The esophagus is a hollow muscular tube connecting the pharynx to the stomach. It courses in the posterior mediastinum behind the trachea, passes through the esophageal hiatus of the diaphragm, and joins the stomach at the cardia. Embryologically, the esophagus and trachea both develop together from the foregut. During the fourth week of gestation, the foregut endoderm differentiates into a ventral respiratory component and a dorsal esophageal component. The separation of the respiratory part from the esophageal part is achieved by the formation of lateral longitudinal tracheoesophageal folds. After 7 weeks gestation, the separation of the esophagus and trachea is complete. Familiarity with the embryologic development of the esophagus and trachea is important to understand the congenital abnormalities that may arise from impairment in the process of septation of the trachea and esophagus.

The length of the esophagus is 8–10 cm at birth and doubles in the first few years of life. The esophageal wall is composed of four layers: mucosa, submucosa, muscularis propria, and adventitia. The esophagus lacks a distinct serosa. The mucosa is lined by nonkeratinizing, stratified squamous epithelium. The muscularis mucosa is the deepest layer of the mucosa and contains longitudinal smooth muscle fibers. The submucosa contains the venous and lymphatic plexuses. The muscularis propria contains the inner circular and outer longitudinal muscle layers. The upper third of the esophagus is composed primarily of striated muscle fibers under voluntary control. The middle third comprises mixed striated and smooth muscle fibers, and the lower third of the esophagus contains only smooth muscle fibers under autonomic control. The mucosa is the strongest layer of the esophageal wall, in contrast to the small intestine, in which the submucosa is the strongest. When the esophagus is divided, the mucosa will retract. Meticulous approximation of the esophageal mucosa is essential for a technically sound anastomosis. Two sphincters control passage of contents into the gastrointestinal (GI) tract: an anatomic upper esophageal sphincter (UES), consisting of the cricopharyngeal and inferior pharyngeal constrictors, and a physiologic lower esophageal sphincter (LES).

The blood supply to the proximal esophagus is derived from the fourth branchial arch. The fourth branchial arch gives rise to the subclavian artery and its branches, including the inferior thyroid artery, which supplies the cervical esophagus. The thoracic esophagus is supplied directly from branches of the aorta (aortoesophageal branches). The abdominal esophagus has a generous vascular supply from the phrenic branches and gastric vessels. The excellent submucosal plexus of the proximal esophagus allows for extensive mobilization without compromise to the vasculature, whereas caution should be taken distally because of the segmental esophageal blood supply.

Lesions of the upper esophagus are best approached through the right chest to avoid problems with the aortic arch. The azygos vein may need to be ligated and divided where it crosses the esophagus. As long as the superior vena cava is patent, the azygos vein can be divided without consequence. Lesions of the lower esophagus may be explored through either the right or left chest. To expose the distal esophagus via the left chest, the inferior pulmonary ligament must be divided, taking care not to injure the inferior pulmonary vein that runs in the upper portion of the pulmonary ligament.

The structure and function of the esophagus may be assessed with radiographic contrast studies, high-resolution-manometry (HRM), and flexible or rigid endoscopy. A barium esophagram provides anatomic information regarding mechanical obstruction or stricture, leak, and extrinsic compression from mediastinal lesions. It also provides some functional information regarding peristalsis, lower esophageal sphincter (LES) relaxation, and the presence/absence of GER. High-resolution-manometry is performed with a flexible catheter to measure pressures at multiple points along the esophagus and is the gold standard for evaluating motility disorders. Diagnostic esophagoscopy is frequently used to evaluate dysphagia and gastroesophageal reflux. Therapeutic esophagoscopy is used to dilate esophageal strictures, evaluate for trauma, aid in sclerotherapy for bleeding esophageal varices, and place gastrostomy tubes. Both rigid and flexible esophagoscopes are available for use in children of all ages.

Flexible endoscopy is the technique of choice for routine diagnostic esophagoscopy in children. The rigid esophagoscope, although useful, is less versatile and requires more experience to use effectively. The main value of rigid esophagoscopy in current pediatric practice is for therapeutic procedures such as dilation of an esophageal stricture or removal of a foreign body, but even these may be supplanted by flexible endoscopic techniques.

