This document discusses congenital anomalies of the esophagus. It begins by classifying anomalies and discussing their embryology. The most common anomaly is esophageal atresia with distal tracheoesophageal fistula, occurring in 84% of cases. Prenatal diagnosis and clinical findings are outlined. Treatment involves repairing the fistula and reconnecting the esophagus. Complications can include anastomotic leaks, strictures, and gastroesophageal reflux disease. The document provides details on surgical techniques, pre- and post-operative management of various anomaly subtypes.

1. Congenital anomalies of esophagus
Dr. Pranaya Panigrahi
BHU, VARANASI

3. CLASSIFICATION
• GROSS CLASSIFICATION
• WATERSTON RISK GROUP
• OKAMOTO MODIFICATION OF SPITZ
CLASSIFICATION

4. ANATOMICAL CLASSIFICATION
1.Esophgeal Atresia with a distal tracheoesophageal
fistula - 84% GROSS TYPE C
2. EA without TEF- 6% GROSS TYPE A
3. TEF without EA- 4% GROSS TYPE E
4. EA with a fistula to both pouches- 1% - GROSS
TYPE D
5. EA with a proximal TEF - 5%- GROSS TYPE B

5. GROSS CLASSIFICATION
A, EA without a TEF
B, Atresia with a proximal
TEF
C, EA with a distal TEF
(the most frequently
encountered form of
esophageal anomaly.)

6. D, Atresia with a double (proximal and distal) fistula.
E, TEF without atresia (H-type fistula).
F, Esophageal stenosis

7. Waterston Risk Groups

8. OKAMOTO MODIFICTN OF SPITZ
CLASSIFCTN

10. EMBRYOLOGY
• No single unifying theory has been proposed that
can explain.
• Half of the cases of EA are isolated and the other
half are associated with other malformations.
• Fusion of invaginating lateral longitudinal ridges
that created a septum dividing the foregut into a
dorsal digestive tract and a ventral respiratory
system.

12. • Formation of the so-called tracheoesophageal
septum is believed to begin caudally and end
cranially.
• Cozzi reported- EA is a component of cephalic
neurocristopathy DUE TO clear association of
neural crest-implicated cardiovascular anomalies
(aortic arch, conotruncal and membranous
ventricular septal defects)
• 50% of cases of EA-TEF are associated with other
anomalies.
• 10% of cases are found in specific chromosomal or
single-gene disorders.

13. • The anomalies are most common in cases of EA
without TEF and are least common in cases of H-
type TEF.
• ASSOCIATED ANOMALIES INCIDENCE
• Cardiovascular 24%
• Genitourinary 21%
• Gastrointestinal 21%
• Musculoskeletal 14%
• Central nervous system 7%
• VACTERL association 20%
• Overall incidence 50%-70%

14. • CARDIAC ANOMALY WITH INCIDENCE:-
VSD > ASD > PDA > TOF
• GI ANOMALIES
ARM > MALROTATION > DUODENAL ATRESIA
• GU ANOMALIES
RENAL AGENESIS / HYPOPLASIA > HYPOSPADIAS

15. • Incidence of the VACTERL association in the EA population is -
20%.
• “Association” - idiopathic occurrence of multiple congenital
anomalies during blastogenesis. Affected patients have no family
history of malformations, no recognizable teratogen, and no
chromosomal abnormality.
• Requires a minimum of three of the components to qualify as a
“VACTERL patient.”
• Vertebral - 17%
• Anal -12%
• Cardiac - 20%
• TEF
• Renal - 16%
• Limb- 10%
VACTERL – H- HYDROCEPHALUS

16. Diagnosis and Clinical Findings
• Prenatal detection of EA by ultrasonography relies
on the finding of
a small or absent stomach bubble and associated
maternal polyhydramnios.
Esophageal pouch in the middle of the fetal neck in
association with polyhydramnios.
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• Earliest clinical sign of EA - usually excessive
salivation that results from pooling of secretions in
the pharynx.
• regurgitation, choking, and coughing

