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1. Esophageal atresia with tracheo- esophageal fistula
Introduction
One of the severe forms and rare congenital malformations of gastrointestinal tract represents
a failure of the esophagus to develop as a failure of the esophagus to develop as a continuous
passage and failure of the trachea and esophagus to separate into distinct structures. These
defects may occur as separate entities or in combination and without early diagnosis and
treatment, they pose a serious threat to the infant's well-being.
Definition
Esophageal atresia is malformation occur due to failure of development of esophagus
resulting in gap in the continuity of the esophagus. Tracheoesphageal fistula is the condition
of abnormal separation/connection of the esophagus and trachea. Two rare malformations
usually occur at the same time.
Incidence
Oesophageal atresia and Tracheoesphageal fistula affect males and females in equal numbers.
The incidence is estimated to be approximately 1 in 4,000 live births in the United States.
Source:- https://rarediseases.org/rare-diseases/esophageal-atresia-andor-tracheoesophageal-
fistula/
According to the Kanti children's hospital medical records from period of 01 Jan, 2018 to
Dec 31, 2019. The prevalence of trachea-oesophageal anomalies was found out to be 30
(9.8%).
Source:- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7654479/
Types
1. Oesophageal atresia without fistula (Type A):- the upper part of the esophagus end
blindly. There is no connection between esophagus and trachea. This is known as pure
type of oesophageal atresia
2. Proximal trachea-oesophageal fistula with oesophageal atresia is very rare (Type B).
In this type the upper part of the esophagus is attached to the trachea, but the lower
part of the esophagus has a closed end.
3. Distal trachea-oesophageal fistula with oesophageal atresia (Type C) is the most
common type. In this type the upper part of the esophagus has a closed end and the
lower part of the esophagus is attached to the trachea, as is shown in the drawing.
4. Proximal and distal fistula with oesophageal atresia (Type D) is the rarest and most
severe. In this type the upper and lower parts of the esophagus are not connected to
each other, but each is connected separately to the trachea.
5. Tracheoesphageal fistula without atresia (H type fistula) Type E: less common type
where the normal trachea and esophagus are connected by a fistula referred as 'H'
type.

2. Etiology
Exact cause of the malformation is unknown, however some associations are following
1. Genetic defect:-mutation in genetic structure of baby during embryonic development
may lead to abnormal development of organ.
2. Teratogenic stimuli:- maternal infection, chemicals, drugs, radiation.
3. Intrauterine causes:- Infection like TORCH can cross placental barrier and can leads
to intrauterine triggering.
4. Prematurity leads incomplete growth of the trachea and esophagus.
5. Defect in cellular growth during embryonic development.
6. Maternal polyhydraminous is found in approximately 50% of the affected baby.
Pathophysiology
The esophagus and trachea both develop from the primitive foregut. During 4th
-5th
weeks of
gestation, foregut normally lengthens and separates longitudinally. With increasing weeks of
gestation, the size of esophagus lengthens and separates longitudinally and each longitudinal
portion fuses to form two parallel channels (esophagus and trachea) are joint only in the
larynx. But in case of anomalies- incomplete fusion of the tracheal folds following separation
or altered cellular growth during embryonic development leads Tracheoesphageal Atresia and
Fistula
Clinical presentation
1. Excessive salivation
2. Constant drooling and frothing of saliva from the mouth and nose
3. Large amount of secretions from nose.
4. Coughing and choking on feeds may be associated with cyanosis.
5. Cyanosis:- Intermittent unexplained cyanosis due to laryngospasm caused by
accumulated saliva.
6. Regurgitation of feeding may be entering into respiratory system through fistula during
inspiration.

3. 7. Distended abdomen in case of distal fistula and scaphoid abdomen (boat shaped
abdomen) if no fistula.
Diagnosis
1. History taking.
2. Physical assessment:-assessment of clinical presentation.
3. Inserting a nasogastric tube in the upper pouch and taking a radiographic film that
demonstrates coiling of the tube in the upper pouch in newborn that has symptoms at
birth.
4. X-ray detect blind pouch of esophagus, presence of gas in stomach.
5. Bronchoscopic examination to visualize the fistula.
6. Presence of maternal polyhydraminous (>200ml).
7. USG is also useful to identify the type of anomaly.
Therapeutic Management
The infant with tracheoesphageal fistula should be managed immediately. Surgical
repairment is the main management with supportive management. Management includes
Pre-operative Management
a. Child is admitted in surgical ICU for management
b. Maintenance of patent airway: Suctioning (oral and pharynx), positioning (30-40ºhead
elevation), endotracheal intubation
c. Oxygen therapy
d. Withholding oral feeding
e. Fluid electrolyte and nutrition management: IV fluid
f. Prevention of aspiration (pneumonia): frequent suctioning
g. Maintain body temperature
h. Antibiotic therapy and other aseptic technique to prevent infection
i. Gastric decompression (gastrostomy tube drainage)
Surgical Management
The definitive treatment includes one to two or more than two operations needed to complete
the treatment. Surgery includes:-
1. Thoracotomy is performed in which division and ligation of the tracheoesophageal
fistula and end to end anastomosis of the esophagus will be done and chest tube is
placed for drainage.
2. If insufficient length of the 2 segment of the esophagus, repairment of esophagus
using part of colon.
3. Cervical esophagostomy makes stoma in the neck and allow drainage of saliva
through stoma.
4. Endotracheal intubations (supportive care).
Nursing Management
Assessment:-
Assess respiratory status, chocking, and baby's general condition during delivery, etc
Nursing Diagnosis
(i) Impaired gaseous exchange related to abnormal opening between esophagus and
trachea as evidenced by cyanosis.

