Overview: GER is defined as the retrograde passage of gastric contents into the esophagus.³ When symptoms or lesions occur as a result of GER, it is referred to as gastroesophageal reflux disease (GERD).⁴ The primary mechanism of GER is transient relaxation of the lower esophageal sphincter. This relaxation can be triggered by vasovagal reflex initiated by gastric distension or cardiopulmonary receptors or by a swallow that does not trigger esophageal peristalsis.³ In children, severe GERD has several known risk factors, including neurologic disorders such as spastic quadriplegia and cerebral palsy, esophageal atresia, chronic lung disease such as cystic fibrosis, and hiatal hernia.^5,6

Clinical Presentation: GER is ubiquitous in infants, occurring in 100% of 3-month-olds, 40% of 6-month-olds, and 5% to 20% of 1-year-olds.4,6 In children and adolescents, GERD increases in incidence with age. Symptoms of GERD are reported to occur on a weekly basis in 2% of 3- to 9-year-olds and 5% to 8.2% of children aged 10-17 years.⁷

The symptoms of GERD depend on age. In infants, symptoms include irritability, feeding difficulty, poor weight gain, and sleep disturbance. In older children, symptoms include heartburn, abdominal pain, regurgitation or vomiting, and dysphagia.³ Extraesophageal symptoms of GERD also can occur and include chronic cough, asthma, apnea, bradycardia, sore throat, dental erosions, and recurrent otitis or sinusitis.³ When compared with adults, children report fewer episodes of heartburn, dysphagia, and chest pain and more episodes of vomiting and regurgitation.⁴

GERD often is diagnosed on the basis of symptoms and a trial of acid-reduction therapy. When symptoms are not specific or are atypical, confirmatory testing can be performed. Intraesophageal pH monitoring traditionally has been thought of as the gold standard because of its ability to measure the pH in the esophagus over a long period. The main limitation of this technique is that patients may have abnormal esophageal acid exposure without symptoms of GERD or retrograde bolus movement in the esophagus.3,8 To evaluate for the retrograde passage of gastric contents and esophageal acidity, combined pH/impedance probes are used.⁸

Imaging: UGI imaging often is performed in the setting of GER to evaluate for an anatomic abnormality; however, it should not be used as the primary method of diagnosing GER or GERD. UGI has multiple limitations in diagnosing GER, including its nonstandard technique, lack of correlation with symptoms, and use of ionizing radiation. Methods used to provoke reflux such as the Valsalva maneuver, positional changes, abdominal compression, or leg lifting may increase the sensitivity of detecting GER but lower the specificity.³ Because more sensitive diagnostic tests are available and the radiologic findings do not correlate with symptoms, the collaborative practice guideline set forth by the American College of Radiology and the Society for Pediatric Radiology do not recommend provocative maneuvers or prolonged fluoroscopy for the detection of GER.⁹

Even though UGI imaging should not be prolonged to identify GER, GER often is present (Fig. 99-1). The percentage of patients who have GER on UGI imaging decreases with age from 80% of infants <18 months to 30% of adolescents between 12 and 18 years.³ It should be noted that although the height of reflux often is reported as a surrogate for its severity, no correlation exists between the height of GER on UGI imaging and symptoms of GERD.³

Technetium-99m sulfur colloid also can be used to diagnose GER. Although scintigraphy is a sensitive method of detecting GER, it has many of the same limitations as UGI mainly, the presence of GER does not correlate with symptoms of GERD. In addition to this limitation, scintigraphy also requires the use of ionizing radiation. A limitation unique to scintigraphy is its inability to diagnose some of the complications of GERD. Scintigraphy is thus reserved for cases in which pH monitoring is not able to be performed or when evaluation of gastric motility also is required.

