Esophageal atresia/Tracheo-esophageal fistula
Definition
Congenital obstruction of the esophagus characterized by interrupted the continuity of the esophageal wall with one or more fistulae may be present between the malformed esophagus and the trachea.
Incidence
It is the most common congenital malformation of the esophagus.Incidence approximately 1 out of every 3,000 babies.
Embryology
• Esophageal atresia with TEF
• Posterior deviation of the septum which divides the foregut into ventral laryngeotracheal tube and dorsal esophagus
• Isolated esophgeal atresia :
• Failure of the recanalization of the esophagus during the eighth week of development and is not associated with tracheo-oesophageal fistula.
Types
Type A
Type B
Type C
Type D
Type E
Frequency of associated anomalies in esophageal atresia
Abnormality PercentageCardiac 25Gastrointestinal 22Vertebral/Skeletal 20Renal 15Chromosomal (Trisomy 18 & 21) 7
VACTERL syndrome
Vertebral anomalies
Anorectal malformation
Cardiac anomalies
Tracheo-esophageal fistula
Esophageal atresia
Renal anomalies
Limb anomalies
VACTERL syndrome
Vertebral anomalies
Anorectal malformation
Cardiac anomalies
Tracheo-esophageal fistula
Esophageal atresia
Renal anomalies
Limb anomalies
- 70 %of patients with VACTERL association -small (hypoplastic) vertebrae or hemivertebra -may put the child at risk for developing scoliosis or curvature of the spine.
Sacral agensis
VACTERL syndrome
Vertebral anomalies
Anorectal malformation
Cardiac anomalies
Tracheo-esophageal fistula
Esophageal atresia
Renal anomalies
Limb anomalies
- 55% of patients with VACTERL association -usually noted at birth and often require surgery in the first days of life.
VACTERL syndrome
Vertebral anomalies
Anorectal malformation
Cardiac anomalies
Tracheo-esophageal fistula
Esophageal atresia
Renal anomalies
Limb anomalies
- 75% of patients with VACTERL association -The most common heart defects seen are ventricular septal defect (VSD), atrial septal defects and tetralogy of Fallot. Less common defects are truncus arteriosus and transposition of the great arteries.
VACTERL syndrome
Vertebral anomalies
Anorectal malformation
Cardiac anomalies
Tracheo-esophageal fistulaEsophageal atresia
Renal anomalies
Limb anomalies
- 70 % of patients with VACTERL association, although it can frequently occur as an isolated defect.
VACTERL syndrome
Vertebral anomalies
Anorectal malformation
Cardiac anomalies
Tracheo-esophageal fistula
Esophageal atresia
Renal anomalies
Limb anomalies
- 50% of patients with VACTERL association. - Renal agensis or severe reflux
VACTERL syndrome
Vertebral anomalies
Anorectal malformation
Cardiac anomalies
Tracheo-esophageal fistula
Esophageal atresia
Renal anomalies
Limb anomalies
- 75% of patients with VACTERL association. - include hypoplastic thumb, extra digits (polydactyly) fusion of digits (syndactyly) and forearm defects such as radial aplasia.
- 75% of patients with VACTERL association. - include hypoplastic thumb, extra digits (polydactyly) , fusion of digits (syndactyly) and forearm defects such as radial aplasia.
- 75% of patients with VACTERL association. - include hypoplastic thumb, extra digits (polydactyly) , fusion of digits (syndactyly) and forearm defects such as radial aplasia.
- 75% of patients with VACTERL association. - include hypoplastic thumb, extra digits (polydactyly) , fusion of digits (syndactyly) and forearm defects such as radial aplasia.
CHARGE association
ColobomaHeart anomaliesAtresia choanaeRetarded growthGenital hypoplasiaEar anomalies
CHARGE association
ColobomaHeart anomaliesAtresia choanaeRetarded growthGenital hypoplasiaEar anomalies
CHARGE association
ColobomaHeart anomaliesAtresia choanaeRetarded growthGenital hypoplasiaEar anomalies
CHARGE association
ColobomaHeart anomaliesAtresia choanaeRetarded growthGenital hypoplasiaEar anomalies
CHARGE association
ColobomaHeart anomaliesAtresia choanaeRetarded growth Genital hypoplasiaEar anomalies
CHARGE association
ColobomaHeart anomaliesAtresia choanaeRracheo-esophageal fistulaGenital hypoplasiaEar anomalies
Diagnosis
Clinical picture Investigations
Antenatal
Postnatal
Symptoms
• Drooling of frothy saliva from the angle of the mouth.
• Attacks of chocking and cyanosis on feeding.
• Tachypnea and aspiration pneumonia.
Signs
• Passage of a semi-rigid PVC radio-opaque 10F catheter will be arrested and coiled back.
