broader population of patients with gastroesophageal reflux. They should be considered for antireflux surgery when patient data suggest severe disease or predict the need for long-term medical management. Most patients with BE are symptomatic. Although it has been argued that some patients with BE may not have symptoms, careful history taking will reveal the presence of symptoms in most, if not all, patients. (See Schwartz 9th ed., p 831.)
2. Which of the following manometic findings is indicative of an incompetent lower esophageal sphincter (LES)?
A. Average LES pressure 20 mmHg
B. Average intra-abdominal esophagus 4 cm
C. Average sphincter length 2 cm
D. Average peak pressure 25 mmHg
A mechanically defective sphincter is identified by having one or more of the following characteristics: an average LES pressure of 6 mmHg, an average length exposed to the positive-pressure environment in the abdomen of 1 cm or less, and/or an average overall sphincter length of 2 cm or less. Compared with the normal volunteers, these values are below the 2.5 percentile for sphincter pressure and overall length and for abdominal length. (See Schwartz 9th ed., p 816, and Table 25-2.)
TABLE 25-2 Normal manometric values of the distal esophageal sphincter, n = 50
3. Nonoperative management of an esophageal perforation can be considered in patients who
A. Have mild symptoms
B. Show drainage of extravasated contrast back into the esophagus
C. Have mild signs of sepsis
D. All of the above
Conservative management [of esophageal perforation] should not be used in patients who have free perforations into the pleural space. Cameron proposed three criteria for the non-operative management of esophageal perforation: (a) The barium swallow must show the perforation to be contained within the mediastinum and drain well back into the esophagus (Fig. 25-3), (b) symptoms should be mild, and (c) there should be minimal evidence of clinical sepsis. If these conditions are met, it is reasonable to treat the patient with hyperalimentation, antibiotics, and cimetidine to decrease acid secretion and diminish pepsin activity. Oral intake is resumed in 7 to 14 days, dependent on subsequent radiographic examinations. (See Schwartz 9th ed., p 876.)
FIG. 25-3. Barium esophagogram showing a stricture and a contained perforation following dilation. The injury meets Cameron criteria: It is contained within the mediastinum and drawn back into the esophagus, the patient had mild symptoms, and there was no evidence of clinical sepsis. Nonoperative management was successful.
4. The initial pathologic change that leads to the clinical findings of achalasia is
A. Hypertension of the LES
B. Relaxation of the LES
C. Hypertension of the body of the esophagus
D. Diffuse relaxation of the body of the esophagus
The pathogenesis of achalasia is presumed to be a neurogenic degeneration, which is either idiopathic or due to infection. In experimental animals, the disease has been reproduced by destruction of the nucleus ambiguus and the dorsal motor nucleus of the vagus nerve. In patients with the disease, degenerative changes have been shown in the vagus nerve and in the ganglia in the myenteric plexus of the esophagus itself. This degeneration results in hypertension of the LES, a failure of the sphincter to relax on swallowing, elevation of intraluminal esophageal pressure, esophageal dilatation, and a subsequent loss of progressive peristalsis in the body of the esophagus. The esophageal dilatation results from the combination of a nonrelaxing sphincter, which causes a functional retention of ingested material in the esophagus, and elevation of intraluminal pressure from repetitive pharyngeal air swallowing (Fig. 25-4). With time, the functional disorder results in anatomic alterations seen on radiographic studies, such as a dilated esophagus with a tapering, ‘bird’s beak’–like narrowing of the distal end (Fig. 25-5). (See Schwartz 9th ed., p 851.)
FIG. 25-4. Pressurization of esophagus: Ambulatory motility tracing of a patient with achalasia. A. Before esophageal myotomy. B. After esophageal myotomy. The tracings have been compressed to exaggerate the motility spikes and baseline elevations. Note the rise in esophageal baseline pressure during a meal represented by the rise off the baseline to the left of panel A. No such rise occurs postmyotomy (panel B).
FIG. 25-5. Barium esophagogram showing a markedly dilated esophagus and characteristic “bird’s beak” in achalasia. (Reproduced with permission from Waters PF, DeMeester TR: Foregut motor disorders and their surgical management. Med Clin North Am 65:1244, 1981. Copyright © Elsevier.)
5. The treatment of choice for an asymptomatic Schatzki’s ring is
C. Incision (division of the ring)
D. Resection with end-to-end anastomosis
Schatzki’s ring is a thin submucosal circumferential ring in the lower esophagus at the squamocolumnar junction, often associated with a hiatal hernia.