Esophageal complications of GERD include esophageal strictures and Barrett esophagitis. Esophageal strictures, also known as peptic strictures when they are caused by GERD, typically occur in the lower third of the esophagus and are a result of acidic injury. They are cited as being present in up to 15% of children with GERD and can occur at any age.¹⁰ Many patients who experience strictures have an associated comorbidity, with 25% having a neurologic impairment.¹⁰

Barrett esophagus is defined as metaplasia of cells in the distal esophagus from squamous to columnar epithelium. Its prevalence in children with GERD ranges from 0.25% to 4.8%.6,11 Risk factors for the development of Barrett esophagus in children include severe chronic GERD, congenital abnormalities, neurologic impairment, hiatal hernia, and family history.⁶ Although it is associated with a thirtyfold increase in esophageal adenocarcinoma in adults, the risk of developing adenocarcinoma is not defined in children.⁶

Treatment: Multiple options exist for treating GERD, depending on the patient’s age, comorbidities, and severity of symptoms. Generally, lifestyle changes and pharmacotherapy are first-line options. Lifestyle changes include avoidance of overfeeding, thickening feeds, upright positioning during sleep, and avoidance of second-hand smoke.³ A goal of medical therapy is to decrease the acidity of the refluxed gastric contents, which is generally performed by using proton pump inhibitors and histamine receptor antagonists.³ In patients with continued severe GERD after pharmacotherapy or other comorbidities such as neurologic impairment, antireflux surgery such as Nissen fundoplication is performed. Strictures are treated with esophageal dilation and fundoplication. In a small percentage of patients, severe and recurrent strictures will require extensive and repeated dilatations and may require surgical resection or replacement of the esophagus.¹⁰

Overview: In the United States and Europe, coins are the most commonly ingested foreign body.¹² It is estimated that 4% of all children swallow a coin.¹⁴ Although most coins spontaneously pass through the gastrointestinal tract, they can lodge in the esophagus. Coins typically lodge in one of three locations: the thoracic inlet (60% to 70%), the mid esophagus at the level of the aortic arch (10% to 20%), and just above the lower esophageal sphincter (20%).¹²

Clinical Presentation: Symptoms of chronic foreign body impaction include respiratory distress, asthma symptoms, cough, nausea, vomiting, and dysphagia. Respiratory symptoms are a result of local inflammation surrounding the foreign body.¹⁴ Patients with chronic foreign body impaction are at risk of esophageal perforation.

Imaging: Radiographs are useful to identify the location of the coin and to look for signs of chronic impaction. Coins generally are described as oriented in the coronal plane when they are located in the esophagus on the frontal radiograph, although reports have been made of sagittally oriented coins in the esophagus.¹⁵ Inflammatory changes that develop in patients with chronic foreign body impaction can be visualized as thickening of the space between the esophagus and trachea on lateral chest radiographs (e-Fig. 99-2, A and B); these changes therefore are useful in patients in whom the episode was not witnessed and the time course is not known. An esophagram often is performed in patients with a chronic foreign body impaction to evaluate for perforation or development of a tracheoesophageal fistula.

Treatment: Coins, like other esophageal foreign bodies, can be removed via Foley catheter balloon extraction or endoscopy. Foley catheter extraction has been used as a safe and effective method of coin removal in children older than 1.5 years before esophageal edema has developed.¹⁶ Despite its history of safe extractions and lower cost, Foley balloon extraction has fallen from favor because of concerns about patient safety related to airway compromise or esophageal injury.¹⁷ Endoscopy is now the preferred method for extraction at most centers.

Overview: Button battery ingestion by children is increasing in frequency.¹⁸ Button batteries often are found in objects such as watches, calculators, toys, and hearing aids. Management of battery ingestion is different than that of coins, because batteries lodged in the esophagus can cause severe damage in as little as 2 hours.¹⁸ Tissue damage is caused by one of three mechanisms: leak of alkaline contents, pressure necrosis, and generation of a current causing electrolysis of tissue fluids at the battery’s negative pole.¹⁸

Clinical Presentation: If ingestion is witnessed, extraction should occur before the development of symptoms. If symptoms develop, they include drooling, chest discomfort, choking, gagging, and airway obstruction. Damage can include esophageal perforation or creation of a fistula to the trachea or to a major blood vessel, leading to exsanguination.¹⁸

Imaging: Batteries can be distinguished on radiographs by their characteristic halo appearance with a circle of lucency just within the outer border (Fig. 99-3 and e-Fig. 99-4). An esophagram with water-soluble iso-osmolal contrast can be performed after removal to evaluate for the presence of complications, such as esophageal perforation or a tracheoesophageal fistula.

