Esophageal Atresia PDF

ESOPHAGEAL ATRESIA INTRODUCTION Reference: National Library of Medicine https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3406418/ INTRODUCTION ▪ The esophagus is a muscular tube that transports a food bolus from the pharynx to the stomach. ▪ Esophagus is derived from the endoderm ge...

ESOPHAGEAL ATRESIA INTRODUCTION Reference: National Library of Medicine https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3406418/ INTRODUCTION ▪ The esophagus is a muscular tube that transports a food bolus from the pharynx to the stomach. ▪ Esophagus is derived from the endoderm germ layer which forms the pharynx, esophagus, stomach, and the epithelial lines of the aerodigestive tract. ▪ “Atresia” means that a passageway in the body is missing or closed.  ESOPHAGEAL ATRESIA (EA) A rare birth defect in which a baby is born without part of the esophagus. Instead of forming a tube between the mouth and the stomach, the esophagus grows in two separate segments that do not connect. In most cases, two separate tubes are formed, an upper (proximal) tube connected to the mouth and a lower (distal) tube connected to the stomach. This separated tubes are sealed off creating a pouch on either side; the gap between these pouches can be short or very long. This will cause accumulation of saliva that could result to choking, coughing and cyanosis during attempted feeding of the infant. Esophageal atresia often occurs with a birth defect called TRACHEOESOPHAGEAL FISTULA. TRACHEOESOPHAGEAL FISTULA – abnormal connection between the esophagus and the trachea (windpipe). TRACHEOESOPHAGEAL FISTULA (TEF) Tracheoesophageal fistula might manifest where the connection forms off of the lower pouch or form off of the upper pouch or both. EA/TEF always require surgical treatment to enable the baby to feed. This will be done in an operation under general anesthetic. In newborns with EA/TEF, the trachea may also be affected by a condition known as tracheomalacia and can cause breathing problems. This means the trachea is softer and less rigid than normal and may exhibit a loud and harsh “barking cough”. Some children can have difficulties swallowing certain foods due to the narrowing of the esophagus where it was repaired and may develop a problem with gastro-esophageal reflux where the contents of the stomach flow back up the esophagus causing pain and irritation when they get older.  5 CATEGORIES OF ESOPHAGEAL ATRESIA ▪ Type A is an isolated esophageal atresia without associated tracheoesophageal fistula and has a prevalence of 8%. The upper and lower parts of the esophagus do not connect and have closed ends. In this type, no parts of the esophagus attach to the trachea. ▪ Type B is an esophageal atresia with a proximal tracheoesophageal fistula. It is the rarest with a prevalence of 1%. ▪ Type C is an esophageal atresia is the most prevalent at 84% and involves a proximal EA with distal tracheoesophageal fistula. ▪ Type D is an esophageal atresia with both a proximal and distal TEF. It is rare at 3%. ▪ Type E is an esophageal atresia with just an isolated tracheoesophageal fistula without associated esophageal atresia. It is known as an "H" type, with prevalence around 4%. Reference: Clark DC. Esophageal atresia and tracheoesophageal fistula. Am Fam Physician. 1999 Feb 15;59(4):910-6, 919-20. 5 CATEGORIES OF ESOPHAGEAL ATRESIA Epidemiology of Esophageal Atresia As with other congenital malformations, EA/TEF occurs at an increased rate in twins, but usually affects only one twin. Once a couple has one child with EA/TEF, the risk of having a second child with this anomaly is increased to 1% Reference: National Library of Medicine https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2874026/ Etiology of Esophageal atresia The pathologic mechanism leading to EA/TEF is unknown. But below are the risk factors: Environmental Factors Genetic Factors Various environmental factors have been More than 50% of EA/TEF patients have suggested as risk factors for the development associated anomalies. Certain anomalies, such of tracheoesophageal anomalies, including as cardiovascular