The best form of treatment of a symptomatic Schatzki’s ring in patients who do not have reflux consists of esophageal dilation for relief of the obstructive symptoms. In patients with a ring who have proven reflux and a mechanically defective sphincter, an antireflux procedure is necessary to obtain relief and avoid repeated dilation. (See Schwartz 9th ed., p 846, and Fig. 25-6.)
FIG. 25-6. Barium esophagogram showing Schatzki’s ring (i.e., a thin circumferential ring in the distal esophagus at the squamocolumnar junction). Below the ring is a hiatal hernia.
6. Which of the following is NOT one of the five principles of surgical correction of gastroesophageal reflux?
A. The fundoplication should be maintained in the abdomen by a crural repair
B. The operation should restore the pressure of the LES to 10 times the resting gastric pressure
C. An adequate amount of intra-abdominal esophagus must be obtained (approximately 2 cm)
D. The fundoplication should not increase the resistance above what the peristalsis of the esophagus can overcome (approximately 2-cm wrap)
First, the operation should restore the pressure of the distal esophageal sphincter to a level twice the resting gastric pressure (i.e., 12 mmHg for a gastric pressure of 6 mmHg), and its length to at least 3 cm.
Second, the operation should place an adequate length of the distal esophageal sphincter in the positive-pressure environment of the abdomen by a method that ensures its response to changes in intra-abdominal pressure. The permanent restoration of 1.5 to 2 cm of abdominal esophagus in a patient whose sphincter pressure has been augmented to twice resting gastric pressure will maintain the competency of the cardia over various challenges of intra-abdominal pressure.
Third, the operation should allow the reconstructed cardia to relax on deglutition. In normal swallowing, a vagally mediated relaxation of the distal esophageal sphincter and the gastric fundus occurs. The relaxation lasts for approximately 10 seconds and is followed by a rapid recovery to the former tonicity. To ensure relaxation of the sphincter, three factors are important: (a) Only the fundus of the stomach should be used to buttress the sphincter, because it is known to relax in concert with the sphincter; (b) the gastric wrap should be properly placed around the sphincter and not incorporate a portion of the stomach or be placed around the stomach itself, because the body of the stomach does not relax with swallowing; and (c) damage to the vagal nerves during dissection of the thoracic esophagus should be avoided because it may result in failure of the sphincter to relax.
Fourth, the fundoplication should not increase the resistance of the relaxed sphincter to a level that exceeds the peristaltic power of the body of the esophagus. The resistance of the relaxed sphincterdepends on the degree, length, and diameter of the gastric fundic wrap, and on the variation in intra-abdominal pressure. A 360° gastric wrap should be no longer than 2 cm and constructed over a 60F bougie. This will ensure that the relaxed sphincter will have an adequate diameter with minimal resistance.
Fifth, the operation should ensure that the fundoplication can be placed in the abdomen without undue tension, and maintained there by approximating the crura of the diaphragm above the repair. (See Schwartz 9th ed., p 836.)
7. Traction diverticula in the esophagus are the result of
A. A congenital defect
B. Infection or inflammation
C. Motility disorders
D. Trauma, usually iatrogenic
Diverticula of the esophagus may be characterized by their location in the esophagus (proximal, mid-, or distal esophagus), or by the nature of concomitant pathology. Diverticula associated with motor disorders are termed pulsion diverticula and those associated with inflammatory conditions are termed traction diverticula. Pulsion diverticula occur most commonly with nonspecific motility disorders, but can occur with all of the primary motility disorders. In the latter situation, the motility disorder is usually diagnosed before the development of the diverticulum. (See Schwartz 9th ed., p 853, and Fig. 25-7.)
FIG. 25-7. Illustration of the pathophysiology of midesophageal diverticulum showing traction on the esophageal wall from adhesions to inflamed subcarinal lymph nodes.
8. Which of the following is the treatment of choice in a patient with scleroderma and multiple symptomatic strictures of the esophagus?