Treatment: If a battery is lodged in the esophagus, the clinician who requested the imaging study should be contacted immediately to arrange emergent extraction. Follow-up imaging may be indicated, as previously discussed.

Overview: Impacted food boluses are relatively infrequent in children but may be the initial presenting symptom of eosinophilic esophagitis, peptic stricture, achalasia, vascular rings, or an extrinsic mass.19,20 Eosinophilic esophagitis is the major cause of food bolus impaction in children, accounting for >50% of all cases. The most common remaining cause of food bolus impaction in children is an area of narrowing in a patient with prior esophageal atresia repair or Nissen fundoplication surgery.²¹

Clinical Presentation: Acute impaction of food typically leads to symptoms of dysphagia and drooling; difficulty swallowing may lead to aspiration.

Imaging: On UGI imaging, an impacted food bolus appears as a persistent filling defect in the esophagus (Fig. 99-5). The impacted food can completely obstruct the esophagus, which is seen on UGI imaging as a standing column of contrast that does not pass beyond the food bolus.

Treatment: When a food bolus is identified, it is removed endoscopically. An esophagram can be performed after extraction or passage of the food bolus to evaluate for the underlying cause, if it is not already known. An esophageal biopsy is recommended in patients diagnosed with food bolus impaction, particularly if they do not have a history of prior esophageal surgery.

Overview: Ingestion of caustic materials remains relatively common in childhood. In 2009, a total of 212,263 ingestions of household cleansers were reported to the American Poison Control Centers, more than 75% of which occurred in children.¹³ The age group at highest risk for caustic ingestion is children younger than 5 years,¹³ with a peak around 2 years of age when children are learning to explore their home but are not yet able to distinguish between harmless and harmful substances.²²

Whereas caustic ingestion is usually accidental in children, in adults or adolescents it is usually purposeful. The extent and severity of injury depends on several factors: the corrosiveness of the ingested substance, the quantity ingested, the physical state of the substance, the duration of the contact time of the substance with the esophagus, and subsequent secondary infection.22,23

A variety of substances can cause a caustic injury, including alkalis (pH up to 12) and acids (pH as low as 2). In contrast to acidic substances, which are sour, alkalis have a relatively innocuous taste, leading to ingestion of a greater volume.²² Further, alkaline agents produce liquefaction necrosis and rapid penetration, leading to more severe injury.²³

Clinical Presentation: After caustic ingestion, patients with severe injury typically present with pain, drooling, and airway symptoms; more visible signs of tissue damage include lip swelling, mouth ulcers, and erythema of the tongue.²² Esophageal stricture is an important late complication of caustic ingestion, occurring in 2% to 63% of patients²³; it can form in as little as 3 weeks after injury.²⁴

Imaging: Initial management of patients with caustic ingestion includes a radiograph of the chest and lateral neck to evaluate for pneumomediastinum. An esophagram is not indicated in the acute phase because it delays endoscopy and does not reveal mucosal injuries.²²

When strictures develop, they can be diagnosed on the basis of symptoms and confirmed with an esophagram (Fig. 99-6 and e-Fig. 99-7). Caustic strictures can be focal or can occupy a long segment of the esophagus depending on the substance ingested; acid ingestion typically causes a focal or short segment stricture, whereas alkali substances cause a long segment stricture.²⁴ Strictures most commonly occur in the upper or mid esophagus. Multiple strictures also can occur.

Treatment: Acutely, patients are treated with steroids, proton pump inhibitors, and antibiotics. Endoscopic evaluation is performed to assess the extent of damage and to grade the injury. Strictures are first treated with balloon dilation under fluoroscopy. The advantage of balloon dilation over dilation using a bougie is that balloons dilate strictures in a radial direction rather than in a longitudinal direction. This is thought to be less likely to cause an esophageal perforation.²² After the balloon is inflated and the waist is seen to disappear, an esophagram is performed to evaluate for a leak that occurs in 4% to 30% of patients.²⁴ If balloon dilation fails, operative treatment with either stricture resection or esophageal replacement is performed.

