Gross classification of anatomic patterns of esophageal atresia.
A, Esophageal atresia without a tracheoesophageal fistula. This malformation
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is almost invariably associated with a "long gap."
B, Atresia with a proximal tracheoesophageal fistula. It is an uncommon anomaly; the abdomen
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is airless, and the diagnosis may be missed unless contrast studies or endoscopy is performed.C, Esophageal atresia with a distal tracheoesophageal fistula, the most frequently encountered
form of esophageal anomaly.
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D, Atresia with a double (proximal and distal) fistula. Though rare, this form is found more
often than original y thought.
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E, Tracheoesophageal fistula without atresia (H-type fistula). This anomaly may be missed in thenewborn period because swal owing is possible. It is associated with recurrent cough,
pneumonia, and abdominal distention.
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F, Esophageal stenosis.
Incidence of Various Esophageal Atresia--
Tracheoesophageal Fistula Anomalies
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1. Esophageal atresia with a distal tracheoesophageal fistula
84%C
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2. Esophageal atresia without tracheoesophageal fistula 6%A3. Tracheoesophageal fistula without esophageal atresia 4%E
4. Esophageal atresia with a fistula to both pouches 1%D
5. Esophageal atresia with a proximal tracheoesophageal fistula
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5%BWaterston Risk Groups and
Current Survival Figures
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Group Survival (%) Waterston Classification
A 100 Birth weight > 2500 g and otherwise healthy
B 85 Birth weight 2000-2500 g and well or higher
weight with moderate associated anomalies
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(noncardiac anomalies plus patent ductusarteriosus, ventricular septal defect, and atrial
septal defect)
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C 65 Birth weight < 2000 g or higher with severe
associated cardiac anomalies
Okamoto modification of the Spitz Classification:
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Predictors of Survival in Cases of Esophageal Atresia
Class Description Risk Survival
Class I No major cardiac anomaly, BW >2000 g Low 100%
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Class II No major cardiac anomaly, BW < 2000 g Moderate 81%Class II Major cardiac anomaly, BW >2000 g Relatively high 72%
Class IV Major cardiac anomaly, BW < 2000 g High 27%
ASSOCIATED ANOMALIES
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Incidence of Associated Anomalies
? Cardiovascular 24%
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? Genitourinary 21%? Gastrointestinal 21%
? Musculoskeletal 14%
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? Central nervous system 7%
? VACTERL association 20%
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? Overall incidence 50%-70%ANTENATAL suspected/ possibility
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? Maternal polyhydramnios? Absent stomach bubble (Pure EA)
? Maternal Ultrasound/ Fetal MRI
DIAGNOSIS
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PRE-OP MANAGEMENT? Oxygen by hood
? Mechanical ventilation support
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? Mucus Suction by Replogle tube/ mucus
sucker/ low pressure suction
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? Vitals monitoring (T/P/R)? Temperature maintenance (incubators)
? Rule out associated anomalies (VACTERL)
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? I V Fluids, Antibiotics and Inotropic support
? Routine blood investigations
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(CBC/KFT/CRP/blood culture if sepsis +)SURGERY
? Position: Right Posterolateral thoracotomy
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? Approach: Extraplueral (preferred)/ Transplueral? Surgery:
v Type B,C,D : Trache-oesophageal fistula ligation and end to end
anastomosis
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v Type A: Cervical esophagostomy + Feeding gastrostomy; later
esophageal replacement surgery
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v Type E: Fistula ligation through cervical approachLong Gap EA
? Occasionally in infants with EA-TEF, the upper pouch is high
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and the distance between the upper and lower esophagealsegments limits the ability to easily complete end-to-end
esophago-esophagostomy with acceptable tension.
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? Surgical Maneuvers in Long-Gap Esophageal Atresia
During the Initial Procedure:
? Anastomosis under tension
? Tension-relieving procedures
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? Flap technique? Suture fistula
Delayed Primary Anastomosis:
? With bougienage: proximal,proximal and distal,magnetic
? Without bougienage
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Long Gap EA
Esophageal lengthening techniques:
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?Flaps,?Spiral / Circular myotomy
?Gastric division
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?Traction sutures- Foker's/ Kimura extrathoracic
?Transmediastinal "Thread"
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?With and without "olives"?Kato technique
Esophageal Replacement:
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Colonic interposition
Gastric tube esophagoplasty
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Jejunal interpositionGastric transposition
PREDICTORS OF OUTCOME
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? twin birth,? preoperative intubation,
? birth weight less than 2500 g,
? long-gap atresia,
? Anastomotic leak,
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? postoperative intubation longer than 4 days,? and inability to feed at the end of 1 month
COMPLICATIONS
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Early? Anastomotic leak (13-16%)
? Anastomotic stricture (upto 80%)
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? Recurrent tracheoesophageal Fistula (3-14%)Late
? GERD (30-70%)
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? Tracheomalacia (10-25%)? Respiratory disease,
? Disordered esophageal peristalsis.
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