A. Medical therapy alone (PPIs)
B. Dilation and PPI’s
C. Toupet fundoplication, if dilation and PPI are ineffective
D. Nissen fundoplication, if dilation and PPI are ineffective
Traditionally, esophageal symptoms [in patients with scleroderma] have been treated with PPIs, antacids, elevation of the head of the bed, and multiple dilations for strictures, with generally unsatisfactory results. The degree of esophagitis is usually severe and may lead to marked esophageal shortening as well as stricture. Scleroderma patients have frequently had numerous dilations before they are referred to the surgeon. The surgical management is somewhat controversial, but the majority of opinion suggests that a partial fundoplication (anterior or posterior) performed laparoscopically is the procedure of choice. The need for a partial fundoplication is dictated by the likelihood of severe dysphagia if a total fundoplication is performed in the presence of aperistalsis. Esophageal shortening may require a Collis gastroplasty in combination with a partial fundoplication. (See Schwartz 9th ed., p 847.)
9. Which of the following treatments should be considered in a patient with dysphagia and a T4N1M0 esophageal cancer of the gastroesophageal junction?
A. Neoadjuvant chemotherapy, esophagectomy
B. Esophagectomy, postoperative chemotherapy
C. Palliative intraluminal stent
D. Palliative radiation therapy
Therapy of esophageal cancer is dictated by the stage of the cancer at the time of diagnosis. Put simply, one needs to determine if the disease is confined to the esophagus (T1–T2, N0), locally advanced (T1–3, N1), or disseminated (any T, any N, M1). If cancer is confined to the esophagus, removal of the tumor with adjacent lymph nodes (LNs) may be curative. Very early tumors confined to the mucosa (T in situ, T1a, intramucosal cancer) may be addressed with endoscopic treatment. When the tumor is locally aggressive, modern therapy dictates a multimodality approach in a surgically fit patient. Multimodality therapy is either chemotherapy followed by surgery or radiation and chemotherapy followed by surgery. When given before surgery, these treatments are referred to as neoadjuvantor induction therapy. For disseminated cancer, treatment is aimed at palliation of symptoms. If the patient has dysphagia, as many do, the most rapid form of palliation is the placement of an expandable esophageal stent, endoscopically. For palliation of gastroesophageal junction (GEJ) cancer, radiation may be the first choice, as stents placed across the GEJ create a great deal of gastroesophageal reflux. (See Schwartz 9th ed., p 863.)
10. Patients with Barrett’s esophagitis have a risk for adenocarcinoma of the esophagus that is
A. 5 times the general population
B. 40 times the general population
C. 90 times the general population
D. 200 times the general population
Adenocarcinoma developing in Barrett’s mucosa was considered a rare tumor before 1975. Today, it occurs in approximately one in every 100 patient-years of follow-up, which represents a risk 40 times that of the general population. Most, if not all, cases of adenocarcinoma of the esophagus arise in Barrett’s epithelium. (See Schwartz 9th ed., p 832.)
11. The most common type of hiatal hernia is
A. Type I
B. Type II
C. Type III
D. Type IV
With the advent of clinical radiology, it became evident that a diaphragmatic hernia was a relatively common abnormality and was not always accompanied by symptoms. Three types of esophageal hiatal hernia were identified: (a) the sliding hernia, type I, characterized by an upward dislocation of the cardia in the posterior mediastinum (Fig. 25-8A); (b) the rolling or paraesophageal hernia (PEH), type II, characterized by an upward dislocation of the gastric fundus alongside a normally positioned cardia (Fig. 25-8B); and (c) the combined sliding-rolling or mixed hernia, type III, characterized by an upward dislocation of both the cardia and the gastric fundus (Fig. 25-8C). The end stage of type I and type II hernias occurs when the whole stomach migrates up into the chest by rotating 180° around its longitudinal axis, with the cardia and pylorus as fixed points. In this situation the abnormality is usually referred to as an intrathoracic stomach (Fig. 25-8D). In some taxonomies, a type IV hiatal hernia is declared when an additional organ, usually the colon, herniates as well.
When radiographic examinations are done in response to GI symptoms, the incidence of a sliding hiatal hernia is seven times higher than that of a PEH. The PEH is also known as the giant hiatal hernia. Over time the pressure gradient between the abdomen and chest enlarges the hiatal hernia. In many cases the type 1 sliding hernia will evolve into a type III mixed hernia. Type II hernias are quite rare. (See Schwartz 9th ed., p 842.)