Patients with a history of caustic ingestion are at risk of developing squamous cell carcinoma of the esophagus. Given this risk, these patients should undergo surveillance with endoscopy as adults.²²

Overview: Pill esophagitis is an esophageal injury caused by medications; the drugs most frequently implicated are doxycycline and alendronate.²⁵ Other drugs reported to cause esophageal injury include nonsteroidal antiinflammatory drugs, potassium chloride, ferrous sulfate, phenytoin, and quinidine.²⁵ Characteristics of medications that are more likely to cause esophageal injury include the acidity of the medication and a capsule formulation that renders it more likely to stick in the esophagus.²⁶ Pill esophagitis is also more likely to occur in patients with delayed esophageal transit time. Factors that delay esophageal transit include taking a pill with little or no water immediately before going to bed, decreased saliva production, and anatomic areas of narrowing.²⁶

Clinical Presentation: Patients with pill esophagitis typically present with dysphagia, odynophagia, and chest pain. Pill esophagitis often can be diagnosed by history alone.

Imaging: UGI imaging is not commonly performed due to the classic history but can show a circular area of ulceration. If endoscopy is performed, a circular ulceration is identified with normal surrounding mucosa. The ulceration typically occurs at an anatomic area of narrowing such as at the level of the aortic arch, left mainstem bronchus, or gastroesophageal junction.²⁶

Treatment: Pill esophagitis is treated by changing pill-taking habits (e.g., taking pills with more water or taking pills long before going to bed), crushing pills, taking a liquid suspension, or switching medications.

Overview: Iatrogenic trauma is the most common cause of esophageal perforation in children, representing 75% to 85% of all cases.²⁷ Esophageal perforation can be life-threatening because the esophagus lacks a serosa. The surrounding loose areolar connective tissue is unable to prevent the spread of infection; thus oral flora and digestive enzymes are able to spread to the mediastinum.²⁷ The most common cause of iatrogenic perforation in children is from stricture dilation. Other causes include complications of nasogastric tube placement, endotracheal intubation, endoscopy, pedicle screw placement, and sclerotherapy of esophageal varices.^28–30 The typical location of perforation depends on the age of the patient. In neonates, perforation typically occurs in the cervical esophagus at the pharyngeal/esophageal junction.²⁷

Clinical Presentation: Neonates often present after a difficult intubation with nonspecific symptoms, including drooling, choking, or coughing with feedings. In older children, perforation is more common in the thoracic esophagus. They typically present with respiratory distress, chest pain, or subcutaneous emphysema.²⁷

Imaging: The initial diagnostic examination is often frontal and lateral radiographs of the chest. Findings on the chest radiograph include pneumothorax, pneumomediastinum, and pleural effusion. If the event was caused by nasogastric tube placement, an abnormal course of the tube may be seen (e-Fig. 99-8 and Fig. 99-9).²⁷ The location of the perforation often can be determined from the radiograph. Left-sided pneumothorax and effusion are more likely to occur in the setting of an upper thoracic perforation, whereas right-sided findings are more likely to occur with perforations in the distal esophagus.²⁷

UGI imaging can be useful in diagnosing and locating a perforation. If UGI is performed, water-soluble iso-osmolal contrast should be used first. If no contrast extravasation is noted, barium can then be used because it is more sensitive in the detection of an esophageal leak.²⁷ Three patterns of perforation have been described on esophagrams: first, a local cervical leak is identified if there is a retropharyngeal pocket of contrast; a submucosal leak appears as a linear tract posterior and lateral to the esophagus; finally, free perforation appears as contrast flowing into the pleural space.²⁷

CT generally is not used to diagnose an esophageal perforation, but in a complex, acutely ill patient, CT may be the first study performed. Signs of esophageal perforation on CT include pneumomediastinum, mediastinal fluid, esophageal wall thickening, and a catheter extending beyond the esophageal lumen.

