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Congenital Esophageal Diseases Burns Amp Strictures

Congenital Esophageal Disease

1. Congenital esophageal stenosis

A. Esophageal web: middle 1/3, complete or incomplete, dilatation
B. Intraluminal cartilaginous rest: tracheobronchial origin, local resection with primary repair
C. Segmental hypertrophy: mucosa and submucosa, residual web
D. Laryngotracheoesophageal cleft
1) Varying degrees of communication between larynx-trachea and esophagus
2) High incidence of GER
3) 10 - 40% mortality

2. Esophageal atresia +/- TEF

A. Most common congenital esophageal anomaly
B. Salivary regurgitation, abdominal bloating, pneumonia
C. Dx: cannot pass NG tube, radiologic confirmation
D. Mortality related mostly to other congenital lesions

3. Esophageal atresia +/- TEF: types

A. Atresia with distal TEF: 87%
B. Atresia without TEF: 8%
C. TEF without atresia (H type): 4%
D. Atresia with proximal TEF: 0.8%
E. Atresia with prox/distal TEF: 0.7%

4. Waterston Classification (pneumonia increases one level)

Congenital Esophageal Atresia

5. Esophagus - Caustic burns

A. Acid or alkaline injury
B. Accidental - children < 5 yrs, household cleaners
C. Intentional - adults with psych disorders, suicide attempts
D. Severity related to strength / duration of exposure (prox < distal)
F. Laryngeal edema - respiratory distress

6. Phases

A. Inflammation, edema, necrosis - initial few days
B. Ulceration, sloughing, granulation - 3 - 4 weeks
C. Cicatrization, scar formation - weeks - months
D. Long-term
1) Recurrent strictures
2) Respond to dilatation
E. Pre-cancerous: may develop up to 40 yrs later
3) Increased cancer risk 1000 fold (debated)

7. Treatment

A. Appropriate dilution, no emesis
B. Fluids, antibiotics, (steroids controversial)
C. Fiberoptic laryngoscopy / intubation
D. Esophagoscopy - 48 hours, stop at first evidence of burn
E. Barium swallow 3 - 4 weeks, intermittently thereafter
F. Later dilatation for strictures
G. Surgery usually not necessary early
H. Sepsis / full-thickness necrosis: cervical esophagostomy, gastrostomy with isolation, later reconstruction, (resection controversial)

8. Esophagus - Strictures

A. Causes
1) Malignancy
2) Peptic stricture - at GE junction
3) Mid-esophageal stricture - usually related to Barrett's esophagus
4) Caustic stricture
5) Schiatzki's ring - submucosal fibrous band at squamocolumnar junction
6) Post-operative/anastomotic
7) Congenital
8) Motility disorders, vascular rings

9. Evaluation and Therapy

A. If cause not obviously apparent, malignancy must be ruled out
B. Barium swallow
C. Esophagoscopy, brushings, biopsy
D. If peptic: anti-reflex therapy
E. If benign: dilatation - up to 50 Fr
F. If mucosa / bx normal: external compression, motility disorder, submucosal lesions

10. Bougienage

A. Usually successful for benign strictures
B. Safest with rigid esophagoscopy, general anesthesia (rarely practiced)
C. May worsen gastroesophageal reflux
D. Retrograde via G-tube (children)
E. Other methods: fluoroscopic bougienage, pneumatic dilatation, stents
F. For tight stricture, wire-guided Savory dilators

11. Surgical Considerations

A. Tight strictures, severe scarring, repeated dilatations increase risk of perforation
B. Consider surgery, particularly in young patient
C. Anti-reflux procedure (peptic stricture)
D. High stricture - intra-op dilatation + anti-reflux procedure
E. Consider motility disorder, pre-op motility studies

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

A. For extensive scarring, multiple or long strictures, Barrett's with dysplasia
B. Consider esophageal replacement
C. Gastric pull-up (intrathoracic anastomosis assoc. with recurrent reflux)
D. Colon interposition
E. Jejunal interposition

EXTENDED OUTLINE Congenital Esophageal Abnormalities

1. 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

2. Esophageal Atresia With Tracheoesophageal Fistula

1) Signs and Symptoms
a) related to esophageal obstruction and respiratory complications resulting from reflux
b) infants have copious salivary secretions and tend to choke during feeds
c) gastroesophageal reflux causes aspiration pneumonia and respiratory distress
d) air is eventually forced through the TEF and creates abdominal distension

3. 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

4. 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

5. 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

6. 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
a) may be associated with laryngospasm and apnea spells
b) 10-50% patients require Nissen fundiplication