Rigid esophagoscopy typically requires general anesthesia with endotracheal intubation and muscle relaxation. For both approaches, the child is positioned supine with a roll under the shoulders to extend the neck. With care taken to protect the teeth, the esophagoscope, with its bevel up, is introduced into the oral cavity along the hard and soft palates to identify the cricopharyngeus muscle and enter the esophagus. Once the most distal aspect of evaluation is reached, it is easy to examine the esophagus fully when withdrawing the scope to identify any lesions or foreign bodies missed on insertion. Occasionally, the use of an air insufflator (such as one from a rigid proctoscope) may be attached to the rigid scope to enhance the view by distending the esophagus.

Flexible endoscopy may be performed under sedation or general anesthesia. The endoscope is passed through the oropharynx and cricopharynx into the upper esophagus under direct vision. The scope should be advanced down the esophagus carefully, being sure to continually maintain visualization of the esophageal lumen. The endoscope should never be advanced blindly. If the lumen is not apparent, the scope should be withdrawn slightly with gentle air insufflation until the lumen is identified. Once the stomach is entered, it can be insufflated to allow inspection of the mucosa. In small infants, overdistention of the stomach may lead to respiratory distress.

Complications related to passage of a rigid or flexible endoscope are typically at the level of the cricopharyngeus muscle. Fortunately, perforation during diagnostic esophagoscopy is exceedingly rare. ^, Perforation of the cricopharyngeal muscle occurs in about 0.10% with flexible endoscopy and 0.07% in rigid esophagoscopy.

Congenital anomalies of the esophagus include EA, TEF, congenital esophageal stenosis, and duplication cysts. EA and TEF are discussed in Chapter 25 .

Congenital Esophageal Stenosis

Congenital esophageal stenosis is a rare childhood condition, with an incidence of 1 in 25,000–50,000 live births. One-third of patients with congenital stenosis have EA. The remaining are isolated cases. Three histopathologic variants are seen: tracheobronchial remnants, membranous diaphragms or webs, and diffuse fibrosis of the muscularis and submucosa. Most infants have normal physical findings at birth. As a result, congenital esophageal stenosis is rarely diagnosed in the neonatal period. The onset of symptoms, most commonly vomiting, dysphagia, and failure to thrive, typically develop with the introduction of solid food at ages 4–10 months. An esophagogram may show an abrupt or tapered stenosis, commonly at the junction of the middle and distal third of the esophagus ( Fig. 24.1A–C ). Additional workup, including esophagoscopy with biopsy and pH impedance, is important to exclude the more common diagnosis of GER-associated stricture and esophagitis. When associated with EA, stenosis is usually found in the distal one-third of the esophagus. Endoscopic ultrasonography or chest computed tomography (CT; Fig. 24.1D ) should be performed to evaluate for tracheobronchial remnants, which may be missed on routine endoscopic biopsy. Pneumatic balloon dilation may be attempted initially, with success approximating 90%–95%. Stenosis due to tracheobronchial remnants responds less well to dilation, with a lower rate of success and higher rate of perforation. Patients who fail to respond to dilation should have a limited resection of the stenosis through the left chest, either open or thoracoscopically, with primary end-to-end anastomosis for long-term relief. ^,

(A–D) (A) This infant presented with significant dysphagia and emesis at 1 month of age. An esophagogram revealed marked narrowing of the distal esophagus ( arrow ). The stenosis was evaluated by endoscopy (B) but could not be dilated, thus a gastrostomy tube was placed, and the child progressed to oral feeds. Subsequent imaging (Upper Gastrointestinal Series [UGI] and CT chest) revealed a tracheobronchial remnant in the distal esophagus (C, D). This child underwent resection of stenosis and primary repair of the esophagus at 9 months of age.

Achalasia is a rare motility disorder of the esophagus characterized by absent or poor esophageal peristalsis and failure of the LES to relax during swallowing. The specific etiology is unknown. Achalasia is rare in children, with a reported incidence of 0.11 cases per 100,000. It has been associated with various syndromes, including trisomy 21, congenital hypoventilation syndrome, glucocorticoid insufficiency, Chagas disease, and Allgrove (Triple A) syndrome (achalasia, alacrima, and adrenocorticotropic hormone insensitivity).

Presenting symptoms of achalasia are age dependent. Infants present with frequent regurgitation, choking, pneumonia, and failure to thrive. Symptoms in older children are comparable to those seen in adults and include dysphagia, regurgitation, retrosternal chest pain, and weight loss. Given its rarity, this disorder is commonly misdiagnosed as GER, or an eating disorder in older children, thus resulting in delayed diagnosis.