Treatment: Treatment usually consists of expectant medical management with broad-spectrum antibiotics, pleural drainage via a chest tube, and nutritional support with total parenteral nutrition or gastrostomy; a nasogastric tube may be placed beyond the perforation under direct visualization. Operative exploration is considered in patients in whom the aforementioned more conservative management fails.²⁷

Overview: The esophagus is rarely injured after chest trauma because of its flexibility and ability to decompress via the mouth and stomach.³¹ Reported causes of esophageal injury include handlebar injury, crush injury, opening a bottle of a carbonated drink in the mouth, and child abuse.^32–35 A tracheoesophageal fistula is an even rarer complication of trauma.

Clinical Presentation: Patients usually present with coughing induced with swallowing. Other findings include subcutaneous emphysema, pneumothorax, dysphagia, dyspnea, and hoarseness.

Imaging: A chest radiograph or CT may show pneumomediastinum and pleural effusion in a child presenting with blunt trauma. UGI can be used to confirm the diagnosis. An esophagram can be performed to diagnose a suspected tracheoesophageal fistula.³⁶

Overview: Boerhaave syndrome is defined as spontaneous rupture of the esophagus, often after vomiting. The rupture typically involves the distal portion of the thoracic esophagus. Boerhaave syndrome is rare in children. When it does occur, nearly 50% of cases occur in neonates.³⁷

Clinical Presentation: Patients typically present with vomiting, lower thoracic pain, and subcutaneous emphysema.

Imaging: In most children the rupture occurs on the right side, in contrast to adults, in whom the rupture is usually left sided,³⁷ and therefore a chest radiograph in an infant will demonstrate a right-sided pneumothorax and pleural effusion. Diagnosis is confirmed with an esophagram and/or endoscopy; however, insufflation during endoscopy may lead to a life-threatening pneumothorax.

Treatment: Although children and neonates are more likely to have a better prognosis than adults, surgery remains the treatment of choice for persons with Boerhaave syndrome. A conservative approach can be used in patients with small tears and is used in patients with presentation or diagnosis greater than 24 hours.³⁷

Overview: Eosinophilic esophagitis is a chronic inflammatory disease characterized by dense esophageal eosinophilia. During the past decade, awareness of the disease has increased along with an increase in the number of cases diagnosed. Currently it is thought that eosinophilic esophagitis occurs with an incidence of up to 1 : 10,000 children per year.³⁸ Eosinophilic esophagitis is three to four times more common in males and can occur at any age.³⁹ To be diagnosed with eosinophilic esophagitis, patients must have both the histologic and clinical features of the disease. Histologically, eosinophilic esophagitis is characterized by an esophageal mucosal biopsy with more than 15 eosinophils per high-power field.

Clinical Presentation: Patients present with esophageal symptoms that mimic GERD. Older children tend to present with vomiting and abdominal pain, although in infants the presentation is more likely feeding difficulties; diagnosis in infants younger than 6 months is rare. Patients often have other signs of atopy, including dermatitis, food allergies, and asthma.³⁹

Imaging: Because the symptoms of eosinophilic esophagitis are protean and simulate those of GERD, diagnosis often is delayed. UGI frequently is performed as part of the diagnostic workup of patients because of their esophageal symptoms. On UGI imaging, the most common finding is a normal esophagus.⁴⁰ The most common positive findings are GER, irregular contractions, esophageal dysmotility, esophageal strictures, esophageal rings, small caliber esophagus, and food bolus impaction (Fig. 99-10).⁴⁰

Treatment: Eosinophilic esophagitis is thought to represent an allergic response to food and is usually treated with attempts to identify and eliminate the offending agent(s), or with an elemental diet. Steroids also may be used alone or combined with diet therapy.³⁹

Overview: Epidermolysis bullosa is a rare group of hereditary blistering disorders caused by mutations in genes expressed at the dermal-epidermal junction. More than 100 distinct genotypes and 20 phenotypes of disease of varying severity have been described.⁴¹

Clinical Presentation: Patients often experience skin blistering and erosions with minor mechanical trauma. Repeated blistering leads to scar formation and causes symptoms such as contractures. Extracutaneous sites also can be affected and include the esophagus, bowel, rectum, anus, bladder, urethra, and trachea.⁴¹

Imaging: In the esophagus and oropharynx, scar formation leads to dysphagia and esophageal strictures. Any segment of the esophagus may be involved, although approximately half of strictures occur in the cervical esophagus.⁴²