defects, renal agenesis, maternal exposure to methimazole , exogenous microcephaly, duodenal atresia, limb reduction sex hormones , maternal alcohol and smoking , defects, and polycystic kidney are especially infectious diseases, and working in agriculture or horticulture. prevalent in patients with EA/TEF Reference: National library of medicine https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2874026/  Stages in the development A. The laryngotracheal diverticulum forms as a ventral. Outpouching from the caudal part of the primitive pharynx. B. Longitudinal tracheoesophageal folds begin to fuse toward the midline to eventually form the tracheoesophageal septum. C. The tracheoesophageal septum completely forms.  At week 4, tracheoesophageal diverticulum developed to tracheoesophageal septum. There is a failure to separation in the septum leading to fistula. During week 8, the primitive gut failure of recanalization leads to atresia Associated Congenital Anomalies Reported Patients with Esophageal Atresia Amulya K Saxena, Esophageal Atresia With or Without Tracheoesophageal Fistula, Medscape. eMedicine, Updated: Apr 25, 2012, link: (http://emedicine.medscape.com/article/935858-overview#showall )  Pathophysiology A fetus with EA cannot effectively swallow amniotic fluid. Polyhydramnios Premature labour Aspiration of saliva or milk, if the baby is allowed to suckle, can lead to as aspiration pneumonitis. ▪ The neonate with EA cannot swallow and there is copious amounts of saliva. ▪ Aspiration of saliva or milk, if the baby is allowed to suckle, can lead to an aspiration pneumonitis. ▪ In a baby with EA and TEF, the lungs may be exposed to gastric secretions. ▪ Air from the trachea can pass down the distal fistula when the baby cries, strains, or receives ventilation. ▪ This condition can lead to an acute gastric perforation, which is often lethal.  Diagnosis Prenatal 2. Prenatal Ultrasonography 1. Polyhydramnios Sensitivity 40% Absence of stomach bubbles with fluid filled loops of bowels Amulya K Saxena, Esophageal Atresia With or Without Tracheoesophageal Fistula, Medscape. eMedicine, Updated: Apr 25, 2012, link: (http://emedicine.medscape.com/article/935858-overview#showall ) Diagnosis Laboratory Studies - CBC count - Electrolytes levels - Venous gas concentration - BUN and serum creatinine levels - Blood glucose level - Serum calcium level - ABG concentration ,as necessary - Genetic testing chromosome analysis microarray genomic hybridization (array GH)  Diagnosis Chest radiography CT scan Diagnosis Laboratory Studies Renal ultrasonography Echocardiography Used to evaluate associated kidney Who have clinical signs of anomalies , ureteral anomalies, or both cardiovascular disease Treatment Preoperative management: 1. The oral pharynx should be cleared. 2. The infant's head should be elevated. 3. IV fluids (10% dextrose in water). 4. Oxygen therapy is used. 5. In infants with respiratory failure, endotracheal intubation should be performed. 6. broad-spectrum antibiotics(such as ampicillin plus gentamicin) Surgical: Techniques vary according to surgeons' preferences and variations in pathologic anatomy. The best esophagus is the patient's esophagus.  SURGICAL TREATMENT: FOKER PROCESS: A technique that stimulates the upper and lower ends of the esophagus to make them grow inside your baby, allowing them to then be joined together after several days or weeks. THE MINIMAL INVASIVE FOKER PROCESS: It involves very small incision on the child’s back and uses minimally invasive instruments and a camera. The surgeon places traction suture on each end of the esophagus but creates the traction system inside the child’s boy by securing the sutures around one of their ribs. Surgeons tighten these sutures every week until the two ends of the esophagus are close to attach to each other. JEJUNAL INTERPOSITION: Uses a section of the jejunum (the middle part of the small intestine) as a replacement for the missing section of esophagus. This procedure generally results in shorter stays and fewer complications than alternatives, such as colonic interpositions or gastric pul-ups. THANK YOU!