CTSN - Congenital Esophageal Diseases

Congenital Esophageal Disease

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1. Congenital esophageal stenosis
Esophageal web: middle 1/3, complete or incomplete, dilatation
Intraluminal cartilaginous rest: tracheobronchial origin, local resection with primary repair
Segmental hypertrophy: mucosa and submucosa, residual web

Laryngotracheoesophageal cleft

Varying degrees of communication between larynx-trachea and esophagus

High incidence of GER

10 - 40% mortality

2. Esophageal atresia +/- TEF

Most common congenital esophageal anomaly

Salivary regurgitation, abdominal bloating, pneumonia

Dx: cannot pass NG tube, radiologic confirmation

Mortality related mostly to other congenital lesions

Esophageal Anomalities
3. Esophageal atresia +/- TEF: types

Atresia with distal TEF: 87%

Atresia without TEF: 8%

TEF without atresia (H type): 4%

Atresia with proximal TEF: 0.8%

Atresia with prox/distal TEF: 0.7%

4. Waterston Classification (pneumonia increases one level)

Congenital Esophageal Atresia [table] 5. ESOPHAGUS - CAUSTIC BURNS

Acid or alkaline injury

Accidental - children < 5 yrs, household cleaners

Intentional - adults with psych disorders, suicide attempts

Severity related to strength / duration of exposure (prox < distal)

Laryngeal edema - respiratory distress

6. Phases

Inflammation, edema, necrosis - initial few days

Ulceration, sloughing, granulation - 3 - 4 weeks

Cicatrization, scar formation - weeks - months

Long-term

Recurrent strictures

Respond to dilatation

Pre-cancerous: may develop up to 40 yrs later

Increased cancer risk 1000 fold (debated)

7. Treatment

Appropriate dilution, no emesis

Fluids, antibiotics, (steroids controversial)

Fiberoptic laryngoscopy / intubation

Esophagoscopy - 48 hours, stop at first evidence of burn

Barium swallow 3 - 4 weeks, intermittently thereafter

Later dilatation for strictures

Surgery usually not necessary early

Sepsis / full-thickness necrosis: cervical esophagostomy, gastrostomy with isolation, later reconstruction, (resection controversial)

8. ESOPHAGUS - STRICTURES

Causes

Malignancy

Peptic stricture - at GE junction

Mid-esophageal stricture - usually related to Barrett's esophagus

Caustic stricture

Schiatzki's ring - submucosal fibrous band at squamocolumnar junction

Post-operative/anastomotic

Congenital

Motility disorders, vascular rings

9. Evaluation and Therapy

If cause not obviously apparent, malignancy must be ruled out

Barium swallow

Esophagoscopy, brushings, biopsy

If peptic: anti-reflex therapy

If benign: dilatation - up to 50 Fr

If mucosa / bx normal: external compression, motility disorder, submucosal lesions

10. Bougienage

Usually successful for benign strictures

Safest with rigid esophagoscopy, general anesthesia (rarely practiced)

May worsen gastroesophageal reflux

Retrograde via G-tube (children)

Other methods: fluoroscopic bougienage, pneumatic dilatation, stents

For tight stricture, wire-guided Savory dilators

11. Surgical Considerations

Tight strictures, severe scarring, repeated dilatations increase risk of perforation

Consider surgery, particularly in young patient

Anti-reflux procedure (peptic stricture)

High stricture - intra-op dilatation + anti-reflux procedure

Consider motility disorder, pre-op motility studies

12. Esophageal lengthening procedure (Collis-Nissen) for shortened, scarred esophagus

For extensive scarring, multiple or long strictures, Barrett's with dysplasia

Consider esophageal replacement

Gastric pull-up (intrathoracic anastomosis assoc. with recurrent reflux)

Colon interposition

Jejunal interposition

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EXTENDED OUTLINE Congenital Esophageal Abnormalities Esophageal Atresia