Treatment: The initial treatment of choice for esophageal strictures in children is balloon dilation under fluoroscopic or endoscopic visualization (Fig. 99-11 and e-Fig. 99-12).^41,42 Patients often require multiple dilations to treat recurrent or persistent strictures.⁴²

Overview: Crohn disease is a chronic inflammatory disease of the gastrointestinal tract characterized by waxing and waning symptoms, transmural inflammation of the bowel wall, and skip lesions. It can affect any portion of the gastrointestinal tract from the mouth to the anus, but it most commonly affects the small bowel and colon in children. Esophageal symptoms of Crohn disease are uncommon; however, endoscopic and histologic findings are more frequent and occur in 7.6% and 17.6%, respectively.⁴³

Clinical Presentation: Dysphagia is the most common symptom of esophageal Crohn disease followed by odynophagia, chest pain, nausea, and vomiting.⁴⁴

Imaging: Although the esophagram often is normal, esophageal Crohn disease can manifest with mucosal irregularity, aphthous ulcers, or stricture.⁴³

Overview: Chronic granulomatous disease is an uncommon primary immunodeficiency characterized by recurrent bacterial and fungal infections occurring at epithelial surfaces. Gastrointestinal manifestations are present in most patients with chronic granulomatous disease. Any portion of the gastroesophageal tract may be involved from the mouth to the anus. Esophageal involvement is uncommon.

Clinical Manifestations: The symptoms overlap those of other entities affecting the esophagus and include vomiting, dysphagia, heartburn, and odynophagia.⁴³

Imaging: In the esophagus, dysmotility and strictures have been seen on UGI.⁴⁵ CT of the chest can demonstrate esophageal wall thickening (Fig. 99-13).⁴⁶

Overview: Behçet disease is an immune-mediated systemic vasculitis that affects multiple systems. It likely represents an exaggerated inflammatory endothelial response. The entity is more prevalent in Mediterranean and Middle Eastern countries, as well as Japan and Southeast Asia.

Clinical Manifestations: Behçet syndrome is a multisystem disorder characterized by pyoderma, gastrointestinal and genital mucous membrane ulceration, uveitis, and central nervous system vasculitis.⁴⁷ Oral ulcerations often are the first manifestation of disease. Because these ulcerations are nonspecific, diagnosis often is delayed until abnormalities of the genitals, gastrointestinal system, or central nervous system develop. Patients also are at risk of experiencing venous thrombosis and arterial aneurysms, which can be life threatening.

Imaging: In the esophagus, dysmotility, ulceration, and stricture have been described on UGI imaging.⁴⁷ These findings often are difficult to distinguish from Crohn disease. Diagnosis is made by the presence of mouth ulcers in association with two other major criteria, including skin lesions, recurrent genital ulcerations, or the presence of an excessive dermal inflammatory response termed “pathergy.”

Treatment: Treatment is geared at reducing the overactive inflammatory response and includes corticosteroids, azathioprine, and interferon.⁴⁵

Overview: Infectious esophagitis typically is seen in immunocompromised children. Affected children may have a congenital or acquired immunodeficiency. Children receiving immunosuppressive therapy such as chemotherapy or posttransplant immunosuppression also may be affected.

Imaging: Endoscopy has replaced UGI imaging as the method of diagnosing infective esophagitis because of its ability to visualize the abnormality directly and to obtain a tissue sample for culture. If UGI is performed for infectious esophagitis, the double-contrast technique is preferred, if possible, to visualize mucosal detail.

Overview: Candida albicans is the most common infective agent causing esophagitis.⁴⁸ Oropharyngeal candidiasis is the strongest risk factor for the development of esophagitis in patients with neutropenia or human immunodeficiency virus (HIV) infection.

Clinical Manifestations: Patients typically present with odynophagia, dysphagia, and chest pain.

Imaging: On endoscopy, the appearance of esophageal candidiasis varies from a few small, raised white plaques to confluent plaques with hyperemia and ulceration.⁴⁸ An esophagram is less sensitive than endoscopy in making the diagnosis.⁴⁸ On esophagram, severe infection is characterized by a shaggy appearance of the esophagus as a result of multiple ulcers and nodular plaques.

