Tracheoesophageal fistula and esophageal atresia, pediatric

Key Concepts:

Terms in this set (22)

Overview
Tracheoesophageal fistula (TEF) is a developmental anomaly characterized by an abnormal connection between the trachea and the esophagus that usually accompanies esophageal atresia and in which the esophagus is closed off at some point
Numerous anatomic variations in malformations exist, most commonly esophageal atresia with fistula to the distal segment
TEF and esophageal atresia are two of the most serious conditions in infants requiring immediate diagnosis and correction.
Overview-Pathophysiology
TEF and esophageal atresia result from failure of the embryonic esophagus and trachea to develop and separate correctly.
Respiratory system development begins at about day 26 of gestation. Abnormal development of the septum during this time can lead to TEF.
The most common abnormality is type C TEF with esophageal atresia, in which the upper section of the esophagus terminates in a blind pouch, and the lower section ascends from the stomach and connects with the trachea by a short fistulous tract. (See Types of tracheoesophageal anomalies.)
In type A, both esophageal segments are blind pouches and neither is connected to the airway.
In type B, the upper portion of the esophagus opens into the trachea with atresia of the distal esophagus.
In type D, the upper portion and distal portion of the esophagus open into the trachea.
Infants with type B or D may experience life-threatening aspiration of saliva or food.
In type E (or H-type) TEF without atresia, the fistula may occur anywhere between the level of the cricoid cartilage and the midesophagus but usually is higher in the trachea than in the esophagus. Such a fistula may be as small as a pinpoint.
Overview-Causes
Exact cause unknown
Commonly found in infants with other anomalies, such as:
congenital heart disease