1. embryology

a. esophagus first appears at four weeks gestation

b. esophageal atresia results from incomplete separation of the laryngotracheal groove between the third and sixth weeks of gestation

2. common forms of esophageal atresia

3. esophageal atresia with fistula occurs in ~1/8000 births

4. incidence of associated anomalies is about 50%

c. cardiac defects are important to identify preoperatively

d. VATER association is common- three or more of the following: vertebral defects, anal atresia, tracheoesophageal atresia and radial and renal dysplasia

e. CHARGE association: choanal atresia, heart anomalies, esophageal atresia and GI tract anomalies

Esophageal Atresia With Tracheoesophageal Fistula

Signs and Symptoms

1. related to esophageal obstruction and respiratory complications resulting from reflux

2. infants have copious salivary secretions and tend to choke during feeds

3. gastroesophageal reflux causes aspiration pneumonia and respiratory distress

4. air is eventually forced through the TEF and creates abdominal distension
Diagnosis

1. some are diagnosed on prenatal ultrasound

2. most are diagnosed after birth

3. if suspected then a rigid nasogastric tube should be placed and a CXR should be obtained which will demonstrate blind esophageal pouch

4. can also use water soluble contrast to study pouch to delineate the anatomy

5. if a right aortic arch is suspected, then echocardiography should be performed

Treatment- Preoperative Management

1. place baby in the semisitting position

2. place sump tube for control of saliva or use frequent suctioning

3. resuscitate in the ICU, correct electrolyte and acid base status

4. antibiotics are given

5. mechanical ventilation if required

a. in patients with increased pulmonary resistance, high frequency ventilation with low mean airway pressure may be more effective than conventional ventilation

b. lower mean airway pressures should help control abdominal distension

Surgical Treatment

1. surgical approach and timing of surgery depend upon the size of the infant and condition

2. primary repair is preferable for almost all infants unless weight is less than 1500 g or they have associated severe respiratory distress syndrome for which delayed primary or staged repair should be completed 1-8 weeks after birth

3. some infants may be delayed secondary to critical congenital cardiac disease

4. most patients do not require gastrostomy before thoracotomy unless a staged procedure is planned

5. in patients that require high ventilatory airway pressures, a gastrostomy may become a source of an air leak exacerbating respiratory status

6. if the infant has an associated doudenal atresia or imperforate anus, these defects are corrected first then the esophageal fistula (author had no deaths in 24 patients with this sequence)

7. approach:

a. right thoracotomy- standard approach

b. left thoracotomy- when there is a right aortic arch

c. can be extrapleural or transpleural

d. division of azygous vein and identification of vagus nerve will facilitate locating fistula

e. fistula is closed utilizing running suture

f. esophagus is reapproximated with either a single or double layer anastomosis

g. the esophagus can be elongated with circular or longitudinal myotomies, but a staged procedure may be required if enough length cannot be attained

Complcations

1. most common complications are pneumonia and atelectasis

2. anastomotic leak

3. anastomotic strictures

a. occur most commonly after leak has occurred

b. most can be dilated

4. gastroesophageal reflux

d. may be associated with laryngospasm and apnea spells

e. 10-50% patients require Nissen fundiplication

Results

1. risk classification by Waterston

group A- birth wt > 2.5 kg in good medical condition

group B- birth wt 1.8-2. Kg with mild pneumonia or an associated congenital anomaly

group C- birth wt < 1.8 kg or higher with severe pneumonia or life threatening anomaly

2. in authors experience with 100 patients there was no mortality with group A or B patients and a 15% early mortality in group C patients

a. overall early mortality was 4.6%

b. late mortality was 10% over 10 year period

Isolated Esophageal Atresia
Signs and Symptoms

1. similar presentation with those with a tracheoesophageal fistula

2. most weigh less than 2.5 kg

3. almost all have associated maternal polyhydramnios

4. scaphoid, gasless abdomen

Surgical Treatment

1. lower esophageal segment is usually short

2. primary repair is not feasible

3. gastrostomy should be performed

4. daily lengthening of the upper esophageal pouch is completed with an orogastric tube