4. (ii) Impaired swallowing related to mechanical obstruction.
(iii)Risk for injury related to surgical procedure.
(iv)Anxiety related to difficulty swallowing, discomfort due to surgery.
(v) Altered family processes related to children with physical defects.
(vi)Risk for aspiration related to difficulty in swallowing.
Nursing Interventions
1. Impaired gaseous exchange
(i) Assess the respiratory status of patient.
(ii) Monitoring oxygen saturation level
(iii)Arterial blood gas analysis
(iv)Provide oxygen
(v) Position baby slightly head up.
2. Impaired swallowing
(i) Place suction equipment at the bedside, and suction as needed;
(ii) Ensure proper nutrition by consulting with physician for enteral feedings, preferably a
PEG tube in most cases.
3. Prevent aspiration.
(i) Check placement of NG tube before feeding,
(ii) Using tube markings, x-ray study (most accurate),
(iii)Colour of aspirate as guides; if ordered by physician, put several drops of blue or
green food colouring in tube feeding to help indicate aspiration.
(iv)Elevate the head of bed to 30 to 45 degrees while feeding the patient and for 30 to
45 minutes afterward if feeding is intermittent.
4. Reduce anxiety.
(i) Allow family caregivers to talk about anxious feelings and examine anxiety-
provoking situations if they are identifiable
(ii) Explain clearly about the activities, procedures, and issues that involve the patient.
(iii)Explain all; use nonmedical terms and calm, slow speech;
(iv)Assist them in developing new anxiety-reducing skills (e.g., relaxation, deep
breathing, positive visualization, and reassuring self-statements)
5. Preoperative management
(i) Keep the newborn in 30º- 40º head up position
(ii) Frequent suctioning of the upper pouch immediately after diagnosis
(iii)Tracheo-broncheal aspiration is done several times/day through ET tube
(iv)Administer oxygen through ET tube to correct hypoxia
(v) Keep NPO
(vi)Keep the child in radiant warmer or incubator to prevent hypothermia
(vii) Administer IV fluid and electrolyte to correct hypoglycemia and hypovolemia
(viii) Continue monitoring of the infant's general condition, vital signs, oxygen
saturation
(ix)Provide pre operative medication as prescribed
(x) Collect all the lab investigation report of the patient
(xi) Counsel the patient and patient party to removed anxiety
(xii) Provide detail information about the disease condition and operative
procedure to caregiver
(xiii) Consent for surgery and other procedure for surgery
(xiv) Prepare the child and the parents for different procedures and treatment
(xv) Promote adequate hydration and nutrition by administering parenteral fluids
and electrolyte as prescribed.

5. 6. Postoperative Management
(i) Ensure ET tube in proper place
(ii) Assess the general condition of patient
(iii)Do not extend the neck
(iv) Maintain the hydration level of the patient by providing IV fluid as indicated
(v) Suction oro-pharynx using soft catheter carefully to avoid injury to incision area.
Insert catheter up to measured mark (usually 7 cm) only.
(vi)Check the inter-coastal thoracic drainage
(vii) Position change 2 hourly (right side preferable)
(viii) Baseline observation: vital sign, oxygenation and respiratory.
(ix)Daily chest X-ray should be done
(x) Chest physiotherapy
(xi)Keep child in radiant warmer /incubator.
(xii) Keep gastrostomy tube on gravity drainage
(xiii) Feeding is started after removal of chest drainage and confirming intergrity of
the esophagus
(xiv) Start oral feeding with sterile water followed by frequent breast feeding
Reference
 Uprety K, Child Health Nursing, fourth Edition (2071 Bhadra), Tara Books and
Stationery, Chhetrapati, Kathmandu, pg 133- 135
 Shrestha T. Essential Child Health Nursing. first Edition 2015,August. Medhavi
Publication; Jamal, Kathmandu Page no.267-270
 Adhikari T, Essential of Pediatric Nursing, first 2014 edition, vidyarthi pustak
bhadar, bhotahity, Kathmandu, page no 171-175
 WilsonD, Rodgers CC, Hockenberry M, Wongs Essential of pediatric Nursing 10 th
edition, ELSEVIER, page no Page 1411-1414
https://www.cdc.gov/ncbddd/birthdefects/esophagealatresia.html
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7654479/
https://nurseslabs.com/tracheoesophageal-atresia/