Barium esophagography will show aperistalsis of the esophagus, a dilated esophagus, and minimal or no opening of the LES, known as the “bird’s beak” sign ( Fig. 24.2 ). HRM is the standard diagnostic test for achalasia, and typical findings include elevated LES pressure; failure of LES relaxation with swallowing; and low amplitude, nonprogressive, or absent peristaltic contractions in the esophageal body.

This barium swallow was performed in a 16-year-old patient with dysphagia secondary to achalasia. The classic “bird’s beak” narrowing of the distal esophagus at the level of the spastic, contracted esophagus is seen. Also, note the dilated esophagus proximal to the lower esophageal sphincter.

There is no cure for the underlying pathology in achalasia. The aim of therapy is to reduce the LES pressure to facilitate esophageal emptying and improve symptoms. Symptoms of achalasia are quantified using the Eckardt score, a numerical symptom scale of 0–12 in which normal is ≤ 3. Treatment has involved several approaches: pharmacologic agents, Botox injection of the LES, mechanical dilation, and esophagomyotomy.

Pharmacologic treatment with nitrates or calcium channel blockers may result in a decrease in the LES pressure, but the response is short lived. The need for long-term medication limits the usefulness in children. Intersphincteric injection of Botulinum toxin (Botox) has been used in recent years for the treatment of achalasia. Botox is a neurotoxin that binds to presynaptic cholinergic terminals in skeletal muscle, inhibiting the release of acetylcholine at the neuromuscular junction, which creates chemical denervation. Although initially effective, there is a high recurrence rate that also limits its applicability in children. Because of the limited success of these therapies, they are not used for primary therapy but may be considered in rare cases in which children are unable to undergo general anesthesia.

Pneumatic dilation involves forceful dilation of the LES with a balloon dilator of adequate size (20–35 mm in older children) to partially disrupt the sphincter muscle complex. Immediate relief of symptoms may be observed in many patients following pneumatic dilation. However, symptoms typically begin to recur around 6 months, requiring either repeated pneumatic dilations or definitive esophagomyotomy. The perforation rate with pneumatic dilation is about 5% and can often be managed conservatively or with immediate operative repair and myotomy based on the patient’s clinical status.

Esophagomyotomy is the definitive treatment of achalasia with disruption of the muscularis propria of the LES without penetrating the esophageal or gastric mucosa. This has been accomplished with various approaches: thoracic or abdominal, open or minimally invasive, as well as by completely endoscopic methods. Since relieving the LES pressure may induce GER in up to 50% of patients, an antireflux procedure is typically performed at the same time. Recent studies demonstrate that the technique favored by most surgeons is an extended laparoscopic Heller myotomy (LHM), with or without partial fundoplication (Dor, Thal, or Toupet; Fig. 24.3A–D ). This approach seems to best balance the competing goals of adequate relief of dysphagia and minimization of reflux. The esophageal myotomy should be extended 4–6 cm above and 2–3 cm distal to the esophagogastric junction. Most surgeons perform the myotomy with a hook cautery or ultrasonic scalpel. Adjunctive techniques have included use of intraoperative endoscopy, EndoFLIP (functional lumen imaging probe), and intraoperative manometry, which allow assessment of the mucosal integrity and adequacy of the myotomy.

(A–D) Port placement for a laparoscopic Heller myotomy in a smaller child (5 kg) is shown in Fig. (A). The myotomy is initiated through the longitudinal muscle to the circular muscle layer and carried down to the submucosal layer using hook cautery and blunt dissection (B, C). If a Dor fundoplication is performed following the esophagomyotomy, the anterior portion of the stomach is fixed to the muscular edge of the myotomy and crus on the patient’s left side (D). It is then secured to the muscular edge of the myotomy and crus on the right side. The anterior stomach is then secured to the lateral portion of the patient’s right crus to keep it in an anterior position. Lastly, the anterior stomach is sutured to the right border of the stomach to take tension off the other sutures.

From Kane TD, Wall JK. Laparoscopic and Endoscopic esophagomyotomy. In: Holcomb GW III, Rothenberg SS, eds. Atlas of Pediatric Laparoscopy and Thoracoscopy . Elsevier; 2022:21–30.