5. after 6 weeks to 3 months repair is attempted

6. it is usually necessary to perform myotomy to gain length

Esophageal Replacement

1. if esophageal replacement is necessary, then a gastrostomy and cervical esophagostomy should be performed

2. these patients are then treated until the age of 1 year or more with G-tube feeds

3. techniques:

a. jejunal interposition is no longer used

b. colonic inerposition with right or left colon

i. substernal

ii. intrathoracic

a. gastric transposition

3. Complications

a. most common complication of colonic replacement is leak

b. most serious complication is gangrene due to congestion

c. peptic ulceration of the cologastrostomy can occur

Results

1. 30 patients operated on without mortality

2. 12 patients received esophageal lengthening, myotomy and delayed primary anastomosis with stricture formation in 10 patients (9/10 dilated successfully) H-Type Fistula

Signs and Symptoms

1. have symptoms of aspiration

2. may present later in life up to 1-2 years of age

3. have difficulty with thin feeds

Diagnosis

1. some advocate use of contrast studies to demonstrate fistula

2. author utilizes endoscopy to make diagnosis

3. bronchoscopy may also demonstrate fistula

4. most fistulae are located low in the neck and rarely in the thorax

Treatment/Results

1. performed electively

2. preliminary gastrostomy not required

3. fistula can usually be approached via a neck incision unless it is thoracic

4. preoperative placement of a fogarty catheter may facilitate identification

5. esophagus is dissected from the posterior trachea and the fistula closed with two layered closure and paraspinous muscle interposition

6. recurrent laryngeal nerve may occur

7. no mortality in 24 patients, one leak

Esophageal Stenosis

1. usually acquired but can be congenital

2. stenosis presents as a web or diaphragm amenable to dilation

3. if associated with cartilaginous tracheobronchial remnants, resection is required

4. may have associated anomalies such as esophageal atresia or anorectal malformations

5. most webs occur in the upper and middle esophagus; those associated with esophageal atresia tend to be located in the lower esophagus

Esophageal Duplications

1. generally lie in the posterior mediastinum

2. usually asymptomatic, found on CXR

3. approximately 30% of mediastinal masses in children are of foregut origin

4. usually do not communicate with the lumen of the esophagus

Diagnosis

1. barium esophagram may reveal filling defect

2. CT scan is procedure of choice

3. USG of abdomen to rule out associated abdominal duplications

Treatment/Results

1. preferred approach is complete resection

2. may be necessary to divide diaphragm or make separate abdominal incision to resect associated abdominal duplication cyst

3. no long-term sequlae encountered, no deaths or serious complications

Esophageal Diverticula

1. extremely rare

2. have complete muscular wall (unlike Zenkers)

3. risk of aspiration

4. resection is treatment of choice

Gastroesophageal Reflux

1. in patients with intact esophagus and no other esophageal anomaly, the most likely cause of GER is failure of maturation of the LES

2. the LES is usually competent by 6-7 weeks of life

3. most common presenting symptoms are frequent regurgitation, upper GI tract bleeding, apneic spells, and chronic intermittent pneumonia

4. if symptoms persist for > 6 months then surgical intervention is recommended

5. diagnosis can be made by 24 hour pH monitoring, upper GI swallow and endoscopy

6. most patients respond to medical therapy- H2 blockade, bethanechol, reglan

7. Nissen fundiplication is procedure of choice

Achalasia

1. uncommon in childhood

2. diagnosed with barium swallow and esophageal manometry

3. trail of pneumatic dilatation is recommended first then surgical therapy if recurrence occurs rapidly (90% success rate is authors experience)

4. cricopharyngeal achalasia may also occur in children which requires myotomy via cervical approach