Treatment: Treatment is with antifungal medications, including fluconazole and nystatin. Caspofungin and amphotericin are reserved for systemic cases and cases resistant to therapy. Prophylactic therapy is used in immunocompromised patients.

Overview: Cytomegalovirus (CMV) is common in the population at large and is one of the more common opportunistic infections in immunocompromised patients. When it affects the GI tract, CMV most commonly involves the esophagus and the colon.

Clinical Manifestations: Patients with CMV involving the GI tract present with chronic diarrhea, abdominal pain, bleeding, perforation, odynophagia, peritonitis, and bowel obstruction.⁴⁹

Imaging: Esophagitis is the second most common gastrointestinal manifestation of CMV infection after colitis.⁴⁹ Endoscopy is the diagnostic method of choice; however, UGI imaging can demonstrate the large, shallow ulcers characteristic of CMV infection.

Treatment: The most effective treatment for CMV infection in immunocompromised patients is restoration of immunocompetence, such as the use of antiretroviral agents in patients with HIV.⁴⁹

Overview: Giant esophageal ulcers can occur in patients with HIV infection. Ulcers in these patients have several different causes, including CMV, Candida albicans, mycobacteria, and herpes simplex virus.⁵⁰ The ulcer is called an HIV-related or idiopathic giant ulcer when the cause of the ulcer cannot be attributed to a known pathogen.

Clinical Presentation: Patients present with esophageal symptoms of odynophagia, dysphagia, and retrosternal chest pain and may experience hematemesis. Complications include bleeding, strictures, and fistulas into the tracheobronchial tree.⁵¹

Imaging: Idiopathic giant ulcers typically occur in the middle to distal third of the esophagus. Smaller satellite ulcers also may be present.⁵⁰ Idiopathic giant ulcers are difficult to distinguish from ulcers caused by CMV infection, although idiopathic giant ulcers are said to be larger and have overhanging edges.⁵⁰

Treatment: Treatment is geared to the infectious agent that is identified. If the ulcer is idiopathic, treatment is with steroids; thalidomide also may be added.⁵¹

Overview: Involvement of the esophagus by tuberculosis is rare, occurring in 0.15% of patients. Several reports have been made of esophageal erosion with formation of a tracheoesophageal fistula in patients with tuberculosis.52,53 Esophageal perforation is thought to occur by one of three methods: rupture of a mediastinal abscess into the esophagus, formation of a traction diverticulum as a result of tuberculous mediastinitis, or erosion as a result of pressure necrosis of adjacent lymph nodes.⁵²

Clinical Presentation: Symptoms depend on the complications; patients with esophagotracheal or esophagobronchial fistulas may experience extensive coughing after the ingestion of fluids, along with hematemesis.⁵⁴

Imaging: When a perforation occurs, an esophagram shows extraluminal extension of contrast into the mediastinum or through the fistula. On CT, large, low-density mediastinal lymph nodes are observed in addition to pneumomediastinum.

Treatment: Treatment is geared to addressing the complications in addition to use of antituberculous medications.

Overview: Esophageal atresia is a congenital malformation occurring in 1 in 2500 births (see Chapter 97, Congenital and Neonatal Abnormalities). It is corrected via surgical repair in the early neonatal period. The repair has several potential complications: recurrent tracheoesophageal fistula, esophageal strictures at the site of anastomosis, and food bolus impaction (Fig. 99-14).

Esophageal dysmotility is nearly ubiquitous in patients with a history of esophageal atresia. Two potential theories for the etiology of esophageal dysmotility have been proposed: primary dysgensis of the esophageal nerve supply, or postoperative damage to the esophageal nerve supply after the initial repair.⁵⁵

Overview: Achalasia is a primary motor disorder characterized by failure of the lower esophageal sphincter to relax with swallowing.⁵⁶ Achalasia typically presents with dysphagia, chest pain, vomiting, regurgitation, or symptoms of GER.⁵⁷ Diagnosis can be made via manometry, upper endoscopy, or UGI imaging. Manometry is the gold standard for diagnosis and shows aperistalsis of the smooth muscle of the esophagus, incomplete relaxation of the lower esophageal sphincter, and elevated resting pressure in the lower esophageal sphincter.⁵⁸ Biopsy confirms the diagnosis of achalasia with hypertrophy of the muscularis and absent ganglion cells.