FIG. 25-8. A. Radiogram of a type I (sliding) hiatal hernia. B. Radiogram of a type II (rolling or paraesophageal) hernia. C. Radiogram of a type III (combined sliding-rolling or mixed) hernia. D.Radiogram of an intrathoracic stomach. This is the end stage of a large hiatal hernia regardless of its initial classification. Note that the stomach has rotated 180° around its longitudinal axis, with the cardia and pylorus as fixed points. (Reproduced with permission from DeMeester TR, Bonavina L: Paraesophageal hiatal hernia, in Nyhus LM, Condon RE (eds): Hernia, 3rd ed. Philadelphia: Lippincott, 1989, p 684.)
12. Which of the following is the best initial treatment for a patient with achalasia and a small hiatal hernia?
A. Balloon dilation
B. Botulinum toxin
C. Myotomy of the LES
D. Long esophageal myotomy
Long-term follow-up studies have shown that pneumatic dilation achieves adequate relief of dysphagia and pharyngeal regurgitation in 50 to 60% of patients with achalasia. Close follow-up is required, and if dilation fails, myotomy is indicated. For those patients who have a dilated and tortuous esophagus or an associated hiatal hernia, balloon dilation is dangerous and surgery is the better option. The outcome of the one controlled randomized study (38 patients) comparing the two modes of therapy suggests that surgical myotomy as a primary treatment gives better long-term results. Several randomized trials comparing laparoscopic cardiomyotomy with balloon dilation or botulinum toxin injection have favored the surgical approach as well. (See Schwartz 9th ed., p 857.)
In performing a surgical myotomy of the lower esophageal sphincter (LES), there are four important principles: (a) complete division of all circular and collarsling muscle fibers, (b) adequate distal myotomy to reduce outflow resistance, (c) “undermining” of the muscularis to allow wide separation of the esophageal muscle, and (d) prevention of postoperative reflux. In the past, the drawback of a surgical myotomy was the need for an open procedure, which often deterred patients from choosing the best treatment option for achalasia. With the advent of minimally invasive surgical techniques two decades ago, laparoscopic cardiomyotomy (Heller myotomy) has become the treatment of choice for most patients with achalasia. (See Schwartz 9th ed., p 859.)
13. Which of the following increases the risk of esophagitis in a patient with gastroesophageal reflux?
A. Total time the esophageal mucosa is exposed to fluid with a pH 4
B. Total time the esophageal mucosa is exposed to fluid with a pH >7
C. Total time the esophageal mucosa is exposed to bile salts
D. All of the above
Complications of gastroesophageal reflux such as esophagitis, stricture, and Barrett’s metaplasia occur in the presence of two predisposing factors: a mechanically defective LES and an increased esophageal exposure to fluid with a pH of 4 and >7. (See Schwartz 9th ed., p 830.)
There is a considerable body of experimental evidence to indicate that maximal epithelial injury occurs during exposure to bile salts combined with acid and pepsin. These studies have shown that acid alone does minimal damage to the esophageal mucosa, but the combination of acid and pepsin is highly deleterious. Similarly, the reflux of duodenal juice alone does little damage to the mucosa, although the combination of duodenal juice and gastric acid is particularly noxious. (See Schwartz 9th ed., p 829.)
14. Which of the following makes curative resection of an esophageal cancer unlikely?
A. Tumor length >6 cm
B. Weight loss >10%
C. Recurrent laryngeal nerve palsy
D. >2 abnormal lymph nodes on CT scan
Clinical factors that indicate an advanced stage of carcinoma and exclude surgery with curative intent are recurrent nerve paralysis, Horner’s syndrome, persistent spinal pain, paralysis of the diaphragm, fistula formation, and malignant pleural effusion. Factors that make surgical cure unlikely include a tumor >8 cm in length, abnormal axis of the esophagus on a barium radiogram, more than four enlarged lymph nodes (LNs) on CT, a weight loss more than 20%, and loss of appetite. Studies indicate that there are several favorable parameters associated with tumors 4 cm in length, there are fewer with tumors between 4 and 8 cm, and there are no favorable criteria for tumors >8 cm in length. Consequently, the finding of a tumor >8 cm in length should exclude curative resection; the finding of a smaller tumor should encourage an aggressive approach. (See Schwartz 9th ed., p 865.)
15. Following a cricopharyngeal myotomy, what is the best option to treat an associated 3-cm-long Zenker’s diverticulum?
A. Nothing, the diverticulum should be left in place
C. Plication of the diverticulum
If a diverticulum is present and is large enough to persist after a myotomy, it may be sutured in the inverted position to the prevertebral fascia using a permanent suture (i.e., diverticulopexy) (Fig. 25-9). If the diverticulum is excessively large so that it would be redundant if suspended, or if its walls are thickened, a diverticulectomy should be performed. (See Schwartz 9th ed., p 849.)
