Disorders of the Esophagus

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107 Disorders of the Esophagus

Amy Feldman, Rose C. Graham

The esophagus is a muscular tube connecting the pharynx and the stomach. Its role is to move material from the mouth to the stomach. It does not produce any digestive enzymes and has no active role in digestion. Disorders of the esophagus can present with chest or abdominal pain, difficulty swallowing, abnormal movement of gastric contents, or gastrointestinal (GI) bleeding. Disorders involving the esophagus include developmental anomalies, motility disorders, gastroesophageal reflux (GER), esophagitis, and traumatic injury.

Developmental Anomalies

Congenital disorders of the esophagus occur in approximately 1 in 3000 to 5000 births. These disorders commonly occur during embryogenesis as the trachea separates from the esophagus, and include atresia with or without tracheoesophageal fistula (TEF), esophageal stenosis, esophageal duplication, esophageal webs and rings, esophageal diverticulum, and esophageal or bronchogenic cysts.

Etiology and Pathogenesis

The esophagus forms from a small ventral diverticulum of the embryonic foregut. This diverticulum separates into the esophagus and trachea around the fourth gestational week of fetal development. Anomalies can occur with abnormalities in any step in esophageal formation and development. One of the most common anomalies is TEF with or without atresia. This occurs when there is a disruption in the elongation and separation of the trachea from the esophagus. There are five types of TEF. The most common is type C in which there is atresia of the proximal esophagus with a fistula from the trachea to the distal esophagus (Figure 107-1). Other categories of TEF, in descending frequency, include isolated esophageal atresia without TEF, isolated TEF, esophageal atresia with proximal TEF, and esophageal atresia with proximal and distal TEF.

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Figure 107-1 Tracheoesophageal fistula.

Congenital disorders of the esophagus are often associated with polyhydramnios as a result of the infant’s inability to swallow and absorb amniotic fluid and with prematurity. As many as 70% of children with esophageal abnormalities may have other congenital anomalies, including imperforate anus, vertebral anomalies, duodenal atresia, and annular pancreas. The VACTERL association is a combination of defects that are commonly found together: vertebral anomalies, anorectal atresia, cardiac anomalies, tracheoesophageal fistula, esophageal atresia, renal anomalies, and limb anomalies.

Clinical Presentation

A neonate with an esophageal congenital anomaly may present with episodes of respiratory distress worsened by feeding, regurgitation, a history of multiple episodes of pneumonia, or failure to thrive. Older children may present with dysphagia, regurgitation, halitosis, or respiratory symptoms. The severity of symptoms depends on the degree of esophageal compression or obstruction.

Evaluation and Management

Esophageal atresia or complete esophageal obstruction can be diagnosed by an inability to pass an orogastric or nasogastric tube into the stomach. TEF may be suggested on abdominal or chest radiograph; however, an isolated TEF (H-type) is best detected by esophagography with contrast. Barium swallow or endoscopy can be useful in diagnosing stenosis or incomplete obstruction from a web, ring, cyst, or diverticulum. Surgical correction is required to treat most congenital anomalies.

Motility Disorders of The Esophagus

The esophagus relies on a coordinated motility effort to drive food forward into the stomach and to clear acidic and bilious secretions that may leak upward from the stomach. If a motility disorder disturbs this muscular endeavor, proper delivery of food and clearance of gastroesophageal fluids cannot occur.

Etiology and Pathogenesis

Striated muscle makes up the upper esophageal sphincter along with the proximal one-third of the esophagus. Smooth muscle composes the remaining two-thirds. The lower esophageal sphincter (LES) is a physiologic sphincter. There is a rich nerve supply to the esophagus. Motility problems can arise from muscular disorders (polymyositis, dermatomyositis, muscular dystrophy, myasthenia gravis), neurologic disorders (stroke, multiple sclerosis, lead poisoning), systemic illness (lupus, scleroderma, sarcoidosis, thyroid disease, diabetes), or infection (tetanus, botulism, Trypanosoma cruzi infection).

Achalasia

Achalasia is a progressive motor disorder of the esophagus resulting in increased lower esophageal pressure, impaired relaxation of the LES during swallowing, and impaired esophageal peristalsis. This produces a functional obstruction at the esophagogastric junction. It is an uncommon disorder in the pediatric population. In the United States, childhood-onset achalasia is most often idiopathic, but systemic disease should be considered in determining the etiology. Histologic changes seen with achalasia include loss of ganglion cells, a decrease in the number of myenteric plexus nerve fibers, and degeneration of the vagus nerve.

Spastic Motility Disorders

Spastic esophageal disorders occur from diffuse esophageal spasm (contraction of more than one esophageal site at the same time), nutcracker esophagus (esophageal contractions of amplitude >180 mm Hg and duration >6 sec), or a hypertensive LES (lower esophageal pressure >44 mm Hg).

Clinical Presentation

Achalasia

Symptoms of achalasia include food and liquid dysphagia, regurgitation, vomiting, choking and coughing episodes, a sense of “food getting stuck,” a gurgling noise coming from the chest, postprandial and nocturnal chest pain, recurrent episodes of pneumonia, and loss of weight. Leiomyoma of the distal esophagus, anorexia nervosa, rumination syndromes, Chagas’ disease, and candidal esophagitis are other diseases with similar symptoms that need to be considered in the differential diagnosis. Allgrove’s syndrome is an autosomal recessive disorder that includes achalasia, alacrima, and adrenocorticotropic hormone insensitivity.

Spastic Motility Disorders

Spastic motility disorders present with dysphagia and substernal chest pain. Unlike cardiac chest pain, pain associated with esophageal dysfunction is nonexertional, occurs at night, is affected by ingestion of food, and responds to antacids. Loss of weight is not usually seen with these disorders.

Evaluation

Achalasia

Chest radiograph may show widening of the mediastinum, loss of the gastric air bubble, or an esophageal air-fluid level. Barium swallow will show impaired contrast movement, abnormal peristalsis, and tapering of the distal esophagus known as the “bird’s beak” sign (Figure 107-2). Endoscopy should always be performed and often shows esophageal dilatation, erythema, and ulceration from stasis. Esophageal manometry assesses LES pressures and function in addition to esophageal contraction and motor pattern. In achalasia, manometry shows increased lower esophageal pressure, abnormal peristalsis, and elevated intraesophageal pressures.

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Figure 107-2 Barium swallow findings seen with achalasia.

Spastic Motility Disorders

Barium swallow may reveal nonperistaltic contractions intermixed with normal peristaltic contractions. Manometry reveals similar pathologic findings observed in achalasia.

Management

Achalasia

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