Recent reports demonstrate excellent outcomes after LHM with partial fundoplication, with resolution of symptoms and normalization of the Eckardt score in 85%–100%. ^{, ,} Significant reflux develops in 9%–17% and can usually be managed with medications. Intraoperative mucosal perforation occurs in 5%–30%. When recognized intraoperatively, this is typically managed by covering the perforation with an anterior fundoplication, usually with no long-term adverse sequelae. Symptom relief is durable in most patients, although reintervention rates for recurrent symptoms are higher in children than in adults. ^, In two recent series, 15%–25% required postoperative balloon dilations or redo LHM with takedown of the fundoplication. ^, Persistence or recurrence of dysphagia following myotomy is thought to be a result of incomplete disruption of the muscle fibers of the distal esophagus or somatic growth in children.

Esophagomyotomy has even been used to effectively treat achalasia in infants. Although little data are available regarding this extremely uncommon indication, infant achalasia is thought to have a different natural history than achalasia in older children. We have operated upon three infants under 1 year of age with LHM without fundoplication with excellent results. These infants did not spontaneously improve, despite expectant management and temporary gastrostomy placement.

Peroral endoscopic myotomy (POEM) is an emerging technique that allows an extended esophagomyotomy to be performed endoscopically ( Figs. 24.4A–E and 24.5A–D ). The submucosal plane is entered in the proximal esophagus with a flexible endoscope, and a distal submucosal tunnel is created. An extended myotomy is then performed using an endoscopic knife and cautery, and the mucosal defect is closed with clips. ^, Outcomes to date appear similar to LHM with partial fundoplication, with dysphagia relief approaching 100% and reflux developing in 11%–46%. Relief from dysphagia also appears to be durable, with a recent series reporting 100% of patients symptom-free at 24 months. Complications included mucosal injury at the myotomy site (18%), pneumothorax requiring chest tube placement (7%), and pneumoperitoneum requiring needle decompression (7%). At present, the procedure is performed by relatively few pediatric surgeons given the requirement for specialized endoscopic equipment and expertise.

(A–E) POEM steps A submucosal wheal is created with methylene blue and a mucostomy is performed with a triangle knife (A, B). A submucosal tunnel is created with blunt and cautery dissection down past the esophagogastric junction (C). The circular muscle myotomy is performed with triangular knife for 2 cm onto the stomach and 4–5 cm on the proximal esophageal side (D). The mucostomy is closed with clips to complete the operation (E).

From Kane TD, Wall JK. Laparoscopic and Endoscopic esophagomyotomy. In: Holcomb GW III, Rothenberg SS, eds. Atlas of Pediatric Laparoscopy and Thoracoscopy . Elsevier; 2022:21–30.

(A–D) Drawings of POEM technique. Submucosal wheal and mucostomy is created approximately 10 cm above the esophagogastric junction (A). Submucosal tunnel is created down past the esophagogastric junction (B). The myotomy is performed for 2 cm onto the stomach and 4–5 cm proximally on the esophagus (C). The mucotomy is closed with clips (D).

In our experience, POEM is the preferred technique for achalasia in children with equivalent if not better results than our experience with LHM. POEM is our preferred technique for esophagomyotomy for achalasia in children, and we now have experience with over 90 patients managed by POEM, with over 30 being 10 years of age or younger. It is important to remember that treatment for achalasia is palliative, not curative. Even following operative intervention for achalasia, children have significantly lower quality of life (QOL) scores than children with inflammatory bowel disease and have QOL scores that are comparable to those of children with chronic constipation. Population-based studies have found a 16-fold risk of developing esophageal cancer following treatment for achalasia. However, the absolute risk of esophageal cancer remains relatively low, and there is no consensus regarding the need for routine surveillance in these patients.

Foreign body ingestion is common in children. When a foreign body becomes impacted in the esophagus, it may erode into the airway, aorta, mediastinum, or pleural space, causing life-threatening complications. Impaction is most likely to occur at one of the four sites of physiologic narrowing in the esophagus: the cricopharyngeus of the UES (upper esophageal sphincter, the narrowest point in the GI tract); the aortic notch; the left mainstem bronchus; and the LES.

The epidemiology of swallowed foreign bodies depends on the cultural context. In Western countries, coins account for most pediatric foreign body ingestions, while pieces of food account for 10%–20%. Infants may present with excessive drooling, refusal to eat, and unexplained coughing or gagging. Older children can have dysphagia, vomiting, chest pain, or respiratory symptoms.

The best initial diagnostic tests are anteroposterior and lateral chest radiographs. The flat surface of a coin is best seen on the anteroposterior view when it is lodged in the esophagus, whereas the lateral view will show the flat surface when it is lodged in the trachea. Radiographs will also differentiate coins from button batteries, which may have a halo sign, as well as the step-off between the positive and negative nodes of the battery ( Fig. 24.6A and B ). If no radiopaque object is seen and suspicion is high, contrast esophagram or diagnostic endoscopy may be performed.