FIG. 25-9. Posterior of the anatomy of the pharynx and cervical esophagus showing pharyngoesophageal myoto my and pexing of the diverticulum to the prevertebral fascia.
16. A patient presents with dysphagia and undergoes a barium swallow. Based on the image in Fig. 25-10, the most likely diagnosis is
A barium swallow is the most useful method to demonstrate a leiomyoma of the esophagus (Fig. 25-10). In profile, the tumor appears as a smooth, semilunar, or crescent-shaped filling defect that moves with swallowing, is sharply demarcated, and is covered and surrounded by normal mucosa. (See Schwartz 9th ed., p 874.)
17. The most appropriate initial treatment for a patient with symptomatic nutcracker esophagus is
A. Acid reducing agents
B. Calcium channel blockers
At the lower end of peak pressure, it is unclear whether nutcracker esophagus causes any symptoms. In fact, chest pain symptoms in nutcracker esophagus patients may be related to GERD rather than intraluminal hypertension. Treatment in these patients should be aimed at the treatment of GERD. (See Schwartz 9th ed., p 852.)
18. In a patient with scleroderma, which of the following would be expected on a barium swallow?
A. Dilated esophageal lumen
B. Narrowed esophageal lumen
C. “Beaking” of the distal esophagus
D. Multiple strictures of the proximal esophagus
The diagnosis of scleroderma can be made manometrically by the observation of normal peristalsis in the proximal striated esophagus, with absent peristalsis in the distal smooth muscle portion. The LES pressure is progressively weakened as the disease advances. Because many of the systemic sequelae of the disease may be nondiagnostic, the motility pattern is frequently used as a specific diagnostic indicator. Gastroesophageal reflux commonly occurs in patients with scleroderma, because they have both hypotensive sphincters and poor esophageal clearance. This combined defect can lead to severe esophagitis and stricture formation. The typical barium swallow shows a dilated, barium-filled esophagus, stomach, and duodenum, or a hiatal hernia with distal esophageal stricture and proximal dilatation. (See Schwartz 9th ed., p 846.)
19. Esophageal Aadenocarcinoma occurs most commonly in the
A. Cervical esophagus
B. Upper thoracic esophagus
C. Mid thoracic esophagus
D. Lower thoracic esophagus
Tumors of the lower esophagus and cardia are usually adenocarcinomas. (See Schwartz 9th ed., p 865, and Fig. 25-11.)
FIG. 25-11. Incidence of carcinoma of the esophagus and cardia based on tumor location.
20. What is the appropriate treatment for a patient with a type III (mixed) hiatal hernia and iron deficiency anemia?
B. Acid reducing agents
C. Repair of the hiatal hernia alone
D. Repair of the hiatal hernia and fundoplication
Many patients with sliding hernias and reflux symptoms will lose the reflux symptoms when the hernia evolves into the paraesophageal variety. This can be explained by the re-creation of the cardiophrenic angle when the stomach herniates alongside the GEJ or becomes twisted in the sac. Repair of the hernia without addressing the reflux can create extremely bothersome heartburn. (See Schwartz 9th ed., p 843.)
21. A patient with dysphagia undergoes a barium swallow. Based on the image in Fig. 25-12, the most likely diagnosis is
B. Diffuse esophageal spasm
C. Nutcracker esophagus
D. Ineffective esophageal motility disorder
(See Schwartz 9th ed., p 852, and Fig. 25-12.)
FIG. 25-12. Barium esophagogram of patient with diffuse spasm showing the corkscrew deformity.
22. In a patient undergoing curative resection for an adenocarcinoma of the gastroesophageal junction, how much proximal normal esophagus should be resected?