17. • Inability to pass a feeding or suction catheter
through the mouth or nose into the stomach.
• Abdomen distends as inspired air passes into
stomach.
• Chemical pneumonitis due to gastric acid reflux
• flexible catheter such as a feeding tube will often
curl up in the upper pouch
• ECHO , KARYOTYPING, USG KUB

19. it is possible to have absence of intestinal air in the circumstance of a
narrow or occluded distal fistula, which is identified at the time of
operation

20. • TEF WITHOUT EA - Repeated coughing or choking
during feedings, often with pulmonary infiltrates on
chest radiograph suggesting aspiration.
• The diagnosis can be made by barium
esophagography in the prone position.
• CONFIRMATION – BRONCHOSCOPY WITH OR W/O
ESOPHAGOSCOPY

21. Contrast is noted
passing from
the esophagus,
through the fistula
(arrow), and filling
the upper part of
the
trachea and larynx.

22. Preoperative Treatment
• Pneumonitis from aspiration of upper pouch
secretions and reflux of gastric acid through the TEF
causing worsening respiratory distress.
• continuous aspirating saliva under low-pressure
suction. (Doub Lumen REPLOGLE TUBE)

23. • Head-up, prone position is most effective at
minimizing reflux.
• Broad-spectrum antibiotic coverage and pulmonary
physiotherapy.
• Routine endotracheal intubation should be avoided.

24. Operative Repair
• Emergency operation for EA with distal TEF is not
necessary.
• 24 to 48 hours between diagnosis and operation
permits full assessment of the infant and treatment
of pulmonary insufficiency including atelectasis and
pneumonitis.
• Position- right posterolateral thoracotomy
• Incision- lower border of the scapula from anterior
axillary line posteriorly to the paravertebral region.
• Identification of the fourth intercostal space

25. • Most continue to advocate the extrapleural approach
for EA-TEF repair because a substantial anastomotic
leak does not result in empyema but merely causes a
esophagocutaneous fistula ( HEALS BY 1-2 WEEK)
• Azygos vein divided.
• Preserve the vagal fibers that supply the distal portion
of the esophagus.
• Close the fistula with absorbable suture such as
polydioxanone which prevents intratracheal granuloma
( nonabsorbable suture.)
• 1 to 2 mm of esophagus on the tracheal end of the
fistula to minimize the risk for postoperative tracheal
stricture, while not leaving so as of tracheal
diverticulum.

26. • Distal mobilization should be minimized to avoid
damaging vagal branches and the segmental blood
supply.
• Identify proximal esophageal pouch.
• A traction suture can be placed through the tip of
the pouch, and even through the suction catheter,
to assist in proximal dissection and avoid the
trauma caused by repeatedly applying forceps to
the proximal pouch.
• Necessitates dissection up to the thoracic inlet.
• This type of extensive dissection does not run a
significant risk for ischemic injury due to cervical
blood supply to the proximal part of the esophagus
is excellent.

27. • The upper pouch mucosa often retracts and can
easily be missed - best to insert the entire back row
of sutures before tying.
• Thoracoscopic repair of EA-TEF
• Superior visualization,
• Improved cosmesis,
• Elimination of the morbidity of neonatal
thoracotomy (scoliosis, winged scapula, chronic
pain, shoulder weakness).
• Limitations- limited working space, lack of
articulation at the end of the instruments and the
necessity of tying sutures under tension.

28. INFANTS WITH EA AND
LARGE DISTAL TEF WITH SEVERE RESPIRATORY DISTRESS
SYNDROME
• Necessary preoperative endotracheal intubation
• High-pressure ventilation may worsen lung
ventilation
• Abdominal distention as inspired air is diverted
through the TEF into the stomach.
• Exacerbating respiratory compromise.

29. • Emergency gastrostomy with underwater seal to
decompress the air-filled stomach.
• Emergent thoracotomy and fistula division in an
infant with severe respiratory distress syndrome
who requires high-pressure ventilation is generally
the best approach.
• Gastric division.
• Banding of the gastroesophageal junction.
• Bronchoscopic Fogarty balloon catheter through the
fistula.

