Congenital Disorders
28.2.1 Tracheoesophageal Fistula
Anatomy, Prevalence, and Associations
Tracheoesophageal fistula (TEF) describes an abnormal communication between the trachea and the esophagus. The prevalence is approximately 1 in 1,400 live births. In approximately 85% of cases, the esophageal lumen ends in a blind pit, esophageal atresia, and the fistula connects this lumen with the anterior tracheal wall ( ▶ Fig. 28.1 a).
If a TEF occurs without esophageal atresia, the fistula connects obliquely from the trachea in a caudal direction toward the esophagus. The fistula enables air to pass from the trachea to the esophagus, and esophageal contents (saliva, milk, and gastric juices) into the trachea, as in the far less common H-type fistula ( ▶ Fig. 28.1 b).
The baby with an H-type fistula can have a normal or near-normal swallow but will aspirate some of the contents of the esophagus into the airway. Diagnosis can be delayed as the baby may continue to feed. 1
Additional major congenital anomalies are present in approximately 50% of babies with a TEF. These include the VATER or VACTERL (vertebral defects, anorectal anomalies, cardiac anomalies, tracheoesophageal disorders, cardiac defects, renal defects, and limb disorders), and the CHARGE (coloboma, congenital heart disease, choanal atresia, renal and genital anomalies, and ear abnormality) complexes of disorders.
Fig. 28.1 Illustration of tracheoesophageal fistula (TEF). (a) Esophageal atresia with distal TEF (common type). (b) H-type fistula (rare). (Reproduced from Tytgat GNJ, Tytgat SHAJ. Grading and Staging in Gastroenterology. Stuttgart/New York: Thieme; 2009, with permission.)
Presentation and Diagnosis
Maternal polyhydramnios can be one sign of esophageal atresia, particularly if there is a TEF with esophageal atresia; there may be suspicion based on prenatal ultrasound scanning.
After birth, the child is reported to be mucus-ridden, with saliva drooling from the mouth. Feeding results in choking, respiratory distress, cyanotic attacks, aspiration into the lungs and pneumonia, or unexplained abdominal distensions. Symptoms are relieved by nasogastric tube feeding unless the baby has esophageal atresia.
Suspect esophageal atresia if a 10-gauge catheter (preferably relatively stiff) passed through the mouth becomes arrested approximately 9 to 13 cm from the gums. To determine if the secretion aspirated from the catheter is neutral or near-neutral (usually pH 4 and higher), pH paper should be used. Normal stomach pH is 3.5 and lower.
Investigations
A plain X-ray of the abdomen and thorax helps differentiate the different forms of TEF: if it shows a gasless abdomen, the child probably has no distal TEF, but may have a proximal TEF or esophageal atresia alone. In a distal TEF, there is air in the abdomen below the diaphragm.
Tracheobronchoscopy can demonstrate an upper pouch fistula or identify the point at which the distal fistula enters the airways ( ▶ Fig. 28.2). Moderate-to-severe tracheomalacia is a common accompaniment to a TEF and will need the attention of a pediatric otolaryngologist. Upper esophagoscopy (rigid or flexible) will not always detect an H-type TEF, but if performed, it will demonstrate the blind-ending esophageal pouch in esophageal atresia.
An upper pouch fistula or the subtle signs of an H-type TEF can be demonstrated by contrast introduced by a catheter placed in the midesophagus under continuous video fluoroscopy (tube esophagoscopy).
Fig. 28.2 Tracheoesophageal fistula evident at bronchoscopy. An H-type fistula can be especially difficult to detect as the baby often feeds normally.
Management
The infant should be stabilized and kept warm with minimal handling and adequate oxygenation. Place the infant in the right lateral position, allowing draining of saliva and preventing gastric contents from coming up. The child will need frequent gentle suctioning and should be transferred as soon as is practicable to a specialist pediatric center for surgery under the care of a suitably trained pediatric surgical team.
28.2.2 Esophageal Strictures, Web, and Rings
Etiology and Presentation
In contrast to acquired strictures (see Chapter ▶ 28.3.1), congenital esophageal strictures are very rare. Esophageal rings are thin, fragile structures that partially or completely obstruct the esophageal lumen. An esophageal web is a thin mucosal fold protruding into the lumen, most commonly located anteriorly in the cervical esophagus, causing focal narrowing in the postcricoid area.
Schatzki “B” rings are smooth, thin mucosal structures at the gastroesophageal junction. They may cause dysphagia. Muscular rings are very rare, typically located within 2 cm of the squamocolumnar junction. 1
Symptoms and Treatment
Most esophageal rings are asymptomatic, are found incidentally, and do not require treatment. If symptomatic, esophageal dilatation is the best treatment. Esophageal strictures, webs, and rings have not been associated with globus pharyngeus. 2
28.3 Acquired Esophageal Disorders
28.3.1 Esophageal Strictures
Etiology
A stricture is a fixed narrowing within the lumen. It may be caused by inflammation, fibrosis, or intraluminal neoplasia. Extraluminal causes include direct invasion by a neoplasm or mediastinal lymph node enlargement. More often a stricture is benign, secondary to the following:
Severe GERD;
Caustic ingestion;
Previous surgery; or
Infection (candida, herpes virus, meningococcus).
Other causes include inflammation (epidermolysis bullosa, chronic granulomatous disease), chemotherapy, or radiotherapy. Some children develop iatrogenic esophageal stricture secondary to sclerotherapy of varices.
Presentation
Infants and children may present with recurrent vomiting, coughing, choking, cyanosis with feeds, and irritability during or after feeds. Older children may present with dysphagia, odynophagia, frequent regurgitation of swallowed food bolus, vomiting, and weight loss.