Clinical Presentation: Patients present with progressive dysphagia, regurgitation, chest pain, nocturnal cough, and heartburn.⁵⁸ Aspiration pneumonia, esophageal perforation, or esophageal cancer can develop in patients with untreated achalasia.

Imaging: On the chest radiograph, the esophagus can appear dilated and filled with air (Fig. 99-15). This appearance is often unusual and can mimic a medial pneumothorax. UGI imaging shows dilation of the proximal esophagus, lack of peristalsis, and smooth tapering of the distal esophagus with a bird’s beak appearance near the gastroesophageal junction.⁵⁸ This appearance of the distal esophagus also can be seen on sonography⁵⁸ and should be recognized if it is captured incidentally during abdominal ultrasound imaging.

Treatment: Achalasia can be treated with balloon dilation or Heller myotomy.⁵⁷

Overview: Although they are rare, leiomyomas are the most common esophageal tumor in children. In contrast to adults, the lesions in children are more likely to be multiple or diffuse.⁵⁹ Diffuse esophageal leiomyomatosis is a rare hamartomatous condition in which proliferation of the smooth muscle of the esophagus occurs. Esophageal leiomyomatosis can be associated with leiomyomas at other sites or with Alport syndrome (i.e., nephropathy, astigmatism, and myopia).⁶⁰

Clinical Presentation: Isolated leiomyomas may be an incidental finding, or patients may present with progressive symptoms of dysphagia, vomiting, regurgitation, weight loss, and retrosternal pain.⁶⁰

Imaging: Two potential findings on a chest radiograph are a tubular posterior mediastinal mass and rightward deviation of the azygoesophageal stripe.59,60 Findings on an esophagram can mimic achalasia and demonstrate a dilated tortuous esophagus with decreased peristalsis, tapered narrowing, and deviation from midline caused by the intramural masses.⁶⁰ If cross-sectional imaging is performed, it can show diffuse circumferential wall thickening of the esophagus; a discrete soft tissue mass protruding from the esophagus also may be present.⁶⁰

Treatment: Diffuse esophageal leiomyomatosis generally is treated with an esophagectomy and gastric pull-through.

Overview: Esophageal varices are seen in the setting of portal hypertension. Varices primarily occur around the esophagus, stomach, spleen, and retroperitoneum. When portal hypertension is suspected, endoscopy is the best method to screen for varices.

Clinical Presentation: Patients present with symptoms of portal hypertension; varices themselves are silent until an episode of bleeding ensues. Variceal bleeding is the most serious complication of portal hypertension and is associated with a mortality of up to 30%.⁶³

Imaging: Varices can be identified on contrast-enhanced CT as dilated serpiginous vessels (Fig. 99-18). On UGI imaging, they appear as a lobulated filling defect in the distal esophagus.

Treatment: Varices typically are treated with sclerotherapy, banding, or placement of a portosystemic shunt.

Overview: Esophageal diverticula are uncommon in children. They can be associated with stricture, prior esophageal atresia repair, prior injury such as with a chronic foreign body, and connective tissue disorders such as Ehlers-Danlos syndrome.64–66

Overview: Plummer-Vinson syndrome is an uncommon disease that typically occurs in middle-aged white women, although it can occur in the pediatric age group.

Clinical Presentation: Plummer-Vinson syndrome is characterized by dysphagia, iron deficiency anemia, and esophageal webs. It is associated with celiac disease and increased menstrual blood loss.⁶⁷

Imaging: The esophageal webs are best seen on esophagram or UGI where they typically occur in the proximal esophagus. They are characterized by one or more thin, horizontal membranes in the esophageal lumen.⁶⁷

Treatment: Patients are treated for the underlying cause of anemia along with iron supplements and esophageal dilation. Esophageal or pharyngeal cancer occurs in 3% to 15% of patients with Plummer-Vinson syndrome.⁶⁷