30. LONG-GAP ESOPHAGEAL ATRESIA
• no precise definition of “long-gap” EA.
• Relative term as per surgeon.
• In EA commonly with LONG GAP, gastrostomy for early
feeding and delayed primary repair.
• The gap between the two ends of the Esophagus tends
to lessen because of spontaneous growth, possibly
related in part to reflux of bolus gastric feedings into
the lower esophagus, which makes primary repair
more feasible.

31. • Multistaged, extrathoracic elongation technique of
Kimura.
• Foker technique- traction sutures on both the
proximal and distal esophageal pouches exit
through the chest wall and are serially pulled in
opposite directions until the pouches approximate.
• Livaditis technique - circular myotomy of the upper
part of the esophagus to decrease the tension.
• Risk of stricture, food impaction, rupture
esophagus, pseudodiverticulum.

32. ISOLATED (H-TYPE) TRACHEOESOPHAGEAL
FISTULA
• Can be successfully divided through a cervical
approach.
• Right-sided, low cervical incision is
determined.
• Identification of the trachea and esophagus.
• Recurrent laryngeal nerve sparing.
• Fistula ligation and repair of both defect.
• Muscle interposition to prevent recurrence

33. COMPLICATIONS
• EARLY
Anastomotic leak
Anastomotic stricture
Recurrent tracheoesophageal fistula
• LATE
GERD
Tracheomalacia
Respiratory Disease
Disordered esophageal peristalsis

34. Anastomotic Leak
• Most leaks are clinically insignificant and can be
managed with adequate drainage and nutritional
support.
• Retropleural approach + patent mediastinal
drainage- 95% heals
• Breakdown of anastomosis- identified in 24-48
hours.
• Infant frequently deteriorates from tension
pneumothorax or mediastinitis.
• Redoprocedure or esophagostomy + gastrostomy to
be done.

35. STRICTURE
• Stricture - presence of symptoms (dysphagia and
recurrent respiratory problems from aspiration or
foreign body obstruction) and narrowing noted on
endoscopy or contrast esophagography.
• Traditionally treated by dilation.
• Ballon catheter is recently used.
• First dilatation at 1month postop . 2- 3 sessions of
dilatation usually suffice. GERD should be prevented or
treated.
• Endoscopic KENACORT or MITOMYCIN C can be tried
• Redo anastomosis or esophageal replacement

36. Recurrent TEF
• Attributed to anastomotic leak with local
inflammation and erosion through the previous site
of TEF repair.
• Symptoms can be typical of those seen with a
congenital H-type TEF including coughing and
choking or cyanosis with feedings.
• Diagnosis may be suggested by an air-filled
esophagus on plain radiographs of the chest.
• Thoracotomy with fistula ligation and division WITH
pleura, intercostal muscle, or pericardium
interposed between the esophagus and trachea.

37. Gastroesophageal Reflux Disease
• GERD occurs in 30% to 70% of patients after repair
of EA.
• REASONS-
1. Shortening of the intra-abdominal portion of the
esophagus due to traction.
2. Manipulation by instruments
3. Esophageal motor dysfunction
4. Congenital

38. DIAGNOSIS
• Vomiting, dysphagia, and recurren anastomotic
stenosis.
• Upper gastrointestinal contrast study.
• Twenty-four-hour Ph probe.
• TREATMENT-
• thickening of Feedings.
• positioning of the infant in a prone or upright posture.
• administration of acid reduction agents such as
histamine-2 blockers, proton pump inhibitors, and
prokinetic agents.
• Nissen fundoplication with floppy wrap.

39. Tracheomalacia
• Tracheomalacia has been noted to affect up to 75%.
• Tracheomalacia - generalized or localized weakness of
the trachea that allows the anterior and posterior
tracheal walls to come together during expiration or
coughing.
• Embryologically , shorter cartilage fails to provide the
support necessary to maintain a patent airway.
• “brassy” or “barking” cough in mild cases to recurrent
pneumonia or acute, life-threatening apneic spells.
• In acute life-threatening events, the operative
treatment of choice is aortopexy.