Investigation and Diagnosis
Diagnosis is by endoscopy (esophagogastroduodenoscopy [EGD]), possibly combined with barium swallow/meal. Computed tomography may be helpful in selected cases ( ▶ Fig. 28.3, ▶ Fig. 28.4).
Fig. 28.3 Filiforme fibrotic stricture secondary to chronic gastroesophageal reflux.
Fig. 28.4 Irregular extensive stenosis in severe gastroesophageal reflux disease.
Management
Children with minimal symptoms may require no treatment. It may be helpful to counsel parents regarding smaller meals and antireflux medications as needed. Balloon dilatation under fluoroscopic guidance may be helpful if symptoms are more severe, and in extreme cases, surgery (stricturoplasty) can be considered. There is a small risk of perforation and recurrence with these techniques. 2
28.3.2 Caustic Esophageal Damage
This is caused by mucosal and mural ulceration brought about by ingestion of chemicals. It has become relatively rare in western communities due to improved safety, awareness, and labeling of household cleaner containers, for example, caustic soda, and increased parental awareness of the dangers of chemical ingestion by children. Caustic strictures are still a major health problem in the developing world (see Chapters ▶ 4 and ▶ 22).
Very significant damage can be caused to the oropharynx, the esophagus, stomach, and the larynx by alkaline liquid agents (e.g., dishwasher fluid) or crystalline drain cleaners, which adhere to the oropharynx, become lodged in the upper esophagus, and cause tissue injury by liquefactive necrosis. Household bleach ( ▶ Fig. 28.5, ▶ Fig. 28.6) may induce severe mucosal burns and edema. 3 “Liquitabs” containing dishwasher fluid or detergent are a particular modern hazard (see ▶ Fig. 20.5).
Fig. 28.5 Bleach crystal pack.
Fig. 28.6 Soluble bleach crystals in esophagus and stomach (depicted here) following accidental ingestion. Note the large amounts that in this case required surgical retrieval through laparotomy, following joint assessment from ear, nose, and throat, gastroenterology, anesthetics, general surgery, and respiratory medicine.
Symptoms
In the acute situation, there may be burns of the hands, face, and oral cavity. The child may present with vomiting, dysphagia, drooling, epigastric and abdominal pain, and refusal to drink. Shock and severe respiratory distress may occur if there is mediastinal penetration.
In long-standing cases, there may be mucosal erythema and edema. Noncircumferential superficial mucosal ulcerations do not always cause long-term complications. Sequelae of esophageal caustic injuries are as follows:
Stricture formation.
Development of achalasia.
“Brachyesophagus.”
Gastroesophageal reflux (GER).
The development of malignancy (a late complication).
Diagnosis
A chest radiograph can detect air in the mediastinum if there is perforation and mediastinitis.
A joint approach by ENT, gastroenterology, surgery, anesthetics, and respiratory physicians should be planned and coordinated to determine the type of injury and to remove toxic material adhering to the mucosa before it causes further tissue damage.
For ingested liquids, flexible endoscopy may be performed with great care within 12 to 36 hours after ingestion to assess the extent of damage. 3 A contrast swallow can be performed 2 to 3 weeks after ingestion.
Treatment
In the emergency situation, make no attempt to induce vomiting or to neutralize the caustic agent due to the risk of exothermic reaction and additional injury. 3
Admit the child to hospital even for an apparently minor injury and commence resuscitation. Early treatment with H2 blocker or proton pump inhibitors (PPIs) is indicated. Nasogastric tubes should not be placed blindly. Usually, parenteral fluids are required until after endoscopic assessment when, depending on the findings, patients may be considered safe to swallow liquids. Children are at ongoing risk of additional complications and need to be monitored for respiratory symptoms on a ward with surveillance facilities (e.g., a high dependency unit).
If “liquitabs” or granules are swallowed, they should be removed either by safe endoscopy (e.g., wrapped in a net and ideally sealed, if the equipment is available), or following diagnostic endoscopy, a laparoscopic surgical removal may be needed to retrieve larger amounts of the substance.
If nasogastric tube placement is considered (e.g., difficult venous access), the placement requires endoscopic guidance (laryngoscopy and flexible esophagoscopy) to avoid a perforation. Depending on the type or amount of ingested chemical, the extent of epidermal or mucosal damage, the presence of respiratory symptoms and signs of systemic inflammation or infection, and the perceived risks of aspiration, admission and treatment on a pediatric intensive care unit may be indicated. This is to facilitate airway protection by intubation and ventilation.
In severe cases and if there is evidence of incipient shock, an intravenous fluid bolus, continued intravenous fluid therapy, or even total parenteral nutrition may be needed.
There is some evidence that early high-dose steroids (1-g methylprednisolone/1.73 m2/day for 3 days) reduces secondary stricture formation. 4 Antibiotics may be used if there is any suspicion of perforation.
In the long term, repeated esophageal dilatation or reconstructive surgery may be needed.
28.3.3 Gastroesophageal Reflux
Introduction
Some reflux of gastric contents into the esophagus above the gastroesophageal junction is physiological, especially in infancy. Reflux as a developmental variation of gastrointestinal (GI) motility resolves as the infant matures. The distinction between this “physiological” and “pathological” GER in infancy and childhood is determined not merely by the number and severity of reflux episodes but also, and most importantly, by the presence of reflux-related complications. These can include failure to thrive, erosive esophagitis, esophageal stricture formation, and chronic respiratory disease ( ▶ Fig. 28.7). 5
Fig. 28.7 Grade III gastroesophageal reflux disease with fresh bleeding and fibrinous ulcerations.
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