(A–C) (A) Anteroposterior and (B) lateral radiographs demonstrate a button battery lodged at the cricopharyngeus muscle. Note that button battery will have two projections of a larger aspect (positively charged) and narrower aspect (negatively charged). (C) UGI of traumatic tracheoesophageal fistula caused by a button battery ( arrow ).

Key principles of endoscopic management of esophageal foreign bodies are to protect the airway, maintain control of the object during extraction, and avoid causing additional damage. Many centers use flexible endoscopy to remove low-risk (smooth-surfaced) esophageal foreign bodies. Coins may be grasped with endoscopic grasping forceps and extracted with the scope through the mouth or pushed gently into the stomach to be retrieved with a basket. Other approaches have been described, including bougienage, Foley balloon extraction under fluoroscopy, and brief observation trials. Around 40%–50% of pediatric esophageal food impactions are associated with eosinophilic esophagitis. Current recommendations are to biopsy the proximal and distal esophagus at the time of endoscopic removal, treat with a proton pump inhibitor, and perform repeat endoscopy in 6–12 weeks.

For other objects or complicated presentations, removal with rigid or flexible esophagoscopy under general anesthesia is acceptable. This procedure has been proven to be highly successful with low complication rates. Patients with foreign bodies causing perforation into the pleural space or other nonvascular structures should undergo endoscopic removal. For early presentations of esophageal perforation, it is recommended that removal be followed by immediate esophageal repair via thoracotomy, depending upon patient stability. Thoracic/pleural drainage and conservative management may also be successful in many cases. Delayed perforations or those involving other essential structures should be managed by positioning an endoscopic feeding tube past the injury, nil per os (NPO), possible feeding gastrostomy tube, and intravenous (IV) antibiotics. If a contrast esophagram demonstrates resolution of the leak, oral feeds can be initiated. Otherwise, delayed repair may be performed.

Button batteries warrant special attention due to their potential for severe complications, including esophageal perforation, TEF ( Fig. 24.6C ), bilateral vocal cord paralysis, and death from aorto-esophageal fistula (AEF) and uncontrollable hemorrhage. Most serious button battery injuries are caused by lithium batteries >20 mm. These batteries cause injury through several mechanisms, the most severe being caustic injury. Mucosa contacts the positive and negative terminals, completing a circuit and allowing current to flow (from the positive to negative terminal direction). This process generates hydroxide radicals, which begin to cause necrosis of tissue within 15 minutes of contact. Severe esophageal damage and perforation can occur within a few hours.

An algorithm for management of button battery ingestion has been recently published. The orientation of the slightly smaller negative pole (anode) on plain radiographs should be noted, as this is the direction of most serious injury. With esophageal impaction, a negative terminal anteriorly facing may lead to TEF whereas negative terminal posteriorly directed may produce an AEF. A helpful mnemonic for remembering which side of the battery will lead to injury is N–N–N (negative-narrow-necrosis). Esophageal impaction at the level of the aortic arch, age <5 years, battery size of ≥20 mm, and prolonged time of impaction are risk factors for more severe complications. The battery should be removed emergently using rigid or flexible esophagoscopy and the degree of esophageal injury noted. Any evidence of injury should prompt admission for observation, withholding of oral intake, and intravenous antibiotics. A contrast esophagram should be performed prior to starting oral feeding. Consideration of a feeding gastrostomy (laparoscopic) may be appropriate if a large injury or tracheoesophageal fistula is identified.

AEF is the deadliest complication of impacted esophageal foreign bodies, with fatality rates of 40%–70%. ^, Stable patients presenting with an impacted foreign body and hematemesis should undergo emergent CT angiography of the chest to evaluate the relationship of the foreign body to the aorta. AEF is strongly suspected when injury is radiographically evident within 2–3 mm of the aorta. Emergent endoscopic removal should be performed with cardiothoracic surgeons immediately available for aortic control and repair. Some authors have observed improved outcomes using endovascular stents for aortic control. In the case of button batteries, patients with any degree of esophageal injury noted at the time of endoscopic removal should also undergo CT angiography of the chest, even if asymptomatic. Evidence of injury extension within 3 mm of the aorta mandates prolonged NPO, IV antibiotics, and serial chest MRI every 5–7 days until the injury is observed to recede away from the aorta. AEF can also present weeks after seemingly uncomplicated removal of an esophageal button battery. Further information about esophageal foreign bodies can be found in Chapter 10 .