A. 3 cm
B. 5 cm
C. 8 cm
D. The entire thoracic esophagus (cervical division)
Because of the propensity of GI tumors to spread for long distances submucosally, long lengths of grossly normal GI tract should be resected. The longitudinal lymph flow in the esophagus can result in skip areas, with small foci of tumor above the primary lesion, which underscores the importance of a wide resection of esophageal tumors. Wong has shown that local recurrence at the anastomosis can be prevented by obtaining a 10-cm margin of normal esophagus above the tumor. Anatomic studies have also shown that there is no submucosal lymphatic barrier between the esophagus and the stomach at the cardia, and Wong has shown that 50% of the local recurrences in patients with esophageal cancer who are resected for cure occur in the intrathoracic stomach along the line of the gastric resection. Considering that the length of the esophagus ranges from 17 to 25 cm, and the length of the lesser curvature of the stomach is approximately 12 cm, a curative resection requires a cervical division of the esophagus and a >50% proximal gastrectomy in most patients with carcinoma of the distal esophagus or cardia. (See Schwartz 9th ed., p 865.)
23. Which of the following disorders is characterized by absent peristalsis in the esophageal body?
A. Diffuse esophageal spasm
B. Nutcracker esophagus
D. Hypertensive lower esophageal sphincter
(See Schwartz 9th ed., p 851, and Table 25-3.)
TABLE 25-3 Manometric characteristics of the primary esophageal motility disorders
Incomplete lower esophageal sphincter (LES) relaxation (75% relaxation)
Aperistalsis in the esophageal body
Elevated LES pressure ≤26 mmHg
Increased intraesophageal baseline pressures relative to gastric baseline
Diffuse esophageal spasm (DES)
Simultaneous (nonperistaltic contractions) (>20% of wet swallows)
Repetitive and multipeaked contractions
Intermittent normal peristalsis
Contractions may be of increased amplitude and duration
Mean peristaltic amplitude (10 wet swallows) in distal esophagus ≥180 mmHg
Increased mean duration of contractions (>7.0 s)
Normal peristaltic sequence
Hypertensive lower esophageal sphincter
Elevated LES pressure (≥26 mmHg)
Normal LES relaxation
Normal peristalsis in the esophageal body
Ineffective esophageal motility disorders
Decreased or absent amplitude of esophageal peristalsis (30 mmHg)
Increased number of nontransmitted contractions
Source: Reproduced with permission from DeMeester TR, et al: Physiologic diagnostic studies, in Zuidema GD, Orringer MB (eds): Shackelford’s Surgery of the Alimentary Tract, 3rd ed, Vol. I. Philadelphia: W.B. Saunders, 1991, p 115. Copyright © Elsevier.
24. A patient presents after vomiting 500 ml of bright red blood. Endoscopy shows a tear in the mucosa at the gastroesophageal junction (Mallory-Weiss syndrome). The most appropriate treatment is
B. Administration of anti-emetics
C. Placement of a Sengstaken-Blakemore tube
D. Surgical gastrotomy and oversewing of the tear
In the majority of patients [with Mallory-Weiss syndrome], the bleeding will stop spontaneously with nonoperative management. In addition to blood replacement, the stomach should be decompressed and antiemetics administered, as a distended stomach and continued vomiting aggravate further bleeding. A Sengstaken-Blakemore tube will not stop the bleeding, as the pressure in the balloon is not sufficient to overcome arterial pressure. Endoscopic injection of epinephrine may be therapeutic if bleeding does not stop spontaneously. Only occasionally will surgery be required to stop blood loss. The procedure consists of laparotomy and high gastrotomy with oversewing of the linear tear. Mortality is uncommon, and recurrence is rare. (See Schwartz 9th ed., p 876.)
25. Which of the following is NOT a risk factor for squamous carcinoma of the esophagus?
A. Barrett’s esophagitis
C. Human papillomavirus
D. Zinc deficiency
Barrett’s esophagitis is a risk factor for adenocarcinoma of the esophagus.
Squamous carcinoma accounts for the majority of esophageal carcinomas worldwide. Its incidence is highly variable, ranging from approximately 20 per 100,000 in the United States and Britain, to 160 per 100,000 in certain parts of South Africa and the Honan Province of China, and even 540 per 100,000 in the Guriev district of Kazakhstan. The environmental factors responsible for these localized high-incidence areas have not been conclusively identified, though additives to local foodstuffs (nitroso compounds in pickled vegetables and smoked meats) and mineral deficiencies (zinc and molybdenum) have been suggested. In Western societies, smoking and alcohol consumption are strongly linked with squamous carcinoma. Other definite associations link squamous carcinoma with long-standing achalasia, lye strictures, tylosis (an autosomal dominant disorder characterized by hyperkeratosis of the palms and soles), and human papillomavirus. (See Schwartz 9th ed., p 862.)