1 Esophageal Atrasia.pptx7777777777777777777777777777777777

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1. Topic: ESOPHAGEAL ATRESIA By: Dr. J. K. Sesay Senior Registrar, MoHS Specialist: General Surgeon Dip. Int. Health, M.D., M.Med. Surgery USLTHC, Connaught

2. Outline Summary Epidemiology Classification Pathophysiology Clinical Features Diagnosis Differential Diagnosis Treatment Prognosis Reference

3. Summary Esophageal  atresia  is a congenital defect in which the upper  esophagus  is not connected to the lower  esophagus , ending blindly instead. It is caused by the abnormal development of the tracheoesophageal septum. Esophageal  atresia  with a  fistula connected distally to the  trachea is the most common kind of esophageal  malformation  (classified as Gross type C). It manifests immediately after  birth  with  cyanotic  attacks, foaming at the mouth, and  coughing , and prevents any attempts to pass a feeding tube into the  stomach . X‑ray is mandatory for classifying the  atresia  and should show an air‑filled pouch situated at the level of the third  thoracic vertebra .  Infants with suspected esophageal  atresia  cannot be fed orally because of the risk of  aspiration pneumonia . Curative surgery must, therefore, be performed within the first 24 hours after  birth .

4. Epidemiology Incidence : approx. 1:4,300  live births  in the US  Epidemiological data refers to the US, unless otherwise specified. CLASSIFICATION Types Description Percentage Type A Esophageal atresia without tracheoesophageal fistula ∼ 8% of cases Type B Esophageal atresia with tracheoesophageal fistula to the proximal esophageal segment ∼ 3% of cases Type C Esophageal atresia with tracheoesophageal fistula to the  distal esophageal segment ∼ 84% of cases Type D Esophageal atresia with tracheoesophageal fistula to the proximal and distal esophageal segments ∼ 1% of cases Type E H‑type  tracheoesophageal fistula without atresia ∼ 4% of cases

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6. Pathophysiology A wedge of  mesoderm  called the tracheoesophageal septum separates the developing  foregut  ( esophagus ) from the  trachea . Esophageal  atresia  and  tracheoesophageal fistula  are caused by a  defect in mesodermal differentiation About 50% of cases are associated with other mesodermal defects ( VACTERL  association) V ertebral anomaly A nal  atresia C ardiac anomaly T racheoesophageal  fistula E sophageal  atresia R enal anomaly L imb  malformation

7. Clinical Features Prenatal Polyhydramnios : the fetus is unable to swallow  amniotic fluid   associated with an increased risk of  premature birth. Postnatal Esophageal  atresia  → pooling of secretions →  excessive secretions/foaming at the mouth Tracheoesophageal fistula: an abnormal connection between the  trachea  and  esophagus If the  fistula  is connected to the  proximal  esophageal segment:  aspiration  and subsequent  aspiration pneumonia Coughing  spells   Rales Cyanotic  attacks  due to reflex laryngospasms that prevent reflux  aspiration If the  fistula  is connected to the  distal  esophageal segment:  gastric distention

8. Diagnosis Placement of a feeding tube : the feeding tube cannot pass through the  esophagus  in the case of esophageal  atresia X-ray  of the thorax/abdomen   Esophageal pouch  (except with an H‑type  fistula )  Large gastric bubble: air in the  stomach  (gross types A and B present with a gasless abdomen) Further diagnostics concerning  VACTERL  anomalies Ultrasound  of the abdomen Echocardiography

9. Esophageal atresia Gross type C (Vogt 3b) An x-ray of the thorax and abdomen of a newborn shows a nasogastric tube coiled in the esophagus and air in the stomach, indicating an esophageal atresia with tracheoesophageal fistula to the distal esophageal segment, otherwise known as Type C (or Vogt 3b).

10. Esophageal atresia type C X-ray of the thorax and abdomen of a newborn, with contrast administered via gastric tube (white hatched line) The contrast medium only fills the esophagus down to the level of vertebra Th4, which reveals that the esophagus is dilated and has a blind ending (green overlay). There are air-filled intestinal loops in the abdomen, which indicate a tracheoesophageal fistula, through which air reaches the intestine. This finding confirms the diagnosis. An endotracheal tube is also visible (red overlay).

11. Differential Diagnosis Double aortic arch Definition : an embryonic  malformation  resulting in a  double aortic arch  ( vascular ring anomaly ) with subsequent constriction of the  trachea  and  esophagus Pathophysiology : the right and left  pharyngeal arch arteries  persist postnatally → formation of a  vascular ring  ( double aortic arch ) → constriction of the  trachea  and  esophagus Clinical findings   Typically manifests within the first weeks of life, especially in cases of  tracheal  compression  Tracheal  constriction : inspiratory or  expiratory stridor ,  dyspnea , respiratory arrest Acute episodes of severe constriction and/or  apnea  with  cyanosis  may occur (can be life-threatening) Hyperextension of the head to improve airflow Esophageal constriction :  dysphagia , choking, retching, vomiting Diagnostics Chest x-ray  (anteroposterior and  lateral ): shows  anterior   tracheal  bowing and narrowing MRI  scan of the thorax (imaging method of choice): to visualize the defect Treatment : surgical division of the minor arch 

12. Treatment Preoperative Placement of an esophageal or nasoesophageal tube for continuous suction of secretions to prevent  aspiration  and facilitate breathing Upper body elevated, left  lateral   decubitus position Antibiotics  in case of  aspiration pneumonia Surgery Surgical treatment should be performed within the first  24 hours  of  birth . The goal is to  reconnect  the upper  esophageal pouch  and the lower  esophagus . A long gap between both ends of the  esophagus  may not allow primary repair. In this case, a  gastrostomy  tube  is necessary to allow  enteral feeding .

13. gastrostomy  tube  

14. Treatment contd. Postoperative Uncomplicated surgery: transition to a normal diet after 2–3 days Radiological examination with a contrast agent ( esophagram ) one week after surgery to identify complications: e.g.,  esophageal stricture  or  anastomotic leak

15. Prognosis Overall good prognosis, but surgical complications occur frequently: Dysphagia  (50% of cases) Gastroesophageal reflux disease  (40% of cases) Anastomotic stenosis  (30–40% of cases) Wheezing (35% of cases) Infections of the respiratory tract (24% of cases) Anastomotic insufficiency (10–15% of cases) Recurrence of esophageal  tracheal   fistula Esophageal stricture

16. Reference 1.CDC. Facts about Esophageal Atresia.  https://www.cdc.gov/ncbddd/birthdefects/esophagealatresia.html#ref . Updated: November 1, 2018. Accessed: November 1, 2019. 2.Puri P, Höllwarth M.  Pediatric Surgery: Diagnosis and Management . Springer; 2009 3.Pinheiro PF, Simões e silva AC, Pereira RM. Current knowledge on esophageal atresia.  World J Gastroenterol  .2012; 18(28): p.3662-3672. doi:  10.3748/wjg.v18.i28.3662 . | Open in Read by QxMD 4.Polin RA, Abman SH, Rowitch D, Benitz WE.  Fetal and Neonatal Physiology . Elsevier; 2016 5.Clark DC. Esophageal Atresia and Tracheoesophageal Fistula.  Am Fam Physician  .1999; 59(4): p.910-916. url:  http://www.aafp.org/afp/1999/0215/p910.html .