Esophageal perforation is a rare but life-threatening event in children. Perforations are most commonly iatrogenic, resulting from stricture dilation, endoscopy, or nasogastric or endotracheal tube placement. Other causes include foreign body ingestion, caustic ingestion, infection, or trauma. The most common locations of perforation are the pharyngoesophageal junction (the narrowest point of the esophagus) in neonates and the thoracic esophagus in older children.

Depending on their location, full-thickness perforations allow leakage of bacteria and digestive enzymes into the cervical soft tissues, mediastinum, pleural space, or peritoneal cavity, generally causing rapid development of severe inflammation and sepsis. Patients with thoracic esophageal perforations may present with chest pain, respiratory distress, dysphagia, fever, or subcutaneous emphysema. Any child who is symptomatic following an endoscopic or esophageal dilation procedure should be evaluated for esophageal perforation. The initial study is a chest radiograph in the anteroposterior and lateral views. Findings suggestive of esophageal perforation include pneumothorax, pleural effusion, subcutaneous emphysema, pneumopericardium, or pneumomediastinum. A contrast esophagram is the diagnostic study of choice to determine the presence of an esophageal perforation ( Fig. 24.7 ). Frequently, water-soluble contrast can be used initially, followed by barium if a leak is not seen. A 10% false-negative rate has been reported with esophagography alone. CT of the chest with intravenous contrast may increase the sensitivity. Aside from obvious extravasation of oral contrast, CT findings suggestive of esophageal perforation include pneumomediastinum, pleural effusion, and esophageal thickening. ^,

(A, B) (A) This infant developed a stricture at the anastomosis after esophageal atresia repair. Multiple balloon dilations were required. (B) Unexpectedly, at the time of one of these dilations, an esophageal perforation developed. Note the contrast leak through the esophageal perforation. This child was managed nonoperatively, and the perforation eventually sealed without the need for operative intervention.

Treatment is guided by the location, timing, and extent of injury as well as the clinical status of the child. Key treatment goals are control of infection and provision of adequate nutrition. Most patients with thoracic and cervical esophageal perforation can be successfully managed with an aggressive nonoperative approach. All patients are made NPO and started on broad-spectrum antibiotics. If feasible, a soft nasoenteric tube may be placed beyond the perforation into the stomach or small bowel under radiographic guidance. This provides access to administer enteral nutrition during the healing process. Otherwise, parenteral nutrition is started. Contained perforations, in which contrast is seen to extravasate but immediately drain back into the esophagus, often heal with supportive care only. ^, The finding of a fluid collection or free extravasation of the contrast into the pleural space mandates tube drainage to create a controlled fistula. Recently, the use of endoscopic vacuum-assisted closure (EVAC) for esophageal perforation or leaks following esophageal repair has been described. This technique involves the endoscopic placement of a wound vacuum sponge into an esophageal perforation, followed by gradual removal over several days and replacement with conventional Replogle tube suctioning of the esophagus until the perforation heals.

Operative management is indicated in patients with an abdominal esophageal perforation that communicates with the peritoneal cavity, extensive contamination or tissue necrosis, or clinical deterioration despite treatment. Primary repair of the perforation may be possible if surrounding inflammation is not too severe, which typically corresponds to the first 24 hours after the perforation occurs. The perforation is closed with absorbable suture in a single layer and reinforced with pericardial fat, pleura, stomach, diaphragm, or omentum. As the mucosa is the strongest layer of the esophagus, it is essential to repair the full extent of the mucosal injury. The defect in the muscular layer may need to be extended proximally and distally to fully evaluate the underlying mucosal defect. Surrounding tissues should always be widely drained and consideration given to distal feeding access with a gastrostomy and/or jejunostomy. If the tissues are too inflamed to hold sutures, wide drainage and distal feeding access alone should be performed. Cervical esophagostomy is an option primarily of historical interest. Emerging treatments for perforations in the adult population include covered stents and endoluminal vacuum therapy, but little data are available in children. ^,

The morbidity and mortality of esophageal perforation is directly related to delay in the diagnosis and treatment. There is a five-fold increase in complications following a delay in diagnosis of greater than 24 hours. The mortality of esophageal perforation in the pediatric population is 4%, which is lower than reported for adults.