• Signs of respiratory distress
• Abdomen is distended in cases of large fistula
• Other anomalies eg. Imperforate anus, skeletal anomalies
Causes of respiratory distress in EA/TEF
1. Regurgitation of saliva2. Regurgitation of milk on feeding attempts3. Reflux of gastric acid through distal pouch4. Abdominal distention when large fistula is
present
InvestigationsAntenatal US • Polyhydramnios• Small stomach• Associated anomalies
InvestigationsPlain X-ray chest & abdomen
Blind upper pouch with a marker tube (coiled tube).
Signs of aspiration pneumonia.
Stomach full of air from the distal fistula.
Also, aortic arch location, presence of vertebral or rib anomalies can be detected.
InvestigationsPlain X-ray chest & abdomen
Blind upper pouch with a marker tube (coiled tube).
Signs of aspiration pneumonia.
Gasless abdomen indicates no distal fistula.
Investigations
Other anomalies
Spinal US EchocardiographyAbdominal USGenetic counselling
Treatment
Preoperative care
Neonatal ICU admission and incubation Head elevation at 45° Continuous suctioning IV fluids Antibiotics Intramuscular Vitamin K Ventilation if required ( avoid face mask )
Timing and selection of surgical intervention
Determined by:
The degree of prematurity Patient weight Overall clinical condition Other associated anomalies Length of the gap between the ends of the
esophagus
EA with TEF• In healthy near term or full-term infants without
other severe anomalies and minimal pneumonitis:
Primary repair
• Within the first 24 to 72 hours after birth.• Right transverse extrapleural thoracotomy• Lower pouch is disconnected from the trachea by
ligation & division of the fistula• End to end anastmosis between the 2 pouches• Rertropleural drain placement & closure
Primary anastmosis
Long gap atresia
• Livatidi’s circular myotomy• Slim spiral myotomy• Mucosa-muscular flap from the upper pouch• Foker’s technique• Staged procedure Oesophagostomy to drain saliva & prevent aspiration Gastrostomy for feeding Ligation of the fistula to prevent reflux to the lung Later, esophageal replacement commonly by retrosternal colon
transposition, less commonly by stomach pull or jejunal interposition
Mucosa-Muscular flap from the upper esophageal pouch
Esophageal replacement
Colon transposition
Gastric pull up
In infants with severe pneumonia, prematurity or other significant medical problems that increase the risk of
major surgery.
• Gastrostomy tube placement is an effective method to decompress the intestinal tract and allow for medical optimization prior to esophageal repair.
• Gastrostomy tube placement to allow enteral feeding and the overflow of saliva from the upper pouch is controlled by frequent suction
• Estimation of the distance between both both pouches radiologically and management is done accordingly
In infants with esophageal atresia without tracheesophageal fistula
Postoperative care
• Oral feeding starts on 3rd day• esophageal study at 5th day• Daily assessment of the retropleural drain• Post-operative check for leak – UGI study with
head up 30°, water-souble contrast [not barium] and NGT proximal to anastomotic site
Complications
• Gastresophageal reflux• Anastmotic leakage• Recurrent tracheo-esophageal fistula• Tracheomalacia• Stricture
Minor leakage
Fistula following repair
Anastmotic stricture
Impacted food
PrognosisWaterstone’s risk classification
Survival (%)
Birthweight > 2.5 kgotherwise healthy
100
Birthweight 2.0 – 2.5 kg orhigher mild pneumonia ormoderate cardiac anomalies
85
Birthweight < 2.0 kg or higher, severe pneumonia orsevere associated cardiac anomlalies
65
Summary
Esophageal atresia
Type
C/P
Investigation
Prognosis
Post op. complications
Proximal atresia & distal fistula
Failure to pass NGT
X-ray chest showing coiled NGT
Cardiac anomalies
GE Reflux
Quiz
• A 2.8-kg. neonate with excessive salivation develops respiratory distress. Attempts to pass an orogastric catheter fail because the catheter coils in the back of the throat. A chest film is obtained and shows right upper lobe atelectasis and a gasless abdomen. Which of the following types of EA would be consistent with this findings?
A. Type A B. Type BC. Type CD. Type DE. Type E
The initial treatment for a pure esophageal atresia is:
A. Repair of the esophageal atresiaB. Repair of the esophagcal atresia with
placement of a gastrostomyC. Repair of the esophageal atresia,
Nissen fundoplicalion and placement of a gastrostomy
D. Gastrostomy alone
A baby boy was delivered at 38-weeks gestation, who despite initial suctioning, appears to be salivating excessively. He has mild tachpnea, but is pink.
A) How can the cause be established?B) What are the other anomalies that
are likely to be present?C) What would you do before
proceeding to surgery?