ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA: ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA BY: PERCIVAL BRUCE, BSc . JOEL ZUTAH TETTEH, BSc . Embryology : Embryology • Derived from primitive foregut •4thweek of gestation tracheoesophageal diverticulum forms from the laryngotracheal groove •Tracheoesophageal septum develops during 4th-5thweeks, muscular + submucosal layer of T + E formed •Elongates with descent of heart and lung •7thweek>>> reaches final length DEFINITION: DEFINITION Esophageal atresia is when the upper part of the esophagus does not connect with the lower esophagus and stomach. Tracheoesophageal fistula (TEF) is a connection between the upper part of the esophagus and the trachea or windpipe. TYPES: TYPES INCIDENCE: INCIDENCE Incidence: 1:4000 live births M > F (25:3) 10-40% are preterm Antenatal history: polyhydramnios (60%) CAUSES: CAUSES The exact cause is not known. one of the risk factors is a fetus: trisomy 13, trisomy 18, trisomy 21). ASSOCIATED DISORDERS/RISK FACTORS : ASSOCIATED DISORDERS/RISK FACTORS These two conditions are not thought to be inherited, and cases in which multiple family members are affected are rare. Other digestive tract problems, such as diaphragmatic hernia, duodenal atresia, or imperforate anus ASSOCIATED DISORDERS/RISK FACTORS (CONT.): ASSOCIATED DISORDERS/RISK FACTORS (CONT.) Heart problems, such as ventricular septal defect, tetralogy of Fallot Kidney and urinary tract problems, such as polycystic kidney disease, absent kidney, or hypospadias Muscular or skeletal problems VACTERL Association, which involves vertebral, anal, cardiac, TE fistula, renal, and limb abnormalities SIGNS AND SYMPTOMS OF TEF OR ESOPHAGEAL ATRESIA : SIGNS AND SYMPTOMS OF TEF OR ESOPHAGEAL ATRESIA Symptoms of these two conditions are usually noted quite soon after birth. They may include: Frothy white bubbles in the mouth Coughing or choking when feeding Vomiting Blue color of the skin, especially during feeding Difficulty breathing Very round, full abdomen (TEF) Unusually flat abdomen (isolated esophageal atresia) DIAGNOSIS OF TEF AND ESOPHAGEAL ATRESIA : DIAGNOSIS OF TEF AND ESOPHAGEAL ATRESIA Prenatal Ultrasound =polyhydramnios, absent stomach, MRI >>>Reveals a blind distended esophageal pouch Postnatal A complete medical history physical examination Chest x-ray abdominal X-rays DIAGNOSIS: DIAGNOSIS Bronchoscopy Level of fistula Exclude upper pouch fistula Identify laryngoesophageal cleft Gastroscopy SURGICAL REPAIR: SURGICAL REPAIR Primary surgery Staged surgery Slide 14: Preoperative CARE Minimize pulmonary complication IV access NPO head-up position sump tube (repogle) on low continuous suction gastrostomy under local anesthesia CXR, abdominal x-ray, renal ultrasound 12-L EKG and Echocardiogram POST OPERATIVE: POST OPERATIVE On return from theatre, the infant will have a transanastomotic tube (TAT) in place. The purpose of this tube is to act as a stent for the repair site, as well as for nasogastric feeds once they commence. The tube is marked with green and yellow striped tape. UNDER NO CIRCUMSTANCES SHOULD THE TUBE BE REMOVED WITHOUT THE SURGEONS’ PERMISSION. If the TAT is accidentally removed, do not attempt to reinsert it, and contact the surgeons immediately. 2. Aspirates should be measured, described, and recorded as for any nasogastric tube. Slide 16: FEEDING Gastrostomy feeding---oral feeding POST-OP CARE: POST-OP CARE OBSERVATION Vital signs such as TPR may be frequently monitored usually every 15 to 30 minutes to assess any degree of shock and early signs of haemorrhage etc. Any abnormalities in the vital signs should be reported Observe the colour of the skin for cyanosis and pallor Monitor intake and output level to rule out any imbalance. Observe the infusion for adequate flow rate and insitu Monitor the level of consciousness. POST-OP CARE: POST-OP CARE Oxygen may be administered, and a mechanical respirator may also be necessary. Blood and urine tests may be performed to evaluate the infant's overall condition. Scans may be performed to evaluate esophageal functioning. Post-op Pain Management : Post-op Pain Management Assess pain based on units policies(pain scale that is most frequently used) IV narcotics epidural infusion: 0.1% bupivacaine + fentanyl 0.5 mcg/ml at 01.-0.2 ml/kg/hr 3. rectal Tylenol + LA infiltration of incision POST-OP CARE: POST-OP CARE CARE OF THE DRAINAGE TUBE Connect drainage tube to appropriate receptacle and establish drainage. Keep the tube patent Drainage tube may be removed 2- 5 days post operative depending on the hospitals policy. The infant will be fed intravenously or will have a gastrostomy tube placed directly into the stomach until oral feedings can be swallowed and digested. To educate parents for home care : To educate parents for home care 1. Teach the parents regarding:- Positioning the baby at home (preferably head end elevated especially during and immediately after the feeds) 2. Inform them about the danger signs of respiratory failure 3. Explain to the parents the signs of contracture formation (refusal to eat, dysphagia, increased coughing) LACTATION: LACTATION 1. Explain to the mother the importance of expression of breast milk (even if the baby is not getting oral feeds) to continue lactation. 2. Encourage the mother to express milk regularly to promote lactation. POSSIBLE COMPLICATIONS WITH TEF AND ESOPHAGEAL ATRESIA : POSSIBLE COMPLICATIONS WITH TEF AND ESOPHAGEAL ATRESIA Recurrent fistula (re-operation is required in 1% of cases) Esophageal strictures caused by scarring (10% to 15% of cases) Gastroesophageal reflux (approximately 50% of cases) Aspiration (uncommon) Slide 24: Esophageal dysmotility . Dysmotility refers to weakness of the muscular walls of the esophagus. Some degree of dysmotility is expected to occur in all infants undergoing esophageal repair. This complication requires special precautions when the child is eating or drinking. Swallowing difficulties (dysphagia). Dysphagia can cause food or pills to become stuck in the esophagus at the site of the surgical repair. Medications should be taken with water to prevent ulcers from developing. Breathing difficulties and choking. These complications are related to the slow passage of food, food stuck in the esophagus, or aspiration of food into the trachea. Chronic cough. The cough is characteristic of TEF repair; it is caused by weakness in the trachea and does not indicate a cold or illness. Increased susceptibility to colds, respiratory infections, and pneumonia. Precautions should be taken to avoid contact with other sick children. Parents and caregivers can seek advice about strengthening the child's immune system through appropriate nutrition and supplements. LONG-TERM OUTLOOK FOR CHILDREN WITH TEF OR ESOPHAGEAL ATRESIA : LONG-TERM OUTLOOK FOR CHILDREN WITH TEF OR ESOPHAGEAL ATRESIA Some children born with esophageal atresia have long-term problems. Swallowing food or liquids may be difficult due to: Absent or abnormal contraction of esophageal muscles Scarring in the esophagus can partially block the passage of foods reference: reference www.cincinnatichildrens.org/health/info/ent/diagnose/trach-fistular.htm Boxwell, G. (Ed), (2000). Neonatal intensive care nursing. London: Routledge; Chapter 15, Surgical Aspects; pp 347 - 352 Chambers MA, & Jones, S. (eds).2007. Surgical nursing of children, Edinburgh: Butterworth Heinemann Elsevier, Chapter 9, Neonatal Nursing.