genitourinary abnormalities

imperforate anus

intestinal atresia

skeletal anomalies

central nervous system anomalies
congenital heart disease
genitourinary abnormalities
imperforate anus
intestinal atresia
skeletal anomalies
central nervous system anomalies
Overview-Incidence
Esophageal atresia occurs in about 1 of every 3,500 live births; about one-third of these neonates are born prematurely.
The condition affects boys slightly more commonly than girls.
Overview-Complications
Aspiration of secretions into the lungs, leading to respiratory distress, pneumonia, or cessation of breathing
Death if untreated
Overview-Postoperative Complications
Anastomotic leak
Esophageal dysmotility or dysphagia
Esophageal stricture
Gastroesophageal reflux
Recurrent TEF
Chest wall deformities
Recurrent respiratory tract infections
Reactive airway disease
Tracheomalacia
Failure to thrive
Assessment-History
History of prenatal polyhydramnios
Coughing and choking after eating
Assessment-Physical Findings
Respiratory distress: Tachypnea, especially after feeding, retractions, crackles, or wheezes
Excessive salivation requiring frequent suctioning
Emesis during the first feeding and with attempted subsequent feedings
Drooling
Scaphoid or flat abdomen (suggesting type A or B); abdominal distention (suggesting type C or D)
Imperforate anus
Diagnostic Test Results-Imaging
Chest X-rays after the insertion of a nasogastric or an orogastric feeding tube demonstrate the position of the catheter and can also show a dilated, air-filled upper esophageal pouch, pneumonia in the right upper lobe, or bilateral pneumonitis. Both pneumonia and pneumonitis suggest aspiration.
Abdominal X-rays may show the absence of air in the stomach and lower GI tract (types A and B) or distention (types C and D).
An upper GI with contrast reveals types A and C.
Prenatal ultrasonography may reveal an absent stomach bubble or decreased intraluminal fluid in the gut.
Diagnostic Test Results-Other
A size 6 or 8 French catheter passed through the nose meets an obstruction (esophageal atresia) about 4" to 5" (10 to 12.5 cm) distal to the nostrils. Aspiration of the gastric contents is less acidic than normal.
Esophagoscopy identifies strictures or fistulas in the upper pouch; bronchoscopy confirms the presence of a fistula.
Treatment-General
Upright positioning
Suction catheter placement to reduce secretions
Treatment-Diet
Nothing by mouth (NPO)
Gradual introduction of oral feedings postoperatively, initially via a nasoduodenal tube and then orally after anastomoses heal
Treatment-Medications
I.V. fluids, such as 10% dextrose and hypotonic sodium chloride solution
Broad-spectrum antibiotics for superimposed infection and postoperatively to reduce infection risk
Oxygen therapy
Treatment-Surgery
Type and timing of the surgical procedure based on the nature of the anomaly, the infant's general condition, and the presence of coexisting congenital defects; both disorders usually considered surgical emergencies
Primary end-to-end anastomosis via thoracotomy or thoracoscopy as the desired surgery
Gastrostomy only when primary anastomosis isn't immediately feasible
Other options (less than optimal), such as gastric pull-up, neoesophagus creation with colonic tissue, and extrathoracic elongation
Nursing Considerations-Nursing Diagnoses
Compromised family coping
Imbalanced nutrition: Less than body requirements
Ineffective airway clearance
Risk for aspiration
Risk for infection
Risk for injury
Nursing Considerations-Expected Outcomes
seek support systems and exhibit adequate coping behaviors
maintain an adequate weight
maintain a patent airway and develop no respiratory complications
show no signs of aspiration
exhibit signs of positive parent-child bonding
remain free from signs and symptoms of infection
avoid or minimize complications.
Nursing Considerations-Nursing Interventions
Maintain a patent airway.
Administer oxygen, as needed, based on the infant's oxygen saturation levels or arterial blood gas results.
Perform pulmonary physiotherapy and suctioning, as needed.
Place the infant in an upright sitting position. Minimize handling of the infant to reduce oxygen demands. Maintain the head upright to reduce the risk of aspiration.
Maintain NPO status. Administer I.V. fluids as ordered; initiate I.V. access if not already present and maintain I.V. patency.
Obtain a daily weight.
Administer antibiotics as ordered to reduce the infant's risk of infection or to treat current infection.
Provide a humid environment.
Offer the parents support and guidance in dealing with their infant's acute illness and possible other anomalies that may be present. Encourage them to verbalize their feelings and fears, participate in care, and hold and touch their infant as much as possible to facilitate bonding. Model appropriate bonding and parenting behaviors, and point out positive aspects of the infant.
Prepare the infant and parents physically and psychologically for surgery. Explain all treatments and care measures, such as care in the neonatal intensive care unit, ventilator support, and drainage tubes as well as expectations for after surgery.
Postoperatively, provide aggressive pulmonary therapy to reduce the risk of respiratory infection and atelectasis and initiate oral feedings slowly as ordered.
Nursing Considerations-Monitoring
Airway patency
Respiratory status, including respiratory rate and depth and lung sounds
Signs and symptoms of aspiration
Signs and symptoms of respiratory distress
Fluid balance and hydration, including intake and output
Nutritional status, including weight
Signs and symptoms of infection
Coping strategies
Parent-infant interaction
Nursing Considerations-After Surgery
Vital signs
Cardiopulmonary function
Surgical site
Chest tube function and drainage, if appropriate
Feeding tolerance
Nutritional status, including weight
Signs and symptoms of infection
Parent-infant interaction
Nursing Considerations-Associated Nursing Procedures
Blood pressure assessment, neonate
Chest tube drainage system setup, pediatric
Chest tube insertion, assisting, pediatric
Family therapy
Feeding
Feeding tube insertion
Oxygen administration, nasal prongs, neonate
Oxygen administration, oxygen hood, neonate
Postanesthesia care, pediatric
Postoperative care
Preoperative care, pediatric
Preparing a patient for abdominal surgery, OR
Preparing a patient for thoracic surgery, OR
Pulse assessment, pediatric
Respiratory rate assessment, neonate
Surgical wound dressing application
Weight measurement, neonate
Patient Teaching-General
disorder, diagnosis, and treatment, including the need for immediate surgery to correct the condition
rationale for and importance of maintaining NPO status before surgery
postoperative feeding procedures and monitoring of nutritional status via weight gain
signs and symptoms of complications, such as new problems related to respiratory and GI function
signs and symptoms of aspiration and respiratory distress, including the need to notify a health care provider immediately if any occur
proper positioning for the infant before and after surgery
need for continued follow-up to evaluate the condition and possible complications, such as X-rays within the immediate postoperative period and then again 1 and 3 months later to evaluate the surgical repair.
Patient Teaching-Discharge Planning
Refer the infant and parents to social services for additional support and resources.
Refer the infant and parents to home care services for additional follow-up and teaching.
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