7. Results

A. risk classification by Waterston
1) group A- birth wt > 2.5 kg in good medical condition
2) group B- birth wt 1.8-2. Kg with mild pneumonia or an associated congenital anomaly
3) group C- birth wt < 1.8 kg or higher with severe pneumonia or life threatening anomaly
B. in author’s experience with 100 patients there was no mortality with group A or B patients and a 15% early mortality in group C patients
1) overall early mortality was 4.6%
2) late mortality was 10% over 10 year period
Isolated Esophageal Atresia

8. Signs and Symptoms

A. similar presentation with those with a tracheoesophageal fistula
B. most weigh less than 2.5 kg
C. almost all have associated maternal polyhydramnios
D. scaphoid, gasless abdomen

9. Surgical Treatment

A. lower esophageal segment is usually short
B. primary repair is not feasible
C. gastrostomy should be performed
D. daily lengthening of the upper esophageal pouch is completed with an orogastric tube
E. after 6 weeks to 3 months repair is attempted
F. it is usually necessary to perform myotomy to gain length

10. Esophageal Replacement

A. if esophageal replacement is necessary, then a gastrostomy and cervical esophagostomy should be performed
B. these patients are then treated until the age of 1 year or more with G-tube feeds
C. techniques:
1) jejunal interposition is no longer used
2) colonic inerposition with right or left colon
a) substernal
b) intrathoracic
(1) 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

11. Results

A. 30 patients operated on without mortality
B. 12 patients received esophageal lengthening, myotomy and delayed primary anastomosis with stricture formation in 10 patients (9/10 dilated successfully) H-Type Fistula

12. Signs and Symptoms

A. have symptoms of aspiration
B. may present later in life up to 1-2 years of age
C. have difficulty with thin feeds

13. Diagnosis

A. some advocate use of contrast studies to demonstrate fistula
B. author utilizes endoscopy to make diagnosis
C. bronchoscopy may also demonstrate fistula
D. most fistulae are located low in the neck and rarely in the thorax

14. Treatment/Results

A. performed electively
B. preliminary gastrostomy not required
C. fistula can usually be approached via a neck incision unless it is thoracic
D. preoperative placement of a fogarty catheter may facilitate identification
E. esophagus is dissected from the posterior trachea and the fistula closed with two layered closure and paraspinous muscle interposition
F. recurrent laryngeal nerve may occur
G. no mortality in 24 patients, one leak

15. Esophageal Stenosis

A. usually acquired but can be congenital
B. stenosis presents as a web or diaphragm amenable to dilation
C. if associated with cartilaginous tracheobronchial remnants, resection is required
D. may have associated anomalies such as esophageal atresia or anorectal malformations
E. most webs occur in the upper and middle esophagus; those associated with esophageal atresia tend to be located in the lower esophagus

16. Esophageal Duplications

A. generally lie in the posterior mediastinum
B. usually asymptomatic, found on CXR
C. approximately 30% of mediastinal masses in children are of foregut origin
D. usually do not communicate with the lumen of the esophagus

17. Diagnosis

A. barium esophagram may reveal filling defect
B. CT scan is procedure of choice
C. USG of abdomen to rule out associated abdominal duplications

18. Treatment/Results

A. preferred approach is complete resection
B. may be necessary to divide diaphragm or make separate abdominal incision to resect associated abdominal duplication cyst
C. no long-term sequlae encountered, no deaths or serious complications

19. Esophageal Diverticula

A. extremely rare
B. have complete muscular wall (unlike Zenker’s)
C. risk of aspiration
D. resection is treatment of choice

20. Gastroesophageal Reflux

A. in patients with intact esophagus and no other esophageal anomaly, the most likely cause of GER is failure of maturation of the LES
B. the LES is usually competent by 6-7 weeks of life
C. most common presenting symptoms are frequent regurgitation, upper GI tract bleeding, apneic spells, and chronic intermittent pneumonia
D. if symptoms persist for > 6 months then surgical intervention is recommended
E. diagnosis can be made by 24 hour pH monitoring, upper GI swallow and endoscopy
F. most patients respond to medical therapy- H2 blockade, bethanechol, reglan
G. Nissen fundiplication is procedure of choice

21. Achalasia

A. uncommon in childhood
B. diagnosed with barium swallow and esophageal manometry
C. trail of pneumatic dilatation is recommended first then surgical therapy if recurrence occurs rapidly (90% success rate is author’s experience)
D. cricopharyngeal achalasia may also occur in children which requires myotomy via cervical approach